CARDIOLOGY Antithrombotic Therapies

CARDIOLOGY Antithrombotic Therapies Anticoagulants: for Vitamin K antagonists Warfarin (Coumadin) -Impairs hepatic synthesis of thrombin, 7, 9, and 10 treatment of venous -Interferes with both clotting and anticoagulation = need to use another med for first 5 days of therapy clots or risk of such -Must consume consistent vit K as factor V Leiden -Pregnancy X disorder, other -Monitor with INR and PT twice weekly until stable, then every 4-6 weeks clotting disorders, Jantoven -Rarely used, usually only if there is a warfarin allergy post PE, post DVT Marvan Waran Anisindione (Miradon) Heparin -IV or injection -Short half-life of 1 hour -Monitored with aPTT, platelets for HIT -Protamine antidote LMWH Ardeparin (Normiflo) -Inhibit factors 10a and thrombin Dalteparin (Fragmin) -Injections can be done at home Danaparoid (Orgarin) -Useful as bridge therapy from warfarin prior to surgery Enoxaparin (Lovenox) -Monitor aPTT and watch platelets initially for HIT, then no monitoring needed once goal is reached? Tinzaparin (Innohep) -Safe in pregnancy Heparinoids Fondaparinux (Arixtra) -Direct 10a inhibitor Rivaroxaban (Xarelto) -Only anticoagulant that does not affect thrombin Direct thrombin Dabigatran (Pradaxa) -Monitor aPTT inhibitors Lepirudin (Refludan) Bivalirudin (Angiomax) Antiplatelets: used COX inhibitors Aspirin -Blocks thromboxane A-2 = only 1 platelet pathway blocked = weak antiplatelet for arterial clots or -Only NSAID where antiplatelet activity lasts for days rather than hours risk of such as ADP receptor inhibitors Ticlopidine (Ticlid) -No monitoring needed stroke, TIA, -May ease migraines atherosclerosis, Clopidogrel (Plavix) -Needs monitoring during initiation due to risk of blood count abnormalities CAD, MI, angina, Ticagrelor (Brilinta) PVD, post PCI, post Prasugrel (Effient) CABG, a-fib PPD inhibitors Cilostazol (Pletal) -Contraindicated in CHF -May be useful in PVD as it widens leg arteries -Pregnancy X Glycoprotein IIB/IIIA Abciximab (ReoPro) -IV only inhibitors Eptifibatide (Integrilin) Tirofiban (Aggrastat) Defibrotide Adenosine reuptake Dipyridamole (Persantine) -Strong treatments for prevention of recurrent stroke inhibitors

CARDIOMYOPATHIES -A group of diseases of the myocardium associated with mechanical or electrical dysfunction that usually exhibit ventricular hypertrophy or dilation -Current major society definitions of cardiomyopathies exclude heart disease secondary to CV disorders such as HTN, CAD, or valvular disease -Etiologies may be genetic, inflammatory, metabolic, toxic, or idiopathic Type Info Signs & Symptoms Workup Management Prognosis Dilated Cardiomyopathy: -Common etiologies: viral, -CHF -Treat CHF symptoms dilation and impaired contraction genetic, alcoholism -Arrhythmias -ICDs of one or both ventricles -Systolic dysfunction -Sudden death -Eval for transplant -Exercise intolerance -Fatigue or weakness -Dyspnea Hypertrophic Cardiomyopathy: -Caused by genetic mutations -Varied presentation, may be asymptomatic -Differential: athlete’s -β-blockers to reduce O2 -Annual disorganized hypertrophy of left -Diastolic dysfunction -CHF heart (physiologic LVH), demand mortality of 1% ventricle and occasionally right -Usually asymptomatic until -DOE: the most common sx HTN, aortic stenosis -CCB to reduce -May progress ventricle childhood or adolescence -Orthopnea and PND -Valsalva will increase contractility and improve to dilated -Athletes with underlying -Exertional chest pain HCM murmur and diastolic relaxation cardiomyopathy HOCM at greater risk for lethal -Atypical chest pain decrease aortic stenosis -Pacer or AICD arrhythmia during exertion -Syncope and presyncope murmur -Surgical myectomy, -May have abnormal SAM -Palpitations -EKG: prominent Q mitral valve surgery, or movement of mitral valve -Postural hypotension waves, P wave ethanol ablation to -Fatigue abnormalities, LAD destroy thickened -Edema -Echo septum -Arrhythmias -Holter monitor -Harsh crescendo systolic murmur ± mitral -Exercise stress test regurg -Screen relatives -S4 -Displaced apical impulse or thrill -Sudden death -Stroke Restrictive Cardiomyopathy: -Etiologies: scleroderma, -R CHF as pulmonary pressures must increase to -Differential: constrictive diastolic dysfunction  normal amyloidosis, genetic, HOCM, deliver blood pericarditis contractility but rigid and stiff DM, chemo, HIV ventricular walls -Uncommon in US Arrhythmogenic Right -Genetic cause -Ventricular arrhythmias Ventricular Cardiomyopathy/Dysplasia: RV wall replaced with fibrous tissue Unclassified Cardiomyopathies -Includes stress-induced cardiomyopathy and left ventricular noncompaction

ARRHYTHMIAS AND CONDUCTION DISORDERS Atrioventricular Block Type Signs & Symptoms/Info/Workup Management EKG First degree -Asymptomatic -Treat reversible -EKG showing lengthened PR interval causes such as -Determine site of block using EKG ischemia, increased findings, atropine, exercise, or vagal vagal tone, or meds maneuvers -Pacemaker usually not recommended

Second Wenckebach -Typically asymptomatic -Treat reversible degree (Mobitz type I) -EKG shows progressive PR causes such as prolongation for several beats prior to ischemia, increased nonconducted P wave vagal tone, or meds -Beats classically occur in ratios of 3:2, -Pacemaker if there is 4:3, or 5:4 symptomatic -Can be a result of inferior MI bradycardia

Mobitz type II -May be asymptomatic or have signs of -Treat reversible hypoperfusion or HF causes such as -PR interval remains unchanged prior to ischemia, increased a nonconducted P wave vagal tone, or meds -Most patients will require a pacemaker

Third degree -May have dizziness, presyncope, syncope, v-tach, v-fib, worsening HF, or angina -P waves don’t correlate to QRS -Escape rhythm takes over for QRS (junctional or ventricular)

Bundle Branch Block -Occurs when block in left or right BB delays depolarization Differential Workup to a ventricle -Any BBB: ventricular rhythm or ventricular pacing -RBBB: if asymptomatic and no other evidence of cardiac -Variation is “intermittent Mobitz” where there is a RBBB or -RBBB: Brugada syndrome disease, no further w/u indicated LBBB plus intermittent BBB of opposite side  can progress -LBBB needs further w/u for cardiac cause to 3° AV block BBB and acute MI -LBBB interferes with dx of ventricular hypertrophy, myocardial Management Causes ischemia, and acute MI because of associated Q waves (early -Permanent pacemaker insertion for symptomatic BBB or -Structural heart disease: cor pulmonale, pulmonary ventricular depolarization is affected) as well as ST changes progression to AV block embolism, MI or ischemia (both branches receives blood (ventricular repolarization is affected) supply from LAD), myocarditis, HTN, congenital heart -RBBB usually does not interfere with dx of Q wave MI (early Prognosis disease ventricular depolarization is not affected) but can inflict ST -LBBB in older individuals associated with increased mortality -Iatrogenic: R heart cath, ethanol ablation segment changes

EKG -Joined QRS’s or “rabbit ears” -May have accompanying ST or T wave change due to altered sequence of repolarization -RBBB will be prominent on R heart leads (V1 and V2) -LBBB will be prominent on L heart leads (V5 and V6)

Sick Sinus Syndrome -A combination of unhealthy SA that stops pacing Signs & Symptoms Management intermittently + unresponsive supraventricular foci -Lightheadedness -May eventually need pacemaker -Seen in the elderly with heart disease and in kids with -Presyncope or syncope congenital and acquired heart disease after corrective -DOE Workup cardiac surgery -Worsening angina -Appears as sinus bradycardia -Palpitations -May also see intermittent SVT  Causes bradycardia-tachycardia syndrome -SA node tissue becomes replaced with fibrous tissue -Compromised blood supply to SA node (atherosclerosis, inflammation, emboli) -Lyme disease -Drugs causing depressed SA node function: β-blocker, clonidine, methyldopa, digitalis, Li, amiodarone

Ventricular Tachycardia Torsades de pointes is a Signs & symptoms Management EKG polymorphic form of VT -Can remain alert and stable with -Torsades: remove offending med, use short runs anti-arrhythmics Ventricular flutter is a rapid (240- -Prolonged runs  hypotension, -Treat if > 30 s with antiarrhythmics 280) unstable form of VT that can myocardial ischemia, syncope, (amiodarone, lidocaine, procainamide) deteriorate to VF chest pain, dyspnea -Cardioversion if pt remains unstable -Sudden cardiac death Causes -Electrolyte imbalances -Acid/base abnormalities -Hypoxemia -MI -Drugs

Ventricular Fibrillation Causes Signs & Symptoms Management -Underlying ischemia or -Can remain alert and stable with short -Treat underlying cause LV dysfunction runs -Electric defibrillation -Prolonged runs  hypotension, myocardial ischemia, syncope, chest pain, dyspnea -Sudden cardiac death

Atrial Fibrillation -Associations: valvular disease, Acute management Subsequent management dilated cardiomyopathy, ASD, -Hemodynamically stable  rate and -Cardioversion w/ antiarrhythmic maintenance vs. rate control w/ HTN, CAD, thyrotoxicosis, rhythm control, discuss cardioversion long-term anticoagulation alcohol excess, pericarditis, (with warfarin for several weeks before -Rate control with β-blockers, CCB, and/or digoxin chest trauma, OSA, pulmonary and after if this has been going on > 48 -Assess risk using CHADS2 disease, stimulants hours) -Anticoagulation with warfarin to an INR of 2-3 to prevent stroke -2/3 of pts will spontaneously -Hemodynamically unstable (HR > 150 (unless low risk) convert within 24 hours of 1st or symptomatic)  ER for -New drug dabigatran does not require INR monitoring episode cardioversion

Atrial Flutter -Single irritable atrial focus fires rapidly with every 2-3 flutters Workup EKG reaching AV node that is not refractory  QRS production -Can use vagal maneuvers to inhibit AV node and get -Sawtooth flutter pattern clearer picture of flutters -Atrial rate ~300 Causes -Mitral valve disease Management -Post cardiac surgery -Pharmacologic rate control (diltiazem or verapamil): -Pericardial disease more difficult than in afib -Prior heart surgery -Cardioversion in pts in whom pharmacologic rate -Acute or chronic pulmonary disease control is ineffective or poorly tolerated or who have hemodynamic instability Signs & symptoms -Consider radiofrequency ablation s/p cardioversion -Palpitations to prevent recurrence -Fatigue -Pts with persistent atrial flutter should be considered -Lightheadedness for anticoagulation in the same manner as afib -Mild SOB Supraventricular Tachycardia (SVT) Paroxysmal Differential Signs & symptoms Management SVT = AV -Atrial tachycardia: will see spiked P’ waves -Pounding heart -Vagal maneuvers to inhibit AV node node reentrant -Multifocal atrial tachycardia -SOB -Chemical cardioversion with adenosine (short AV node block) tachycardia = -Afib or aflutter with rapid ventricular response -Chest pain -Prevention or rate control with diltiazem, verapamil, or metoprolol (or SVT with -Sinus node reentrant tachycardia -Dizziness sotalol or amiodarone if AV node is not involved) abrupt onset -AV node reentrant tachycardia -Loss of consciousness -Synchronized cardioversion and termination -AV reentrant tachycardia -Radiofrequency ablation for recurrent SVT -AV junctional tachycardia (junctional ectopic tachycardia): may have wider Causes QRS due to aberrant ventricular contraction, inverted P waves from retrograde EKG -Reentry depolarization -Rate 150-250 -Automaticity -Nonparoxysmal junctional tachycardia -Regular rhythm -Ventricular tachycardia: can look similar to SVT -Sinus tachycardia: will usually be < 150 bpm while SVT is usually > 150 bpm ***Consider dx of Wolf-Parkinson-White syndrome with rapid ventricular response of SVT (accessory Bundle of Kent pathway aids conduction)  If you can’t distinguish atrial from junctional tachycardia (can’t find the P waves), just call it SVT

Premature Beats -Occurs when an irritable focus spontaneously fires Causes Management -Some are not serious while some are warning signs -Irritable atrial or junctional focus: epi, caffeine, amphetamines, -Treat underlying cause cocaine, ↑ sympathetic stimulation, digitalis toxicity, EtOH, -May need antiarrhythmics hyperthyroidism, stretch, hypoxia Premature atrial beat -P’ that is sooner than expected and may be inverted depending on origin (Premature atrial -Wide QRS due to aberrant contraction from semi-refractory bundle branch OR absence of QRS following P’ if contraction or PAC) totally refractory & reset of AV node

Atrial bigeminy = when focus fires prematurely at end of each normal beat

Atrial trigeminy = when focus fires prematurely at end of every 2 normal beats

Premature junctional -Premature depolarization of ventricles may retrograde depolarize the atria  inverted P’ waves beat before, after, or within the QRS & reset of SA node -Wider QRS because ventricular bundle branch may still be refractory

Junctional bigeminy = when focus fires prematurely at end of each normal beat

Junctional trigeminy = when focus fires prematurely at end of every 2 normal beats Premature ventricular -Considered pathological if > 6 -Occurs early in cardiac cycle contraction (PVC) PVCs/min (esp if they look the same) or -Wide, deep, opposite polarity QRS if PVCs occur in runs (geminy) -Does not affect SA node -Early warning sign of hypoxia -3+ successive PVCs are considered Ventricular bigeminy = when focus ventricular tachycardia fires prematurely at end of each -Severe hypoxia (MI) can cause normal beat multifocal PVCs -PVC on a T wave is bad (“R on T”) Ventricular trigeminy = when -Differential: ventricular parasystole focus fires prematurely at end of (ectopic ventricular focus pokes through every 2 normal beats normal sinus rhythm Ventricular quadrigeminy = when focus fires prematurely at end of every 3 normal beats

CONGENITAL HEART DISEASE -Acyanotic = left-to-right -Cyanotic = right-to-left -All left-to-right shunts have the potential to revert to right-to-left shunts due to increasing pulmonary congestion (Eisenmenger’s syndrome)

Investigation of suspected heart defect -Most cases are diagnosed prenatally by US screening @ 16-20 weeks -Some defects don’t emerge until several days or weeks have passed since birth due to transition of circulation  adult levels of pulmonary vascular resistance -Neonate will usually have symptoms within 24 hours Atrial Septal Defect Coarctation of the Aorta -Acyanotic -Obstructive

Signs & symptoms Signs & symptoms -May be asymptomatic unless there are other defects -Poor perfusion to LEs  diminished femoral pulses, cyanosis, -R heart failure cardiogenic shock, cold extremities, claudication -Pulmonary edema -Association with Turner’s syndrome, Shone’s syndrome, and bicuspid -Increased pulmonary vasculature aortic valve -Midsystolic pulmonary flow or ejection murmur accompanied by a fixed split S2 Workup -Measure BPs on all 4 extremities  HTN in UEs with low or Management unattainable BP in LEs -Refer to pediatric cards for echo -Refer for echo -Surgical repair at age 2-3 for most -Small defects in boys don’t need closure if RV size is Management normal -Reopen truncus arteriosus within 4 days of birth with prostaglandins

Patent Ductus Arteriosus Tetralogy of Fallot -Acyanotic -The most common cyanotic heart defect -Pulmonary stenosis  RV hypertrophy, overriding aorta, VSD Signs & symptoms -VSD may be right-to-left or left-to-right -Harsh continuous machine murmur -Usually asymptomatic Signs & symptoms -May have exertional dyspnea or heart failure -Progressive -May appear healthy and pink at birth Management -Cyanotic “tet spells” where child turns blue, squats to -Refer to valsalva pediatric cards -Harsh systolic ejection murmur for echo and for -May also have right aortic arch, Down’s or DeGeorge’s meds to make syndrome ductal tissue regress or Management surgical repair -Surgical correction in early infancy

Complications -Brain abscess -Stroke -CNS injury 8

Ventricular Septal Defect Transposition of the Great Vessels -The most commonly diagnosed -Cyanotic congenital heart defect -Aorta and pulmonary trunk are switched so that -May be single or multiple deoxygenated blood gets pumped through the aorta to -May be associated with other lesions systemic circulation while the oxygenated blood gets pumped through the pulmonary artery back through the Signs & symptoms lungs -Holosystolic murmur -Coexisting left-to-right shunt must also be present for life -May have thrill or diastolic rumble ex utero -Heart failure -Down’s syndrome association Signs & symptoms -Severe cyanosis at birth Management -Loud S2 -Most will get smaller and disappear on their own Management -Surgical repair indicated for intractable CHF, failure to thrive -Requires arterial switch for long-term survival

HEART FAILURE Chronic Congestive Heart Failure -Most often a result of ischemic heart disease Signs & symptoms Other Management (systolic HF)  myocardial remodeling -R CHF  ascites, + hepatojugular reflex, weight gain, JVD, -Salt restriction, daily weights -Other causes: bad valves, HTN (diastolic hepatomegaly, edema, abdominal distension, mostly clear lungs with -Avoid NSAIDs, CCBs HF), myocarditis, pericarditis, alcoholism (R dullness at the bases, ↑ JVP with hepatojugular reflux, tricuspid regurg, -ATP III recommends giving aspirin to reduce prothrombotic state HF), substance abuse, COPD or other lung peripheral edema -Exercise training program for stable NYHA class II to III disease (R HF) -L CHF (mostly heart and lung sx)  dyspnea, cough, S3 or S4, crackles, -Devices: AICD, intra-aortic pump, LVAD -Usually associated with low cardiac output wheezes, dullness at bases, frothy or pink sputum, pulse alternans but can be high (alternating strong-weak pulse), palpitations, fatigue, diaphoresis, displaced PMI, mitral regurg, pulmonary edema, orthopnea, PND Acute/flash pulmonary edema with acute  But research shows there are no hard and fast physical exam MI, severe illness, PE, HTN, end stage differentiations for R vs L CHF; all of these s/s can overlap! valvular disease Workup Beware acute HF with massive MI, -Referral for echo tachyarrhythmias, or endocarditis with valve -EKG for LVH rupture: severe SOB, cool skin, diaphoresis, -Stress test AMS, pallor, cyanosis -CXR for pulmonary edema (Kerley B lines) -Labs: BNP, CBC, CMP, fasting glucose, lipids Decompensated HF with new or worsening -Cardiovascular MRI can help distinguish ischemic heart disease from sx, new murmur, pt is “cold and wet”, CHF cardiomyopathy “decision rule” predicts 30 day mortality, avoid β-blockers

Drugs with proven mortality benefit -Note that there is only evidence for systolic HF for these drugs; use with diastolic CHF is not yet substantiated -ACEI or ARB -Hydralazine + isosorbide dinitrate: most beneficial for black man as an add on therapy to those already on β-blocker and ACEI that are still symptomatic -β-blockers -Aldosterone antagonists

Prognosis -Diet and prescription noncompliance are a major cause of hospital readmission for CHF -Main causes of death are arrhythmia and progressive pump failure

Acute Congestive Heart Failure -The development of acute dyspnea associated with elevated left sided Signs & symptoms Workup Management filling pressures ± pulmonary edema -Tachypnea with use of accessory -ACS workup: enzymes, -Treat underlying cause muscles EKGs -Supplemental O2: nonrebreather if necessary Differential -Wheezing (“cardiac asthma”) -BNP (most helpful in -Diuresis with IV loop diuretic -PE -Tachycardia comparison to previous) -Vasodilators -Pneumonia -HTN -CXR -Bipap if respiratory failure present -Asthma -Many have new or changed murmur -ABG if respiratory distress -Severe symptoms  IV + inotropes, of S3 or S4 present mechanical cardiac assistance, ultrafiltration Causes -Elevated JVD if R sided filling -BMP -Hold ACEI and β-blockers until -New onset HF (MI, valvular dz, afib and other arrhythmias, cardiotoxic pressures are ↑ -Echo to assess cardiac fcn hemodynamically stable or unless acute CHF agents, RV pacing dyssynchrony) -Peripheral edema with chronic CHF is mild -Exacerbation of chronic CHF (dietary indiscretion, medication -Flash pulmonary edema -Maintain aldosterone antagonist therapy or nonadherence, iatrogenic volume overload, drug effects) begin prior to discharge if not already on -Severe HTN -Renal failure -Anemia -Hypo or hyperthyroid -Infection -PE -Uncontrolled DM

HYPERTENSION Hypertension -95% of cases are essential hypertension Pharmacologic treatment options: single agents only lower by 10-20 mm Hg, may need a 2nd agents -Secondary cause workup: renal disease, renal Thiazides: HCTZ, chlorthalidone -DOC for HTN but can’t use once CrCl < 30 stenosis, aortic coarctation, hyperaldosteronism from -Ca sparing = good for osteoporosis pts tumor or hyperplasia, Cushing’s, pheochromocytoma, -Need to check BMP before and after starting OSA -Chlorthalidone has the most evidence -May hear S4 from forceful atrial contraction Loops: furosemide K-sparing: spironolactone, eplerenone Not very potent JNC- 7 classifications and initial treatment of HTN -Pre-HTN is 120/80 to 139/89  lifestyle changes ACEIs: benazepril, enalapril, lisinopril -Cough -HTN stage I is 140/90 to 159/99  thiazide (or loop -Can cause renal failure = need to monitor BMP 1 week and 1 month after starting and if renal pt) periodically after that, STOP if serum Cr ↑ by 30% -HTN stage II is >160/>100  thiazide + 2nd agent -Ok to use in patients with no renal function left -Pregnancy D Hypertensive urgency = stable or no end organ ARBs: irbesartan, losartan, olmesartan, -Same AEs as ACEIs and also pregnancy D damage, no raised ICP valsartan -May have SOB, headache, BPs usually > 220/110 CCBs: dihydropyridine (nifedipine, -Useful in the elderly -Tx is to lower BP in clinic slowly over several hours amlodipine) and non-dihydropyridine -FDA warning about amlodipine, verapamil, and diltiazem use with simvastatin (≤160/100) with labetalol, clonidine, captopril (verapamil and diltiazem) -Contraindicated in heart failure -Close follow-up Other direct vasodilators: hydralazine, minoxidil Hypertensive emergency = rapidly progressing end α-blockers -Clonidine: only for refractory HTN due to risk of falls organ damage -Methyldopa: DOC for HTN in pregnancy β-blockers -Questionable role in the treatment of essential HTN unless patient also has CHF or MI Monitoring HTN: -Need strict β-1 blockers for asthma/COPD patients so that bronchial relaxation is not blocked -Annual urine microalbumin (atenolol or metoprolol) -Annual BMP -Propranolol and labetalol block at multiple sites -Annual lipids -Can mask signs of hypoglycemia -Baseline EKG, look for LVH -Contraindications: heart block Preferred drug classes for initial treatment of HTN with comorbid conditions Diabetes MI CAD CHF Pregnancy Older patients CKD Recurrent stroke Thiazide β-blocker Thiazide Thiazide Clonidine CCB ACEI/ARB prevention β-blocker ACEI β-blocker β-blocker Methyldopa Thiazide ACEI/ARB AA ACEI ACEI/ARB ACEI CCB CCB AA Malignant Hypertension (Hypertensive Emergency) -HTN with signs of acute end-organ damage Signs & Symptoms Management -BP ~ 220/140 -Goal is lower DBP by 10% first hour, then 15% next 3-12 hours Causes -Retinopathy: blurred vision, retinal hemorrhages or (otherwise could cause ischemic stroke or MI) -Longstanding uncontrolled HTN exudates, papilledema -IV nitroprusside is the fastest acting -Self d/c of HTN meds -HA, confusion, n/v, seizures -Labetalol also a good option for most cases -Acute aortic dissection -Acute CHF -Underlying CAD  nitroglycerin -Post-CABG -Renal: AKI, oliguria -Switch to outpatient oral therapy with goal of lowering DBP to 85-90 -Acute MI over 2-3 months -Unstable angina -Eclampsia -Head trauma or burns 11

HYPOTENSION Hypotension Causes Signs & Symptoms Workup -Low stroke vol: dehydration, hemorrhage, vomiting, diarrhea, burns, 3rd spacing, pneumothorax, PE, cardiac tamponade, -SBP <90 or >30 below baseline -Echo if suspecting cardiogenic shock myocardial ischemia or cardiomyopathy, aortic stenosis or insufficiency, mitral regurg, aortic dissection, ventricular -AMS septum or free wall rupture -Cyanosis Management -Abnormal HR: brady or tachy -Oliguria -Treat underlying cause -Low systemic vascular resistance: sepsis, adrenal insufficiency, anaphylaxis, vasodilating drugs, neurogenic shock -Cool, clammy extremities -IVF -Orthostatic/postural: antipsychotics, diuretics, ACEI, vasodilators, methyldopa, polyneuropathy, Parkinson’s -Generalized weakness -Pressors: dopamine, dobutamine -Postprandial: a result of autonomic dysfunction -Presyncope or syncope -Intra-aortic balloon pump Cardiogenic Shock Causes Signs & Symptoms Workup Management -Acute MI -Hypotension -Echo to assess wall motion and -Supplemental O2 -End-stage CHF -AMS function -Optimize heart rate and rhythm: β-blockers, antiarrhythmics -Tachyarrhythmia -Cyanosis -Optimize volume status: fluids vs diuretics -Acute mitral or aortic regurg -Oliguria -Reduce afterload with vasodilator -Aortic or mitral stenosis -Cool, clammy extremities -Improve pump function: dobutamine to ↑ CO, consider device like intra-aortic -Traumatic cardiac injury -Elevated JVD balloon pump or ventricular assist device -Myocarditis -Peripheral edema

CORONARY HEART DISEASE Coronary Artery Disease -Risk of developing CAD for 40 year olds in Signs & Symptoms Workup Management of new disease or worsening symptoms the US is 49% for men and 32% for women -Angina -PE findings: S4, -Referral to cardiology -Risk factors: age, males, FH, sedentary -SOB arterial bruits, -ER via ambulance if EKG shows new ischemic changes: lifestyle, tobacco, HTN, DM, ↑ lipids -Sudden cardiac death is the first symptom in 15% abnormal ST depression or elevation, inverted T waves or there is funduscopic exam, hemodynamic instability Classification Chest pain differential corneal arcus, -Class I = no limitations or symptoms with -Atherosclerosis xanthelasma, Management of stable disease normal activity -Vasospasm from cocaine or stimulants tendinous xanthoma, -LDL goal <100 or <70 -Class II = slight limitations and normal -Prinzmetal’s angina: women under 50 CHF, murmurs -β-blocker (proven mortality benefit), CCB, statin, activity results in symptoms -Coronary artery or aortic dissection -EKG clopidogrel, nitrates PRN -Class III = marked limitation and minimal -Congenital abnormality -Refer for stress test -New drug ranolazine for refractory chest pain activity results in symptoms -Aortic stenosis if pt has low to -PCP visits every 6 months: annual CBC to check for -Class IV = symptoms persist with minimal -HCM intermediate anemia, annual lipids, FBG activity and rest -Coronary thrombus or embolus probability of CAD -Cardiologist every 1-2 years -Non-cardiac: costochondritis (reproducible on palpation), -Refer for cardiac -Consider early revascularization for significant Screening intercostal shingles, cervical or thoracic spine disease cath if pt has high narrowing of LAD, left main CAD, LVEF < 30%, or -Consider stress test in asymptomatic pts (reproducible with specific movements of the head or neck, probability of CAD large area of myocardium at risk with multiple risk factors causes paresthesias), PUD, GERD, cholecystitis, PE, pneumonia, pneumothorax (dyspnea)

Acute Myocardial Infarction Signs & symptoms Workup Emergent Management (any ACS) Treatment of Cocaine-Related ACS -Sudden onset chest pain, -Obtain 12 lead within 10 min of arrival -Oxygen: only if sats < 90% or resp distress) -Benzos every 15 minutes PRN nausea, vomiting, and repeat every 10 minutes if initial -Aspirin + NG -DON’T give β-blockers diaphoresis, SOB EKG is not diagnostic (1st EKG is -Morphine for continued chest pain despite NG -Jaw, neck, scapular, negative 40% of the time) -Treat HF if present with NG, furosemide throat, or arm pain -Look for early peaked T waves, ST -Give β-blocker if HF is not present in order to Post-ACS Treatment -DOE elevation, Q waves, J point elevation reduce myocardial oxygen demand -Continue drugs used during hospitalization: β-blocker, statin, ASA, SL -Chest pain > 30 min not -NSTEMI does not have EKG changes -Begin 80 mg atorvastatin for pts not already nitrates PRN (and possibly PO as well), aldosterone antagonist & ACEI responsive to NG because the infarction is in an on (if DM, HF, LV EF < 40%, or HTN), clopidogrel if intervention was done -Hypovolemia electrically silent area -Echo to determine cardiac function -Pre- or postdischarge stress test (depending on whether or not -HTN or hypotension -Presence of LBBB or pacing spikes intervention was done) -Tachy or bradycardia makes EKG difficult to interpret in the Additional STEMI Treatment -S3 or S4 setting of MI! -Antiplatelet and anticoagulant therapy for all Prognosis -Signs of CHF -Emergent cardiac consult for patients patients (in addition to aspirin) -33% are fatal, with most deaths caused by v-fib -Systolic murmurs with cardiogenic shock, left heart -Emergent stent if < 3 hours from symptom -Complications: CHF, RV infarction, ventricular rupture, arrhythmias, -Friction rub if day 2 or failure, or sustained ventricular onset mural embolus, stroke, pericarditis, postinfarction angina later tachyarrhythmia -Alternative is lytic therapy if not -For USA and NSTEMIs, can estimate 14-day risk of death, recurrent MI, -Change from stable -Electrolytes, coagulation studies, H/H contraindicated, symptoms < 12 hours, and PCI or need for urgent revascularization using TIMI score (Skyscape) angina to ACS = angina at -Serial troponins: specific cardiac unavailable within 90-120 minutes rest, new onset angina that damage marker, including damage from -CABG rarely performed during acute MI markedly limits activity, defibrillation, arrhythmias, cardiac more frequent angina, long procedures, CHF, vasospasms, PE, Additional NSTEMI Treatment duration angina, or angina myocarditis; elevation begins within 1 -Antiplatelet therapy for all patients (in addition occurring with less hour and remains ↑ for 5-14 days to aspirin; clopidogrel, ticagrelor, etc.) exertion than previous -CK: found in skeletal muscle -Anticoagulant therapy for all patients (heparin) -Remember that women, throughout the body; shows up in 1-6 -Invasive intervention based on presence of the elderly, and diabetics hours and lasts up to 1.5 days high risk factors (recurrent angina at rest, may have atypical -CK-MB: cardiac specific CK; shows elevated troponin, ST depression, high risk presentations up in 2 hours and declines after 24-72 stress test result, EF < 40%, hemodynamic hours instability, sustained VT, recent PCI, prior -Coronary angiography to determine CABG, TIMI score) location of lesion -Glycoprotein IIA/IIIB inhibitor in addition to all other meds for a subset of select pts who will undergo early PCI

Angina Pectoris Differential -AMI -Pneumonia -Syndrome X: myocardial ischemia in the setting of normal coronary arteries; due to disease of -Pleuritis coronary microvasculature -Bronchitis -Prinzmetal’s angina: caused by coronary vasospasm; usually at rest; typically in younger women; -GERD pain relieved by nitrates; dx via angiography with injection of provocative agents -PUD -Pericarditis -Biliary disease -Aortic dissection -Pancreatitis -PE -Cervical or thoracic disk disease, thoracic outlet syndrome -Tension pneumothorax -Costochondritis -Esophageal rupture -Anxiety or panic attack -Shingles 13

Stable Angina Unstable Angina Signs & symptoms Signs & symptoms -Gradual onset chest pain due to myocardial ischemia that occurs predictably and reproducibly on -Chest pain refractory to NG treatment or chest pain at rest or nocturnally exertion -May be associated with SOB, nausea, diaphoresis -May also have SOB -Relieved by rest or NG Management -Usually lasts 2-5 minutes -Treat like other ACS: admission, MOANS, serial troponins, EKGs -Diffuse discomfort -Stabilize -Antiplatelet therapy and possible reperfusion for select patients Workup - β-blockers -Stress test -Statins -Coronary angiography if needed -ACEI with DM, HF, LV EF < 40%, or HTN -CV risk reduction Management -Goal is to relieve symptoms and prevent future cardiac events -Nitrates and β-blockers are initial DOCs -CCB for refractory symptoms -Exercise -Daily aspirin -CV risk reduction: BP control, smoking cessation, statins, weight reduction, glycemic control -Periodic reevaluation with EKGs -Revascularization therapy an option for select candidates

VASCULAR DISEASE Aortic Dissection -Occurs when tear in the inner wall of the aorta Signs & Symptoms Management causes blood to flow between the wall layers  -Sudden onset of “ripping” retrosternal and back pain -Achieve hypotension and creation of false lumen -HTN bradycardia with β-blocker -Acute or chronic -Hypovolemia and nitroprusside -Usually in ascending aorta -Syncope -Surgical repair for Stanford -Typically in men 60-70 -Shock type A -Debakey and Stanford classifications -Pulse discrepancies -Stanford type B admitted to -Cardiac tamponade ICU for medical management: Risk Factors -May have focal neuro deficits or CVA due to poor morphine for pain control, β- -Connective tissue disorders perfusion of the brain blockers and nitroprusside -Bicuspid aortic valve -Coarctation of the aorta Workup Prognosis -HTN -EKG may show infarct pattern or LVH -Greater than 20% intra-op -CXR will show widened mediastinum, L sided pleural mortality Causes effusion -50% mortality within 10 -Usually a result of HTN -Bedside TEE test of choice, CT if unavailable years of all hospital survivors -Increased risk in pregnancy, connective tissue disease, bicuspid aortic valve, aortic coarctation

VASCULAR DISEASE Aortic Aneurysm -Occurs when blood collects between the aortic Risk Factors Differential Prognosis vessel layers, with true aneurysms involving all -Smoking -Pseudoaneurysm: a collection of blood and -Can spontaneously rupture 3 layers (intima, media, adventitia) -HTN connective tissue located outside of the vessel -Post-op complications: MI, reduced blood -Most commonly occurs below the kidney -Hyperlipidemia wall flow to LEs from emboli, AKI, mesenteric or -Male spinal cord ischemia, device migration or -Atherosclerosis endoleak with graft placement -FH Abdominal Aortic Aneurysm Thoracic Aortic Aneurysm -Normally AA is 2 cm, becomes aneurysmal Workup -Further classified as ascending, descending, or arch Workup when > 3 cm -Abdominal US for diagnosis -Ascending thoracic AA usually due to elastin degradation, -CXR for widened mediastinum, enlarged -Caused by atherosclerosis and -Abdominal CT for further which can be normal aging or accelerated by HTN, aortic knob, tracheal displacement inflammation characterization and measurement connective tissue disorder, RA, or bicuspid aortic valve -MRI or CTA are test of choice for -Categorized based on morphology -Descending thoracic aneurysm is caused by atherosclerosis characterization and dx -Usually infrarenal in location Management -Arch aneurysm seen in trauma or deceleration injuries -Echo -Surgical repair indicated when > 5 Screening cm; may be endovascular (stent) or Signs & Symptoms Management -USPSTF recommends US screen in all men open graft repair; endovascular has -Aortic insufficiency symptoms from dilation of valve -BP control, β-blockers preferred 65-75 who have ever smoked lower short-term -CHF -Re-image with CT or MRI every 6 mos -May also want to screen women with mortality/morbidity but open repairs -Compression of SVC by enlarging aorta  SVC syndrome -Surgical management is risky and complicated cardio risk factors and anyone > 50 with a have better long-term outcome -Tracheal deviation = rarely done, need to weigh risk of rupture FH -Watchful reimaging and risk -Cough -Surgical repair indicated for thoracic AA ≥ 6 reduction if < 5 cm: smoking -Hemoptysis cm, rapid expansion of aneurysm, or Signs & Symptoms cessation -Dysphagia symptomatic aneurysm -Usually asymptomatic and discovered -Consider elective nonrepair and -Hoarseness incidentally on abdominal exam cessation of surveillance imaging if -Steady, deep, severe substernal, back, or neck pain Prognosis -Abdominal or back pain life expectancy is < 2 years -Less likely to spontaneously rupture than -May have signs of limb ischemia AAA Arterial Embolism (Acute Arterial Occlusion) Causes Signs & symptoms Workup Management -Embolism or thrombus lodges or forms -5 P’s of critical limb ischemia: pain, -Heparin drip in a usually already-diseased vessel pallor, pulselessness, poikilothermia -Thrombolytics (cold), paresthesias/paralysis -Surgical emergency, requires correction Risk factors via thrombectomy or bypass within 6 -Afib hours of symptom onset or amputation -Valvular disease will be necessary -Prosthetic device -Ischemic disease Prognosis -Trauma -With intervention limb loss approaches -Hypercoagulable disorder 30% and overall mortality of 20% due to underlying cardiopulmonary disease

Giant Cell Arteritis (Temporal Arteritis) -Rheumatic disease, most often affects med-large head & Signs & symptoms Workup neck vessels -Weight loss -Arterial biopsy -Usually in white patients over 50 -Night sweats -↑ ESR or CRP -Often co-exists with polymyalgia rheumatica -Fever -Jaw claudication Management -Temple tenderness -Immediate steroids while awaiting biopsy results in order to prevent blindness -Vision loss with pale optic disc -Monitor for thoracic aortic aneurysm (increased risk) -New headache -Scalp tenderness Thrombophlebitis of Superficial Veins -Usually along a recent IV or PICC site (commonly the saphenous) Disposition Prognosis -May be spontaneous in pregnancy or postpartum or with varicose veins or -If any fever or chills, progressive ER -Inflammatory rxn subsides in 1-2 trauma -Stable and localized  heat, NSAIDs weeks -Can be associated with malignancy -20% mortality if septic -20% of cases will also have DVT -May not see significant swelling -Linear rather than circular

Deep Venous Thrombosis Signs & Symptoms -Palpable cord -Calf pain -Ipsilateral edema, warmth, tenderness, erythema

Workup -Homan’s is only + 50% of the time -Determine probability with Well’s criteria  < 2 indicates unlikely, > 6 highly likely -Further investigation using D-dimer -US for at least moderate Well’s score

Management -Immediate anticoagulation with heparin, LMWH, or fondaparinux -Lytics or thrombectomy for select cases -3 months of anticoagulation for initial distal DVT or consider IVC filter if not a good candidate

Peripheral Vascular Disease -Risk factors: smoker, DM, HTN, ↑ lipids, obesity Workup -Ankle/brachial index: PVD if <0.9, will have intermittent claudication if <0.7, pain at risk if <0.4, Signs & symptoms impending necrosis if <0.1 -LE disease: claudication (ppt by exercise, relieved by rest) in butt, hip, thigh, calf, or foot; diminished peripheral pulses; femoral bruits; nighttime pain from ischemia Management -UE disease: difference in systolic BPs between aroms, arm pain with exertion, dizziness during -Smoking cessation arm exertion (subclavian steal syndrome) -Walking program -Cool skin or abnormal skin color -Antiplatelet therapy -Poor hair growth -Revascularization if necessary via open surgery or stent -Ulceration or tissue necrosis -BP, lipid, sugar control -Signs of atherosclerosis elsewhere in the body Leg Ulcers Risk factors -Poor circulation, venous insufficiency, disorders of clotting, diabetes, sickle cell, neuropathy, renal failure, HTN, lymphedema, inflammatory skin diseases, smoking, genetics, malignancy, meds Diabetic (Neurotrophic) Ulcers Chronic Venous Insufficiency Ulcers Arterial Insufficiency (Ischemic) Ulcers Screening -Classified by CEAP system, which helps Workup Signs and symptoms -Recommended annually with distinguish initial disease severity as well as -ABIs to r/o PAD -PAD symptoms: pain and claudication visual examination and changes over time -Duplex US to eval degree of venous with walking that is relieved by rest monofilament test (checks most -Varicose veins in the absence of skin changes are obstruction or reflux (however may have more pain with leg common sites of ulceration) NOT chronic venous insufficiency! elevation in severe disease) Management -Ulcers are usually on the feet at points of Signs & symptoms Risk factors -Leg elevation, exercises, and friction and appear punched-out -Ulcers with punched-out -Advancing age, FH, increased BMI, smoking, h/o graduated compression stockings -Feet will turn red when dangled and pale borders with calloused LE trauma, prior DVT, pregnancy -SCDs for patients refractory to white or yellow when elevated surrounding skin stockings -Underlying neuropathy Signs & symptoms -Horse chestnut seed extract for -C/o tired, heavy legs, leg pain, or leg swelling patients who can’t tolerate or are Workup -Telangiectasias, reticular veins, and varicose veins noncompliant with compression -ABIs to r/o PAD -Edema, inflammation, pruritic dermatitis therapy -Ulcers with irregularly shaped borders along the -Aspirin therapy to accelerate healing Management medial ankle or saphenous veins that are tender, of ulcers -Comprehensive shallow, exudative, and have a base of granulation -Invasive options: sclerotherapy, laster assessment of ulcer tissue therapy, endovenous ablation and patient’s overall -Skin discoloration or redness, may appear shiny or techniques, vein stripping medical condition tight -Skin moisturizers -Classification of -20% of symptomatic patients will have no visible -Wound debridement PRN wound at each clinical signs -Barrier creams to protect adjacent skin follow-up -Selection of proper wound dressing -Debridement, local -NOT effective: topical antibiotics, wound care, debriding enzymes, growth factors, or pressure relief, honey infection control, and proper dressing selection -Compression bandages for severe -Negative pressure wound therapy following edema, weeping, eczema, or ulceration debridement after infection, necrosis, or amputation -Aspirin therapy accelerates healing -Revascularization for critical wound ischemia -Referral to subspecialty for slowly healing ulcers, persistent dermatitis, or recurrent cellulitis

Varicose Veins -Usually occur in the saphenous veins Signs & Symptoms Differential Management -Dull or aching pain in legs that is worse after -Claudication -Compression stockings Causes standing -Superficial thrombophlebitis -Leg elevation -Incompetent valves from damage or venous -Pruritus -Arthritis -Venous ablation dilation -May have h/o DVT -Peripheral neuropathy -Sclerotherapy -AV fistula -Brownish thinning of the skin above the -Great saphenous vein stripping -Congenital venous malformations ankles Prognosis -Complication of thrombophlebitis

VALVULAR DISEASE ***Stenosis causes HYPERTROPHY while regurgitation causes DILATION Aortic Stenosis (Aortic Valve Stenosis) -Causes obstruction  ↑ LV Signs & symptoms Workup pressure  LVH  CHF -May have asymptomatic murmur early in -EKG for LVH -Normal valve is 3-4 cm2 disease: harsh systolic < > murmur at RUSB, -CXR for cardiomegaly with radiation to the carotids bilaterally -Echo is diagnostic Etiologies -Late: DOE, SOB, angina, syncope, CHF, -Age-related calcification PND, orthopnea, carotid pulsus parvus et Management (inflammation + lipids) tardus -Proven benefit with statins -Bicuspid aortic valve -Sudden cardiac death -Valve replacement if symptomatic -Rheumatic fever -Arrhythmias -Aortic balloon valvotomy as bridge to surgery or for palliation -Endocarditis -Bleeding predisposition Prognosis -High risk of sudden death without valve replacement (life expectancy of 2-3 years)

Aortic Regurgitation (Aortic Insufficiency) -Causes increased afterload in LV  Signs & Symptoms Workup LV dilation  increased end-diastolic -Acute: flash pulmonary edema -EKG for LVH pressure in LV  backup into -Chronic: left-sided CHF, increased pulse pressure -CXR for cardiomegaly pulmonary circulation -Classic murmur is soft blowing < diastolic murmur -Echo is diagnostic -May have S3 gallop Causes -Austin Flint murmur: mid-diastolic, low frequency murmur at Management -Congenital: bicuspid or unicuspid aortic the apex from regurgitant flow competing with inflow from left -Acute: nitroprusside and emergent aortic valve valve atrium replacement -Infectious: rheumatic fever or infective -DeMusset sign: head bob with each heartbeat -Vasodilators (ACEI or ARB or hydralazine + nitrates) endocarditis -Water hammer pulse (Corrigan pulse): radial and carotid pulses to reduce afterload -Inflammatory: SLE or RA are abrupt and distensive with fast collapse -Endocarditis prophylaxis in certain patients -Aortic root disease: Marfan, syphilis, -Traube sign (pistol shot femoral): booming S1 and S2 over -Aortic valve replacement if having symptoms of CHF, ankylosing spondylitis, aortic dissection, femoral artery hemodynamic compromise, or EF < 55% trauma -Muller sign: systolic pulsations of the uvula

Mitral Stenosis -Causes elevated LA pressure  LA Signs & symptoms Workup hypertrophy  transmission of high -Dyspnea is commonly the only symptom -Echo to stage pressures to pulmonary vasculature  -A-fib from disruption of electrical conduction pulmonary edema  possible R-sided in hypertrophied tissue Management CHF -Fatigue -If asymptomatic, only watchful waiting -Apex murmur: loud S1 with post S2 opening -HTN management Etiologies snap, followed by low-pitched rumble -Afib management -Most commonly due to rheumatic -RV heave with progression to pulmonary HTN -β-blockers to prevent pulmonary edema heart disease -Pregnancy may bring on symptoms -Anticoagulation for any embolic event -Congenital malformation -Follow with echoes every 1-5 years depending on -Connective tissue disease stage -Mitral valve replacement or balloon valvuloplasty

Mitral Regurgitation (Mitral Insufficiency) -Causes increased afterload in LA  Signs & symptoms Workup dilation of LA to accommodate  increased -Acute: flash pulmonary edema, -EKG for LVH end-diastolic pressure in LA  backup into cardiogenic shock -Echo pulmonary circulation -Chronic: progressive L-sided -Cardiac cath to grade severity CHF, afib Causes -Holosystolic murmur at apex Management -Weakening of connective tissue with radiation to axilla with -ACEI to reduce afterload -Mitral valve prolapse severity correlated to duration -Diuretics -Ischemic LV function post MI rather than intensity (↑ with -Digoxin -Dilated cardiomyopathy valsalva, ↓ with squatting) -Endocarditis prophylaxis -Rheumatic fever -↑ JVD -Surgical repair if acute -Papillary muscle dysfunction or rupture -Laterally displaced apical -Mitral annulus calcification impulse -Bacterial endocarditis

Mitral Valve Prolapse -Displacement of an abnormally thickened mitral valve Signs & symptoms Management leaflet into the LA during systole  elongated chordae -Usual presents in early adulthood -Initial  refer to cardiology tendinae and mitral regurg -May be asymptomatic or have fatigue, atypical -β-blockers for palpitations -More common in women chest pain, palpitations, anxiety disorder, -Aspirin for clot risk postural orthostasis -Endocarditis prophylaxis no longer recommended Risk factors -Rarely progresses to mitral regurg -Periodic echos if there is progression to mitral -Collagen vascular disease: lupus, RA, ankylosing -Mid-systolic click regurg spondylitis, Ehlers-Danlos, Marfan’s -Valve repair vs. replacement if severe

Tricuspid Stenosis -Results in elevated RA pressures  edema, Signs & Symptoms Management hepatosplenomegaly, ascites -Fatigue and weakness -Balloon valvuloplasty or valve replacement if -Soft, high-pitched diastolic murmur that is ↑ symptomatic Causes with inspiration -Usually due to rheumatic disease -Visible hepatojugular reflux -Rarely an isolated disease, usually will see signs of mitral or aortic defects

Tricuspid Regurgitation (Tricuspid Insufficiency) -Results in leakage into the RA during systole Signs & Symptoms Management -Can be normal if present in small amounts -Symptoms of RV failure: anasarca, ↑ JVD -Treated only if severe with hepatojugular reflux, pulsatile liver -Diuretics for R-sided CHF Causes -Holosystolic murmur that is ↑ with inspiration -Digoxin for arrhythmias -Ebstein’s anomaly (displacement of valve -Afib -Treat pulmonary HTN towards apex) -Surgical repair better outcome than -Rheumatic disease replacement -Carcinoid -Endocarditis

Pulmonary Stenosis (Pulmonic Valve Stenosis) Causes Signs & Symptoms Workup -Most commonly congenital -DOE -Echo to look for doming of pulmonic valve -Rheumatic fever -Fatigue during systole and grade severity -Complication of arrhythmia ablation -Presyncope procedure -Cyanosis Management -↑ JVP -Based on Doppler gradient obtained during -Split S2 with soft P2 echo -Ejection click followed by < > systolic -Balloon valvulotomy murmur -May hear S4

Pulmonary Regurgitation (Pulmonic Valve Regurgitation) -Results in backward flow of blood into the Signs & Symptoms Workup RV during diastole -Can be tolerated asymptomatically for many -Echo years if it is the only defect Causes -Palpable RV heave Management -Dilated annulus from pulmonary HTN -Low-pitched < > diastolic murmur -Valve replacement -Connective tissue disorder -RV failure symptoms if pulmonary HTN -Infection endocarditis present -Surgical complication -Congenital malformation -Syphilis -Carcinoid -Rheumatic fever

OTHER HEART DISEASES Bacterial Endocarditis -Mostly affects the elderly Signs & symptoms Workup -New regurgitant murmur -Blood cultures x 3 Agents -HF -EKG -Staph aureus is most common -Evidence of embolic events -Echo -Viridans strep -Peripheral lesions (petechiae, splinter hemorrhages, Roth  Use Duke criteria to determine probability -Enterococci spots, etc) -Fever Management Risk factors: IVDU, prosthetic heart valve, structural heart -Prosthetic valves may need to be replaced disease, prior endocarditis -Empiric therapy initiated with IV vanco, gentamicin, and either cefepime or a carbapenem; subsequent AB therapy targeted to Prevention culture results -New guidelines from AHA suggest only prophylaxing the -Treat for at least 6 weeks highest risk groups prior to procedures likely to result in bacteremia: prosthetic heart valves, h/o endocarditis, unrepaired Prognosis cyanotic congenital defect, repaired congenital defect with -Complications are common: heart failure, stroke and other embolic prosthetic material, cardiac valvulopathy in a transplanted heart events, perivalvular abscess, pericarditis, fistulas, aortic valve -Usual AB is amoxicillin 2 g 30-60 min prior to procedure dissection, meningitis or encephalitis -In-hospital mortality rate of 18-23% -6 month mortality rate of 22-27% Acute Pericarditis Causes Signs & Symptoms Workup Management -Viral: Coxsackie virus B, -Chest pain that is worse with deep -EKG shows diffuse ST elevation -Treat underlying cause hep B, CMV breathing, cough, or lying down without reciprocal lead depression -NSAIDs, may need steroids -Bacterial: Staph, Strep, -Pericardial friction rub -Cardiac enzymes are – -Pericardiocentesis if tamponade or large effusion present TB -Cardiac tamponade: hypotension, -Echo shows pericardial effusion -Post-MI tachycardia, DOE, distended neck veins, Sequelae -Drugs (procainamide) narrow pulse pressure, pulsus paradoxus, -May develop constrictive pericarditis, a thickening and fibrosis of the -Malignancy mets indistinct heart sounds pericardium -SLE or other collagen -May develop cardiac tamponade from accumulation of effusion fluid vascular disease 21

Cardiac Tamponade Causes Signs & Symptoms Management -Pericarditis -Hypotension, tachycardia, DOE, distended neck veins, narrow -Pericardiocentesis -Trauma pulse pressure, pulsus paradoxus, indistinct heart sounds -Post-heart surgery -Myocardial rupture -Hypothyroidism

PULMONARY SYSTEM INFECTIOUS DISORDERS Acute Bronchitis Acute bronchitis = less than 3 weeks Treatment is supportive Differential -Almost always VIRAL! even if sputum is -Fever  antipyretics -Asthma purulent and you hear wheezes and rhonchi -Wheezing or cough with activity  short-acting bronchodilator (albuterol) -Allergic rhinitis -Only consider bacterial if pt does not get -Antitussives  dextromethorphan has the best evidence (Robitussin, Vick’s DayQuil Cough, codeine/guaifenesin (Cheratussin -Pneumonia better AC – may need Rx in NC), benzonatate (Tessalon – Rx required, comes in capsules), chlorpheniramine/hydrocodone (Tussionex -Pertussis – may need Rx in NC) -CHF -Peds over 2  children’s Robitussin or Triaminic Long-Acting -GERD -Meds Post-tussive emesis  concern for pertussis  give azithromycin if high suspicion and isolate for 5 days -Environmental Chronic bronchitis = more than 3 months Treat as exacerbation if there is a change in sputum color or amount from baseline, deterioration in respiratory function or exposure each year for at least 2 years, all other causes increased dyspnea excluded -Antibiotics: Augmentin, cephalosporins, macrolides, respiratory FQ (not cipro, S. pneumo resistance) -Think smokers, COPD Acute Bronchiolitis -Research definition = first episode of wheezing in a child younger than Agents Differential 12 to 24 months who has physical findings of a viral respiratory infection -Usually RSV -Asthma and has no other explanation for the wheezing, such as pneumonia or -Rhinovirus -Foreign body atopy -Human metapneumovirus -Broader definition = an illness in children <2 years of age characterized -Influenza Workup by wheezing and airway obstruction due to primary infection or -Parainfluenza -Diagnosis is usually clinical reinfection with a viral or bacterial pathogen, resulting in inflammation of -Adenovirus -CXR showing hyperinflation, interstitial pneumonitis, infiltrates the small airways/bronchioles -ELISA for RSV available -Mostly in infants < 2 months Signs & symptoms -Prophylaxis with Synagis given to high risk infants during first RSV -Concomitant URI Management season -Conjunctivitis or OM -Supportive -Wheezing, tachypnea, retractions, crackles -Humidifier -Oxygen if needed for severe disease -Bronchodilators or steroids for select patients

Acute Epiglottitis Agents Differential Management -H. flu -Croup -Send to ED for inpatient management -Strep pneumo or Strep pyogenes -Peritonsillar abscess and antibiotics as any manipulation of -Staph aureus -Foreign body glottis could result in airway -Trauma -Diptheria obstruction

Signs & symptoms Workup -Abrupt onset of high fever, sore throat, stridor, dysphagia, drooling, trismus -Lateral x-ray for “thumb sign” -Sitting child that won’t lie down, head leaning forward (sniffing or tripod position) Croup Agents Differential Management -Usually parainfluenza virus -Epiglottitis -Supportive: cool mist humidifier -RSV -Neoplasm -Send to ED for inhaled -Human metapneumovirus -Bacterial tracheitis epinephrine if severe or if there is -Pharyngeal abscess stridor at rest Signs & symptoms -Foreign body -Steroids -Average child is 18 months of age -Stridor, hoarseness, barking seal cough, low-grade fever Workup -Rales, rhonchi, wheezing -CXR showing “steeple sign” -Symptoms worse at night Influenza Influenza vs. common cold Workup Treatment -Flu = abrupt onset (sx worsen over 3-6 hours) with fever > -Nasopharyngeal swab (may be -Antipyretic/analgesic 101.5, severe myalgias, headache, malaise, painful dry cough, done just for epidemiologic -Albuterol neb sore throat, rhinitis purposes) -Ipratropium inhaler (Atrovent) for secretions -Cold = slow, insidious onset, usually no headache or chills, sore -Consider steroids throat, stuffy nose, sneezing, mild aches -Consider antivirals (oseltamivir, zanamivir) for influenza A or B only with hospitalization, severe or progressive disease, age under 2 or over 65, and for outbreak control in institutions or Prevention health care workers, AND MUST BE within 48 hours of start of symptoms to help at all -Inactive vaccine starting at 6 months (first-time vaccination in kids under 9 requires 2 doses) Sequelae -Live vaccine if 2-49 and healthy (warning: viral shedding) Secondary Staph aureus pneumonia may follow Pertussis Prevention Signs & symptoms Management -Dtap vaccine series for -Initial: cold-like; rhinorrhea, lacrimation, dry cough with episodes of severe cough, low-grade fever; post-tussive emesis -Macrolides are DOC kids -Paroxysmal stage: coughing becomes more severe and may persist up to 10 weeks at this stage; paroxysmal whooping may be -Septra is an alternative -Tdap vaccination for heard adults to protect kids -Convalescent stage: coughing diminishes as patient recovers and disappears over 2-3 weeks but may recur with subsequent URIs Prognosis -May be infectious for several Workup weeks if untreated -Bordetella culture or PCR from nasopharyngeal swab

Pneumonia Microbial etiology of community-acquired pneumonia in patients Prevention with pneumococcal vaccination who underwent comprehensive testing 23 valent -Adults over 65 (Pneumovax) -Persons aged 19-64 years with chronic cardiovascular disease (including CHF and cardiomyopathy), chronic pulmonary disease (including asthma and COPD), DM, alcoholism, chronic liver disease (including cirrhosis), CSF leak, cochlear implant, cigarette smoking - Persons aged 19-64 years who are residents of nursing homes or long-term care facilities -Singe revaccination recommended if adult was < 65 and it was more than 5 years ago when they got it, and in immunocompromised 5 years after initial dose 13 valent -Adults who are immunocompromised (should get 23 valent also, but not at same time) -Routine for all kids under 5 -Kids 6-18 who have sickle cell disease, HIV or other immunocompromising conditions, cochlear implant, or CSF leak

7 valent -No longer being used

Signs & symptoms -Rigors, sweats, fever or subnormal temp, cough ± sputum, dyspnea, pleuritic chest pain, fatigue, myalgias, abdominal pain, anorexia, headache, AMS -Pleural effusion: pulmonary consolidation, crackles, dullness to percussion, ↓ breath sounds

Outpatient Inpatient Workup Management HAP = pneumonia appearing > Non-HCAP Management -CXR: may lag behind PE findings! - -CAP  macrolide 48 hours after admission, or PNA -Non ICU  initial therapy with anti-pneumococcal β-lactam Urine test for Legionella -Underlying comorbidity (higher risk = need to in a recently hospitalized pt (ceftriaxone, ertapenem, or ampicillin-sulbactam) + macrolide -CBC, BMP cover resistant Strep pneumo, enterics, Moraxella, (cover atypicals), or monotherapy with a FQ anaerobes)  antipneumococcal FQ like levo, or HCAP = PNA in non- -ICU patients  initial therapy same as non-ICU, add vanco if CXR Findings macrolide + β-lactam (cefpodoxime, cefuroxime, hospitalized pt that has had suspecting MRSA, add anti-pseudomonal drug for COPD or -Can’t tell explicitly viral vs amox HD, ceftriaxone) extensive healthcare contact frequent steroid or AB users (β-lactam + FQ) pneumonia by patterns (old myth!) (group home, SNF, IV therapy, -Clinical improvement should occur within 72 hours -Lobar pneumonia: suggests Strep Disposition HD, etc) -Switch from IV to orals with clinical improvement pneumo, H flu, Legionella -Use PORT score or CURB-65 to estimate risk -F/u CXR for patients over 50 at 7-12 weeks -Patchy infiltrates in multiple lung (QX Calculate app) and determine outpatient vs VAP = ventilator-associated areas (bronchopneumonia): suggests inpatient pneumonia Empiric HAP/HCAP/VAP Management Staph aureus, gram negs, atypicals, -ER if RR > 30, HR > 125, SBP < 90, -Need to cover MRSA: vanco or linezolid viruses comorbidities Additional Workup -Need to cover Pseudomonas and other gram negs: Zosyn, -Fine dense granular infiltrates -ICU or EtOH or pleural cefepime, ceftazidime, aztreonam (only for severe PCN allergy (interstitial pneumonia): suggests Prognosis effusion blood culture, sputum b/c it’s not as effective) influenza, CMV, PCP -Fever clears after 2-4 days of treatment culture, Legionella & -Need additional coverage for gram negs and atypicals: cipro, -Lung abscess: suggests anaerobes -CXR clears after 30 days (up to 6 mos if elderly) pneumococcal antigen testing levo, gentamycin, tobramycin, or carbapenem -Nodular lesions suggests fungal Respiratory Syncytial Virus -Highly contagious, transmitted via aerosols or fomites Management -The most common cause of fatal acute respiratory infections in infants and young children -Supportive -Causes a spectrum of disease from URTs, LRTs, pneumonia -May need hospitalization with fluid and respiratory support -Most serious disease is in preemies, chronic lung disease, heart defects, asthma, -Albuterol trial -Steroids only in older kids, not infants immunocompromised, and the elderly -Ribavirin for select infants 24

Tuberculosis Signs & symptoms Workup Active TB drug regimens Monitoring -Latent or primary infection: Asymptomatic -If high suspicion, most clinics don’t workup but put a -Initial for 2 months: isoniazid, rifampin, -Sputum smears and cultures -Active infection: cough, fever, weight loss, mask on and send to ER pyrazinamide, ethambutol throughout treatment night seats, hemoptysis, fatigue, decreased -CXR: active infection (infiltrates in mid or lower fields, -Continuation for 4-7 months: isoniazid and -Vision checks and color vision appetite, chest pain hilar adenopathy, cavitation, emyema) or previous rifampin testing with ethambutol (pulmonary nodules, apical fibrosis, Ghon lesion) -CMP, CBC, and bili -TB skin test, AFB smear Latent TB drug regimens -9 months of isoniazid or 4 months of rifampin

NEOPLASTIC DISEASE Lung Cancer (Bronchogenic Carcinoma) -85% of cases occur among smokers -Bone, brain, liver, or adrenal symptoms from mets -Other contributing causes include radon gas, asbestos, and -Axillary or supraclavicular adenopathy environmental pollutants -Digital clubbing -2 major groups (small cell and non-small cell) account for 95% Differential of lung cancers -TB -Other lung cancers are rarer and include primary pulmonary -Fungal infection lymphoma, carcinoid tumors, bronchoalveolar cancers, and -Mets to the lung mesotheliomas -Sarcoidosis -Overall survival rate of 14% Workup Small Cell Carcinoma -Begin with CXR -Metastasize rapidly to regional lymph nodes and distant sites -F/u masses with CT -Classified as limited or extensive disease -Sputum cytology -Very responsive to chemo -Bronchoscopy -Remission is common but so is recurrence  overall survival -Transthoracic needle biopsy of 5% -Node sampling via transbronchial biopsy, mediastinoscopy, or mediastinotomy Signs & symptoms -Lung cancers are more like to cause paraneoplastic syndromes Management such as hypercalcemia, SIADH, ectopic ACTH secretion, -Assess feasibility of surgical resection and overall Lambert-Eaton myasthenic syndrome, and hypercoagulable patient health/quality of life issues states -Radiation for advanced disease or nonsurgical -Nonspecific cough or dyspnea candidates -Chest pain -Combination chemotherapy for candidates -Hemoptysis -Monitoring for recurrence -Anorexia, weight loss, fevers, night sweats -Hoarseness due to compression of the recurrent laryngeal nerve -Facial or UE swelling from SVC syndrome

Non-Small Cell Carcinoma -Arises as discrete masses within the lung parenchyma that can spread to regional lymph nodes -Limited response to chemo and then metastasize to distant sites -Surgical resection of limited tumors can be curative -Squamous, adeno, and large cell carcinomas -Staged by TNM Bronchial Carcinoid Tumor Squamous Cell Carcinoma Adenocarcinoma -Previously known as bronchial adenoma -Associated with slow growth and late metastasis -Peripheral -Rare group of pulmonary neoplasms characterized by -Rapid growth with mets neuroendocrine differentiation and relatively indolent clinical -Associated with lung scarring, not smoking course -Can also arise in the thymus, GI tract, and ovary -Surgical resection is treatment of choice Pulmonary Nodules Classification Differential Workup Further Evaluation Solitary pulmonary nodule = -Bronchogenic carcinoma -Review old radiographs < 5% probability of malignancy  < 3 cm, round, isolated opacity -Infectious granuloma or abscess -Follow-up suspicious CXR watchful waiting with serial outlined with normal lungs (no -Hamartoma nodules with hi-res chest CT imaging infiltrate, atelectasis, or -Mets -Infection clues: doubling < 30 -Age under 30 adenopathy) -AVM days -Lesions stable over 2 years -Resolving pneumonia -Benignity clues: doubling > 1 -Benign calcification pattern -Rheumatoid nodule year, well-defined borders, -Pulmonary infarction central calcification on CT Intermediate probability of -Carcinoid -Malignancy clues: ill-defined malignancy  refer for TTNA, -Pseudotumor borders, lobular borders, bronchoscopy, PET, or VATS Multiple pulmonary nodules -Mets, bronchogenic cancer, lymphoproliferative cancer, TB, abscess, granuloma, spicules on CT, peripheral halo fungus, sarcoidosis, silicosis, coal worker’s pneumoconiosis, MAC, AVM, rheumatoid on CT, stippled or eccentric > 60% probability of malignancy  nodules, hamartomas, Wegener’s granulomatosis, methotrexate, eosinophilic calcification on CT, cavitary refer for staging and excision granuloma, echinococcosis, paragonimiasis lesions with thick walls

OBSTRUCTIVE PULMONARY DISEASE Asthma Management and monitoring PCP treatment for adult acute exacerbation PCP treatment for pediatric acute -Refer for methacholine challenge if not sure it’s asthma -Peak flow > 70% predicted (dyspnea on exertion) exacerbation -Home peak flow monitoring: have pt establish baseline by measuring am and noon readings  inhaled SaβA up to 3x, can be managed at home -Assess severity using Pulmonary Index over 2-3 weeks, establish green, yellow, and red zones -Peak flow 40-69% of predicted (dyspnea limiting Score -Assess efficacy of treatments at each visit: nighttime awakenings, use of emergency inhalers, usual activity)  inhaled SaβA up to 3x, then oral -Mild: SaβA neb up to 3 doses, with oral ER or urgent care visits corticosteroids if no improvement steroids given after 1st dose if no -Step up meds if nighttime awakenings are 2+ times per month -Peak flow < 40% predicted (dyspnea with inability improvement -Office spirometry is preferred over peak flow measurement but not all PCPs will have this to speak, diaphoresis)  ER -Moderate: oxygen if needed, SaβA + ipratropium neb up to 3 doses, with oral steroids after 1st dose

Medication Notes SAβAs: albuterol, levalbuterol, -Use of more than one canister per month indicates need to step pirbuterol up -Use more than twice per week indicates need to step up Anticholinergics: ipratropium, -Alternative to SaβAs or as adjunct for severe exacerbation tiotropium Adult Meds: Systemic corticosteroids -For 3-10 days after exacerbation Inhaled corticosteroids: -Use a spacer Ages 12+ budesonide, beclomethasone, flunisolide, fluticasone, mometasone LAβAs: salmeterol, formoterol, -Only use for shortest time needed to control symptoms indacaterol -Only use long-term for someone whose asthma is not controlled on other meds -Don’t use without ICSs, increased risk of death!  make use of combo inhalers to comply Leukotriene modifiers: -Good for exercise-induced asthma montelukast, zafirlukast, zileuton Mast cell stabilizers: cromolyn -Good for seasonal asthma and exercise-induced bronchospasm -Takes 2 weeks for therapeutic response Theophylline -An adjunct to ICSs for management of nighttime symptoms -Requires serum monitoring Omalizumab -For severe allergic asthma in patients with frequent exacerbations, already on high steroid dose

Pediatric Meds: Ages Pediatric Meds: Ages 0-4 5-11

Asthma Exacerbation Signs & Symptoms Workup Management -Expiratory wheezing -CXR to eval for other causes of -Duoneb tx (albuterol + -Cough SOB ipratropium) q 20 minutes x 3 -Chest pain/tightness -Peak flow (exac if < 80% -MDI albuterol is just as -SOB with prolonged expiration baseline, severe exac if < 50% effective as nebulized albuterol -Tachypnea & tachycardia baseline) -IV or PO steroids if inadequate -Beware the quiet chest with -Check ABG for severe distress response intercostal retractions and -Supplemental O2 if needed accessory muscle use! -DON’T use ICS -MgS for refractory cases (has bronchodilator effect) -Mechanical vent if peak flow persists < 25% -Admit if not responding in 4-6 hours to treatments -Nonstandard therapies: montelukast, helium, furosemide, ketamine, macrolides

Cystic Fibrosis -Autosomal recessive inherited defect of protein regulating chloride channels, bicarb, and Workup other ions (CFTR protein)  defective mucociliary clearance  mucus obstruction, -Newborn screens detect only severe disease inflammation, infection, and fibrosis -Genetic screens of 23 most common mutations only identify CF in Caucasians -Also affects the pancreas and vas deferens -Buccal DNA swab for other mutations -Most commonly affects Caucasians -Sweat chloride test is confirmatory

Signs & symptoms Management -Will be on a continuum depending on % of normal CFTR functioning -Dietary support: higher BMI associated with better lung functioning, need high caloric intake to -No known abnormalities until there is < 10% normally functioning CFTR proteins; < 10%  combat malabsorption, salt supplements, pancreatic lipase supplements, fat-soluble vitamin absence of vas deferens, < 5%  sweat abnormality, < 4.5%  progressive pulmonary supplements infections, < 1%  pancreatic deficiency -Promote mucus clearance: percussion and chest compression vests, upside-down coughing, huff -Recurrent pulmonary infections with atypical bacteria (Staph aureus in infancy and breathing, oral oscillators, exercise, CPAP, saline mist, albuterol Pseudomonas in adulthood), poorly controlled asthma, failure to thrive, meconium ileus, -Infection control: cyclic use of antibiotics against Pseudomonas, intermittent IV antibiotics, oral pancreatitis, vitamin deficiencies, nasal polyps, sinusitis, fatty liver, liver fibrosis, portal HTN, antibiotics for 2-3 weeks after exacerbation gallstones, jaundice, osteoporosis or frequent fractures from vit D deficiency, rectal prolapse -Frequent office visits with PFTs, sputum culture, diabetes screens, bone densitometry, CBC, from thick stools, intestinal strictures, appendicitis, GERD, infertility, delayed puberty, PT/PTT, UA, vitamin levels, LFTs, albumin, immunizations smooth muscle growth around bronchioles, respiratory symptoms, diabetes, enlarged or deficient spleen Prognosis -Acute exacerbation (will be bronchial rather than pneumonia): increased cough of sputum, -Lung function declines at about 2% per year, but this will speed up with increasing exacerbations sputum color change, dyspnea, fatigue, decreased exercise tolerance, poor appetite, new -Patients are unable to return to previous baseline with each exacerbation tachypnea, retractions, wheezing, rhonchi, weight loss, fever, new findings on CXR, ↓ PFTs, -Median survival age is 38 hypoxia 28

Chronic Obstructive Pulmonary Disease (COPD) Chronic bronchitis = proximal predominant Management of stable COPD -“Blue bloaters” = depressed respiratory drive with use of -Goal is to reduce exacerbations requiring hospitalization accessory muscles  acidosis, productive cough, wheezing, -Get PFTs for diagnosis and classification of disease stage and rhonchi, hyperinflation of lungs, cor pulmonale to follow course of disease (FEV1/FVC < 70% with FEV1 < 80% are diagnostic criteria) Emphysema = distal-predominant -Add treatments in a stepwise fashion as needed -“Pink puffers” = high RR/dyspnea due to damaged vascular -New drug roflumilast (PPD-4 inhibitor) indicated for severe beds, distant breath sounds, hyperinflation of lungs, low COPD with chronic bronchitis and history of acute cardiac output exacerbations -Not recommended: expectorants, antitussives (COPD cough  Can have both chronic bronchitis and emphysema, and a not centrally mediated), respiratory stimulants subset of these patients also have asthma -Increased survival when oxygen therapy is used > 18 hours per day -Chronic airway inflammation  systemic release of inflammatory cytokines  CAD, renal insufficiency, Management of acute exacerbations neuromyopathy, osteoporosis, cachexia, downward spiral -SaβA + inhaled anticholinergic -Airway obstruction is not fully reversible -10-14 day steroid taper -Antibiotics if there is increased sputum purulence or vol or Workup increased dyspnea -PFTs demonstrating FEV1/FVC ratio < 0.70 -Supplemental oxygen to 90-94% saturation -NPPV is the preferred form of ventilation if needed

Bronchiectasis -Permanent abnormal dilation and destruction of bronchial walls Workup Management -Caused by an infectious insult + impaired drainage, airway -Abnormal CXR such as linear atelectasis or dilated and -Outpatient acute exacerbation  FQ obstruction, or a defect in host defense (ex. FB aspiration, thickened airways -Inpatient acute exacerbation  begin 2 IV antipseudomonal smoking, CF) -Labs: CBC, Ig levels drugs -An obstructive lung disease -Sputum smear and culture -Chest physiotherapy -PFTs -Inhaled steroids only for severe wheeze or cough or acute Signs & symptoms -Chest CT is diagnostic exacerbation -Cough -Daily production of mucopurulent sputum for months to years -Dyspnea, hemoptysis, wheezing, pleuritic chest pain

PLEURAL DISEASES Pleural Effusion -Excess fluid in the pleural space between Signs & Symptoms Exudates Transudates the lung and chest wall -Dyspnea -Caused by local ↑capillary and -Caused by systemic hydrostatic -Fluid may be serous, pus, lymph, or blood -Cough membrane permeability, impaired or oncotic pressure imbalances, or -Chest pain lymph drainage, or fluid shift from less commonly from fluid shift Causes peritoneal space from another space -Usually due to ↑circulatory hydrostatic Workup -Defined by 1+ presence of Light’s -Usually CHF pressure or permeability: L heart failure, -Frontal CXR shows blunting of costophrenic margins, can see free fluid Criteria: pleural protein/serum protein -Also cirrhosis, ↓albumin, pneumonia in LLD position, should also get lateral view > 0.5, pleural LDH/serum LDH > 0.6, nephrotic syndrome, acute -Decreased circulatory oncotic pressure: -May only be able to see very small pleural effusions on CT or pleural LDH > 2/3 ULN of serum atelectasis, myxedema, peritoneal ↓albumin -Thoracentesis is diagnostic 75% of the time and is indicated with any LDH dialysis, PE, Meig’s syndrome, -Decreased pressure in pleural space: new pleural effusion, esp with atypical clinical situation or failure to -Usually infectious obstructive uropathy pneumothorax progress as anticipated and is used to determine transudate vs exudate -Also malignancy, trauma, pulmonary -Impaired lymphatic drainage: malignancy -Routine pleural fluid tests: cell count, pH, protein, LDH, glucose infarction, PE, autoimmunity, Management -Movement of fluid from peritoneal space: -Additional pleural fluid tests for inconclusive results: amylase, pancreatitis, ruptured esophagus, -Thoracentesis ± chest tube ascites cholesterol, TG, Gram or AFB stains, bacterial or AFB cultures, post-CABG, drug reaction -Treat underlying cause cytology Pneumothorax Types Signs & Symptoms Workup Management -Primary spontaneous: occurs most commonly in thin -Sudden onset dyspnea -CXR: will show -Primary spontaneous  aspiration, may need pleural abrasion to 20-40 year old males, but can also occur due to ruptured -Pleuritic chest pain mediastinal and tracheal prevent recurrences blebs in smokers -Decreased or absent breath sounds push away from tension -Secondary spontaneous or traumatic  chest tube unless very small -Secondary spontaneous: occurs in setting of underlying on affected side pneumo, black space from -Flutter dressing if open pneumothorax from penetrating trauma lung disease like COPD and is more life threatening collapsed lung -Tension pneumo requires emergent needle thoracostomy (2nd -Traumatic: a result of penetrating or blunt chest injuries intercostal space) to convert it to a simple pneumothorax, followed -Tension: occurs when air continues to fill the pleural by chest tube insertion (5th intercostal space) for definitive treatment space and compresses the heart and great veins

PULMONARY CIRCULATION DISORDERS Cor Pulmonale -A subset of R-sided CHF where diastolic R ventricular failure Signs & symptoms Workup occurs as a result of pulmonary HTN associated with diseases of -DOE -Concomitant pulmonary HTN workup the lung, upper airway, or chest wall (most commonly COPD) -Fatigue -Does NOT include R sided CHF as a result of L sided CHF or -Lethargy Management congenital heart defect -Exertional syncope or angina -Treat underlying cause -Usually slow and progressive but may be acute -S4 -Diuretics -↑ JVP -Peripheral edema Pulmonary Embolism Etiologies Differential Management -Most arise from LE DVT -Pneumonia -Supplemental O2 if hypoxic -Stasis: surgery, heart failure, chronic venous stasis, immobility -Infection -Give empiric heparin while -Blood vessel injury: fractures, surgery -Obstructive lung disease waiting for imaging results -Hypercoagulability: postpartum, malignancy, OCPs, protein C/S/antithrombin III -CHF (depending on level of suspicion deficiency, lupus anticoagulant, factor V Leiden, prothrombin gene mutations, -Msk disease as well as timeframe to get test hyperhomocysteinemia -Acute MI results back) -Anxiety -IVC filter for repeat clots or 30

Classification poor anticoagulant candidates -Massive = sustained hypotension, pulselessness, persistent bradycardia, or need for Workup -Consider lytics for massive PE inotropic support -D-dimer is only useful if PE is very unlikely! Otherwise risk is too great -Consider surgical embolectomy -Submassive = pt is normotensive with myocardial necrosis that there will be a false negative for failed or contraindicated -Minor/nonmassive = normotensive with no myocardial necrosis -PE highly unlikely in ED if pt meets these criteria: age < 50, HR < 100, anticoagulation or lytic therapy SpO2 ≥ 95%, no hemoptysis, no estrogens, no prior h/o DVT or PE, no -Can be managed outpatient for Signs & symptoms unilateral leg swelling, no surgery or hospitalization in past 4 weeks select stable patients with no -Onset does not have to be sudden! -ABG if respiratory distress present: will usually show respiratory comorbidities -Dyspnea, pleuritic or anginal chest pain, cough, wheezing alkalosis, overall not very useful in diagnosing PE -Continue outpatient -Leg swelling or pain -Troponin anticoagulation for at least 3 -Hemoptysis -EKG sometimes shows S1Q3T3 months -Palpitations, syncope -Troponins: May be + in moderate to large PEs from acute R heart -Tachycardia and tachypnea, loud P2 from pulmonary HTN overload Prognosis -Diaphoresis -May have concomitant DVT detected by US -30% are fatal without treatment -Fever -CXR may show edema, cardiomegaly, prominent pulmonary vein, left -Less than 10% mortality if -Homan’s sign sided pleural effusion, or atelectasis treated by anticoagulation -Orthopnea -VQ scan, spiral CT pulmonary angiography (test of choice), or -↓ Breath sounds pulmonary angiography (gold standard but hi morbidity, requires femoral -JVD cath) -“Massive PE”  hypotension -Pregnant? VQ scan vs CTA and radiation dose is debated

Pulmonary Hypertension Etiologies Workup Management -Arteriolar narrowing in the lungs (idiopathic, familial, scleroderma, portal -Diuretics for fluid retention HTN, HIV, drugs or toxins, chronic hemolytic anemia) -Anticoagulation therapy if cause is -Left heart disease familial, drug, or idiopathic -Lung disease or hypoxia: COPD, interstitial lung disease, OSA, chronic high -Supplemental oxygen for resting altitude hypoxia -Chronic thrombotic or embolic disease -CCB -Splenectomy -Last resort is atrial septostomy or  May be secondary to multiple “hits” lung transplant -F/u every 3 months Signs and symptoms -Dyspnea, fatigue, chest pain, palpitations, LE edema, dizziness, syncope Prognosis -Tachypnea and tachycardia -Progressive and fatal if untreated -Evidence of R heart failure: JVD, ascites, edema -Average time for correct diagnosis -Loud P2 from elevated pulmonic pressures slamming valve shut is 15 months because symptoms -Tricuspid regurgitation murmur because it can’t shut all the way due to the other than SOB may not be present dilation of muscle and there is a lot to rule out -Pulmonic regurgitation murmur -Pulsatile liver

Differential -Left CHF -CAD -Liver disease -R heart cath showing mean pulm artery pressure > 25 mm Hg at rest is -Budd-Chiari syndrome (hepatic or vena cava thrombosis) diagnostic

RESTRICTIVE PULMONARY DISEASE Idiopathic Pulmonary Fibrosis -A chronic, progressive fibrotic disorder of the lower respiratory Signs & symptoms Management tract -DOE -No evidence that any treatment improves survival or quality -Can be genetic or acquired -Persistent nonproductive cough of life -Usually affects adults over 40 -Crackles or Velcro rales -Supportive care: oxygen, pulmonary rehab -Risk factors: smoking, environmental pollution, chronic -Digital clubbing -N-acetylcysteine pills microaspiration -Clinical trial enrollment Workup -Don’t use steroids as monotherapy -PFTs -Lung transplant -Chest CT -Acute exacerbation  broad spectrum antibiotics, high dose -Lung biopsy or bronchoalveolar lavage steroids, and azathioprine -Labs: CMP, CBC, CK -UA -EKG Pneumoconiosis -Occupational lung disease and restrictive lung disease caused by the inhalation of dust Signs & Symptoms Workup Management -SOB -CXR shows patchy -Bronchodilators Types -Cough bibasilar interstitial -Supplemental O2 -Coal worker’s pneumoconiosis (aka miner’s lung, black lung, or anthracosis) -Weight loss infiltrates and/or -Aggressive management of respiratory -Asbestosis -Fatigue honeycombing tract infections with abx -Silicosis -Pleuritic pain -Referral for transplant with advanced -Bauxite fibrosis -May be acute, chronic, or accelerated disease -Siderosis -Byssinosis Sarcoidosis -A rheumatic disease Signs & symptoms Workup -Cause is unknown -Dyspnea -Myopathies or myositis are uncommon -CXR for staging (I-IV) -More common in black -Cough -Neurologic: CN palsies, meningitis, brain -Ophtho exam patients -Chest pain lesions, neuroendocrine dysfunction -PFTs -Course of disease may -Fatigue -Cardiac: arrhythmias, conduction delays, -UA be acute and severe or -Eye manifestations pulmonary HTN, CHF, pericarditis -Labs: serum ACE, BMP mild and chronic -Skin manifestations (with predilection for -GI: abdominal pain, esophageal -CBC scarred or tattooed areas): lupus pernio or involvement -EKG Differential erythema nodosum -Liver (90% of pts): jaundice, varices, -TB test -Leukemia granulomatous hepatitis -Granuloma biopsy -Multiple myeloma -Endocrine: hypercalcemia, goiter, thyroid  A diagnosis of exclusion -Amyloidosis nodules -Diabetes -Renal: calculi, failure, nephritis Management -Thyroid or parathyroid -GU: epididymitis, AUB -Aimed at site of disease disease -Pulm  steroids, lung transplant -IBD -Arthralgias  steroids, NSAIDs, colchicine, biologics -Hemochromatosis -Polyarthritis in the ankles, knees, wrists, -Steroid sparing agents: methotrexate, cyclosporine, mycophenolate or elbows mofetil, azathioprine, cyclophosphamide -Gum hyperplasia Prognosis -Acute presentation has a high rate of spontaneous resolution

OTHER PULMONARY DISEASE TOPICS Acute Respiratory Distress Syndrome (ARDS) -Noncardiogenic pulmonary edema caused by capillary leaking from Stages Workup infection or inflammation  parenchymal inflammation and edema  -Stage I: clear CXR, infiltration of PMNs Diagnostic criteria: known clinical insult < 1 week ago, bilateral impaired gas exchange and systemic release of inflammatory mediators  begins opacities consistent with pulmonary edema on CXR or CT, not further inflammation, hypoxemia, and frequently multiple organ failure -Stage II: develops over 1-2 days with explained by cardiac failure or fluid overload, hypoxemia present on patchiness on CXR with edema and type I minimal ventilator settings (PaO2/FiO2 < 300) Acute lung injury (ALI) = a less severe form of ARDS alveolar cell damage Management -Stage III: develops over 2-10 days with -Treat underlying cause! Inciting Events diffuse infiltration on CXR, exudates, -Usually mechanical ventilation is needed, using low tidal volumes -Pneumonia of any kind proliferation of type II alveolar cells and PEEP -Chemical inhalation functioning as repair cells -Use of steroids is controversial -Chest trauma -Stage IV: develops > 10 days with diffuse -Supportive care: sedatives, nutritional support, BG control, DVT -Sepsis infiltration on CXR, involvement of prophylaxis, GI prophylaxis, neuromuscular blockade for vent -Pancreatitis lymphocytes  pulmonary fibrosis dyssynchrony to ↓O2 demand, keep fluid balance even to negative -Connective tissue disease: lupus -Vasculitis Prognosis -Hypersensitivity rxn to blood transfusion -Overall mortality 40-60% -Burns Neonatal Respiratory Distress Syndrome (Hyaline Membrane Disease) -A result of surfactant deficiency  alveolar collapse and diffuse atelectasis Signs & symptoms Workup -Typically occurs in preterm infants -Respiratory distress and cyanosis -CXR showing diffuse ground glass appearance soon after birth with air bronchogram Prevention -Tachypnea -Antenatal glucocorticoid treatment for women at risk for preterm delivery prior to 34 weeks of gestation -Tachycardia Management -If gestation is greater than 30 weeks, the fetal lung maturity may be tested by sampling the amount of -Chest wall retractions -Inpatient with fluid balance, CPAP, exogenous surfactant in the amniotic fluid by amniocentesis -Abdominal breathing surfactant Foreign Body Aspiration -Most common site is the right lung, followed by left lung, trachea/carina, and larynx Workup -Send to ED for bronchoscopy if severe symptoms Signs & symptoms -CXR is problematic because most swallowed objects are radiolucent -Choking episode followed by symptom-free period -Respiratory distress -Cyanosis -AMS -Generalized wheezing -Coughing -Recurrent pneumonia -Diminished breath sounds

Cough Duration Differential Workup/treatment Acute = less than 3 weeks -Viral URI or postviral most common cause -CXR for smokers, weight loss, persistent cough without prior URI, -Bacterial URI abnormal VS Persistent = greater than 3 -Postnasal drip -Pulse ox weeks -Allergic rhinitis -Acute bronchitis  short-acting bronchodilators -Pneumonia: VS abnormalities or findings of consolidation -Postnasal drip  antihistamine, decongestant, or nasal steroid Subacute = postinfectious -Pulmonary edema -Antitussives: not to be used in kids under 2, dextromethorphan has the cough lasting 3-8 weeks -PE best evidence Chronic = greater than 8 -Postviral -Irritant inhalation 1.) D/C ACEI, pertussis testing, r/o post-infectious cough weeks -RSV -Tumor 2.) Clear CXR  2-4 week treatment for asthma, postnasal drip, or -Parasites -ILD disease GERD -COPD -Sarcoidosis 3.) 2 week prednisone trial -Bronchiectasis -Chronic aspiration 4.) Referral to pulm or ENT for chest or sinus CT, EGD, barium swallow, -CF -GERD etc. -Bacterial: MAC, TB, pertussis, Mycoplasma, -Sinusitis Chlamydophila -Laryngitis -Asthma -TM irritation -β-blocker -ACEI -Chronic bronchitis in smokers -Psychogenic

GASTROINTESTINAL SYSTEM ESOPHAGUS Mallory-Weiss Tear -A tear of the distal esophagus at the Signs & Symptoms Management gastroesophageal junction, typically -Middle aged male presenting with -Most will resolve bleeding spontaneously occurring after a bout of vomiting hematemesis -May require injection or thermal coagulation -Major cause of upper GIB -May have recent h/o alcohol ingestion Prognosis Risk Factors Workup -Risk of rebleeding -Underlying portal HTN -Endoscopy is test of choice

Esophageal Neoplasms Benign Malignant Leiomyoma Usually occurs in males 50-70 Signs & symptoms Management -Tumor of smooth muscle -Progressive solid food dysphagia -Surgical resection with -Surgical removal if symptomatic Types -Weight loss gastric pull-up or -Squamous cell carcinoma: occurs in the upper 2/3 of the esophagus, -Usually is late stage by time patient is symptomatic colonic interposition Adenoma risk factors are alcohol use, tobacco, achalasia, caustic esophageal -Tumor arising from glandular tissue injury, head and neck cancers, Plummer-Vinson syndrome, black Workup -Palliative radiation -Usually found in areas of Barrett’s esophagus ethnicity, male -CXR showing mediastinal widening, lung or bony mets -Chemo -Adenocarcinoma: occurs in the lower 1/3 of the esophagus, risks are -Barium swallow showing many infiltrative or ulcerative -Palliative stenting Esophageal Papilloma Barrett’s esophagus, white ethnicity, males lesions and strictures -Associated with transformation to SCC -Lymphoma: very rare in esophagus -Chest CT  Recent trend towards adenocarcinoma -Endoscopic US for staging 34

Esophagitis Type Etiologies Signs & symptoms Differential Workup Management Prognosis Medication- -Major offenders are -Sudden onset -Only necessary for severe -Most cases induced tetracyclines, anti- odynophagia, retrosternal presentations or atypical heal inflammatories, KCl, pain after ingestion of a symptoms without quinidine, alendronate pill -Endoscopy or barium intervention swallow within a few days Eosinophilic -Allergic response -Dysphagia -Upper endoscopy with -Allergy referral -Heartburn unresponsive biopsy -Elimination diet to medications -Acid suppression -H/o environmental -Topical glucocorticoids (swallowed fluticasone) allergies or atopy -Esophageal dilation to treat strictures -Vomiting -Repeat endoscopies for change in symptoms -Abdominal pain GERD -Transient lower -Heartburn -Infectious esophagitis -Endoscopy with biopsy for -Lifestyle modification: ↑ HOB, avoid food before esophageal sphincter -Regurgitation -Pill esophagitis alarm symptoms (dysphagia, sleep, avoiding trigger foods, weight loss, smoking relaxation -Dysphagia -Eosinophilic odynophagia, weight loss, cessation -Hypotensive lower -Chronic cough esophagitis iron deficiency anemia), -Change therapy as needed every 2-4 weeks esophageal sphincter -Hoarseness -PUD symptoms refractory to -Maintain optimal therapy for 8 weeks -Anatomic disruption -Non-ulcer dyspepsia empiric PPI trial, new onset -Recurrent symptoms within 3 months suggest of the -Biliary tract disease symptoms in a patient over disease best managed with continuous therapy gastroesophageal -CAD 50, or symptoms > 10 years -Acid suppression will reduce the acid but not the junction, often with -Esophageal motility -Ambulatory pH monitoring reflux! hiatal hernia disorder for negative endoscopy with -Therapeutic regimens in order of increasing persistent symptoms potency: OTC antacids or H2 blockers, rx H2 -Esophageal manometry for blockers BID (take 30 min to work), PPI for 2 suspected motility disorder weeks, 20 mg omeprazole daily, 20 mg omeprazole BID or 40 mg daily -Acid suppression with symptoms worse than mild or intermittent  PPI recommended over H2 antagonist -EGD every 3 years for patients with known Barrett esophagus Other -HSV: mostly in immunocompromised -CMV -Candida

Esophageal Motility Disorders Achalasia -Absence of normal Signs & symptoms Workup Management esophageal peristalsis -Months to years of symptoms -Manometry is the gold standard -Smooth muscle relaxers like CCB, with increased tone of the -Gradual, progressive dysphagia of both -CXR showing enlarged, fluid-filled esophagus nitrates LES solids and liquids -Barium swallow showing “bird’s beak” from acute tapering -Balloon dilation of LES: high perf -Regurgitation, sometimes nocturnally of LES at gastroesophageal junction rate! Etiologies -Substernal discomfort or fullness after -EGD to look for other cause -Surgical myotomy -Chagas’ disease eating -Botox injection to relax LES -Others -Poor esophageal emptying Diffuse Esophageal Spasm -Simultaneous, nonperistaltic contractions of the esophagus Workup Management -Uncommon -Barium swallow showing corkscrew contractions or “rosary bead” appearance -Disease is -Manometry shows intermittent, simultaneous contractions of high amplitude not related to usually self- Signs & symptoms swallowing along with periods of normal peristalsis limiting -Intermittent dysphagia -Anterior chest pain unrelated to exertion or eating -Provocation by stress, large food boluses, hot or cold liquids

Scleroderma Esophagus -Atrophy and fibrosis of esophageal smooth muscle  loss of Signs & symptoms Workup LES competency, decreased peristalsis, and decreased gastric -Severe acid reflux -Manometry showing diminished peristalsis emptying -Strictures with low pressures, relaxed LES -Can also occur with progressive systemic sclerosis, Raynaud’s, -Erosions -Barium swallow showing dilated, flaccid or CREST -Heartburn esophagus -Dysphagia

Esophageal Strictures Causes Signs & Symptoms Workup -Most benign strictures are from long-standing GERD -Pyrosis -Barium swallow or endoscopy -Esophagitis -Bitter or acid taste in mouth -Dysfunctional LES or motility -Choking Management -Hiatal hernia -Coughing -Treat underlying condition -Radiation -SOB -Mechanical dilation -Esophageal sclerotherapy -Frequent belching or hiccups -PPI tx to avoid further stricturing -Caustic ingestions -Dysphagia -Intralesional steroid injection for refractory strictures -Surgical anastomosis -Hematemesis -Rare derm conditions -Weight loss -TB

Esophageal Varices -Dilated submucosal veins in the Signs & Symptoms Management lower esophagus as a result of portal -If ruptured  hematemesis, -Octreotide (or vasopressin + NG) to ↓ HTN melena, hematochezia, dizziness portal vein inflow -Sx of cirrhosis and portal HTN -Endoscopic therapy is treatment of Risk Factors choice: sclerotherapy or band ligation -Portal vein thrombosis Workup -TIPS procedure for failed endoscopic -Liver disease: alcoholic, viral -Labs will show typical values therapy hepatitis, etc. of liver dz: ↑LFTs and bili, -For variceal hemorrhage: pRBCs as ↓albumin and cholesterol, needed Prevention ↑coags -Pts with cirrhosis should have -Endoscopy is test of choice for Prognosis diagnostic endoscopy to screen for diagnosis -Bleeding varices resolve varices based on Child score, may spontaneously half the time need prophylaxis with β-blocker or -Continued bleeding associated with ligation increased mortality

STOMACH Gastroesophageal Reflux Disease (GERD) -Common cause is impaired LES function Differential Management -Can be associated with hiatal hernia -PUD -Mild, intermittent symptoms  lifestyle modifications -Severity of symptoms does not correlate -Gastritis like elimination of triggers, weight loss, avoid lying down with tissue damage -Non-ulcer dyspepsia after meals, elevate HOB; PRN antacids -Cholelithiasis -Oral H2 agonists have a 30 minute delay Signs & Symptoms -Angina pectoris -Troublesome frequent symptoms  PPI for 4-8 weeks, -Heartburn 30-60 minutes after meals and/or -Infectious esophagitis: then consider chronic PPI use if symptoms relapse or on reclining Candida, CMV, HSV persist -Regurgitation of gastric contents -Pill esophagitis -Known GERD complications  long-term PPI therapy -Hoarseness -Esophageal motility -If unresponsive to PPI  verify drug compliance, refer for -Loss of dental enamel disorder endoscopy -Relief with antacids -Radiation esophagitis -Antireflux surgery such as Nissen fundoplication is last -Alarm symptoms: dysphagia, odynophagia, -Gastrinoma resort weight loss, iron deficiency anemia, -Delayed gastric emptying symptom onset after age 50, symptoms Complications persistent despite PPI therapy  refer for -Barrett esophagus occurs in 10% of patients with chronic urgent workup and upper endoscopy reflux  screening EGD recommended for adults 50 or -Asthma older with 5-10 year history of GERD, and EGD q 3 years for patients with known Barrett -Peptic strictures  progressive solid food dysphagia -Esophagitis -Asthma, laryngitis, or chronic cough

Gastritis Differential -Viral Workup -PUD -Indigestion -Urgent EGD for alarm symptoms: severe pain, weight loss, -Nonulcer/functional dyspepsia -Angina pectoris vomiting, GIB, anemia -GERD -Severe pain  esophageal rupture, gastric volvulus, ruptured -Hiatal hernia AA Management -Biliary disease -Erosive gastritis s/p NSAIDs or other meds, alcohol, stress due -PPIs are superior to H2 blockers for treating NSAID-related -Pancreatitis to severe medical or surgical illness, portal HTN dyspepsia and healing related ulcers -Gastric or pancreatic cancer -Pernicious anemia gastritis -Propranolol for portal HTN -Eosinophilic gastritis -Larval infection from raw fish or sushi -H. pylori eradication therapy -Hypertrophic gastropathy Gastric Neoplasms Benign Malignant Gastric Polyps Leiomyosarcoma Adenocarcinoma -Many can undergo -Arises from smooth muscle -High incidence in Korea, Japan, and China malignant transformation so -Rare -Usually occurs after age 60 these should be biopsied -Requires local resection, rarely metastasizes -Risk factors: pickled foods, salted foods, smoked foods, H. pylori, atrophic gastritis, polyps, radiation -Signs & symptoms: early disease is asymptomatic; indigestion, nausea, early satiety, anorexia, weight loss, Others Lymphoma palpable stomach, hepatomegaly, pallor, Virchow’s nodes (L sided supraclavicular) or Sister Mary Joseph -Lipoma -Either MALT or diffuse large B cell lymphoma nodes (umbilical); advanced: pleural effusions, SBO, bleeding -Fibroma -Workup: EGD preferred, endoscopic US to assess tumor depth, barium swallow, CT of pelvis, chest, and -Glomus tumor Sarcoma abdomen to look for mets -Hemangioma -GIST -Management: depends on stage; resection (Billroth or roux-en-Y), chemo, radiation, adjuvants if needed -Prognosis: difficult to cure, most will die of recurrent disease even after resection Peptic Ulcer Disease Causes Risk Factors Workup Treatment -H. pylori -Older age -Barium upper GI -H. pylori eradication with triple therapy and -Chronic NSAIDs -Anticoagulation series films are less confirmation via stool or urease breath test -Excess acid -Steroids sensitive (beware, resistance is on the rise!) -Cigarette smoking -NSAIDs -EGD with gastric -Bland diet NOT shown to be helpful -CMV in transplant pts -Chronic disease biopsy for definitive -Quit smoking as it retards ulcer healing -Crohn’s diagnosis -H2 blockers block 65% of acid secretion and Signs & symptoms -H. pylori test: fecal can heal ulcers in 6-8 weeks Prevention -Gnawing, dull, or aching pain localized to the epigastrium that is non-radiating or urea breath test -PPIs block 90% of acid secretion and can heal -Prevent with cox-2 inhibitors vs -Gastric ulcers are worse after meals preferred over ulcers in 4-8 weeks, preferred agent NSAIDs, PPIs, high dose H2 blockers, -Duodenal ulcers are better after meals antibody test misoprostol if must use NSAIDs -Pain frequently awakens patients from sleep between 2-3 am Sequelae -May be asymptomatic -PUD is the #1 cause of upper GIB Pyloric Stenosis -Typically in 3-6 week olds, usually firstborn males Workup -Rare after 12 weeks -KUB showing “caterpillar sign” of distended, hypertrophic stomach Signs & symptoms -US showing thickened stomach muscle (preferred imaging) -Projectile nonbilious vomiting -Ravenous hunger Management -Palpable pyloric olive -Refer for surgical pyloromyotomy -Poor weight gain -Visible peristaltic waves

GALLBLADDER Biliary Disease

Biliary Disease Info Risk Factors Signs & Symptoms Differential Workup Management Complications Cholelithiasis -Most stones are cholesterol, -Frequently asymptomatic and -RUQ US -Lap chole is fewer are pigment stones discovered incidentally surgical treatment of -Biliary colic: infrequent episodes of choice -Obesity steady severe pain in epigastrium or -Nonsurgical -Rapid weight RUQ with radiation to right scapula -Perforated candidates: loss (Boas’ sign) peptic ulcer dissolution therapy, -DM -May be ppt by large or fatty meal -Acute shockwave -Bile peritonitis Cholecystitis -Usually due to stone lodged -High carb -Severe pain and tenderness in right pancreatitis -CBC shows lithotripsy, -Subhepatic in cystic duct (but can be diet hypochondrium or epigastrium -Appendicitis leukocytosis percutaneous stone abscess acalculous)  secondary -Crohn’s -N/v with high-lying -↑ serum bili, removal or drain -Gallstone bacterial infection (usually disease -Fever appendix AST/ALT, ALP placement pancreatitis Klebsiella or E. coli) -High TG -Murphy’s sign, guarding, rebound -Perforated Amylase may be ↑ -Pancreatitis -Pregnancy tenderness colonic -Cholecystitis: US is s/p endoscopic Choledocholithiasis -Stone has traveled to -Hemolytic -Can be jaundiced carcinoma initial test of choice -Endoscopic sphincterotomy common bile duct anemia -Referred shoulder pain not -Liver abscess -HIDA if suspecting sphincterotomy to -Cirrhosis commonly seen -Hepatitis acalculous allow stone passage -Estrogens -Dark urine or light stools with -Pneumonia cholecystitis through sphincter of -TPN choledocholithiasis with pleurisy -Choledocholithiasis: Oddi -Native endoscopic US or Ascending -When choledocholithiasis American -Charcot’s triad: fever, RUQ pain, MRCP are tests of -Zosyn or Cholangitis progresses to infection jaundice choice ceftriaxone + -Consider in any pt with h/o -Reynold’s pentad: Charcot’s + -Cholangitis: ERCP metronidazole biliary disease who presents confusion and hypotension or abdominal US -Biliary drainage via with recurrent symptoms shows common bile ERCP -Mortality of 50% duct dilation

Biliary Cancer -90% are fatal Cholangiocarcinoma Gallbladder Adenocarcinoma Other Malignancies -Pts are usually asymptomatic -Arises in bile ducts -Accounts for 80% of gallbladder malignancies -SCC -Endoscopic US is imaging of choice for workup -Risk factors: primary sclerosing cholangitis, -S/s: RUQ pain, weight loss, anorexia, nausea, -Neuroendocrine tumors choledochal cysts, Clonorchis sinensis infection obstructive jaundice, ascites -Lymphoma Risk Factors -S/s: Courvoisier’s sign (palpable nontender -Management: surgical resection -Sarcoma -Gallbladder polyps gallbladder + jaundice) -Gallstone disease -Congenital biliary cysts -Anomalous pancreaticobiliary junction -Chronic infection -Porcelain gallbladder

LIVER Cirrhosis Etiologies Workup Management Prognosis -Alcoholic: average consumption of 8 -High INR and low albumin from -For ascites: salt restriction, diuretics, therapeutic paracentesis, TIPS if refractory, AB for -Variable 12 oz beers, 1 L wine, or ½ pint of decreased ability to make proteins secondary infection depending on spirits per day for 20 years -Elevated conj. bili due to inability -For encephalopathy: lactulose to reduce ammonia comorbidities -Non-alcoholic: a result of chronic of liver to process bilirubin, with -Treatment of viral hepatitis if present and etiology inflammation eventual unconj bili ↑ -Alcoholic abstinence -Child-Pugh -US to check for ascites and portal -Hep A and B immunization classification Signs & symptoms vein thrombosis -Transplant estimates 1-year -Portal HTN -Diagnostic paracentesis to survival rates -Ascites: + fluid wave, shifting determine true ascites vs. bacterial -MELD score dullness peritonitis (true ascites is a used for liver -Gastro-esophageal varices difference between serum albumin transplant -Splenomegaly  thrombocytopenia and peritoneal fluid albumin > 1.1) evaluation -Encephalopathy from lack of toxin -Liver biopsy clearance -Screen for varices with EGD

Hepatitis Etiologies Signs & symptoms -Most are due to the hepatitis viruses -Acute: malaise, myalgias and arthralgias, fever, n/v/d, headache, anorexia, dark urine, scleral -Toxins: alcohol icterus, abdominal pain, tender hepatomegaly, lymphadenopathy, splenomegaly -Meds -Chronic: malaise, weakness, cirrhosis symptoms if severe -Industrial organic solvents -Other infections -Autoimmune disease -NASH Type A B C D E Source Fecal-oral Blood & assoc fluids, Blood & assoc fluids, Blood & assoc Fecal-oral parenteral, sex, , dialysis, parenteral, sex, IVDU, fluids tattoing transfusions Incubation 28 days 45-180 days 2-26 weeks Transmission Fecal-oral Percutaneous/mucosal Percutaneous/mucosal Percutaneous or Fecal-oral Transplacental mucosal Prevention Immunization, esp Immunization of all Blood donor screening, Immunization Safe drinking water for travelers, MSM, infants, adolescents and don’t share needles, barrier for HBV drug users, chronic adults in high risk groups, protection during sex liver disease perinatal prevention Presentation ACUTE CHRONIC in 5% unless Usually no acute flare, just CHRONIC ACUTE RUQ pain, n/v you clear it becomes chronic Silently progressive Investigation ↑ ALT/AST + surface Ag with active + AB in present or + AB in present + AB + IgM if acute infection previous infection or previous + IgG if prior/vacc + surface AB with + RNA in active infection infection previous infect/vacc + RNA in active + core AB with active or infection prior infect (NOT vacc) + E Ag with active viral replication + E AB in chronic infect w/o replication, + blood DNA in infection Treatment IFN, antivirals Type 1  direct antivirals, Benign and self- Usually clears pegylated IFN, ribavirin limiting spontaneously Type 2 or 3  pegylated IFN, ribavirin Chronic No Yes, especially in kids Yes in 70% Yes No infection? under 5 Special notes “Infectious “Serum hepatitis” #1 cause for liver Requires Increased severity in hepatitis” Chronic increases risk for transplant coinfection with pregnant women Complications of cirrhosis and HCC 6 genotypes, with 1 most hep B Rare in US fulminant hepatitis, common and hardest to Endemic in India, cholestatic hepatitis treat Mexico, Iraq, North Prevalent in Alaska Liver biopsy useful for Africa, etc. natives, American staging chronic Indians

Liver Neoplasms -Most pts are asymptomatic -Diagnostic approach depends on risk factors of patient and size of lesion -Workup: ↑AFP indicates malignancy Benign Malignant Hemangioma -Risk factors: EtOH, autoimmune hepatitis, viral hepatitis, alpha-1 antitrypsin deficiency, -The most common benign liver tumor Wilson’s disease -Small, asymptomatic -Finding is incidental Hepatocellular carcinoma -Usually occurs with chronic liver disease or cirrhosis Hepatic adenoma -High risk pts should be screened every 6 months via US -Associated with long-term estrogen use -Heightened suspicion for malignancy in previously compensated cirrhosis pts who develop -Can rupture and bleed, so it should be resected decompensation -Lab findings will be nonspecific, but baseline AFP will rise Focal nodular hyperplasia -Diagnostic imaging shows multiphasic tumor -May be a response to a congenital malformation -Treat by resection or radiofrequency ablation, palliative embolization, or liver transplant -Should be resected Metastatic Disease Others -The most common malignant hepatic neoplasms in the Western world -Hamartoma -Cysts: simple, infectious, polycystic liver, biliary cystadenoma, Von Meyenburg complex

PANCREAS Acute Pancreatitis -Occurs with inappropriate activation Signs & symptoms Workup of trypsin within the pancreas  -Range of severity from mild illness to severe -↑ Amylase: not specific, can be ↑ in appendicitis, cholecystitis, perf, enzymatic damage to the pancreas multiorgan failure ectopic pregnancy, or renal failure; elevated for 24 hours -Constant epigastric pain radiation to the back -↑ Lipase: more specific for pancreatitis, but can be elevated in renal -Nausea and vomiting failure; stays elevated for 3 days Etiologies -Tachycardia secondary to hypovolemia from leaky  Elevated amylase or lipase alone without clinical signs are NOT -Gallstones are the most common vessels and 3rd spacing pancreatitis! cause -Fever  Amylase/lipase #s DON’T correlate to severity of disease! -Alcohol use -Sepsis -Bili will be elevated if there is an obstruction blocking it from leaving -Other obstructions: pancreatic or -Icterus or jaundice if there is biliary obstruction the liver ampullary tumors, sphincter of Oddi -Abdominal tenderness with rigidity and guarding -Elevated BUN and hct with vol depletion dysfunction, pancreatic malformation -Acute interstitial pancreatitis: mild, with pancreatic -US showing large, hypoechoic pancreas -Meds: diuretics, azathioprine, 6- edema -CT showing pancreatic enlargement and peripancreatic edema (imaging of choice for pancreatitis) mercaptopurine, sulfa drugs, ACEIs, -Acute necrotizing pancreatitis: severe, with -MRCP or ERCP HIV meds necrosis of parenchyma and vessels  Gray- -Infections: mumps, rubella, Turner’s sign and Cullen’s sign Management Coxsackie, echovirus, EBV, HIV -If mild  NPO with IFV, correction of electrolytes, pain control; -Metabolic: ↑TG, hyperCa Differential resolves in 3-7 days -Toxins: methanol, ethanol, scorpion -Acute cholecystitis or cholangitis -Severe  ICU monitoring, early NGT with tube feeds sting in Trinidad -Penetrating duodenal ulcer -Acute necrotizing pancreatitis  imipenem  -Vascular: vasculitis, ischemia -Ischemic colitis -Gallstone pancreatitis sphincterotomy if suspecting risk of -SBO cholangitis, otherwise plan for lap chole after recovery -Abdominal trauma -AAA -Post-ERCP -Nephrolithiasis Prognosis -Inherited causes -Pancreatic pseudocyst -Complications: inflammatory cascade can cause ARDS, sepsis, or renal failure; pancreatic necrosis or abscess, pancreatic pseudocyst 42

Chronic Pancreatitis -Chronic inflammation Signs & symptoms Management leads to irreversible -Recurrent episodes of epigastric and LUQ pain -Abstinence from alcohol fibrosis of the pancreas -Steatorrhea -Pancreatic enzyme replacement + PPI + low fat diet -Fat soluble vitamin deficiency -Insulin Etiologies -Diabetes -Surgical options for refractory cases: decompression, -Chronic alcohol use resection, or denervation procedures -Chronic pancreatic Workup duct obstruction -Amylase and lipase won’t be elevated because the -Malnutrition pancreas is burned out by now -Autoimmune -Secretin stimulation test to see if the pancreas still works -Hereditary -Abdominal x-ray showing pancreatic calcifications -Idiopathic -CT showing calcifications and atrophy -ERCP showing “chain of lakes” or areas of dilation and stenosis along the pancreatic duct Pancreatic Neoplasms Benign Malignant -Usually asymptomatic and found -Resection via Whipple procedure incidentally -Eval further using MRI, Ductal Adenocarcinoma endoscopic US with FNA -Includes signet ring cell carcinoma, adenosquamous carcinoma, undifferentiated carcinoma, and mucinous non-cystic carcinoma -Accounts for > 95% of pancreatic malignancies Serous Cystadenoma -Typically occurs in ages 70-80 -Most common benign pancreatic -Most commonly in the head of the pancreas lesion -Risk factors: tobacco use, chronic pancreatitis, exposure to dye chemicals, -Low malignancy potential DM2 in nonobese person arising after age 50, history of partial -Resection not recommended gastrectomy or cholecystectomy, genetic factors including BRCA2 -Signs & symptoms: pain, steatorrhea, weight loss, jaundice, Courvoisier’s Mucinous Cystadenoma sign (palpable gallbladder due to compression of bile duct), Trosseau’s -Moderate malignancy potential sign (hypercoagulable state created by the malignancy  migratory thrombophlebitis throughout body) Intraductal Papillary Mucinous -Workup: ALP, bili, initial imaging with RUQ US and ERCP, CT for Neoplasm “double duct sign” (dilation of the common bile and main pancreatic -High malignancy potential if ducts), endoscopic US if other imaging is not convincing, confirmation located within main duct with histologic diagnosis -Management: serial CA-19-9 to follow trend; surgical resection + Solid Pseudopapillary Neoplasm radiation (if there is no invasion, lymphatic involvement, or mets), locally -Low to moderate malignancy advanced disease  radiation only; chemo, pain control, and palliative potential stents for metastatic disease -Should be resected -Prognosis: half of all pancreatic cancers are metastatic by the time of diagnosis, with a life expectancy of 3-6 months; resectable disease survival is < 1.5 years; locally advanced disease survival is 6-10 months

Others -Acinar cell carcinoma -Pancreatoblastoma

SMALL INTESTINE, COLON, AND RECTUM Appendicitis -Usually Signs & Symptoms Workup Management caused by a -Dull periumbilical pain -CBC may show leukocytosis -Surgical consult fecalith that progresses to focal (but can be late finding) -Presence of abscess or rupture sharp pain with RLQ -Abdominal CT preferred in will require cipro or Zosyn radiation adults and nonpregnant women treatment first followed by -Anorexia, n/v -US preferred in peds and appendectomy in 3-4 weeks in -Low grade fever pregnancy kids or colonoscopy in adults -McBurney’s point -If probability for appendicitis is (colon ca can manifest as tenderness high, can go straight to surg appendiceal abscess) -Obturator sign consult in many cases -Psoas sign -Rovsing’s sign Celiac Disease -Immune-mediated inflammation of small intestine due to Signs & symptoms Workup gluten sensitivity (wheat, barley, rye, ?oats) -Malabsorption: -Can have false neg IgA serology as many celiac pts are IgA -Found primarily in white/European patients diarrhea, steatorrhea, deficient -Genetic factors weight loss, vitamin -Definitive dx is endoscopy with small bowel biopsy showing deficiencies villous atrophy Screening -Majority have only -Screen for skin dz, DM1, thyroiditis, bone density -Serologies recommended for pts with failure to thrive, chronic minor GI complaints -Positive serology with negative biopsy may indicate latent diarrhea, chronic constipation, recurrent abdominal pain, or can be celiac disease dental enamel hypoplasia, idiopathic short stature, pubertal asymptomatic delay, iron deficiency anemia not responsive to supp -Secondary Management -Serologies also recommended for 1st degree relatives of pts hyperparathyroidism -Gluten-free diet with celiac, autoimmune thyroiditis, DM1, Down’s, Turner’s, from vitamin D -Repeat bowel biopsy if dx is uncertain Williams, IgA deficiency, and autoimmune hepatitis deficiency -Pneumovax as celiac is associated with hyposplenism -Comorbid autoimmune dz: dermatitis herpetiformis, DM1, -Latent celiac disease should be f/u with repeat monitoring thyroiditis and treated if symptoms develop

Adult Constipation -May refer to < 3 stools per week, difficult-to-pass stools, sense of Workup Pharmacologic Therapies incomplete evacuation, abdominal distension, bloating, or pain -Rectal and abdominal Fiber laxatives Osmotic laxatives -Generally considered to be a functional disorder with 3 subtypes: exam -First-line therapy for constipation -Onset within 24 hours slowed colonic transit, obstructive defecation, constipation- -Alarm symptoms  -Bran powder: may cause gas -MgOH (milk of magnesia, epsom salts) predominant IBS further workup via CBC, -Psyllium (Metamucil, Perdiem) -Sorbitol or lactulose TSH, BMP, referral for -Methylcellulose (Citrucel) -Polyethylene glycol (Miralax) Differential EGD or flex sigmoidoscopy -Ca polycarbophil (FiberCon): pill form -Sodium phosphate (Visicol, OsmoPrep, -Most common: low fiber, sedentary activities, inadequate fluid -Guargum (Benefiber) Fleet’s): not to be used in pts over 55, kidney intake Treatment: disease, or pts taking meds affecting kidney -Systemic: endocrine (hypothyroid, hyperparathyroid, DM), -Increase fiber gradually function metabolic (hypokalemia, hypercalcemia, uremia, porphyria), neuro -Increased # of daily meals (Parkinson, MS, sacral nerve damage, paraplegia, autonomic -Laxatives intermittently or Stimulant laxatives Opioid-receptor antagonists neuropathy) chronically for constipation -Onset in 6-12 hours if oral or 15-60 min if -Effectively blocks peripheral opioid receptors -Meds: opioids, diuretics, CCBs, anticholinergics, psychotropics, unresponsive to lifestyle rectal without affecting central analgesia = good Ca and Fe, NSAIDs, clonidine, cholestyramine change -Bisacodyl: cramping, avoid daily use option for patients on chronic opioids -Structural: anorectal, perineal descent, colonic mass, colonic -Senna (ExLax, Senekot): cramping, avoid -Methylnaltrexone stricture, Hirschsprung daily use -Slowed transit: idiopathic, psychogenic, eating disorder, chronic -Cascara (Nature’s Remedy) pseudoobstruction -Lubiprostone: category C, expensive -Pelvic floor dyssynergia Stool surfactants/emollients -IBS -Not for constipation but to soften stool for patients who aren’t supposed to strain -Mineral oil -Docusate sodium (Colace): marginal benefit Pediatric Constipation ***Encopresis is managed similarly, only without the use of laxatives as long as constipation has been excluded as a cause -Usually begins with an acute Differential Workup Rome III criteria for the diagnosis of functional constipation in episode of constipation then is -Imperforate anus -Criteria: symptoms must be present for 1 month in children self-perpetuating as kids may -Hirschsprung disease repair toddlers and infants and 2 months in older children hold stool to avoid painful BMs -Crohn’s perianal disease -Labs only for kids not responding to an intervention or going at school  chronic -Psychogenic program rectal distension  increased -Hypothyroidism threshold for conscious need to -Tethered cord Management defecate -Spina bifida -Initial disimpaction with enema or Golytely (or -Anterior displacement of the anus lactulose or sorbitol-containing juices in infants) Signs & symptoms -Intestinal pseudo-obstruction followed by maintenance with Miralax (if > 2 years -Encopresis -Cystic fibrosis old, but safety has also been demonstrated in infants) -UTIs -Celiac -Adjust maintenance therapy to goal of 1 soft stool per -Chronic abdominal pain -Lead intoxication day -Poor appetite -Botulism -“Rescue plan” to use stimulant laxative, enema, or -Lethargy -Cow’s milk constipation suppository if there are signs of constipation -Rectal skin tags recurrence -Behavioral modification with toileting regimen and bowel training  sit on toilet for 5-10 min after each meal, give sticker or game reward for each effort, record BMs and symptoms with log

Diverticular Disease Diverticulosis -A 20th century disease associated with Western lifestyle of low fiber, red meat, obesity, and Signs & symptoms increasing age -Many cases of colonic diverticula are asymptomatic and discovered incidentally -Diverticula are outpouchings of the mucosa and submucosa through the muscular layer of the -Chronic constipation, abdominal pain, fluctuating bowel habits colonic wall; they can become perforated and infected with constipation or increased -May have mild LLQ tenderness intraluminal pressure Management -May reduce complications of diverticulosis with fiber supplements Diverticulitis Signs & symptoms Management Prognosis -Mild to severe LLQ or suprapubic pain ± palpable mass -Uncomplicated/simple = empiric treatment with 7- -Complications: lower GIB, -Acute GIB that is painless and maroon in color 10 days of outpatient antibiotics to cover aerobes intra-abdominal abscess or -Fever, malaise, constipation, diarrhea, cramping, bloating, and anaerobes (cipro + metronidazole), clear liquids peritonitis secondary to nausea, vomiting, dysuria, and urinary frequency diet until clinical improvement (2-3 days), surgical perf, fistulas, obstruction consult if no improvement or worsening of -30-40% of cases will have Differential: perforated colonic carcinoma, Crohn’s, symptoms in 72 hours episodic abdominal cramps appendicitis, ischemic colitis, C. diff, ectopic pregnancy, -Refer for colonoscopy, CT colonography, or barium without frank diverticulitis ovarian cyst, ovarian torsion enema with flex sig 2-6 weeks after recovery to -30% of cases will go on to evaluate extent of diverticulosis and exclude have a second attack of Workup malignancy (don’t want to do this right away due to diverticulitis -CBC shows leukocytosis with left shift risk of causing perforation) -CT with contrast is imaging of choice to assess severity -Complicated (peritonitis, obstruction, perf, abscess, -Plain films for free air, ileus, or obstruction or fistula) = hospitalization with IV antibiotics (ampicillin, gentamicin, and metronidazole), IVF, pain management, and antiemetics Intussusception -The most common cause of intestinal obstruction in infants < 1 year Differential -Most cases are between 6 months and 3 years of age -Malignancy if child is over 3 -Can occur multiple times Workup Etiologies -Plain films showing SBO -Idiopathic: most cases -US showing “pseudokidney sign” or “lasagna sign” (test of choice) -Viral Management -Underlying condition: Meckel diverticulum -Refer for emergent reduction via enema or surgical repair

Sings & symptoms -Periodic colicky abdominal pain -Vomiting -Bloody “currant jelly” stools -Palpable mass or “sausage” in RUQ -Lethargy Lactose Intolerance -May be primary or secondary due to bacterial overgrowth, enteritis, Celiac disease, IBD, etc. Management -High prevalence among Native Americans, patients of African descent, and Hispanics -Avoid milk and ice cream as they have the highest amount of lactose -Lactase supplementation (variable results) Signs & symptoms -Add Lactaid to milk and let sit overnight before drinking -Abdominal pain, bloating, farts, diarrhea, and possibly vomiting after ingestion of lactose -Utilize yogurt or cheese for dietary calcium needs, or supplement 46

Inflammatory Bowel Disease -Both are autoimmune -Not everyone needs continued treatment or any treatment at all; treat the affected area -Incidence highest in 15-40 year olds and > 60 year olds -Response to any given treatment is only 30-70% -Tend to run in families -Use steroids sparingly to induce remission -Extraintestinal manifestations possible: eye (uveitis, episcleritis), skin (erythema nodosum, pyoderma -During flare, check WBCs, H/H, f/u with endoscopy referral if not improving gangrenosum), liver, joints -Diagnosis relies on a combination of endoscopy, histology, radiography, labs, and clinical data Crohn’s Disease Ulcerative Colitis -Can affect any portion of GI tract from lips Workup -Disease begins in the rectum and is Workup to the anus and has transmural involvement, -Labs are not specific or reliable limited to the colon with superficial -Labs: ↓ serum albumin, CBC for anemia, however most common site is ileum -Initial imaging is upper GI series with small bowel follow- penetration of the mucosal wall ↑ ESR -Disease skips areas  skip lesions through -Bouts of flares and periods of -Negative stool cultures -Bouts of flares and periods of remission -Colonoscopy shows cobblestoning with varying degrees of remission -Sigmoidoscopy with biopsies showing mucosal ulceration crypt abscesses, chronic colitis for dx Signs & symptoms Signs & symptoms -Barium enema may show “stovepipe” -Aggravated by smoking Management -Proctitis colon due to loss of haustral folds -Fistulas and abscesses -Steroids for flares -Tenesmus -Perianal disease -Gentle wiping, sitz baths, perianal pads for perianal disease -Lower abdominal or pelvic cramping Management -Obstructions -Low-roughage diet only for obstructive symptoms -Bloody diarrhea -Distal colitis  DOC is topical -Prolonged diarrhea and abdominal pain -Mesalamine trials show that it is not effective for Crohn’s -Mucus or pus per rectum mesalamine, hydrocortisone suppositories -Fatigue -Antibiotics during flares have shown little to no efficacy -Fever PRN, second-line therapy is oral -Weight loss -Steroid courses PRN: budesonide has fewer side effects sulfasalazine -Immunomodulating agents for pts unresponsive to steroids Differential -Mild-mod colitis (above sigmoid colon) Differential or requiring chronic steroids (refer to rheumatology): -Infectious colitis: Salmonella, Shigella,  oral 5-ASAs, add hydrocortisone foam -Ulcerative colitis azathioprine, mercaptopurine, methotrexate Campylobacter, amebiasis, C. diff, or enema if needed, refer for -IBS -Annual colonoscopy recommended with > 8 year disease enteroinvasive EC, CMV immunomodulating agents if no response -Appendicitis history -Ischemic colitis -Severe flare  send to ED for -Yersinia enterocolitica enteritis -Crohn’s disease hospitalization -Mesenteric adenitis Complications -Diverticular disease -Screening colonoscopies ever 1-2 years -Intestinal lymphoma -Small bowel strictures -Colon cancer for patients with > 8 year history of disease -Segmental colitis: ischemia, TB, amebiasis, -Fistulae to bowel, bladder, vagina, or skin -Antibiotic-associated diarrhea or Chlamydia -High oxalate from malabsorption of ingested fat (binds Ca) pseudomembranous colitis Complications -Diverticulitis with abscess  kidney stones, gallstones -Infectious proctitis: gonorrhea, -Toxic megacolon -NSAID-induced colitis -Often require surgical management Chlamydia, HSV, syphilis -Extension of colonic disease -Perianal fistula: lymphogranuloma -Radiation colitis or proctitis -Perforation venereum, cancer, rectal TB -Strictures Irritable Bowel Syndrome -Chronic abdominal pain and Signs & symptoms Workup Management altered bowel habits in the -Pain relieved with defecation -Need to r/o IBD, infections, cancer -Patients with elevated IgG can try elimination absence of any organic cause -More frequent stools at onset of pain -FOBT diets (lactose, gluten) = functional -Passage of mucus -CBC, CMP, ESR, serum albumin -Psychiatric eval for anxiety or depression -Subtypes of constipation- -Bloating -Consider TSH -Reassurance that there is no change in life predominant, diarrhea- -Sense of incomplete evacuation -Celiac panel if diarrhea expectancy although there is no cure predominant, and mixed -Urgency -Manning and Rome criteria for diagnosis -Abdominal pain  antispasmodics, -Increased risk of developing -Red flags for something that is NOT IBS that need a antidepressants after acute infectious Differential: dietary, infection, IBD, colonoscopy: abnormal exam, fever, + FOBT, weight loss, -Diarrhea  loperamide cautiously gastroenteritis psychogenic, malabsorption, tumors, onset in older patient, nocturnal awakening, low Hb, -Constipation  bulking agents endometriosis ↑WBCs, ↑ESR

Ischemic Bowel Disease -Most cases are acute Signs & symptoms Management -Risk factors: age, -Diarrhea -Restoration of intestinal blood flow atherosclerosis, low cardiac -Fever -Hemodynamic support output, arrhythmias, severe -Hyperactive phase: passage of bloody stool, -Correction of metabolic acidosis valvular disease, recent MI, severe abdominal pain -Initiation of broad spec AB intra-abdominal malignancy -Paralytic phase: diffuse abdominal pain, tender -NGT for gastric decompression abdomen, bloating, no further bloody stools, -Bowel rest Etiologies absent bowel sounds -Low blood pressure -Shock phase: fluids leaking through damaged Prognosis -Clot colon lining  metabolic acidosis, dehydration, -Most patients make a full recovery without sequelae -Vasoconstriction hypotension, tachycardia, confusion -Idiopathic Colonic blood supply. Pink - supply from superior mesenteric artery (SMA) and Workup its branches: middle colic, right colic, ileocolic arteries. Blue - supply from -Mesenteric angiography is the gold standard inferior mesenteric artery (IMA) and its branches: left colic, sigmoid, superior -Surgical consult rectal artery. 7 is for so-called Cannon-Böhm point (the border between the areas of SMA and IMA supplies), which lies at the splenic flexure

Anal Fissure -A tear or erosion in the epithelium of the anal canal Signs and Symptoms -Acute or chronic -Tearing pain with BMs, although less painful if chronic -Small amount of bright red blood on toilet paper Causes -Usual location is posterior midline -Usually due to large or hard-to-pass stool -Perianal pruritus or skin irritation -Infectious: TB, syphilis, HIV, occult abscess -Acute fissures appear like a paper cut -Carcinoma -Chronic fissures usually have raised edges with external -Granulomatous disease skin tags and hypertrophied pillae -IBD -Prolonged diarrhea Differential -Anal sex -Perianal ulcer: IBD, TB, STDs -Childbirth -Anorectal fistula: differentiate from fissure by tract formation Prevention -Avoid constipation with high fiber and fluid intake Management -Wiping with moist cloth -Stool softeners -Sitz baths -1% hydrocortisone cream -2% nitroglycerine cream: ↑ blood flow and reduces pressure on internal anal sphincter -Surgical consult if not improving in 6 weeks; possible need for internal sphincterotomy

Small Bowel Neoplasms -Malignant tumors very rare when compared to incidence in large Signs and Symptoms Risk Factors for Malignancy bowel -Crampy, intermittent abdominal pain -Familial cancer syndromes: HNPCC, Peutz-Jeghers, FAP -Usually located in the ileum -Weight loss, nausea, vomiting -Chronic inflammation: IBS, celiac disease -GI bleed -Intake of alcohol, refined sugar, red meat, or salt-cured or smoked foods -Intestinal obstruction -Smoking? -Usually asymptomatic if benign -Obesity? Benign Malignant Adenoma Adenocarcinoma Lymphoma -Villous adenomas can transform to malignancy -Arises from glandular tissue -Almost always non-Hodgkin -Duodenal adenomas associated with increased risk for colon cancer -Occurs in duodenum -Includes MALT lymphoma, diffuse large B-cell -Tubular adenomas most common in the duodenum lymphoma, mantle cell lymphoma, and Burkitt lymphoma Carcinoid Tumors Leiomyoma -A neuroendocrine cell tumor arising from the Sarcoma -Arise from intestinal submucosa enterochromaffin cells of the gut -Tumor of the mesenchymal cells -Most common type of small bowel malignancy -Most common type is GI stromal tumor (GIST): may be Lipoma -Usually occurs in the ileum considered benign but all have the potential for malignant -Arise from submucosal or subserosal adipose -With mets can have carcinoid syndrome: watery diarrhea, transformation; should be resected if > 2 cm; consider flushing, sweating, wheezing, dyspnea, abdominal pain, imatinib (Gleevec) as neoadjuvant prior to resection if Other benign small bowel tumors: desmoid tumor, hemangioma, hypotension large; rarely metastasizes fibroma Colorectal Neoplasms Benign Malignant: Adenocarcinoma Non-Neoplastic Polyps -Accounts for 95% of primary colon cancers Signs and symptoms -Hyperplastic polyps = not pre-malignant  more -30% will be in the rectum, 25% on the right colon -Rectal bleeding frequent screening not needed -Risk factors: age, FH (up to 30% have a genetic component), DM2, metabolic -Iron deficiency anemia -Hamartomatous polyps syndrome, ethnicity, IBD, high red meat or processed meat consumption, -Fatigue and weight loss -Inflammatory polyps inactivity, obesity, smoking, heavy alcohol use -Obstruction -Lymphoid polyps -Prevention: diet with plant foods, healthy BMI, limited red meats, physical -Change in stool quantity or caliber activity -Abdominal mass or pain Neoplastic Epithelial Polyps -Weakness -These are pre-malignant  need screens more Associated Familial Syndromes -Mets to the liver and lung frequently for monitoring -FAP: also incurs risk of thyroid, pancreas, duodenal, and gastric cancers -Tubular adenoma -HNPCC: associated with endometrial, ovarian, gastric, urinary tract, renal cell, Workup -Tubulovillous adenoma biliary, and gallbladder cancers -Colonoscopy for biopsy -Villous adenoma -Most occur after age 50 -Abdominal/pelvis CT for staging (“apple core” lesions) -CXR for mets Leiomyoma Screening -Labs: CBC, CMP, baseline CEA for f/u -Tumor of smooth muscle -Begin assessing risk at age 20 -PET -Can occur in colon or rectum -Begin screening at 40-45 for AA patients, at 50 for all other patients of average risk; continue until life expectancy is estimated to be less than 10 years or 85 Management Others years at the latest -Early stage tumors may be removed endoscopically -Lipoma -Begin screening those with FH at least 10 years before the age at which the -Hemicolectomy with lymph node dissection -Neuroma youngest affected family member was diagnosed -Local treatment of mets -Hemangioma -Colonoscopy every 10 years -Chemo to eradicate micromets -Lymphangioma -CT colonography or flexible sigmoidoscopy every 5 years -Radiation not typically used due to its high toxicity in the -FOBT annually for patients in whom imaging or visualization is not possible gut 49

Bowel Obstruction -Obstruction can be Causes of Small Bowel Obstruction Differential Workup mechanical (intrinsic: post- -#1 cause is adhesions from previous surgeries -Paralytic ileus: occurs post-op or after -BMP shows electrolyte derangements from fluid shifts op) or functional (paralytic: -Hernias peritonitis (will see dilated small bowel -Check lactate if concerned for bowel strangulation or electrolyte abnormality, DM) -Neoplasm in presence of dilated colon on KUB) ischemia -Strictures -Intestinal pseudo-obstruction: recurrent -Initial imaging with KUB shows distended loops of small Types -Intussusception abdominal distension in the setting of no bowel, air-fluid levels, free air under diaphragm if perforated, -Simple obstruction = blood -Meckel’s diverticulum mechanical obstruction “swirl sign” where bowel has twisted on its mesentery, and supply intact -Volvulus -Gastric outlet obstruction “bird’s beak” or “corkscrew” if volvulus is also present -Strangulated obstruction = -Intramural hematoma -Intestinal malrotation -Can f/u KUB with CT for further localization compromised blood supply -SBO in absence of prior abdominal surgery should trigger -Closed loop Signs & Symptoms malignancy workup -Obstruction can be -Crampy, generalized abdominal pain complete, partial, or -No signs of peritonitis Management intermittent -Abdominal distension with diffuse midabdominal -IVF tenderness to palpation -Antibiotics Causes of Large Bowel -Suspect ischemia with localized TTP -NPO with NGT decompression Obstruction -Nausea -Volvulus: rectal tube for decompression followed by surgical -#1 is neoplasms -Vomiting, may have coffee-ground emesis or feculent repair to prevent recurrence -Diverticular disease material -Ischemia or perforation: immediate surgical intervention -Volvulus: usually sigmoid -Reduced urine output or cecal -Inability to pass gas -Adhesions -However, pts may still be passing gas and having flatus up to 12-24 hours after onset of obstruction, since the colon requires this much time to empty distal to the obstruction Toxic Megacolon -A potentially lethal complication of colitis that is Signs & symptoms Management characterized by total or segmental nonobstructive colonic -Severe bloody diarrhea -Fluid resuscitation dilation + systemic toxicity -Correction of abnormal labs Workup -IV vanco and metronidazole Etiologies -Abdominal plain film showing R colon -Complete bowel rest -IBD dilation > 6 cm, dilation of transverse -Bowel decompression with NGT -Infectious colitis colon, absence of normal colonic -Surgical consult for subtotal colectomy with end-ileostomy for -Ischemic colitis haustral markings, and air-filled pts not improving on medical management -Volvulus crevices between large pseudopolypoid -Diverticulitis projections extending into the gut lumen -Obstructive colon cancer

Rectal Abscess -Most arise from infected anal glands Workup Management -Pelvic MRI for nonvisible nonpalpable abscess -I&D with culture for pts needing abx or with Signs & symptoms -Use Goodsall’s rule to assess tract location if pain out of proportion to clinical findings -Severe anal or rectal pain fistula is suspected -Complicated abscess location may need OR -Fever or malaise -Antibiotics only for pts with valvular heart -Area of fluctuance or patch of erythema may be visible disease, immunosuppression, extensive externally or many only be palpable on DRE cellulitis, or DM -Fistula may form with non-healing anorectal abscess -Surgical management if fistula present

Hemorrhoids -Engorgement of the venous plexuses of the rectum, anus, or with; with protrusion of the mucosa, anal margin, or both -Classified as internal or external based on position in relation to dentate line

Causes -Constipation or straining -Portal HTN -Pregnancy

Internal Hemorrhoids External Hemorrhoids Classification Signs & symptoms • Grade I hemorrhoids are visualized on anoscopy and may bulge into the lumen but do not extend below the dentate line. -Rarely bleed but are extremely painful, especially if • Grade II hemorrhoids prolapse out of the anal canal with defecation or with straining but reduce spontaneously. thrombosed (exquisitely tender blueish perianal nodule) -Itching • Grade III hemorrhoids prolapse out of the anal canal with defecation or straining, and require the patient to reduce them into their -Visible externally on perianal exam normal position.

• Grade IV hemorrhoids are irreducible and may strangulate. Management -Sitz bath Signs & symptoms -1% hydrocortisone -Painless bleeding after defecation -Stool softeners -Visible during anoscopy -May need to remove thrombosed clot -Not palpable or painful on DRE -Surgical referral if refractory to medical management

Management -1% hydrocortisone -Refer to GI for rubber band ligation if prolapsed (bulging out of anus)

HERNIAS Inguinal Hernias -Risk factors: h/o or FH of hernia, older age, chronic cough, chronic constipation, strenuous exercise, abdominal wall injury, h/o AAA, smoking, ascites -Differential: hydrocele, inguinal adenitis, varicocele, ectopic testis, lipoma, hematoma, sebaceous cyst, hidradenitis, psoas abscess, lymphoma, metastatic neoplasm, epididymitis, testicular torsion, femoral hernia, femoral adenitis, femoral aneurysm -Workup: groin US if uncertain of mass etiology Direct Inguinal Hernia Indirect Inguinal Hernia Femoral Hernia -When intestine plows through -Most common type of hernia -Occurs through the femoral canal, which is just below weak abdominal tissue in area of -Occurs when intestine slips the inguinal ligament Hesselbach’s triangle (bordered by through an abnormally open inguinal ligament, inferior inguinal canal (patent epigastric vessels, and rectus processus vaginalis) abdominis) -Variation is a pantaloon Causes hernia which is a combined -Increased intra-abdominal direct and indirect inguinal pressure hernia where both hernias -Weakening of tissue due to age or straddle each side of the smoking inferior epigastric vessels

Signs and Symptoms Signs and Symptoms -Bulge in area of Hesselbach’s triangle -Bulge in scrotum due to herniation through inguinal canal -Only mild, intermittent pain or discomfort unless incarcerated or strangulated Management -Bordered by femoral vein laterally, lacunar ligament -Signs of sepsis in an incarcerated hernia -Higher risk of strangulation so surgical repair is indicated medially, and Cooper’s ligament below -More common in females Management Prognosis -If only mild symptoms or asymptomatic  consider watchful waiting -Risk of postoperative pain syndrome from damage to ilioinguinal Signs and Symptoms -Attempt manual reduction of incarcerated hernias nerve -Commonly presents emergently as an incarceration or -If symptomatic  surgical hernia repair, usually laparoscopic if strangulation bilateral or recurrent Management Prognosis -Surgical repair -High post-op recurrence Umbilical Hernia -Technically a type of ventral hernia since it is an abdominal wall defect Management -Caused by open umbilical ring, which usually closes in all kids by 5 years but may be slower to -Referral for surgical repair indicated when hernia is incarcerated, extremely large, or symptomatic close in black children -May interfere with feeding if it contains bowel -Rarely become incarcerated or strangulated in kids Ventral Hernias -Caused by defects in the abdominal wall Signs and Symptoms Management -Diastasis recti is an abdominal wall defect but is not a true hernia and does not -Spigelian hernias may not be detected on physical -Most incisional hernias should be repaired (mesh is preferred) due to require repair exam but pts present with mid or lower abdominal risk of incarceration unless very small or large, or upper abdominal and pain and swelling lateral to rectus muscle asymptomatic Types -Incisional: occurs through site of previous surgical incision Workup -Epigastric hernias have low risk for incarceration and only need repair if -Epigastric: occur between umbilicus and xiphoid process -CT to visualize Spigelian hernia symptomatic -Spigelian: hernia through Spigelian fascia -Surgical repair of Spigelian hernias due to high risk of strangulation 52

Other Hernias Internal Hernia Obturator Hernia Littre Hernia Richter Hernia Richter Hernia -Occurs after abdominal surgeries -Occurs when small bowel herniates -Any groin hernia that contains -Occurs when a knuckle of -Any hernia that contains intra-abdominal when the bowel gets trapped as a into the obturator canal Meckel’s diverticulum bowel protrudes into a hernia organs result of new anatomic defect, but only a portion of the relationships circumference is involved and the bowel lumen remains patent Diarrhea Acute Diarrhea -Less than 2 weeks’ duration Non-inflammatory Management -Viral or noninvasive bacteria -Rehydration: ½ tsp salt, 1 tsp baking soda, 8 tsp sugar, 8 Etiologies -Infectious causes: virus, preformed toxin, toxin producing bacteria, oz OJ diluted to 1 L with water -Most common causes are infectious agents, bacterial protozoa (ETEC, Staph, Bacillus cereus, Clostridium, viruses, Giardia) -Antidiarrheals for mild to moderate illness toxins, or drugs -Watery, nonbloody = no fecal leukocytes -Loperamide (non-systemic opioid) as long as there is no -Anal sex: Neisseria gonorrhoeae, syphilis, -Diarrhea may be voluminous blood, high fever, or systemic toxicity lymphogranuloma venereum, HSV -May have periumbilical cramps, bloating, n/v -Bismuth subsalicylates (Pepto Bismol) good for traveler’s -Noninfectious: drug reaction, UC, Crohn’s, ischemic -Prominence of vomiting suggests food poisoning or viral enteritis diarrhea as it is antibacterial and anti-inflammatory colitis, fecal impaction, laxative abuse, radiation colitis, -Typically only eval if persists beyond 7 days or worsens -Empiric antibiotic treatment only for emotional stress immunocompromised, significant dehydration, mod-severe When to evaluate further fever, tenesmus, bloody stools, or presence of fecal Inflammatory -Signs of inflammatory diarrhea: fever > 101.3, bloody diarrhea, abdominal lactoferrin  cipro, Septra, or doxycycline -Invasive or toxin-producing bacteria pain -Antibiotics are not recommended in nontyphoid -Causes: shigellosis, salmonellosis, Campylobacter, -Passage of > 6 loose stools in 24 hours Salmonella, Campylobacter, EHEC, Aeromonas, or Yersinia, C. diff, EHEC, Entamoeba histolytica, Neisseria -Profuse watery diarrhea and dehydration Yersinia gonorrhoeae, Listeria -Frail older patients -Antibiotics are recommended in shigellosis, cholera, -Blood, pus, fever = fecal leukocytes usually present -Immunocompromised patients extraintestinal salmonellosis, traveler’s diarrhea, C. diff, -Diarrhea is typically smaller in quantity -Hospital-acquired diarrhea giardiasis, and amebiasis -Associated LLQ cramps, urgency, tenesmus  Tests indicated: fecal leukocytes, routine stool culture, C. diff if recent -Hospitalization for severe dehydration, severe or -Workup with stool cultures, C. diff, ova, parasites hospitalization or antibiotics, 3x ova and parasites if > 10 d, travel, worsening bloody diarrhea, severe abdominal pain, signs of community water outbreak, HIV, MSM sepsis, or worsening diarrhea in patients > 70 Chronic Diarrhea -Greater than 4 weeks’ duration Workup Management -Not attributed to viruses or bacteria other than C. diff -Ask if diarrhea occurs at nighttime or while fasting -Loperamide -Exclude causes of acute diarrhea, lactose intolerance, -Diphenoxylate with atropine Differential and signs/symptoms IBS, previous gastric surgery, parasitic infections, -Codeine and deodorized tincture -Osmotic (lactose intolerance or other osmotic agents, factitious Mg overuse or laxative use): stool meds, systemic disease of opium: only for intractable volume changes with fasting, ↑ stool osmotic gap -Initial tests: CBC, CMP, Ca, P, albumin, TSH, chronic diarrhea -Secretory (hormonally mediated, factitious, villous adenoma, bile salt malabsorption, meds): > 1L vitamin A, vitamin D, INR, ESR, CRP, IgA for Celiac -Clonidine stool per day, little change with fasting, normal stool osmotic gap, nonanion gap metabolic acidosis, -Stool studies: ova, parasites, electrolytes, fat stain, -Octreotide: for neuroendocrine hyponatremia occult blood, leukocytes or lactoferrin tumors and AIDS diarrhea -Inflammatory (UC, Crohn’s, microscopic colitis, malignancy, radiation): fever, hematochezia, -Consider antigen detection for Giardia and -Cholestyramine abdominal pain, anemia, hypoalbuminia, ↑ ESR or CRP Entamoeba -Meds: SSRIs, cholinesterase inhibitors, NSAIDs, PPIs, ARBs, metformin, allopurinol -Consider acid stain for Crypto and Cyclospora -Malabsorption: weight loss, elevated fecal fat, anemia, hypoalbuminia -Refer for colonoscopy with biopsy -Motility disorders (IBS): systemic disease or prior abdominal surgery -Further testing: 24 hour fecal fat, neuroendocrine -Chronic infections (parasites, AIDS-related) tumors

VITAMIN AND NUTRITIONAL DEFICIENCIES Childhood Nutritional Deficiencies -Supplements indicated for children from neglected or deprived environments, anorexia, inadequate appetite, lead poisoning, failure to thrive, limited sunlight exposure, with chronic disease affecting absorption and utilization of nutrients, who are trying to lose weight, or are on restrictive diets Vitamin Iron Vitamin D Calcium Screening -Hb routinely checked at 12 months, 3 years, annually in -Screen kids with risk factors (premature, -Ask about milk consumption at well child visits teen females, once in teen males exclusively breast fed, vegetarian diet, high altitude, -Screen at 15-18 months for high risk infants malabsorption) Signs of deficiency -Anemia -Rickets -Rickets -Impaired psychomotor or mental development -Osteomalacia -Susceptibility to fracture -Susceptibility to infection -Decreased exercise capacity -Thrombosis Workup -Hb or CBC -25-OH vitamin D level -DEXA scan -Ferritin, Hb electrophoresis, B12, folate -FOBT -Celiac workup -IBD workup Recommendations -Iron supplements for preterm infants until 12 months -At least 400-600 IU daily -Whole milk from 1-2 years of age -Iron-fortified infant formulas -Follow supplementation with laboratory testing -Kids 1-3 need 700 mg of Ca (~2 cups of milk) -No cow’s milk until 12 months -Kids 4-8 need 1000 mg of Ca (~2-3 cups of milk) -Supplement as needed with oral iron -Kids 9-18 need 1300 mg of Ca (~3+ cups of milk) -Recheck CBC every 4 weeks during therapy -Decrease soda intake (P in it associated with bone fx) -Other sources: white beans, broccoli, fortified OJ, salmon, sweet potatoes -Calcium in spinach is not bioavailable! Adult Nutritional Deficiencies Nutrient Copper Iodine Selenium Zinc At risk -X-linked transport mutation -Consumption of uniodized salt -Chinese diet devoid of selenium -Low protein intake -Malabsorption after GI surgery or gastric -TPN without supplementation -From developing country bypass -Ingestion of high doses of Zn Signs & -Anemia -Skin depigmentation -Goiter -Cardiomyopathy -Growth retardation -Immune dysfunction symptoms -Ataxia -Edema -Hypothyroidism  ↓ growth, -Skeletal muscle dysfunction -Hypogonadism -Night blindness -Myeloneuropathy -Osteoporosis development, and cognitive function -Oligospermia -Impaired wound -Fragile hair -Hepatosplenomegaly -Alopecia healing -Impaired taste -Skin lesions Nutrient Chromium Iron Magnesium Vitamin B12 Vitamin D At risk -Hospitalized patients with increased metabolic -Menstruating -ICU -Vegetarians -Elderly -Celiac or IBD demands, especially diabetics women -Homebound -CKD -Short bowel syndrome -Limited sun -Gastric bypass -Burns or trauma exposure -Cystic fibrosis -TPN without supplementation -Obesity -Anticonvulsants Signs & -Impaired glucose tolerance -Microcytic -Insulin resistance -HTN -Lethargy -Osteoporosis symptoms anemia -Constipation -Fibromyalgia -Unwanted weight loss -Nonspecific msk pain -Migraines -Weakness -Mental status change -Rickets -RLS -Trosseau and Chvostek -Anxiety -Alopecia -Cramping signs -Depression 54

METABOLIC DISORDERS Phenylketonuria -Autosomal recessive disorder Signs & symptoms Differential Management -Screened for in newborn metabolic screening -Intellectual disability -BH4 deficiency -Dietary restriction -From defective conversion of phenylalanine to -Epilepsy -Frequent phenylalanine and tyrosine tyrosine -Abnormal gait, posture, or stance Workup monitoring -Phenylalanine is found in breast milk and -“Mousy” urine or body odor -Elevated serum phenylalanine standard formulas -Eczematous rash

OTHER GASTROINTESTINAL SYSTEM TOPICS Hyperbilirubinemia -Jaundice is common in newborns since it is formed at high levels during this time -Minor risk factors: predischarge total bili in the high intermediate risk zone, gestational age 37-38 Management and not cleared as well as in adults weeks, jaundice observed before discharge, previous sibling with jaundice, macrosomic infant of -Total bili values are compared in -Hyperbilirubinemia puts infant at increased risk for encephalopathy and diabetic mother, maternal age > 25 years, male gender percentiles (Bhutani nomogram) kernicterus -Decreased risk factors: total bili in low risk zone, gestational age > 41 weeks, exclusive bottle -Calculate risk zone of infant based -Jaundice within first 24 hours of life is worrisome feeding, black race, hospital d/c after 72 hours on risk factors and total bili values -Jaundice developing in 72-96 hours is physiologic and resolves in 1-2 weeks -Admit for phototherapy if needed -“Breast milk jaundice” begins in the first week after birth, peaks at 2 weeks, and Signs & symptoms -Admit for exchange transfusion if then declines; it is not dangerous and is probably due to the infant’s immature -Appearance of jaundice begins in the face and progresses to the chest, abdomen, arms, and then needed: initiated when liver and intestines legs phototherapy has failed or infant has signs of neuro dysfunction Risk and protective factors Screening -Home measures for low-risk -Major risk factors for infants ≥ 35 weeks’ gestation: predischarge total bili in the -Usually done routinely at time of metabolic screening prior to discharge (USPSTF grade I); infants: increasing frequency and high risk zone, jaundice in first 24 hours, positive DAT or known hemolysis, infants with total bili > 95th percentile are at increased risk efficacy of breastfeeding, gestational age 35-36 weeks, previous sibling received phototherapy, -Routine follow-up appointments after discharge are timed to assess developing jaundice, with f/u supplementing inadequate cephalohematoma or significant bruising, exclusive breastfeeding, East Asian race in 3 days for infants d/c before 24 hours (or sooner if high-risk), and later for infants d/c after 48 breastfeeding with formula hours or beyond

Pediatric Abdominal Pain Chronic Abdominal Pain = greater than 1-2 months duration -Most digestive tract pain is perceived in the midline, so any lateralizing is usually the gallbladder, kidney, ureter, ascending/descending colon, or ovary Organic Etiologies Functional Etiologies

Management -Dietary changes: removing lactose or increasing fiber -Goal is to return to normal function vs. complete elimination of pain -Set plan for return to school (may begin part-time but homeschooling is discouraged) -Pain diaries -Medications for pain triggers: acid, constipation, altered motility -Biopsychosocial model of care receives higher satisfaction in this setting -Refer to GI for alarm symptoms of active or persistent bleeding, weight loss, early satiety -Relaxation techniques with peptic symptoms, loss of appetite, persistent chest pain, persistent vomiting, or failure to improve with medical therapy

Pediatric Acute Abdominal Pain Differential

Neonate 2 months-2 years 2-5 years >5 years

Colic Gastroenteritis Gastroenteritis Gastroenteritis Intraabdominal abscess Dietary protein Viral illness Viral illness Viral illness Ruptured ovarian cyst allergy Trauma (including inflicted injury) Trauma (including inflicted injury) Appendicitis Cholecystitis Volvulus Incarcerated hernia Appendicitis Trauma Pancreatitis Necrotizing Intussusception Pharyngitis Constipation Urolithiasis enterocolitis Urinary tract infection Constipation Pharyngitis Hepatitis Testicular torsion Foreign body ingestion Urinary tract infection Pneumonia (lower lobe  diaphragm irritation) Meckel's diverticulum Adhesions Sickle cell syndrome vasoocclusive Pneumonia Urinary tract infection: may also cause diarrhea Perforated ulcer crisis Intussusception Diabetic ketoacidosis Adhesions Dietary protein allergy Foreign body ingestion Sickle cell syndrome vasoocclusive crisis Hemolytic uremic syndrome Tumor Sickle cell syndrome vasoocclusive crisis Henoch Schönlein purpura Myocarditis, pericarditis Hirschsprung disease Henoch Schönlein purpura Ovarian torsion Primary bacterial peritonitis Adhesions Ovarian torsion Testicular torsion Familial Mediterranean fever Hemolytic uremic syndrome Intraabdominal abscess Inflammatory bowel disease Abdominal migraine Toxin Tumor Meckel's diverticulum Adhesions Hepatitis Hemolytic uremic syndrome Hepatitis Meckel's diverticulum Toxin Primary bacterial peritonitis

Workup -Urine HCG for all menstruating females Management -Unnecessary for kids that are otherwise healthy, well-appearing, -Rapid Strep test -Pain control with morphine is shown to not affect exam and have normal Pes -Imaging for kids with hx of trauma, peritoneal irritation signs, results -CBC with smear for infection and red cell morphology obstructive signs, masses, distension, or focal tenderness or pain -Rule out life-threatening etiologies -Hct for bleeding (for pediatric appendicitis, consult with pediatric surgeon before -Liver enzymes and amylase for suspected hepatitis, cholecystitis, ordering imaging)  abdominal film for obstruction, upper GI or pancreatitis series with contrast for volvulus, US or contrast enema for -BMP for DKA intussusception, CT with contrast when a wide variety of dx are -Urinalysis being considered, US or non-contrast helical CT for urolithiasis

MUSCULOSKELETAL SYSTEM FRACTURE BACKGROUND Pediatric Fractures Types of Fractures General Information -Bowing and greenstick fx are unique to kids due to their -May involve part or all of bone cortex skeletal immaturity -Open = skin or overlying mucous membrane is breached; -Growth plate fx are classified by Salter-Harris closed = no damage to skin or mucous membrane -Most fx only require closed reduction -Kids heal faster due to more active periosteum and Presentation higher % cartilage -Will always cause pain -Tender, swollen, and with mobility at the fracture site Fractures Associated with Child Abuse -Loss of limb function -Metaphyseal corner fx: child abuse until proven otherwise Workup -Posterior rib fx: child abuse until proven otherwise -All suspected fractures need at least 2 views for -Any fracture in a child under 1 radiographs: AP, lateral /-LE fracture in a non-ambulatory child -CT for subtle stress fractures or for inability to detect on -Multiple fractures in various stages of healing x-ray but with high suspicion -Sternal or scapular fx: high impact mechanism such as -MRI: T1 for new fractures, T2 for older fractures MVC required or else it may be child abuse -Spinous process fracture Complications -Lower specificity: clavicular fx, long bone fx, linear -Most commonly DVT or PE skull fx -Compartment syndrome -Avascular necrosis -Nerve injury -Malunion, nonunion, or delayed union -Complex regional pain syndrome form injury to sympathetics (burning pain, skin changes, swelling, excessive sweating at site of injury)

DISORDERS OF THE SHOULDER Dislocations, Separations, and Fractures Injury Type Information Signs & Symptoms Workup Management & Prognosis Shoulder -Usually anterior -Pt will support affected arm with the other arm -Posterior dislocation can be Dislocation -Shoulder may appear flattened life-threatening  immediate -Displaced greater tuberosity and unusual subclavicular ortho consult bulge -Manual relocation -May have loss of sensation over shoulder due to -Can resolve spontaneously axillary nerve entrapment after a few weeks

Injury Type Information Signs & Symptoms Workup Management & Prognosis Clavicle -Usually a pediatric fracture -Affected extremity held close to body -Immobilization in figure 8 Fracture -MOI: direct force to lateral shoulder -Shoulder is slumped downward, forward, and inward dressing from a fall or sporting injury Scapula -MOI: direct violent trauma -May also have injury to ribs, chest wall, or shoulder -Immobilization with sling Fracture girdle and swathe dressing -Shoulder is adducted and arm is held close to the body AC joint -MOI: direct blow to shoulder -Step deformity -Crossover test -Depends on classification separation -Tenderness over joint -X-rays -May need surgery

Common Shoulder Disorders Injury Type Information Signs & Symptoms Workup Management & Prognosis Subacromial -Shoulder pain with motion as well as rest -Bursal fluid aspiration for if signs of -Indomethacin or other NSAID at anti- bursitis -Tenderness of bursa inflammation present inflammatory doses -Steroid injection for severe or persistent pain Biceps tendon -Includes tendonitis, -Pain over anterior shoulder that radiates to biceps -Speed’s and Yergason’s tests -Rest injury tendinosis, subluxation, -Pain aggravated by lifting, pulling, or overhead -US or MRI -NSAIDs partial tears, and complete motion -PT tears -Click -Subacromial or biceps tendon sheath -Night pain injections -Ecchymosis and swelling with rupture -Refer to ortho for rupture -May have other associated injuries such as SLAP tear or rotator cuff injury Rotator cuff -Rotator cuff tendinopathy: night pain, pain with -US is gold standard -Cryotherapy, rest, ice, NSAIDs injury overhead motion -MRI -6 weeks of PT -Rotator cuff tear: weakness, difficulty reaching, -1-2 steroid injections for pain refractory to may be asymptomatic orals -Tears can be chronic or acute -Refer to ortho if suspecting tear or with no improvement in 6-9 months Shoulder -Refers to compression of -Increased translation of humeral head -R/o rotator cuff tear and adhesive capsulitis -PT impingement the GHJ with shoulder -Abnormal acromion morphology -Neer and Hawkins impingement tests -Refer to ortho after 3 months of failed syndrome elevation -Osteophytic changes of the AC joint -Radiographs conservative treatment -Pain with overhead activity -US Shoulder -A result of excessive laxity -Recurrent episodes of shoulder instability and -R/o unidirection cause of instability such as -PT instability of the GHJ  failure to keep pain: looseness, crepitus, anterolateral shoulder a tear -Referral to orthopedic surgery for persistent head of humerus centered in pain -Positive sulcus sign pain or recurrent dislocation episodes glenoid fossa -Crepitation or popping -Positive apprehension test 59

Injury Type Information Signs & Symptoms Workup Management & Prognosis Adhesive -Contraction of GHF capsule -Painful stage for up to 3 months -R/o rotator cuff tear -PT capsulitis -Usually secondary to -Followed by adhesive stage for 3-6 months (less -NSAIDS immobilization after an pain but increased stiffness and reduced -Injections injury movement) -Surgical lysis of lesions -Can also be from thyroid -Recovery stage: ↓pain, slow ↑ ROM, issues, chemo, radiation, DM spontaneous but incomplete recovery

DISORDERS OF THE ARM Arm Fractures Injury Type Information Signs & Symptoms Workup Management & Prognosis Humeral shaft -Typically from trauma in the -Extensive bruising of upper arm -Wrist splinting and casting over site of fracture elderly -Wrist drop from radial nerve damage break

DISORDERS OF THE ELBOW Elbow Dislocations and Fractures Injury Type Information Injury Type Information Nursemaid’s -MOI: being pulled up too hard Medial -MOI: acute valgus sress during Elbow (Radial by hand or wrist  radial head epicondyle FOOSH, posterior stress, Head slipping out of annular ligament fracture chronic muscular traction Subluxation) -S/s: crying, screaming, holding (throwing) arm flexed against belly, refusal -Associated with elbow to use arm dislocation or subluxation -X-ray & assess neurovascular involvement -Reduce with flexion and supination of the arm (usually occurs during x-ray positioning)

Supracondylar -Pediatric fracture Radial head -MOI: FOOSH fracture -Usually involves distal humerus fracture -Decreased ROM in elbow -S/s: Limb ischemia if branchial -Difficult to see on x-ray, may see artery is damaged displacement of fat pad, elbow -X-ray showing posterior sail effusion sign, anterior humeral line drawn will not bisect the capitate

Lateral condylar -MOI: FOOSH with extended fracture elbow, traction forces, or acute varus stress

Common Elbow Disorders Injury Type Information Signs & Symptoms Workup Management & Prognosis Lateral or -A chronic tendinosis from repetitive -Pain with flexion (tennis or racquet) -Ice -Iontophoresis medial motion and overuse rather than acute -Pain with extension (golfing) -Stretch -Surgical release epicondylitis inflammatory process -Strap brace -Injections Olecranon -Onset may be from trauma or may -Red, swollen joint -R/o infection, gout, and triceps rupture -Compression sleeve bursitis be idiopathic -May be painful -Anterior splint -Often seen in truck drivers from -Rarely bursectomy if chronic repetitive leaning

DISORDERS OF THE WRIST Wrist Fractures Injury Type Information Injury Type Information Colles Fracture -MOI: FOOSH  posterior displacement Chauffeur’s -MOI: FOOSH  fracture of radial styloid, of Distal Radius of wrist (“dinner fork deformity”) Fracture of usually due to compression against the -Casting alone if nondisplaced Distal Radius scaphoid -Closed reduction followed by casting if slightly displaced -ORIF & short arm cast if displaced

Smith Fracture -MOI: opposite Colles = fall on back of Scaphoid -MOI: FOOSH of Distal Radius hand Fracture -S/s: fullness or pain in the anatomical -ORIF & short arm cast snuffbox -Difficult to see on x-ray  4 view x-ray & repeat imaging in 10-14 days if negative -Immobilize in thumb spica -Risk of scaphoid necrosis due to poor blood supply

Common Wrist Disorders Injury Type Information Signs & Symptoms Workup Management & Prognosis DeQuervain’s -Tendinosis of sheath surrounding abductor pollicis longus and -Radial wrist pain -Ice -Iontophoresis tenosynovitis extensor pollicis brevis tendons -Rest -Injection -Common in new moms picking up babies -Thumb spica -Last resort is surgical release Scapholunate -Traumatic scapholunate ligament tear -Wrist pain and -Radiograph showing -Surgical dissociation instability Letterman sign of -Can lead to degenerative arthritis and scapholunate increased SL joint space collapse without repair 61

Injury Type Information Signs & Symptoms Workup Management & Prognosis Triangular -Acute or from repetitive use -Tenderness over -Splint fibrocartilage TFCC -NSAIDs complex -PT (TFCC) tear -Injections -Surgical repair

DISORDERS OF THE HAND Hand Fractures Injury Type Information Injury Type Information Boxer’s Fracture -MOI: blow of closed fist against Hook of the -Bust in hamate due to forceful impact as in of Distal 5th another object hamate racquetball Metacarpal -Splinting vs percutaneous pinning fracture -Check ulnar nerve -May need CT to see -Need to excise fragment or cast

Bennet’s -Fracture of the thumb at the Phalangeal -Distal: splint or CRPP if displaced fracture metacarpal base  abductor pollicis fracture -Middle or proximal: buddy tape or ORIF if displaced pulls fragment away -ORIF if unstable

Common Hand Disorders Injury Type Information Injury Type Information Depuytren’s -Occurs when thickened Skier’s thumb -Torn ulnar collateral ligament due to contracture palmar fascia forms nodules (gamekeeper’s abduction stress (breaking rabbit over the flexor tendons  thumb) necks) flexion contracture -Thumb spica if nondisplaced -Surgery indicated for -Surgical repair if displaced or contractures > 30 ° complete tear

Injury Type Information Injury Type Information Trigger finger -AKA stenosing tenosynovitis Mallet finger -Traumatic rupture of extensor tendon distal to -Congenital thickened flexor tendon or DIP nodule at pulley  locking, stiffness, pain -Splint in finger -More common in RA, OA, DM -Injection at the finger flexor crease into sheath -Surgical release

Jersey finger -Traumatic rupture of flexor digitorum profundus at DIP (grabbing football jersey as player pulls away) -Surgical repair within 2 weeks or it will be permanent

DISORDERS OF THE BACK & SPINE Fractures Injury Type Information Injury Type Information Jefferson fracture -Fracture of anterior and posterior arches of atlas Spondylolysis -Stress fx of pars interarticularis, usually (atlas burst) -A result of axial load on back of the head or L5 hyperextension of neck -Seen in gymnasts, football players, -May be accompanied by other cervical spine weight lifters fractures -Pain adjacent to midline and aggravated -Treatment depends on stability of injury = with extension and rotation whether or not transverse ligament is intact -May be asymptomatic -Stable = rigid C collar for 3 months -X-ray showing scotty dog with collar -Unstable = halo for 3 months -Modification of activities -Spinal fusion for large displacements -Core strengthening

Odontoid fracture -Break in the dens from Chance -Compression injury to the anterior portion of the hyperflexion or hyperextension fracture vertebral body as well as a transverse fracture -Can also have Jefferson fracture through the posterior vertebra/vertebral body -Treatment is reduction and halo -MOI: MVA where seatbelt immobilizes pelvis immobilization for 3 months while thorax is thrust forward -Can have permanent neurologic -Unstable injury -Lateral view radiograph

Injury Type Information Injury Type Information Hangman’s -Fracture of both pedicles of the Burst -Vertebral body collapse fracture (C2 axis Fracture -From high height fall with land on feet or butt spondylolisthesis) -Unstable -Unstable -Immobilize in halo -May have neuro sx from fragments extending -ORIF if severe into the spinal canal

Wedge fracture -Collapse of anterior vertebral body with intact Spinous -AKA clay shoveler’s fracture if at C7 posterior wall process -From sudden forceful ligamentous -From hyperflexion +/- osteoporosis fracture traction on or below the spinous process -Stable -Stable

Common Cervical Spine Disorders Injury Type Information Signs & Symptoms Workup Management & Prognosis Cervical strain -Trapezial strain will have localized pain that is reproducible on palpable, may have torticollis, treatment is supportive or sprain -Strain from whiplash may have associated ligamentous injury  get x-ray to eval stability -Acute cervical sprain is ligamentous injury and is graded depending on severity of disruption  investigate with x-rays, will need hard color or immobilization for subluxation Cervical -Degenerative OA of the vertebral -Neck pain -MRI or CT myelogram -Supportive spondylosis discs from repetitive strain or -Radiculopathy -Face injections trauma -Myelopathy (location will point to area of -Surgical decompression with diskectomy -Usually in C5-C6 problem!) or laminectomy -Risk factors: frequent lifting, smoking, excessive driving Cervical stenosis -Narrowing of the cervical spinal -Often asymptomatic until neuro sx -Severity assessed with radiograph and -Conservative treatment if no neuro deficits canal Torg’s ratio (canal:vertebral body width) -Decompressive laminectomy for -May be congenital or acquired progressive neuro deficit -Usually in C5-C6 Brachial plexus -Stretch injury of the brachial -Sudden burning or numbness in lateral arm, -MRI fi symptoms persist > 15 minutes or -ROM neurapraxia plexus thumb, and index finger are repeated -Strengthening (stinger or -Lasts 1-2 minutes burner) Common Thoracic Spine Disorders Injury Type Information Signs & Symptoms Management & Prognosis Hyperkyphosis -Occurs in 20-40% of older adults -Thoracic pain -Exercise-based treatment focusing on postural alignment, flexibility, and -Decreased pulmonary functioning strengthening -Increased fractures -Incurs increased risk of mortality

Common Lumbar Spine Disorders Injury Type Information Signs & Symptoms Workup Management & Prognosis Spondylolisthesis -Displacement of vertebrae -May go unnoticed until adulthood -Radiograph -If asymptomatic  core exercises, no anteriorly or posteriorly -Graded depending on degree of displacement restrictions -Often seen in dancers and -If symptomatic  bracing and core gymnasts exercises -Causes: congenital, traumatic, -If progressive or deficits  surgery degenerative, mets Lumbar strain -Accounts for 70% of low back -Tender paravertebral or erector spinae -Modified activity with exercise pain muscles with minimal radiation -Short-term pain relief Herniated -When torn annulus  -Sciatica -R/o -NSAIDs nucleus pulposus impingement of nucleus on spinal -Positive SLR and FLIP infection or -Muscle relaxers cord -Pain worse on back extension tumor -Exercise -Usually at L5 -Nerve deficits according to root -MRI or CT -Some may need surgery involved

Sacroiliac -Acute or chronic injury to the SI -SI pain with + FABER dysfunction joint -No discogenic pain (no radiculopathy or pain with flexion) Cauda equina -Compression of L2-L4 nerve roots -Loss of bowel and bladder control -Differential: central disc herniation, abscess, -Emergency surgical decompression syndrome  paralysis without spasticity -Bilateral LE weakness and sensory hematoma deficits Lumbar spinal -Progressive degeneration of the -Leg cramping -MRI -PT with core strengthening stenosis disc and facet joints  narrowing -Radiculopathy -NSAIDs of the canal and compression of -Increased pain with sitting or spinal -Decrease impact and bending nerve roots extension with relief of pain with spinal -Surgical decompression for progressive flexion = pt walks stooped over disease -Sensory disturbances -Decreased DTRs -Mild weakness Piriformis -Irritation of the sciatic nerve -Sciatic notch tenderness -R/o herniated nucleus pulposus -Rest, ice, stretching syndrome beneath the piriformis muscle -Injection -Caused by trauma, spasm, or anatomic defect

Injury Type Information Injury Type Information Injury Type Scoliosis -Defined as Cobb angle > 10° -May be detected incidentally -Arm span measurement to -Adolescents with curves at low risk for -Causes: congenital, -Severe curves may result in restrictive detect Marfan’s progression may be followed by primary neuromuscular, idiopathic (the pulmonary disease -Skin examination for care most common kind) -Pain or rapid progression of curve neurofibromatosis, spinal -Refer to orthopedic surgery for increased -Many schools provide screening suggests non-idiopathic etiology dysraphism, tumor, or rotation or Cobb angle, or progression of but efficacy is not proven Marfan’s or Ehlers-Danlos Cobb angle by more than 5° (USPSTF grade D) -Leg length examination to -Refer to specialist for severe pain or neuro -UpToDate recommends routine detect compensatory symptoms screening at well-child visits, scoliosis -Efficacy of bracing is disputed especially before growth spurts -Foot examination and full -Most patients with untreated idiopathic -Bright Futures: begin after age 8 neuro exam (esp abdominal scoliosis have little functional limitation or reflex) to detect pain in adulthood neuromuscular disease -Adams forward bend test -MRI for associated neuro signs, associated pain, early onset with rapid progression, or abnormalities on x-ray -Calculate Cobb angle -Assess skeletal maturity to determine risk for progression of curvature

Ankylosing -Inflammatory back pain > 3 -Bilateral sacroiliitis -Spine film showing syndesmophytes (bony growth -Initial therapy with NSAID like spondylitis months’ duration that is improved -May also have involvement of hips, within spinal ligament)  bridging and fusion of indomethacin with trial for at least 4 weeks by exercise and worsened with rest shoulders, and joints of the LEs, as well vertebral bodies  “bamboo spine” -Augment with other non-opioid and low -Strong association with HLA-B27 as extra-articular manifestations in the -Pelvis film showing erosions and sclerosis at the potency opioid analgesics as needed eye and heart SI joints -Exercise program or PT -Schober’s test shows loss of lumbar -Joint injections for persistent peripheral flexion joint involvement, enthesitis, or sacroiliitis pain -Nonbiologic DMARDs are ineffective for axial disease, need to use anti-TNF agent if nonresponse to NSAIDs -For peripheral disease, can use sulfasalazine or methotrexate -Surgical intervention for severe cases: joint replacement, wedge osteotomy -Poor prognostic indicators are severe hip disease, early age of onset, persistent elevation of ESR

DISORDERS OF THE HIP Hip Dislocations and Fractures -Typically occurs in elderly females -Majority will require corrective surgery -Mortality 20-35% in the first year Injury Type Information Signs & Symptoms Workup Management & Prognosis Hip -Usual posterior (MVA, knee -Hip flexed, -Radiograph -Pain relief dislocation slamming into dashboard) adducted, -Assess neurovascular involvement -Reduce with Allis maneuver internally rotated

Extracapsular -Does not affect blood supply to -H/o fall or trauma -Internal fixation fracture femoral head = complications of -Leg may be shortened nonunion are rare and externally rotated if -Stable vs unstable (detached displacement is present fragment of lesser trochanter) -May also have fx at Intracapsular -Can affect blood supply to femoral another site, usually -Internal fixation if no fracture head, especially if displaced = proximal humerus or displacement commonly complications with distal radius -Hemiarthroplasty often the nonunion and avascular necrosis -Rarely neurovascular treatment of choice due to high risk injury, but can have of avascular necrosis sciatic nerve injury

Femoral stress -Usually occurs in the femoral neck -Groin pain with running that progresses to ADL pain -Hip may be radiographically negative -If nondisplaced  no weight fracture -Seen in thin, young endurance -Pain limits extremes of hip internal and external rotation  need bone scan in 2-8 days bearing for 6-8 weeks athletes -If displaced  ORIF Avulsion -Occurs where sartorius originates -Pain over ASIS and with resisted hip flexion -X-ray -Nondisplaced  RICE, splint in fracture of the -MOI: knee flexion with hip knee flexion with progressive ASIS hyperextension weight bearing -If displaced  ORIF Avulsion -Occurs where hamstrings originate -Pain in buttock -Nondisplaced  RICE with fracture of the -Vigorous hip flexion with knee -Tenderness at ischial tuberosity progressive weight bearing ischial hyperextension -If displaced  ORIF tuberosity

Common Hip Disorders Injury Type Information Signs & Symptoms Workup Management & Prognosis Avascular -Compromise of bone vasculature  death of -Groin, thigh, or buttock pain -Radiograph: can remain normal for months after -Goal is to preserve native joint necrosis bone and marrow cells -Weight-bearing pain symptom onset, early findings are mild density for as long as possible -Causes: femoral neck fx, dislocation, minor -Rest and night pain changes followed by sclerosis and cysts, trauma, steroid administration, alcohol use, sickle pathognomonic crescent sign from subchondral cell, SLE, radiation collapse -MRI if radiograph is nondiagnostic Developmental -Pediatric disease -Abnormal newborn hip exam -US is initial imaging of choice -Referral to orthopedic surgeon dysplasia -Abnormal development of acetabulum and (Barlow-Ortolani) proximal femur with mechanical instability of the -Abnormal leg creases hip joint -Excessive lordosis -Risk factors: breech, FH, female -Trendelenburg gait Groin pull -Strain of hip adductors from forced abduction -R/o hernia or torsion -Rest during fall or collision -Ice -Stretching & strengthening Hip pointer -Contusion of the iliac crest from direct blow -Swelling, tenderness, ecchymosis -X-ray to r/o fracture -Pain meds at iliac crest -Ice -Compression -Progressive stretching

Legg-Calve- -Pediatric disease -Pt is usually age 2-11 -X-ray shows Perthes -Avascular necrosis of the femoral head -Limp mottled femoral disease -Insidious groin and thigh pain head -Loss of internal and external hip rotation

Slipped capital -Occurs when femoral head is displaced from the -Unilateral or bilateral, with many -X-ray -An orthopedic emergency, femoral femoral neck uni cases progressing to bi requires surgical repair epiphysis -Obese, hypogonadic adolescent males are at -Limp increased risk -Affected leg turns out and appears shorter -Loss of hip flexion, internal rotation, and abduction

Injury Type Information Signs & Symptoms Workup Management & Prognosis Snapping hip -When ITB or iliopsoas tendon snaps over ASIS -Hip pain that is worse with -Ice syndrome activity -Activity modification -Snapping with flexion -Strengthening & stability -Increased pain with resisted hip -Injections flexion Trochanteric -Inflammation of burse of greater trochanter -Extreme point tenderness over -Hip stretching bursitis bursa -NSAIDs -Pain at night when lying on -Injection affected side -Pain with hip flexion and extension -Crepitus over greater trochanter

DISORDERS OF THE KNEE Knee Dislocations & Fractures Injury Type Information Injury Type Information Patellar Fracture -MOI: direct blow -MOI: impact, direct axial -S/s: knee pain, difficulty load, or shearing force walking, swelling and bruising, -Lower extremity point tenderness immobilization and no weight -Aspiration will show bearing hemarthrosis with fat globules -Lower extremity immobilization and no weight bearing

Patellar -AP, lateral, merchant, sunrise, dislocation and Laurin x-ray views -Tx: extension brace, quad strengthening, consider surgical repair with multiple recurrences

Common Knee Disorders Injury Type Information Signs & Symptoms Workup Management & Prognosis Torn meniscus -Knee swelling, locking, and catching -Positive Apley’s grind test -May have Baker’s cyst MCL tear -MOI: valgus stress -Medial pain over joint line -Positive valgus stress test -NSAIDs -PT -Graded depending on degree of -Rest -Surgery is rarely tear -Bracing required

LCL tear -MOI: varus stress -Positive varus stress test -Conservative vs surgical depending on -Graded depending on degree of degree of tear tear ACL tear -May have associated Segond fracture (avulsion -MRI to r/o other causes of injury -PT prehab fx of tibial plateau) -NSAIDs -Surgical repair with postop brace PCL tear -Positive posterior drawer, recurvatum, and quad active tests Plica syndrome -When folds of synovial membrane get -Painful snapping -MRI to r/o other causes of injury -PT -Injection stuck in joint spaces  catching -Local swelling -Icing after exercise -Surgical excision -Palpable plica -NSAIDs Osteochondritis -Avascular necrosis  death of bone  -Swelling after -Small effusion -No weight bearing for 6+ weeks dissecans loss of support for articular cartilage  exercise -Tender femoral particles of bone and cartilage rub around in -Locking and catching condyles joint space -Vague pain IT band -Irritation of ITB due to rubbing at the -Snapping hip or knee -PT syndrome femoral head or lateral femoral epicondyle -Instability -Injections -Orthotics -Changing running surface or shoes Patellofemoral -Pain involving the patella and retinaculum -Anterior knee pain that may be acute or -Positive patellofemoral -PT syndrome -Usually an overuse injury gradual compression test -Short-term NSAIDS -May be precipitated by trauma -Positive patellar glide test -Exacerbated by squatting, running, prolonged sitting, or when climbing or descending stairs -Tight hamstrings Osgood- -MOI: anterior tibial tuberosity avulsion -Anterior knee pain that increases gradually -X-ray to rule out fracture -Self-limiting, pain typically subsides after Schlatter due to overuse over time closure of the tibial growth plate at 14-18 disease -Most common in males age 10-14 -Worse with kneeling, running, jumping, years of age squatting, or stairs -Activity as tolerated -Relieved by rest -Stretching, strengthening, and icing -Recent growth spurt -Patellar brace -Recent increased activity -Localized pain and swelling -Step-offs

DISORDERS OF THE LEG, ANKLE, AND FOOT Fractures

Fracture Type Information Signs & Symptoms Workup Management & Prognosis Ankle -Involves lateral, medial, or -Tenderness in these areas suggests fracture vs -Ottawa ankle rules help determine need for x-ray -Elevation and ice Fracture posterior malleolus strain or sprain (Ottawa ankle rules) -Standard AP and lateral views on x-ray (plus AP view with -Short leg cast -MOI: eversion or lateral rotation on 15° internal rotation if suspecting ankle fracture) the talus -Beware commonly missed “FLOAT” fractures

Foot Fracture -Involves talus, calcaneus, metatarsals, or phalanges

Toddler -Spiral fx of distal tibia -Limp -X-ray: may -Long-leg casting Fracture of -Typically in 1-3 year olds -Refusal to bear weight show subtle Distal Tibia -Salter-Harris classification -May not be painful fracture only on 1 view

Common Ankle and Foot Disorders Injury Type Information Signs & Symptoms Workup Management & Prognosis Lateral ankle -Usually an ATFL injury -Ottawa ankle rules help determine need for x-ray -PRICE sprain -The most common ankle injury -NSAIDs -MOI: ankle inversion  stretch -Crutches until weight bearing without limp or tear of lateral ligaments -MRI for symptoms persisting beyond 6-8 weeks -Prevent recurrence with lace-up supports and PT

Medial ankle -From injury to deltoid ligament -May have medial fracture -Ottawa ankle rules help determine need for x-ray -Stretching and band exercises sprain -Rare! such as Maissoneuve fx

Syndesmosis -AKA “high ankle sprain” -Minimal swelling -Positive squeeze test -Refer to ortho sprain -Pain over ATFL -Positive external rotation test -Non-weight-bearing followed by walking boot Achilles -H/o shot-like sound -Positive Thompson test -Serial casting with PT rupture followed by pain -Surgical repair Achilles -Gradual onset of posterior -Short-term heel lift tendonitis pain with activity -NSAIDs -Icing after activity -PT Medial tibial -Posterior tibial muscle tendonitis -Pain worse with activity -Differential: stress fx if presenting with point tenderness and -Ice stress -Point tenderness night pain, compartment syndrome if presenting with -NSAIDs syndrome numbness, pain, and swelling -Decrease mileage (shin splints) -Orthotics -Stretch & strengthen Sever’s -Repetitive microtrauma to the -Pain at calcaneus and -Ice disease calcaneal growth plates Achilles tendon insertion -Massage (calcaneal -Common in 7-15 year olds -Heel pain worse with -Stretch apophysitis) activity -Heel cups or orthotics Haglund’s -Overgrowth of bone on lateral -Change shoes deformity and posterior calcaneus due to -Padding (pump recurrent friction -Ice bump) -Excision of overgrowth

Injury Type Information Signs & Symptoms Workup Management & Prognosis Plantar -Excessive pull at origin of -May have heel spur -Orthotics fasciitis plantar fascia on calcaneus  -Higher heeled shoes inflammation -Ice -Heel cord stretch -NSAIDs

Sesamoiditis -Repeated or direct trauma to the -Metatarsal lift pad sesamoids at the 1st metatarsal

Turf toe -1st MTP joint sprain from -Swelling and tenderness -Graded based on degree of tear -PRICEMM hyperextension of big toe -Decreased ROM at 1st MTP joint

Morton’s -Fibrosis of plantar nerve from -Feels like walking on a -Injection neuroma injury such as repeated walking in marble -Excision tight high-heeled shoes

Injury Type Information Signs & Symptoms Workup Management & Prognosis Bunion -Lateral deviation of the big toe at -Shoe modification (hallux the MTP joint -Osteotomy valgus) -Caused by tight shoes

INFECTIOUS DISEASES Osteomyelitis Agents Signs & symptoms Management -GAS or GBS -Onset can be insidious or chronic -IV AB for 4-6 weeks -Staph aureus -Pain, swelling, tenderness, warmth, overlying cellulitis, fever, chills, n/v -MSSA  nafcillin, oxacillin, or -Polymicrobial in IVDU -Progression of diabetic foot ulcer cefazolin -H/o direct trauma  Staph or Pseudomonas -MRSA or Staph epidermidis  -Salmonella in sickle cell patients Workup vanco -Gold standard is bone biopsy with culture -Surgical draining and Etiologies -Can also treat based on + blood cultures debridement -Hematogenous seeding (usually monomicrobial) -CBC showing leukocytosis -Gram negs  cipro, levo, -Contiguous spread from adjacent soft tissue or joint (polymicrobial) -X-rays only good for chronic osteomyelitis ceftazidime, cefepime -Direct inoculation of bone from trauma or surgery (polymicrobial) -MRI best modality for obtaining details of bone marrow and soft tissue inflammation -Empiric  vanco + Zosyn Septic Arthritis Agents Signs & symptoms Workup -Staph aureus -More likely to manifest in joint with -Synovial fluid aspiration -Streptococci previous arthritis -Plain radiographs in kids to ID joint -Kingella kingae in kids -Usually monoarticular in nongonococcal -Neisseria gonorrheae arthritis (usually knee) -GC test -Syphilis -Fever, chills, n/v Management -Overlying cellulitis -IV antibiotics for 4-6 weeks based on Gram stain Etiologies -Hematogenous spread (most cases) -Bite or other trauma -Joint surgery -Spread of infection from adjacent bone

NEOPLASTIC DISEASE Bone Tumors -Lesions are classified according to matrix produced Malignant lesions Workup -Osteosarcoma: peak age 13-16 -X-ray Benign lesions -Chondrosarcoma -Lesion biopsy if in doubt of nature -Osteoid osteoma -Ewing’s sarcoma -Osteoblastoma -Angiosarcoma Management -Osteochondroma -Fibrosarcoma/malignant fibrous histiocystoma -Surgical excision for giant cell tumor due to aggressive natu -Solitary enchondroma -Chordoma -Surgical management of malignant lesions +/- radio or -Enchondromatosis -Adamantinoma chemotherapy -Periosteal chondroma -Chondroblastoma -Chondromyxoid fibroma Signs & symptoms -Fibrous dysplasia -Usually asymptomatic and discovered incidentally -Osteofibrous dysplasia -Localized pain, swelling, or deformity -Nonossifying fibroma -Pathologic fracture -Unicameral bone cyst (simple bone cyst) -Aggressive or malignant lesions may have lung mets -Aneurysmal bone cyst -Langerhans cell histiocytosis -Giant cell tumor Ganglion Cysts -Swelling in Signs & symptoms Workup Management wrist from -Commonly occur on the wrist, finger joints, or -Light penetration test -Drainage leakage of joint top of foot -Surgical excision fluid -Swelling -Pain -Difficulty moving joint

OSTEOARTHRITIS (DEGENERATIVE JOINT DISEASE) Causes Differential Nonpharmacologic management -Primary -CPPD -Beneficial nonpharmacologic therapy: exercise programs, weight -Secondary: due to trauma, congenital disorder, -Inflammatory loss, wedged sole inserts, canes to offload weight, moist heat crystal disease, or other bone/joint disorder osteoarthritis -Benefit of acupuncture or TENS therapy is controversial -Connective Risk factors tissue disease: Topical drugs -Age > 40 RA, SLE, -Topical NSAID benefit appears to wane after several weeks of use -Female scleroderma, -Topical capsaicin appears to have symptomatic benefit -Obesity Sjogren’s -Inadequate exercise -Trauma Oral drugs -Repetitive low-impact exercise only on -Avascular -DOC is acetaminophen 650 mg q 6 hours or 1000 mg TID neuroanatomically abnormal joints necrosis -NSAIDs shown to be better at relieving overall pain but have greater -Repetitive high-impact exercise on normal joints -Sickle cell GI risks = consider only for failed acetaminophen or mod-severe pain -FH of OA -Tendonitis -Avoid indomethacin for long-term treatment of hip OA (↑ joint -Bursitis damage) Signs & symptoms -Polymyalgia -Only use COX-2 for severe GI risks factors and no CV risk factors -May be localized or generalized rheumatica -Tramadol is useful as add-on therapy to acetaminophen, NSAID, or -Commonly affected joints: cervical or lumbar spine, -Reflex sympathetic dystrophy COX-2 1st CMC, PIP (Bouchard’s nodes, also associated -Seronegative spondyloarthropathy: reactive arthritis, psoriatic -Opioid analgesics should be avoided long-term with RA), DIP (Heberden’s nodes), hip, knee, arthritis, ankylosing spondylitis, IBD -Colchicine for frequent acute inflammatory episodes that don’t subtalar joint, 1st MTP -Infectious arthritis: septic arthritis, Lyme, hep B or C, parvo B19, respond to NSAIDs, injections, or joint irrigation -Rarely affected: shoulder, wrist, elbow, MCP joints rubella -Investigational meds: doxycycline (anti-inflammatory) -Pain that is typically exacerbated by activity and relieved with rest Workup Supplements -Morning stiffness that resolves < 30 min after -Arthrocentesis if pain is severe or acute: synovial fluid aspirate will -Glucosamine and chondroitin appear to have little clinically relevant awakening show clear fluid, few WBCs, normal viscosity, no crystals benefit -Gelling -X-ray will show joint space narrowing, osteophytosis, subchondral -Crepitus sclerosis, subchondral cysts Injections -Bony enlargement -Chondrocalcinosis is a sign of a metabolic, endocrine, or heritable -Intraarticular glucocorticoids can be useful in painful joints despite -Decreased ROM disorder predisposing to OA NSAID use or when NSAIDs are contraindicated -Malalignment -Normal ESR/CRP, RF, anti-CCP -Intraarticular hyaluronans may have some benefit -Tenderness to palpation -May have comorbid CPPD Prognosis -Course is generally slowly progressive

OSTEOPOROSIS Risk factors Screening Management of low T score PTH Antagonists -Meds associated with bone loss: glucocorticoids, -DEXA preferred over peripheral measurements -Treat all postmenopausal women with -Calcitonin: comes in a anticoagulants, anticonvulsants, aromatase inhibitors, -Screen women of average risk > 65 with DEXA established osteoporosis, fragility fracture, nasal spray cyclosporine, tacrolimus, GnRH agonists, barbiturates, Li, -Screen women younger than 65 who have risk factors and select postmenopausal women with -Teriparatide: costs $950 Depo-Provera, chemo, TPN -Screening for men generally not recommended unless osteopenia with pharmacologic therapy per month, AEs -Previous fracture there is evidence of radiographic osteopenia, h/o trauma -First-line therapy is oral bisphosphonates, -Parental history of hip fracture fx, loss of > 1.5 in in height, taking risky meds, androgen 2nd line is raloxifene Hormonal -Low body weight deprivation therapy for prostate cancer, hypogonadism, -Re-check DEXA after 2 years -Raloxifene: black box -Current cigarette smoking hyperthyroidism, etc. warning for risk of DVT -Excessive alcohol consumption -Women with initially good DEXA results need not be Bisphosphonates and fatal stroke with -Rheumatoid arthritis screened again for 10-15 years -MOA: inhibit bone resorption women with CHD -Hypogonadism, premature menopause, malabsorption, -Women with osteopenia on their initial DEXA should be -Alendronate, resideronate, ibandronate, -AEs: DVTs, hot flashes, chronic liver disease, or IBD re-screened anywhere from 1-5 years later, depending on zoledronic acid edema, arthralgia, flu -Advancing age how low their T-score was -Must take on empty stomach and wait 30-60 syndrome, MI, breast minutes before eating and drinking while cancer, stroke Prevention Signs & Symptoms sitting upright (causes acid reflux) -Ca and vit D supplementation recommended for all -Low trauma fracture -AEs: hypocalcemia, dysphagia, esophageal Monoclonal AB patients with inadequate intake -Decreasing height inflammation, gastric ulcer, visual d -Prevent osteoclast -Ca carbonate: Tums, Caltrate, Os-Cal, Viactiv  should isturbance, arthralgia, HA, myalgia, fever formation be 500-600 mg BID Workup after first dose, possible atypical femoral fx = -Costs $825/injection -Ca citrate: Citracal  should 215 mg QID -DEXA: diagnostic if BMD is < 2.5 SD below the young take a break every 5 years, a-fib?, possible -Lots of AEs -Vit D supplements: should be 800-1000 IU daily normal mean at the hip or spine osteonecrosis of the jaw in cancer pts -Exercise, smoking cessation, counseling on fall -Osteopenia is diagnosed if the BMD is 1.0-2.5 SD below receiving IV treatment prevention, avoidance of heavy alcohol use the young normal mean -Contraindications: inability to sit upright for  Also recommended for all postmenopausal women with -If premenopausal, also need to check CMP, CBC, Ca, P, 30 min, esophageal strictures, hypocalcemia osteoporosis vit D, TSH, 24 hour urine for Ca and Cr

RHEUMATOLOGIC CONDITIONS Fibromyalgia -Chronic myalgias and arthralgias in the absence of joint or muscle Differential inflammation on physical exam or laboratory findings -RA -Thought to be caused by alterations in CNS pain processing as well as -SLE genetic and environmental factors -Sjogren’s -Ankylosing spondylitis Signs & symptoms -Polymyalgia rheumatica -Multiple tender points at specific soft tissue locations -Inflammatory myopathy -Comorbid sleep disorder, depression, anxiety, inflammatory rheumatic -Hypothyroidism disease, or noninflammatory msk pain -Peripheral neuropathy -Multiple sclerosis -Myuasthenia gravis -Myofascial pain syndrome -Functional somatic syndromes -IBS -Migraine -Chronic fatigue syndrome -Myofascial pain: will be localized to one anatomic region

Workup -CBC -ESR or CRP -TSH -BMP

Management -Treatment is individualized -Education about uncertain natural history of fibromyalgia -Exercise -Initial DOCs are amitriptyline, duloxetine (good for fatigue), milnacipran, or pregabalin (good for sleep) -Cyclobenzaprine is an alternative -No evidence for opioids -Combination drug therapy for refractory pain, such as SNRI + pregabalin, or fluoxetine + amitriptyline -Continued refractory pain  refer for CBT or to specialist such as rheum for further w/u

Gout -Monosodium urate deposition  arthritis and inflammation Signs & symptoms Management -Peak incidence in males ages 40-50 -First attack is usually podagra (sudden inflammation of the 1st -Do not treat asymptomatic hyperuricemia! -Risk factors: infection, trauma, weight loss, hospitalization, MTP) -Acute attack  continue taking any gout prophylaxis dyslipidemia -Warmth, swelling, dusky red appearance just like a septic joint meds, treat arthritis first with NSAIDs, consider -Usually manifests at night due to fluid shifts giving colchicine for continued symptoms, then treat Etiologies -Systemic signs may be present the hyperuricemia 24 hours after resolution of attack -Usually a result of decreased renal clearance of uric acid (alcohol, -Maximum severity in 12-24 hours (allopurinol ± probenecid, pegloticase, febuxostat) CKD, low urine vol, HTN, diuretics, aspirin, vasopressin, lactic -If chronic: tophi in articular structures, tendons, bursae, or bone -Oral or injectable steroids only for suboptimal acidosis, myxedema, respiratory acidosis, preeclampsia, MI, renal response to NSAIDs & colchicine insufficiency) Differential -Consider pharmacologic prophylaxis with uric acid -Sometimes due to increased renal production of uric acid (purine -Septic arthritis lowering agents (and colchicine bridge if needed to consumption, alcohol use, myeloproliferative disorders, polycythemia, -Trauma avoid ppt attack) if > 2 attacks per year, x-ray leukemia, EBV, psoriasis, drugs) -CPPD/pseudogout erosions, nephrolithiasis or uric acid nephropathy, or -Also influenced by body type, diet, insulin resistance, CHF, and -Other inflammatory arthritis chronic polyarticular gout (wait at least 4-6 weeks organ transplantation after most recent attack and treat for 3-12 months until Workup uric acid goal is reached then d/c) Stages of gout -Joint aspiration with Gram stain, culture, and microscopy is 1.) Hyperuricemia: typically asymptomatic mandatory if possible for all new cases of monoarticular 2.) Acute gout: development may take decades after onset of inflammation, will see urate crystals in joint fluid Prognosis hyperuricemia -Otherwise can diagnose clinically using Rome, New York, or ACR -Without urate-lowering treatment there is greater risk 3.) Intercritical gout: period between attacks diagnostic criteria of tophus formation, development of chronic gouty 4.) Chronic tophaceous gout: caused by repeated attacks  resembles -Plain films will show well-defined erosions in random distribution arthritis, nephrolithiasis, and chronic urate RA with chronic symmetric polyarthritis and tophaceous deposits, less with overhanging edges a well as tophi nephropathy sudden attacks that take longer to resolve -MRI only used to detect early changes Juvenile Rheumatoid Arthritis -Classification of subtypes is still a work in progress -Most commonly used classification is ILAR, with further classification of each group based on age at onset, duration and pattern, and presence of ANA or rheumatoid factor ILAR Classification of Idiopathic Arthritides of Childhood Group Information Signs & symptoms Differential Workup Management Prognosis Systemic -An autoimmune -High fever -Postinfectious arthritis -Diagnosis is clinical, -NSAIDs for 6-12 weeks for -Follows one of three arthritis condition probably -Macular, salmon pink -Reactive arthritis based on presence of mild and nondisabling patterns: systemic symptoms unrelated to other forms rash related to fever -SLE and other connective intermittent fever for at symptoms and no progressive arthritis, of childhood arthritis, spikes tissue diseases least 2 weeks and -Add steroid taper or biologics persistent systemic requiring different -Hepatomegaly -Malignancy arthritis for severe cases or for those symptoms and progressive therapy -Lymphadenopathy -Malaria -Labs will show unresponsive to NSAID trial, arthritis, or resolution of -Accounts for 10-20% of -Arthralgias typically in increased WBCs, followed by DMARD systemic symptoms with cases the wrists, knees, and thrombocytosis, anemia, -Anticytokine therapy for progressive destructive -Can present in kids as ankles high ESR refractory disease arthritis young as 1 Polyarthritis -Involves at least 4 joints -Younger kids: begins -Reactive arthritis -No characteristic labs, -NSAID trial for 3 weeks, -Will be chronic and during first 6 months of with 1-2 affected joints -Psoriatic arthritis may have elevated ESR, followed by a different progressive without illness then spreads -Spondyloarthropathy anemia NSAID if no response treatment -Older kids: rapid onset -SLE -Methotrexate or biologic -Systemic vasculitis in multiple joints -Sarcoidosis -Usually symmetric -IBD -Sausage fingers -Epiphyseal dysplasia -Uveitis -Minocycline-induced autoimm

Group Information Signs & symptoms Differential Workup Management Prognosis Pauciarthritis -Involvement of < 5 -Limping without -Psoriatic arthritis -Diagnosis is clinical -NSAIDs -Many cases resolve within 6 (oligoarthritis) joints during the first 6 complaint -Enthesitis-related arthritis based elimination of -Intraarticular steroids months months of disease onset -Usually large joints are -Infection other causes and on -Methotrexate or biologics -May recur -May involve more joints affected but not the hips -Malignancy presence of arthritis in a rarely required -Uveitis is the most serious over time ( = extended -Swollen, tender joints single joint for at least 3 complication and occurs in pauciarthritis) months or 2+ joints for at 20% of cases least 6 weeks -ANA is usually + -No rheumatoid factor Enthesitis- -Includes childhood -Arthritis + enthesitis -NSAIDs for 3-6 months: -May progress to psoriatic related spondyloarthropathies -Arthritis + 2 or more of frequently diclofenac or arthritis arthritis the following: SI joint piroxicam are used tenderness, inflammatory -Sulfasalazine, biologics, or spinal pain, FH, uveitis, + HLA-B27 DMARDs if no improvement -Gradual onset that may first be recognized following fever or msk trauma Psoriatic -Psoriasis + arthritis -May need to search for -Reactive arthritis -Usually seronegative -NSAIDs: typically don’t -Clinical remission achieved arthritis hidden psoriasis lesions -Ankylosing spondylitis -No specific tests induce remission in most patients after 5 years -Joints tend to be less -Steroid injections into joints of treatment tender than other -DMARDs inflammatory arthritides -Nail pitting or -Add second DMARD or onycholysis biologic if needed -Pitting edema -Monitor for uveitis -Uveitis -Dactylitis Polymyositis -A persistent inflammatory muscle disease that Signs & symptoms Workup causes proximal weakness of the skeletal muscles -Insidious onset with nonspecific symptoms -Elevated CK, LDH, aldolase, and LFTs -Caused by killer T-cells attacking muscle cells -Proximal muscle weakness (can’t get up from chair, can’t raise arms above head) -Autoantibodies (anti-Jo) and + ANA expressing MHC class I (slow fibers)  muscle -Muscle atrophy if long-standing -EMG alteration fiber necrosis, degeneration, and inflammatory cell -Dysphagia, nasal regurgitation, aspiration -Positive muscle biopsy infiltration -Sclerodactyly -Related to dermatomyositis and inclusion body -Low-grade fever Management myositis -Peripheral lymphadenopathy -High dose steroid taper -May be triggered by certain cancers -Interstitial lung disease -DMARDS for patients unresponsive to -Frequent co-occurrence with other systemic autoimmune diseases, another connective tissue steroids disease, or bacterial or viral infection

Vasculitis Syndromes -The vasculitides are characterized by inflammatory Signs & symptoms Workup Management leukocytes in vessel walls with reactive damage to -Systemic symptoms in combination with evidence of single or -Ascertain type of vasculitis: -Depends on severity and type mural structures multiorgan dysfunction CMP, CK, ESR, hepatitis of vasculitis -Can be a primary or secondary process -Nonspecific: fatigue, weakness, fever, arthralgias, abdominal pain, serologies, UA, CXR, echo; may -Stop offending drugs -May case bleeding and compromise of lumen  HTN, renal failure, neurologic dysfunction need CSF, CNS imaging, PFTs, -Antihistamines, NSAIDs, or downstream tissue ischemia and necrosis -Specific: mononeuritis multiplex, palpable purpura, combined blood or tissue culture steroid course for inflammation -Affected vessels vary in size, type, and location pulmonary and renal involvement -More specific tests: ANA, -Monitoring with US complement (deficiency in lupus Types Differential and mixed cryoglobulinemia), Prognosis -Large vessel: Takayasu arteritis, giant cell arteritis -Fibromuscular dysplasia ANCA (Wegener’s) -60-80% 5 year survival with -Medium vessel: polyarteritis nodosa, Kawasaki -Cholesterol emboli -EMG if neuromuscular polyarteritis nodosa disease, primary CNS vasculitis -Infective endocarditis symptoms are present -60% survival with Churg- -Small vessel: Churg-Strauss, granulomatosis with -Malignancy -Tissue biopsy of affected organ Strauss polyangiitis (Wegener’s), microscopic polyarteritis, -Mycotic aneurysm with embolization is essential for diagnosis -75% survival with Wegener’s Henoch-Schonlein purpura, essential -Bacteremia -Arteriogram if suspecting -85% + survival with cryoglobulinemic vasculitis, hypersensitivity -Rickettsial infection vasculitis of large and medium hypersensitivity vasculitis, vasculitis, vasculitis secondary to connective tissue -Thrombocytopenia arteries to look for aneurysms, Henoch-Schonlein purpura, disease, vasculitis secondary to viral infection -Radiation fibrosis occlusions, and vascular wall giant cell arteritis, Takayasu  Criteria for classification of the major forms of -Neurofibromatosis irregularities arteritis vasculitis have been established but only include -Congenital coarctation of the aorta characteristics that help distinguish one disorder -Amyloidosis from other vasculitides = good for research but not -Livedo reticularis very helpful clinically -Cocaine abuse -Hereditary disorders: Marfan, Ehlers-Danlos, etc. -Atherosclerosis -Vasospasm

Polyarteritis Nodosa -Rheumatic vasculitis of medium-sized Signs & symptoms Workup arteries with occasional involvement of -Systemic presentation: fever, fatigue, weakness, loss of appetite, weight loss -CBC showing leukocytosis small muscular arteries -Infarctions manifest as renal failure, HTN, edema, oliguria, uremia, skin lesions, arthralgias, -↑ESR or CRP myalgias, peripheral neuropathies, MI, CHF, pericarditis, and GI tract issues, but typically -CMP Etiology spares the lungs -Rheum workup tailored to differential -Most cases are idiopathic -Skin manifestations include tender erythematous nodules, purpura, livedo reticularis, ulcers, -Confirmatory diagnosis should be made by -Hep B or hep C or hairy cell leukemia are bullous or vesicular eruptions (may be focal or diffuse and can progress to infarction and biopsying clinically affected organ linked to some cases gangrene) -Alternative for dx is arteriography or cross-sectional -Limb edema imaging

Polymyalgia Rheumatica -Seen mostly in white female patients over age 50 Differential Workup -Seronegative RA -Elevated ESR/CRP Signs & symptoms -Bursitis or tendonitis -Pain and stiffness of neck or torso, shoulders or proximal regions of the arms, -Spondyloarthropathy Management and hips or proximal aspects of the thighs for at least 1 month -CPPD -Prednisone -Most severe in the morning and lasts at least 30 min -Hypothyroid -Decreased ROM of the shoulders, neck, and hips -Fibromyalgia -No weakness or decrease in muscle strength -Malignancy -Often co-exists with giant cell arteritis -Infective endocarditis -Dermatomyositis or polymyositis -Vasculitides Reactive Arthritis (Reiter Syndrome) -Acute inflammatory Differential Signs & symptoms Management arthritis following 1-4 -Asymmetric oligoarthritis often affected LEs -Treat active infection found, such as Chlamydia weeks after a GI or GU -Postinfectious arthritis (basically any -Enthesitis -NSAIDs at anti-inflammatory doses infection other organism causing arthritis that -Dactylitis (“sausage fingers”) -Intraarticular glucocorticoids are 2nd line isn’t on the proven list of reactive -Inflammatory back pain -Low dose systemic steroids are 3rd lien Agents arthritis agents) -Conjunctivitis or uveitis -Nonbiologic DMARD is last resort -Usually Chlamydia -Septic arthritis -GU symptoms trachomatis -Crystal arthritis -Oral mucosal ulcers Prognosis -Yersinia -Spondyloarthropathy -Constitutional symptoms -Usually spontaneous remission after 6-12 months -Salmonella -Cutaneous manifestations: keratoderma blennorrhagica, circinate -May evolve to chronic spondyloarthritis -Shigella balanitis, psoriasis-like nail changes -Campylobacter -Clostridium difficile Workup -Chlamydia pneumoniae -Elevated ESR -Negative stool and serologies -Nondiagnostic x-rays -Chlamydia test

Rheumatoid Arthritis -Genetic role with strong Signs & symptoms Differential Management association with HLA-DR1 and -Slow, insidious onset with duration of symptoms over weeks to -Post-infectious sequelae -Goals are to prevent further joint HLA-DR4 months -Other systemic rheumatic disease: SLE damage, prevent loss of function, -Risk factors: nulliparity, older -Waxing and waning of symptoms -Lyme arthritis decrease pain, control systemic age, FH, female, cigs, certain -Usually > 5 joints involved, with small bones of hands and feet -Fibromyalgia -Psoriatic arthritis complications, and maximize quantity of infections usually the first to be involved, with later progression to larger -IBD-associated arthritis life -Protective factors: estrogen, tea, joints -CPPD -NSAIDs should not be used alone as high vit D, breastfeeding -Morning stiffness for at least 1 hour -Polyarticular gout they don’t alter disease course -Aggravated by prolonged periods of rest in the same position -Glucocorticoids are usually used long- Pathophysiology -Fatigue, malaise, low-grade fever, weight loss Workup term-DMARDS should be started within -Triggering incident  -Chronic swelling and joint warmth (but usually no erythema), -Bilateral radiographs of hands, wrists, and feet 3 months of diagnosis proliferation of macrophages and decreased ROM  imaging shows bony erosion with -PT/OT fibroblasts -Hands: boutonniere or swan neck deformities of the fingers, ulnar preservation of joint space -Survey for infections, malignancy, -Lymphocytic invasion of the deviation of the fingers -Rheum labs: ESR and CRP, RF, ANA, anti- osteoporosis, and depression perivascular space -Feet: subluxation of MTPs  calluses on bottom of feet CCP -Local blood vessels become -Wrists: synovial proliferation  median nerve compression, -Arthrocentesis if diagnosis is uncertain Prognosis occluded  outpouching of extensor tendon rupture -Diagnostic criteria: inflammatory arthritis of -Disease will be lifelong with 3-5 year synovial membrane (“pannus”) -TMJ syndrome 3+ joints, +RF or +anti-CCP, ↑CRP or ESR, reduction in life expectancy that eventually invades cartilage -Manifestations in the atlantoaxial joint  UE paresis with head symptoms > 6 weeks, exclusion of other -Spontaneous remission can occur and bone  release of cytokines, movements, pain radiation to occiput diseases on differential (however, it is possible -Complications: infection with unusual proteases, and IL  further joint -Less common manifestations: palindromic rheumatism, to have seronegative RA) pathogens from tx, Felty’s syndrome, destruction by T-cells and monoarthritis of large joint, extra-articular manifestations (skin, Bakers cysts, risk of malignancy macrophages CV, pulm, eye, neuro, heme, renal, bone) DMARDs Agent & MOA Methotrexate: inhibits difolate reductase in Hydroxychloroquine: Sulfasalazine: impairs Leflunomide: inhibits synthesis in WBCs Biologics WBCs interferes with Ag lymphocyte transformation presentation and suppresses NK cells Info -First-line DMARD -The best-tolerated -Good for mild disease -Can be used alone or with methotrexate -Anti-TNFs -Best for moderate-severe disease DMARD, best for mild -Takes 1-3 months to work -Costly -Slows radiographic damage and may reduce disease -Slows radiographic -Used in mortality -Takes 1-6 weeks to work progression combination with -Does not slow radiographic other DMARDs damage so should not be used alone Risks & AEs -AEs: n/v/d, anorexia, alopecia, rash, -AEs: n/v/d, myopathy, HA, -AEs: HA, photosensitivity, -AEs: diarrhea, weight loss, HTN, alopecia, -AEs: HA, myelosuppression, liver or renal failure, retinopathy, rash, n/v/d, anorexia, rash, elevated LFTs  monitor LFTs, Cr, infusion reaction, hyperuricemia, oral ulcers, cough, SOB  agranulocytosis, skin myelosuppression, liver and CBC, signs of infection, pregnancy tests abd pain, need to monitor LFTs, CBC, Cr, CXR, liver pigmentation  monitor kidney failure, oligospermia -Pregnancy X and elimination can take up to vomiting biopsy every 1.5 g with eye exams, CBC,  monitor CBC, LFTs, 2 years so couples wishing to conceive must -Pregnancy X neuro exam BMP undergo a cholestyramine washout -Interactions with NSAIDs, penicillins -Interactions with thiazides -Contraindicated in hepatitis or h/o alcohol and warfarin abuse b/c it’s metabolized in the liver

Systemic Lupus Erythematosus -A result of abnormalities in Signs & symptoms Workup apoptotic cell clearance  -Relapsing and remitting symptoms -Findings accumulate over time, dx may take years generation of autoantibodies to -Fatigue, low-grade fevers -ACR has SLE classification criteria for dx nuclear antigens, phospholipids, and -Malar rash, discoid rash (differentiate from tinea corporis by -CBC, Cr, ESR, CRP, ANA, anti-dsDNA, anti-Sm, anti-RNP, antiphospholipid, anti-Ro, anti- other cell surface proteins its non-fluorescence), psoriasis-like rash La -A type III hypersensitivity -Skin photosensitivity -X-rays demonstration Jaccoud’s arthropathy (ulnar deviation, MCP subluxation, swan-neck -Hereditary predisposition based on -Painless oral or nasal ulcers deformities, and diffuse soft-tissue swelling as a result of tendon laxity rather than RA-type MCH II polymorphisms (HLA-DR2 -Inflammatory arthritis, arthralgias, tenosynovitis, tendon bony destruction) or HLA-DRR3) or complement rupture, osteonecrosis, myositis, myalgias -Head CT may show unidentified bright objects of unknown clinical significance deficiencies  can be triggered by -Vasculitis -UA may show proteinuria and RBC casts if there is lupus nephritis an exposure (infection, UV light, -Serositis around the heart or lung drugs, stress) in a genetically -Glomerulonephritis Management susceptible individual -CNS lupus: seizures, psychosis, transverse myelitis, -Always hydroxychloroquine, which is “lupus life insurance” -Most common in nonwhite women depression, peripheral neuropathy, optic neuritis -Sunscreen 15-40 -Autoimmune hemolytic anemia -NSAIDs for msk complaints, fever, HA, and mild serositis -Pneumonitis, pulmonary hemorrhage, pulmonary HTN, -Systemic steroids for patients with renal, CNS, or other significant organ involvement shrinking lung syndrome -Immunosuppressives (methotrexate, cyclophosphamide, azathioprine, mycophenolate, -Myocarditis, CAD rituximab, etc) for patients with significant organ involvement and inadequate response to -IBD, pancreatitis, liver disease, lupoid hepatitis steroids -Lymphadenopathy -CV risk reduction -For resistant disease, consider high dose chemo followed by autologous stem cell transplant Sjogren Syndrome -Inflammatory disorder of the exocrine glands characterized by infiltration of Workup Management glandular tissue by CD4 T-cells -Salivary gland biopsy -Artificial tears or punctal -Anti-Ro and anti-La, plugs for dry eyes Types other autoantibodies -Biotene mouthwash -Primary Sjogren’s: when there is no other autoimmune disease present -Tests for -Frequent water sipping -Secondary Sjogren’s: when there is another autoimmune disease such as RA keratoconjunctivitis -Cholinergic agonists or SLE sicca: Schirmer test -Hydroxychloroquine for (wetting paper), Rose systemic symptoms Signs & symptoms Bengal stain -May need systemic -Sicca syndrome: dry eyes and mouth -CBC therapy if severe -Gritty or sandy feeling in eyes -CMP -Oral candidiasis -ESR or CRP Prognosis -Parotid or submandibular gland enlargement -UA -Increased risk of -Fatigue developing Hodgkin -Myalgias lymphoma if severe -Vaginal dryness and dyspareunia -Recurrent nonallergic rhinitis and sinusitis, dry cough -Extraglandular organ involvement: cutaneous vasculitis, lupus-like lesions, ILD, CNS and PNS abnormalities, interstitial nephritis

Scleroderma -A chronic systemic autoimmune disease due to Workup defective fibroblast metabolism  skin changes, -Autoantibody fibrosis, vascular alterations, and autoantibodies serologies -May also involve the lungs, kidneys, heart, GI -Serum and urine tract, tendon sheaths, and some endocrine organs protein electrophoresis -Increased incidence in women and black patients -High res CT and PFTs -Usually occurs after age 30 for lung concerns

Etiologies Management -Largely known -Antihistamines for -Exposure to environmental triggers in genetically pruritus susceptible hosts? -Lasering of facial telangiectasias Signs & symptoms -Monitor BP, BMP, -Usually begins in fingers, hands, and face UA, urine protein -Skin thickening and hardening -ACEI if HTN present -Sclerodactyly -GI involvement may -Edematous swelling and erythema require PPIs, abx, -Malaise, fatigue prokinetics, TPN, or -Arthralgias and myalgias surgery -Carpal tunnel, trigeminal neuralgia, or tendon -Acetaminophen or rubs NSAIDs for arthritis -Raynaud phenomenon -PPD-5 inhibitors for -Pulmonary involvement: ILD, pulm HTN ED -Renal disease: albuminuria, HTN, ↑ Cr, -ILD: IV monthly cyclophosphamide or azathioprine + low dose steroids, scleroderma renal crisis in 10-20% pts PCP prophylaxis -Cardiac disease -Screen for ILD with initial high res CT for all systemic sclerosis pts as -Myopathies and neuropathies well as regular PFTs -HA, seizure, stroke -Screen for esophageal dysmotility with manometry -Radiculopathy or myelopathy -Screen for pulmonary HTN with echo for all systemic sclerosis pts -GU symptoms -Surgical repair of flexion contractures -Gastric antral vascular ectasia -GI involvement: rigidity and thinning of oral Prognosis mucosa, GERD, esophagitis, strictures, abnormal -Decreased life span motility, malabsorption, bacterial overgrowth, -Increased risk of cancer diarrhea

OTHER MUSCULOSKELETAL TOPICS Compartment Syndrome -Occurs when increased pressure within a Signs & Symptoms Workup Management of acute compartment syndrome compartment bounded by fascial membranes -Acute: pain out of proportion to the -Compartment pressure measures: -Remove any dressing, splint, cast, or other restriction compromises the circulation and function of injury, pain with passive muscle stretch, should be < 30 mmHg difference -Do not elevate limb tissues within the membrane rapid progression of symptoms, motor between compartment pressure and -Pain management deficits are a late finding systemic diastolic pressure; one -Supplemental O2 Causes -Chronic: pain in involved compartment normal reading does not rule out! -IVF for hypotension -Acute: long bone fracture or other trauma, (usually bilaterally) shortly after start of -Fasciotomy is definitive treatment ischemia-reperfusion injury, coagulopathy, exercise with resolution once exercise has venom reaction or other bite, extravasation of stopped Management of chronic exertional compartment syndrome IFV, recreational drug injection, prolonged -Reduce training volume limb compression -Address strength or flexibility deficiencies -Chronic: overuse injury in endurance athletes -Orthotics -Surgical fasciotomy usually successful Crush Injuries -Really a result of acute traumatic ischemia Signs & Symptoms Workup Management -Rhabdomyolysis from sarcolemma failure  permeability of -Fractures -Electrolytes: hyperkalemia, -Can give bicarb before extrication to shift K+ intracellularly muscle membranes  leak of myoglobin and K+ out of cell -Evident soft tissue injury hyperphosphatemia, -Can give Ca carbonate for K+ cardiac membrane with leak in of water, Ca, Na -Dysrhythmias and EKG changes hypocalcemia stabilization post-extrication (different IV from bicarb!) -Also have local vasoconstriction and platelet aggregation  (peaked T waves, loss of P waves) -High myoglobin -Give insulin with D50W to shift K+ intracellularly ischemia from electrolyte imbalances -Elevated CK (officially -Albuterol to raise insulin level  more intracellular K+ shift -Compartment syndrome from increased pressure within -Red-brown urine rhabdo if > 5x ULN) -Kayexalate to reduce K+ via GI tract (slower onset of action) muscle compartments  muscle, tissue, and nerve death -Compartment syndrome: tight, -Elevated Cr due to AKI -Remove any constrictive clothing, jewelry, or splints shiny, pain out of proportion to from trying to clear -Avoid large boluses of fluid if pt is hemodynamically stable Causes exam, pallor, pulselessness, myoglobin -Mannitol: a non-osmotic diuretic to help wash myoglobin -Building collapse paresthesias, paralysis out of renal tubules to protect kidneys -Trapped in machinery Management -Compartment syndrome: fasciotomy, hyperbaric oxygen -Natural disasters -ABCs -MVCs -Cardiac monitoring Prognosis -Prolonged duration of wearing antishock garment -Fluid resuscitation -Degree of physiologic dysfunction is not related to time -Inability to move away from hard surface (CVA, CO, -Pain management elapsed before extrication hypoglycemia, fall, etc)

EYES, EARS, NOSE, AND THROAT EYE DISORDERS Blepharitis -Chronic eye condition Signs & symptoms Management characterized by lid inflammation -Anterior blepharitis: red, itchy eyes with scales along lash -Regular lid hygiene with intermittent acute bases -Warm compresses exacerbations -Posterior blepharitis: inflammation of the inner portion of -Scrub eyelids with the eyelid at the level of the meibomian glands baby shampoo Causes -Burning or gritty eyes -Anterior blepharitis: staph -Mattering in the morning colonization or seborrhea -Posterior blepharitis: meibomian Workup gland dysfunction, rosacea, or -Evaluate for sebaceous cell malignancy with unilateral or seborrheic dermatitis other unusual symptoms 86

Blowout Fracture -The most common orbital Signs & Symptoms Workup Management fx, occurring with blunt -Anesthesia of the infraorbital -Assess visual acuity and EOM function -Ophtho consult force to the globe or orbit area, maxillary teeth, and/or -Slip lamp exam to assess for any involvement of the -Surgical repair if enophthalmos or rim upper lip eyeball persistent diplopia present -Diplopia -Plain films may show teardrop sign from herniation -Antibiotic prophylaxis for any sinus -Rarely enophthalmos of orbital fat into the maxillary sinus or open bomb- involvement (posterior displacement of bay door sign from bone fragments in the sinus eyeball) -Confirm abnormal radiograph with CT

Cataracts -Opacification of the lens Signs & symptoms -Gradual loss of vision Etiologies -Blurred or smoky vision -Age -Glares -Steroids -Decreased vision in bright light or at night -Diabetes -Electrocution Management -Congenital anomaly -Surgical removal when it interferes with ADLs with -Trauma replacement with an artificial lens

Chalazion & Hordeolum Hordeolum Chalazion = subacute or chronic -Infection of sebaceous or apocrine gland -Sebaceous gland cyst from inflammation of blocked gland -Internal or external ( = stye, on lid margin) -May follow an internal hordeolum -Acute onset, lasting 7-10 days -Usually point inside the lid -Tend to be smaller and more painful -Usually painless but can become acutely inflamed -Causes scratchy sensation and blurred vision -Tend to not resolve without intervention -Treat with warm compresses, consider bacitracin or erythromycin ointment -Refer to ophtho for surgical excision or corticosteroid injection if not resolved in a few months -I&D if not resolving in 48 hours Conjunctivitis Etiologies Workup -Kids & adults: adenovirus, Strep pneumo, Haemophilus, Moraxella, -Culture if extremely purulent Pseudomonas -Infant: think Neisseria gonorrhoeae or Chlamydia trachomatis Treatment -Allergic = conjunctivitis verno -All etiologies are usually self-limiting -Conjunctivitis sicca is chronic dry eye related to rheumatic disease -Throw out contact lenses, wash sheets and hands, will be contagious for 2 weeks -Antibiotics  erythromycin ointment, sulfacetamide drops, FQ drops in contact lens wearers (Pseudomonas) -Hard to distinguish bacterial from viral, all etiologies can cause eyes to be stuck -OTC antihistamine drops for viral causes  Ocuhist, Naphcon-A, Visine AC together in the morning -Acute allergy  short-term antihistamine/vasoconstrictor drops like Naphcon-A, Opcon-A, Visine-A -Bacterial tends to be consistently purulent throughout the day and is usually -Chronic allergy  antihistamine + mast cell stabilizer drops like Patanol or Pataday, Optivar, Alocril, Ketotifen, unilateral Alamast, Elestat -Viral tends to feel more gritty and usually affects the 2nd eye 24-48 hours later -Severe allergy: lodoxamide or cromolyn drops -Allergic will be ITCHY = pathognomonic -If no response in 2 days or need for steroid drops refer to ophtho

Hyphema -Layering of RBCs in the anterior chamber Workup Management due to blunt or penetrating trauma -Emergent ophtho referral if bleeding -Eye shield and reading restriction for 1 dyscrasia, sickle cell, or suspected open week or until hyphema resolves Signs and symptoms globe -Elevate head 30° (prevent settling of -Vision loss -Orbital CT for suspected open globe blood) -Eye pain -Complete orbital and ocular evaluation -Pain control: cycloplegic eye drops -N/v required -Emesis control -Microhyphema visible on slit lamp exam if -Severity determined by grading -Steroid drops not evident immediately -Consider antifibrinolytics -Photophobia -Decrease IOP if needed -Elevated IOP -Surgical clot evaluation for large persistent -Corneal blood staining hyphemas > 10 days, early corneal staining, or difficulty controlling IOP

Prognosis -Can result in vision loss

Dacryoadenitis and Dacrocystitis -Dacryoadenitis = inflammation or infection of the lacrimal Signs & symptoms gland from which tears are secreted -Lid pain -Dacryocystitis = infection within the lacrimal drainage system -Excess tearing or discharge due to an obstructed lacrimal duct and sac -Swelling of preauricular nodes -Acute dacryocystitis: swelling of upper lid, erythema, warmth Etiologies -Dacryoadenitis: autoimmune diseases, thyroid eye disease, Management orbital pseudotumor -Simple nasolacrimal duct obstruction  lacrimal duct massage, -Dacryocystitis: EBV, mumps, Staph, gonorrhea warm compresses, referral to ophtho for probing if not improving -Acute dacryocystitis  emergent referral to ophtho and clindamycin or vancomycin due to risk of MRSA -Think malignancy if no improvement Entropion -Folding of the eyelid in ward Signs & symptoms Management -Eye redness and pain -Surgical repair before permanent corneal Causes -Photophobia damage is done -Genetic -Sensitivity to wind -Congenital -Decreased visual acuity -Infection: Chlamydia -Aging -Scarring

Ectropion Causes Signs & symptoms Management -Weakening of tissue or aging -Sagging lid -Surgical repair -Allergic -Dull light reflex -Facial nerve palsy -Eye irritation -Chemo treatments -Congenital

Corneal Abrasion Corneal ulcer = infected corneal abrasion Management -If abrasion is suspected but not visible, need to do Etiologies fluorescein stain under UV light -Traumatic -Rule out FB: flip eyelids-No contact lenses -Foreign body -No steroids -Contact lens -Pain control: cycloplegic, opioids, topical anesthetics -Spontaneous defect in -Don’t leave pressure patch on > 24 hours corneal epithelium =Any corneal infiltrate, white spot, or opacity needs ophtho referral Signs & symptoms -F/u not necessary for most small abrasions as long as -Severe eye pain symptoms improve and vision remains good (except -Foreign body or gritty sensation contact lens wearers) -Photophobia -Excessive tearing Prognosis -Blurred vision -Most abrasions heal regardless of therapy in 24-72 -Headache hours, with return of vision to normal -Blepharospasm -Hazy cornea -Conjunctival injection -↓ visual acuity

Differential -Corneal FB -HSV keratitis

Foreign Body Signs & symptoms Workup Management -Surface FB: pain and -Visual acuity testing -Ophtho consult for intraocular FBs irritation with eye -Flip eyelids -Anesthetic eyedrops movement -Slit lamp exam with -Fine gauge needle to remove FB -Intraocular FB: fluorescein -Metal objects can cause rust rings around them, but these usually resorb discomfort, blurred vision, -CT or plain film if no so don’t try to remove or you could cause damage h/o striking metal or findings on slit lamp -Erythromycin ointment and patching following removal explosion -F/u with ophtho in 48 hours

Macular Degeneration -Damage to the retina causes loss of central vision Risk factors -Age Forms -Genetics -Non-exudative (dry): when drusen accumulates between the -Smoking retina and the choroid  loss of cones; accounts for 90% of -HTN cases -Micronutrient levels -Exudative (wet): when blood vessels grow up from the choroid behind the retina Management -Smoking cessation -Vitamins and supplementation with zinc, beta carotene, copper -Lasering or surgical extraction for neovascularization or macular translocation -Antiangiogenesis therapy

Nystagmus -Regular rhythmic oscillation of the eyes Signs & symptoms Differential Management -Vertigo -Meds: Li, anticonvulsants -Treatment is symptomatic Jerk nystagmus -Oscillopsia -EtOH intox -Treat underlying cause -Alternating phases of a slow drift in one direction with corrective -Abnormal head -Demyelinating disease -Baclofen for periodic alternating nystagmus quick jerks in the opposite directly position -Vertebrobasilar insufficiency -Gabapentin for pendular nystagmus -Caused by imbalance in semicircular canals due to peripheral -Blurred vision -Brainstem stroke -Botox injections vestibular disease or disruption of central vestibular pathways in the -Head trauma -Special prism contact lenses or glasses brainstem -Positional vertigo -Surgical correction of muscle attachments -Thiamine or Mg deficiency Pendular nystagmus -Neoplasm -Slow, sinusoidal oscillations to and fro -Arnold-Chiari malformation -May be acquired or congenital -Encephalitis -Extreme lateral gaze -Normal opticokinetic nystagmus

Glaucoma Preventative Eye Exams Age Black Patients White Patients 20-40 Every 2-4 years No guidelines 40-54 Every 1-3 years Every 2-4 years 55-64 Every 1-2 years Every 1-3 years 65+ Every 1-2 years Every 1-2 years

Closed Angle Glaucoma (Narrow Angle Glaucoma or Acute Angle Glaucoma) -Fluid builds up behind the lens due to Signs & symptoms Management malformed iris and trabecular network -Occurs after being in a dark place or after -Give acetazolamide and timolol, contacting each other  sudden blocking of anticholinergic use apraclonidine, and pilocarpine drainage canal -Prior episodes of blurred vision -Emergent referral to ophtho, monitoring of -Most common in Asians and older folks -Halos around lights IOP q hour until seen -H/o recent eye surgery or uveitis -Nausea/vomiting -Eye redness -Nonreactive pupil -Hazy cornea -Shallow depth of anterior chamber -Optic disc cupping

Open Angle Glaucoma (Wide Angle Glaucoma) -Fluid builds up in front of the lens as a result Signs & symptoms Management of slowly clogging drain  sequential damage -Bilateral eyes affected -Regular aerobic exercise to reduce IOP to optic nerve with progressive loss of visual -Colored halos around lights -Increase outflow with intraocular field -Progressive peripheral vision loss prostaglandin analogues -Most common type of glaucoma -May be asymptomatic until severe visual field -Suppress production with intraocular β- -Most prevalent in blacks over 40 and others loss blockers over 65  screen with visual field -Pupil dilation confrontation

Optic Neuritis -Acute inflammatory demyelinating Differential Management injury to the optic nerve -Ischemia -IV methylprednisone if severe vision -Infection: meningitis, syphilis, Lyme loss of 2+ white matter lesions on MRI Signs & Symptoms -Inflammatory: sarcoid, neoplasm, (NOT oral as it has no benefit) -Painful monocular visual loss over SLE -Interferon treatments if MRI shows several hours to days -Genetic white lesions (may delay development -May have papillitis on funduscopic -Neoplastic of MS) exam -Compression: pseudotumor cerebri -Chronic: afferent pupillary defect, -Toxic/metabolic: drugs, nutritional Prognosis color desaturation, optic atrophy deficiency, radiation -Visual recovery within a few weeks -Trauma -30% will go on to develop MS within 5 years Workup -Gadolinium-enhanced MRI of brain and orbits

Pterygium -Triangular wedge of fibrovascular Signs & Symptoms Management conjunctival tissue extending into the corneal -Redness and irritation -Artificial tears to reduce irritation surface -Growth over months to years -Surgical removal if visual impairment, -Vision impairment restricted eye movement, significant cosmetic Causes -Often bilateral impact, or intractable irritation -UV light exposure -Abnormal angiogenesis Differential -HPV -Conjunctival neoplasia -Pinguecula (no corneal involvement)

-Conjunctivities

Orbital Cellulitis -Usually occurs in kids as a result of Signs & symptoms Workup Management untreated sinusitis or trauma -Eyeball proptosis or chemosis -CT or MRI -Admit for IV antibiotics (amoxicillin or ceftriaxone + vanco for -Edema, erythema, hyperemia, periorbital pain severe cases) Agents -Limited eye movements -Strep pneumo -Reduced vision -H. flu Papilledema -Optic disc swelling due to ↑ ICP Differential Workup -Intracranial mass lesion -Funduscopic exam and visual acuity testing Signs & symptoms -Cerebral edema -Brain MRI -Will usually be bilateral -↑ CSF production or ↓ absorption -LP -Headache -Obstructive hydrocephalus -N/v -Obstruction of venous outflow -Diplopia -Pseudotumor cerebri -Pulsatile machinery-like sound in ear -Intermittent/brief visual symptoms

Retinal Detachment -Occurs when retina peels away Signs & Symptoms Workup Management from underlying support tissue -Sudden painless loss of vision -Emergent ophtho consult -Immediate surgical repair “like a curtain covering the eye” if suspecting Causes -May see flashes or floaters or Prognosis -Increased risk with myopia, trauma, cuts or lines in vision -Will lead to complete vision FH, cataract surgery, diabetic -Defect in confrontational visual loss without surgical repair retinopathy fields

Central Retinal Artery Occlusion -Loss of blood supply to the retina Signs & symptoms Management via embolus -Painless, severe loss of vision in one eye -Emergent ophtho consult with interventions to -Risk factors: a-fib, endocarditis, -Funduscopic exam shows cherry red spot, pale or swollen lower IOP coagulopathies, CAD, optic nerve with splinter hemorrhages, ground-glass retina -Antiplatelets hypercoagulable states, temporal arteritis Workup Prognosis -CV exam for bruits and temporal arteritis -Poor, no treatments proven to improve visual -Carotid imaging outcomes -EKG

Central Retinal Vein Occlusion -When occluded retinal vein backs up and fills Signs & symptoms Management the retina with blood -Painless loss of vision in one eye -Treat underlying medical disorders -Risk factors: HTN, mechanical compression, -Aspirin therapy glaucoma, inflammation of the optic nerve, -Lasering of ischemic retina orbital disease, hyperviscosity disorders -Treat associated glaucoma and macular edema

Diabetic Retinopathy -Occurs when hyperglycemia damages the Screening Signs & symptoms basement membrane of retinal capillaries  -Yearly eye exams for diabetics -Most are asymptomatic loss of pericytes and microaneurysm -Maintain A1c < 7% formation  leakage of capillaries and -HTN control Management macular edema with proliferation of weak -Nonproliferative  panretinal blood vessels photocoagulation if severe -Associated with DM1 and DM2 but not -Proliferative  panretinal photocoagulation gestational diabetes if high risk or severe, early vitrectomy if severe and DM1, intravitreal VEGF inhibitors as therapy adjunct

Strabismus (Tropia) Etiologies Differential Management -Congenital: pseudostrabismus, prenatal drug -Pseudostrabismus -Refer to ophtho for consistent exposure, nerve palsy, familial external -Ocular instability of infancy (normal in first few strabismus at any age, persistent ophthalmoplegia months of life) strabismus after 4 months of age, altered -Acquired: accommodative strabismus, light reflex, deviation with cover test, intermittent exotropia, cataracts, tumors, deviation that changes depending on increased ICP, orbital injury, head trauma, position of gaze, torticollis, parental vascular disorders, botulism, myasthenia Workup concern gravis, nerve palsy, Guillain-Barre, ocular -Affected eye will drift when covered, then moves myopathy, multiple sclerosis, infection, drug or quickly back if cover is removed Sequelae toxin, DM, hypoglycemia, thyrotoxicosis -Differentiate congenital from acquired (may be -If untreated may lead to amblyopia vision-threatening or life-threatening) (vision reduction) or diplopia

Uveitis (Iritis) -Inflammation and leukocyte Causes Signs & symptoms Management infiltration of the iris, ciliary -Infections: CMV, toxoplasma, syphilis, -Depends on area of eye affected -Ophtha consult body, and/or choroid and vitreous HSV, cat scratch disease -Anterior chamber  pain and -Treat any infectious cause humor of the eye -Systemic immune-mediated disease: redness -May need steroids spondyloarthropathies, IBD, -Posterior chamber  painless sarcoidosis, MS floaters or vision loss -Syndromes confined to the eye -Masquerade syndromes: CNS Workup lymphoma -Slit lamp and funduscopic exam

EAR DISORDERS Otitis Media Acute OM Management -Agents: Strep, H. flu, M. cat, or viral (can’t distinguish) -If mild, can watchfully wait with NSAIDs for pain relief as -Signs & symptoms: hearing loss is hallmark, ear pain, ear long as patient is > 2 years fullness, drainage with relief if ear drum is perforated, prior -If infection is obvious or there is a fever, treat with 10-14 d of URI, pulling at ears, fever, irritability high dose amoxicillin, erythromycin, Augmentin, Septra, ceftriaxone Chronic suppurative OM = frequent AOM with otorrhea as a -Refer for surgical management if there is bilateral effusion > 3 result of TM perforation or tube placement months and bilateral hearing deficiency -Chronic  tx with 10 d of FQ, consider chronic therapy with OM with effusion = fluid behind TM without presence of daily amoxicillin during winter and spring with monthly f/u infection, a result of chronic eustachian tube dysfunction, previous AOM, or barotrauma

Otitis Externa Etiology Signs & symptoms Management -Bacterial 90% of the time: Pseudomonas, Strep, Staph -Pain with manipulation of tragus -Bacterial  neo/poly/HC only if TM intact, FQ (use a wick if canal is swollen), systemic -Fungal: Aspergillus, Actinomyces, Candida -Hearing loss therapy if canal is swollen shut or pt is immunocompromised -Eczema if chronic -Otorrhea -Fungal  acetic acid/HC drops, clotrimazole drops -Malignant otitis externa = osteomyelitis of temporal bone -Fullness -Bacterial vs fungal?  CASH powder covers both as a result of chronic infection in DM, not cancerous! -Itching -Chronic  treat eczema with steroid cream, then use vinegar/water washes and avoid Q-tips -Recent exposure to water -Malignant  emergent referral to ENT

Ear Barotrauma Causes Signs & symptoms Management -Water diving -External ear barotrauma: ear pain, bloody otorrhea, petechiae, -Keep ear dry -Ascent into the atmosphere hemorrhagic blebs, or rupture of TM -Pain control -Mechanical ventilation -Middle ear barotrauma: pain and fullness on descent in airplane -Decongestants or antihistamines -URI with failure to pop ears, or may have URI symptoms -Prevent recurrence with decongestants prior to airplane travel Cholesteatoma -Keratinized, desquamated epithelial collection Signs & symptoms Workup in the middle ear or mastoid -May be asymptomatic -CT or MRI if complications present or to -Hearing loss better characterize extent Causes -Dizziness -Primary lesion -Otorrhea Management -Secondary to TM perforation -White mass visible behind TM -Surgical removal -TM retraction -Obstructive polyp

Eustachian Tube Dysfunction -Occurs when blockage of the eustachian tube Signs & symptoms Differential Management allows air to exit middle ear but not come back -TM retraction -Allergies Acute: nasal steroid spray, decongestants in  creation of negative pressure atmosphere -Ear pain -URI -Chronic: tube placement bilaterally in middle ear -Hearing loss -Nasopharyngeal mass -Ear fullness -Abnormal anatomy Prognosis -Risk of cholesteatoma if untreated Ear Foreign Body Causes Management -Embedded earring -Local anesthesia with removal for embedded jewelry -External ear FB: beads, pebbles, tissue paper, -Office removal of small inorganic objects or insects with irrigation (95% ethanol, mineral oil, or 1% lidocaine), followed by forceps or superglue on insects q-tip for further extraction -Systemic antibiotics if perichondritis or chondritis present -ENT referral for button batteries, vegetable matter (will swell with irrigation), penetrating FB, or associated injury to TM Tympanic Membrane Perforation Causes Signs & Symptoms Workup Management -Penetrating trauma -Acute onset pain and hearing loss ± bloody otorrhea -ENT consult for serious -Most cases will heal on their -Noise trauma -May have tinnitus or vertigo hearing loss, otorrhea, facial own nerve paralysis, or -Need f/u hearing testing nausea/vomiting, nystagmus, -Avoid water in ear or ataxia

Hearing Loss -Conductive, sensorineural, or mixed Workup Management -Office hearing evaluation with Rinne -Standard air conduction Etiologies and Weber tests hearing aid -Conductive, outer ear: congenital, external otitis, trauma, -Formal audiologic testing if there is -Bone conduction device for squamous cell carcinoma, exostosis, osteoma, psoriasis, cerumen no obvious etiology congenital atresia, chronic -Conductive, middle ear: congenital, otitis media, cholesteatoma, -Contrasted MRI or CT if there is ear infections, or single- otosclerosis, tympanic membrane perforation, temporal bone progressive asymmetric sensorineural sided deafness trauma, glomus tumor hearing loss -Cochlear implant -Sensorineural: hereditary, congenital, presbycusis, meningitis, -Labs for unexplained sensorineural thyrotoxicosis, viral cochleitis, ototoxic drugs, otologic surgery, hearing loss: glucose, CBC, TSH, RPR Meniere disease, noise, barotrauma, penetrating trauma, acoustic -Urgent referral to ENT for sudden neuroma, meningioma, autoimmune disease, multiple sclerosis, hearing loss cerebrovascular ischemia Hematoma of External Ear -Blood collection after Management Prognosis direct trauma to the ear -Regional auricular block followed by I&D or indwelling catheter placement for large hematomas -Will lead to necrosis, infection, and permanent or needle aspiration if small cauliflower ear deformity if not drained -Refer to ENT for draining if > 7 days old -Give 7-10 days of abx to cover Pseudomonas given tenuous blood supply of cartilage -F/u in 3-5 days to eval for infection or reaccumulation -At least 1 week off from contact sports

Mastoiditis -Complication of acute or chronic OM Signs & symptoms Workup -Usually occurs in kids -Fever -CT scan -Can be serious due to proximity to the -Posterior ear pain posterior cranial fossa, lateral sinuses, facial -Local erythema over the mastoid Management nerve canal, semicircular canals, and tip of -Edema of the pinna -Hospital admission with IV abx temporal bone -Posteriorly and downward displaced auricle -May need mastoidectomy

Vertigo Differential -Psych: panic, anxiety -CV: orthostatic HTN, arrhythmia, CAD -Metabolic: hyperthyroid, menopause -Neuro: acoustic neuroma, TIA, stroke, Parkinson’s, neuropathy, migraine -Orthopedic: cervical disc disease, lower extremity arthritis -Anemia -Geriatric: decreased proprioception, off center of balance, polypharmacy Cause Information Signs & Symptoms Management Benign -Otolith dislodgement into -Intermittent vertigo lasting < 1 Paroxysmal semicircular canals minute Positional -NO hearing loss Vertigo -Better with head held still, worse with R/L movements when lying down -Vertical or horizontal nystagmus + Dix-Hallpike maneuver: affected ear will cause nystagmus when it is downward

Meniere’s -A result of increased -Sudden unilateral SNHL, roaring tinnitus, and vertigo for hours -Diuretics Disease endolymphatic fluid -Ear fullness -Low salt diet -Anti-vertigo meds Acute -Infection or inflammation of the -Severe, disabling vertigo for 24-48 hours followed by weeks of -Steroids Labyrinthitis inner ear, usually due to latent imbalance -PT & Vestibular virus -Vomiting Neuritis -Neuritis = only semicircular -Pts think they are dying canals affected -Labyrinthitis = vertigo + hearing loss Central -Issue with balance centers of the -Symptoms with gradual onset but are constant Vertigo brain -May have nausea or diaphoresis -Vertical nystagmus -Usually no hearing loss Tinnitus -Abnormal perception of sound in the middle Objective tinnitus Differential Workup ear in the absence of a corresponding sound in -When a clinician can perceive an -Excessive noise exposure -Audiometry the external environment abnormal sound emanating from -Meniere’s disease -Refer for imaging for pulsatile tinnitus the pt’s ear -Labyrinthitis Subjective tinnitus -May have clicking if due to -Otitis media Management -A sound only the patient can hear pharyngeal muscle spasm -Eustachian tube dysfunction -Cochlear implants if due to sensorineural hearing loss -Caused by aberrant neurological signalling in -May have breathy sounds if due -Ototoxicity: ASA, aminoglycosides, loop diuretics, -Stop offending meds the brain to abnormally open eustachian cisplatin -Avoid caffeine and nicotine -May be a neurologic response to hearing loss; tube -Glomus tumor -Use background noise high freq. loss will cause high freq tinnitus, -May be pulsatile or bruit-like with -Any cause of sensorineural hearing loss -Tinnitus retraining therapy low freq. loss will cause low freq. tinnitus referred vascular sounds or tumor -Intracranial AV malformation -Resolve underlying problem if due to conductive -Can also be caused be aspirin hearing loss 98

NOSE & SINUS DISORDERS Sinusitis -Acute = symptoms < 4 weeks Management -Subacute = symptoms for 4-12 weeks -Mild cases can be observed for 7 days as 80% of all cases -Chronic = > 3 months improve without any AB within 2 weeks -Treat with AB if mod-severe symptoms have failed to Agents improve after 10-14 days or there is double sickening: -Usually viral amoxicillin or Septra or doxycycline first, Augmentin or -Bacterial is secondary to prior URI: Strep pneumo, M. cat, H. levofloxacin if severe, recent AB use, or failed first-line flu, Staph aureus therapy -Fungal possible if immunocompromised -Nasal saline rinses -Nasal steroid spray Signs & Symptoms -Antihistamine -Headache, localized sinus pain and pressure (esp unilateral), -Decongestant foul or purulent nasal discharge, fever, upper tooth pain, -Mucolytics cough, fatigue -Afrin short-term -No correlation between report of “sinus headache” and -If chronic, consider allergenic causes, CF, anatomic sinusitis found on CT scan abnormalities, unusual organisms, may need to refer -Double sickening more likely to be bacterial -Send to ED if there is facial cellulitis, proptosis, vision -Peds: unusual as their sinuses are not fully formed, but may change, mental status changes have bad breath, subacute or abrupt onset Complications: orbital cellulitis or abscess, osteomyelitis (Pott’s puffy tumor), intracranial extension, cavernous sinus thrombosis Allergic Rhinitis -Typically does not occur in infants under 6 mos Signs & symptoms Differential Management -Seasonal or perennial -Repetitive sneezing -Sinusitis -Instruct patients in allergen avoidance: -Pruritus of nose, eyes, -Rhinitis medicamentosa closed windows, bed cases, washing linens Samter’s triad = syndrome of aspirin sensitivity, nasal polyposis, palate, ears (Afrin!) weekly, removing stuffed animals, cockroach and asthma often seen with allergic rhinitis, frequently leading to -Clear rhinorrhea -Polyps poison, mold precautions, HEPA filters severe pansinusitis -Nasal congestion -Deviated septum -Nasal saline sprays or rinses -Postnasal drip -Adenoid hypertrophy -Oral decongestants -Epistaxis -FB -Nasal steroids: fluticasone, flunisolide -Allergic shiners or Dennie’s lines -Vasomotor rhinitis -1st or 2nd gen antihistamines: cetirizine and -Allergic salute fexofenadine ok for infants > 6 mo -Retracted TMs -Leukotriene inhibitor -Serous effusions -Refer to allergist for kids with mod-severe -Swollen or boggy turbinates disease, prolonged rhinitis despite -Hyperplasia of palate or posterior pharynx intervention, coexisting asthma or nasal polyps, recurrent otitis media or sinusitis

Epistaxis -Almost always anterior Signs & symptoms Workup Management (Kiesselbach plexus) -Anterior bleed is usually unilateral with no sensation of -CBC and coags for profuse bleeds -Anterior: compress nose for 10-15 min, consider blood down the back of the throat vasoconstrictive agents, cautery with silver nitrate or Causes -Posterior bleeding is profuse with strong sensation of electrically, nasal packing last resort -Trauma draining down posterior pharynx -Posterior: nasal packing (high morbidity risk), -Infection embolization, ligation -Allergic rhinitis -Atrophic rhinitis -Coagulopathy -Tumors -Arteriosclerosis in the elderly Nasal Foreign Body Signs & symptoms Workup -Unilateral purulent and foul-smelling nasal drainage -Plain radiographs if unable to visualize FB -Black drainage or epistaxis if button battery present

Management -Urgent ENT referral for button batteries, magnets attached across the nasal septum, penetrating FB, impacted FB, or posterior FB -Nose blowing or mouth-to-mouth blowing using the parent for occlusive FBs -Instrumentation for non-occlusive FBs

Nasal Polyps Etiologies Management -Usually a reaction to bacterial infection in kids -Steroid nasal spray -Allergies -Saline rinses -Chronic sinusitis -Refer for surgical excision but may recur

Signs & symptoms -Stuffiness -Feelings of pressure or fullness in the face -Trouble smelling

MOUTH & THROAT DISORDERS Laryngitis -Etiology is typically viral Treatment -Rest, fluids, smoking cessation Hoarseness differential -Never use antihistamines or steroids because they can cover up the injury  -Acute: postnasal drip, viral laryngitis, hypothyroidism, vocal fold paralysis, recent intubation, vocal hemorrhage permanent damage -Chronic: smoking, vocal strain, GERD, cancer, vocal nodules (vocal misuse) or polyps (GERD) -Refer to ENT for laryngoscopy for hoarseness > 2 weeks

100

Acute Pharyngitis

Differential Agents Signs & symptoms Workup Treatment Viral -Adenovirus, coronavirus, -Concurrent rhinorrhea -Salt water gargles Pharyngitis rhinovirus, influenza, -Erythema, edema, dysphagia, pain, fever, lymphadenopathy, -Lozenges or hard candy parainfluenza, Coxsackie diffusely pink throat, cough, fever -Acetaminophen or ibuprofen -Oral rinse with equal parts lidocaine, diphenhydramine, and Maalox -Benzydamine HCl mouth rinse Strep -GAS -Uncommon in kids under 2-3 -Distinguish from -Penicillin VK Pharyngitis -Sore throat, dysphagia, odynophagia, erythema, airway obstruction, viral by rapid Strep -Cephalosporin bright beefy red demarcated splotches test ± culture -Erythromycin: increasing macrolide resistance -Centor criteria: tender cervical adenopathy, fever > 100.4, no cough, tonsillar exudate -Abdominal pain and vomiting in peds Acute -Viral: Mono -Swollen tonsils with white plaques -Rapid Strep -Antibiotics Tonsillitis -Bacterial: GAS -Monospot Peritonsillar -May follow tonsillitis -Urgent referral to ENT for I&D Abscess -Bulging, asymmetrical soft palate, hot potato voice, severe throat pain, dysphagia, trismus, deviated uvula, salivation, fever, severe malaise Mononucleosis -EBV or CMV -Fatigue, malaise, sore throat with tonsillar edema, erythema, and -Monospot (not + -OTC pain control shaggy white-purple tonsillar exudate, lymphadenopathy, early in disease) -? steroids hepatosplenomegaly -CBC to look for -Splenic precautions -Many will have 2° Strep tonsillitis atypical -Treat tonsillitis but avoid ampicillin due to rxn lymphocytes with mono  rash Fusobacterium -Fusobacterium -Adolescents -May have fever -Very high CRP -Treat to avoid Lemierre’s syndrome (septic emboli Pharyngitis necrophorum -Severe pharyngitis -Unilateral neck pain or swollen neck -↑ WBCs with thrown from internal jugular) with penicillin + -Cervical adenopathy leukocytosis clindamycin -Headache

101

Aphthous Ulcers (Aphthous Stomatitis or Canker Sore) -Intermittent formation of non-contagious Common triggers Differential Management ulcers on the mucous membrane of the oral -Nutritional deficiency -HSV -Topical barriers cavity in otherwise healthy individuals -Local trauma -Erythema multiforme -Topical analgesics -Cause not completely understood but may -Stress -Drug reaction -Topical hydrocortisone + antibiotic ointment involve a T-cell-mediated response after -Hormonal influences -Pemphigus or bullous pemphigoid initiation of a trigger -Allergies -SLE Prognosis -Genetic predisposition -Lichen planus -Large aphthae may take 20-30 days to heal -Herpangina or hand-foot-mouth Signs & symptoms -Acute HIV -Minor aphthous ulceration = lesions < 10 mm -Parvovirus -Major aphthous ulceration = lesions > 10 mm, -Varicella zoster may have scarring -Syphilis -Herpetiform ulceration: crops of vesicles that -Oral candidiasis resembles HSV but is not caused by this! -Behcet’s síndrome -Reactive athritis -IBD -SCC -Necrotizing ulcerative gingivostomatitis Dental Caries Prevention -Early referral to dentist for kids with breast or bottle feeding > 12 months, frequent consumption of sugary beverages and snacks, prolonged use of sippy cups, use of bedtime bottles, use of liquid meds > 3 weeks, insufficient fluoride exposure, visible plaque on upper front teeth, enamel pits or defects, exposure to second-hand smoke -AAP recommends referral to dentist at age 1, Medicaid begins at age 3 -Screen for plaque, white spots, and cavities as soon as first teeth erupt -Instruct parents to clean infant’s gums with soft cloth starting at birth, and to begin brushing teeth when they first appear twice per day -Fluoride varnish: providers in NC may apply from eruption of first teeth up to age 3 -Stop pacifiers by age 3, thumb sucking by age 6

Signs & symptoms -Initial presentation is a white spot Dental Abscess -Caused by entrapment of plaque and Signs & Symptoms Management debris in the periodontal pocket -Painful, red fluctuant swelling of the gingiva -Warm saline rinses and oral penicillin or -Exudate from affected area on probing erythromycin for small abscesses -I&D for large abscesses

102

Signs & symptoms Workup -Abrupt onset of high fever, sore throat, stridor, dysphagia, drooling, trismus -Lateral x-ray for “thumb sign” -Sitting child that won’t lie down, head leaning forward (sniffing or tripod position) Sialadenitis & Parotitis -Inflammation of a salivary gland Workup -Most commonly affects the parotid or submandibular gland -Culture Stensen’s duct drainage if present -Acute, chronic, or recurrent -Agent is usually Staph aureus but can be polymicrobial Management -10-14 days antibiotics (may need IV if severe): Risk factors clindamycin + cipro, or Augmentin -Decreased salivary flow -Warm compresses -Dehydration -Poor oral hygiene Prognosis -Complications: abscess, Ludwig’s angina, cellulitis Signs & symptoms -Painful swelling and edema of the cheek, especially with meals -Reddened skin -Malaise -Purulent exudate from duct punctum

EENT NEOPLASMS Other Ear Neoplasms Nose & Sinuses Neoplasms Salivary Gland Neoplasms Laryngeal Neoplasms Squamous cell Inverted papilloma Squamous cell carcinoma -Most arise in parotid Vocal fold nodules Laryngeal squamous cell carcinoma -Benign tumor caused by HPV -Nasopharynx or sinuses -Most are benign -Benign “Singer’s nodules” carcinoma -Suspect with -S/s: obstruction, hemorrhage -S/s: eustachian tube obstruction, -Need speech therapy -The most common nonresolving otitis -Tx: excise as can be associated serous otitis media malignancy of the larynx externa with SCC Vocal fold polyps or cysts -S/s: new and persistent -High mortality rate Nasopharyngeal carcinoma -Benign hoarseness in a smoker, Juvenile angiofibroma -Associated with EBV -Related to vocal fold persistent throat or ear pain, -Benign vascular tumor trauma neck mass, hemoptysis, -S/s: nasal obstruction and stridor hemorrhage Laryngeal leukoplakia -W/u: CT, laryngoscopy -Tx: surgical excision -Associated with smoking with biopsy, esophagoscopy, -May be associated with bronchoscopy SCC  biopsy -Tx: radiation or partial laryngectomy, chemo if late stage

103

Acoustic Neuroma (Vestibular Schwannoma) -Account for 85% of all cerebellopontine angle Signs & symptoms Management tumors in adults -Unilateral sensorineural hearing loss -Surgical or radiation therapy for large tumors, -Tinnitus young age, or significant hearing impairment Risk factors -Facial nerve symptoms -Observation with serial imaging and -Neurofibromatosis type 2 audiometry for older patients with small -Exposure to loud noise Workup tumors and limited hearing loss -Childhood radiation exposure -MRI -H/o parathyroid adenoma -Cell phone use

Oral Leukoplakia -An early dysplasia of the squamous epithelium Signs & symptoms Prognosis -May be malignant or inflammatory -White patches or plaques on the oral mucosa -Between 1-20% of lesions will progress to carcinoma within 10 -Association with HPV years Workup -Lesions arising in trauma-prone areas of the oral cavity are less Risk factors -Biopsy likely to be dysplastic -Smoking

REPRODUCTIVE SYSTEM UTERUS Leiomyomas (Uterine Fibroids) -Benign tumors arising from the myometrium Signs & Symptoms Workup -Varying locations -Dysmenorrhea and AUB -TVUS is diagnostic -Menorrhagia and possible subsequent anemia -Consider malignancy workout with rapid growth Risk Factors -Dyspareunia -Black -Urinary frequency Management -Obese -Infertility -Only if symptomatic or pursuing pregnancy -Over age 40 -Irregular feeling uterus -Surgical myomectomy (should be hysteroscopic if desiring future pregnancy) -Nulliparity -Abdominal mass -Hysterectomy only for extremely large, painful fibroids with intractable bleeding -Bloating -Mirena IUD or Depo injections to reduce bleeding Protective -Pelvic pain or pressure or feeling of fullness -Menopause-mimicking agent such as ulipristal -Multigravida -Acute pain with torsioned pedunculated -Uterine artery embolization to starve off fibroids -Postmenopausal fibroid -Consider shrinking large fibroids with GnRH agonists prior to surgical removal -Smoker -Miscarriage with submucosal fibroids -Prolonged OCP use intruding on fetus Prognosis -Depo use -Can be asymptomatic -Not associated with malignant transformation -Symptoms improve after menopause

104

Dysfunctional Uterine Bleeding -Bleeding outside normal parameters of menses (24-35 days, < Differential Workup Management 80 mL per cycle) found in the absence of demonstrable -Abnormal uterine -A diagnosis of exclusion -Treat underlying cause if due to abnormal structural or organic pathology that is unrelated to another bleeding (known -Pregnancy test uterine bleeding underlying illness pathology): miscarriage, -Malignancy workup if postmenopausal -Adolescent mild DUB can be treated with iron -Classified as ovulatory or anovulatory gestational trophoblastic -Coagulopathy workup: PT/aPTT, CBC, supplements and observation -Some providers consider DUB to be a subset of abnormal disease, IUD, meds, -Assess ovulatory status: biphasic body temp, -Adolescent mod-severe DUB can be treated uterine bleeding trauma, coagulopathy, progesterone levels, urine LH with OCPs or progestin only regimen adrenal disorder, stress, -Pelvic exam with pap -Patients with contraindication to estrogen Causes pituitary adenoma, -May need endometrial biopsy or hysteroscopy therapy can consider symptomatic management -Usually a hormonal disturbance: menopause, premature smoking, infections, -Consider US evaluation for fibroids, polyps, or with NSAIDs, progestin-only regimen, or ovarian failure, PCOS, prolactinoma, anovulation, immature HP fibroids, malignancy, adenomyosis Mirena IUD axis (adolescents), perimenopause atrophic vaginitis -Consider testosterone and DHEAS levels in -Endometrial ablation an option for women not -Anovulation causes DUB b/c there is no corpus luteum women with signs of virilization wishing to conceive (although will still need formation  no progesterone to oppose estrogen-induced contraception) hyperplasia of the endometrium -Hysterectomy is the definitive treatment Endometrial Neoplasms -Endometrial neoplasia involves proliferation of the Signs & Symptoms Workup Management endometrial glands that can progress to or coexist -Abnormal uterine bleeding -Endometrial biopsy can be done in clinic and -Benign pathology on biopsy watched, no with endometrial carcinoma -Postmenopausal bleeding is 99.6% sensitive in premenopausal women action warranted unless bleeding persists -Endometrial carcinoma is the most common GYN -Abnormal pap cytology and 91% in postmenopausal women -Endometrial hyperplasia on pathology without cancer in the US and is usually adenocarcinoma -Transvaginal US to assess endometrial stripe: atypia is treated with progesterone cream,

Differential for Postmenopausal Bleeding thin stripe < 4-5 mm associated with low risk ovulation induction, or IUD to induce massive Risk Factors -Age > 50 -Atrophy (59%) of cancer while stripe > 5 mm warrants biopsy menses and endometrial sloughing -Uopposed estrogen use -Endometrial polyps -Atypical endometrial hyperplasia needs D&C -PCOS -Endometrial cancer or hysterectomy + BSO -DM -Endometrial hyperplasia -Obesity -Hormonal effects -Nulliparity -Cervical cancer -Late menopause -Tamoxifen use -HNPCC Endometriosis & Adenomyosis Endometriosis Signs & Symptoms Workup -Location of endometrial tissue any place outside of the uterus -Endometriosis typically occurs in young, tall, thin, -US or MRI -May be caused by retrograde menstruation, where sloughed off nulliparous Caucasian women -Laparoscopy for definitive diagnosis (implants will have variable endometrial tissue escapes through the fallopian tubes to implant -Adenomyosis typically occurs in women ages 40-50 coloration and appearance) outside of uterus -Associated with early menarche and late menopause -Could also be caused by Mullerian cell remnants, direct surgical -May be asymptomatic Management transplantation, altered immune response, genetics, or increased -Dysmenorrhea -Endometriosis improves with suppression of ovulation and medical estrogen stimulation -Dyspareunia therapy is first line: OCPs, NSAIDs for cyclical pain, GnRH -Usually occurs in the pelvis, but can occur in the ovary, cul de -Pelvic pain agonists for severe pain (create a hypoestrogenic state) sac, uterine ligaments, fallopian tubes, bladder, rectum, bowel -Sacral backache -Surgical excision for failed medical management cervix, vagina, omentum, umbilicus, vulva, ureter, spinal cord, -Pelvic mass -Adenomyosis is treated with hysterectomy nasopharynx, breast, lung, and kidney -Tenesmus and diarrhea with painful BMs -Urinary frequency Prognosis Adenomyosis -Infertility -Associated with epithelial ovarian cancer but NOT endometrial -Endometriosis within the uterine muscle -Lateral displacement of cervix or stenosed os cancer -Recurrence is common 105

OVARY Functional Ovarian Cysts -Caused by exaggerations of normal Signs & Symptoms Differential Management menstrual cycle rather than true neoplasms -Can be asymptomatic -Ruptured ectopic -Will usually regress spontaneously -Increased risk with smoking -Pelvic pain and dyspareunia if -Mittelschmerz -Treatment only if recurrent or symptomatic large -Ovarian torsion (OCPs, etc) Types -Follicular: pelvic pain if rupture -Degenerating leiomyoma -Ruptured cyst: expectant management if -Follicular cyst: continued growth of follicle -Corpus luteum: adnexal -PID uncomplicated (no hypotension, tachycardia, despite failed ovulation enlargement, one-sided pain, -Acute endometritis fever, leukocytosis, signs of acute abdomen, -Corpus luteum cyst: failure of involution missed menses or US suspicious for malignancy), surgical with enlargement after ovulation and -Torsioned or ruptured cyst will Workup management if complicated continued progesterone secretion cause acute abdominal pain, -Must be differentiated from malignancy (benign = mobile, -Theca lutein cyst: a result of abnormal rebound tenderness cystic, unilateral, smooth, < 10 cm, minimal septations); get Prognosis pregnancy, uncommon pelvic US -Risk of torsion if large or penduculated Ovarian Neoplasms -Vary from annoying and benign to invasive and malignant Malignant Ovarian Neoplasms Signs & Symptoms Management -Functional ovarian cysts (corpus luteum cyst or follicular cysts) are -Adenocarcinoma -Thyrotoxicosis with dermoid tumor -Malignancy: local excision vs NOT considered to be neoplasms because they are a result of a normal -Granulosa cell tumor -Torsioned ovary or cyst  signs of acute abdomen total hysterectomy and bilateral physiologic process -Dysgerminoma -Malignancy symptoms are nonspecific like pelvic SO vs partial bowel resection -Ovarian neoplasms are derived from neoplastic growth of ovarian cell -Clear cell carcinoma pain and bloating depending on stage of cancer, layers -Endometrioid carcinoma usually followed by radiation ± Workup chemo Benign Ovarian Neoplasms Risk Factors -Transvaginal US: signs indicative of malignancy -Benign neoplasms will persist -Mucinous cystadenoma -Nulliparity include large amounts of free fluid in the abdominal unless excised, which is usually -Serious cystadenoma -Fertility treatments cavity, solid ovarian enlargement or mixed cystic done to prevent ovarian torsion -Endometrioma (chocolate cyst) -FH of breast or ovarian cancer and solid enlargement, thick-walled or complex -Simple cysts in a -Fibroma ovarian cysts postmenopausal woman may be -Brenner tumor Protective Factors -Serum CA-125: will also be elevated in infection, followed by serial US and CA- -Thecoma -Prolonged OCP use endometriosis, ovulation, and trauma 125s -Sertoli-Leydig cell tumors -Pregnancy -Staging and grading of malignancies -Dermoid cyst (teratoma): can contain hair, teeth, sebaceous glands, and -Tubal ligation or hysterectomy thyroid cells producing TH -Uterine leiomyoma Ovarian Torsion -A gynecologic emergency caused by ovarian Risk Factors Workup ischemia as a result of complete or partial -Ovarian mass -Pelvic US rotation of the ovary on its ligamentous -Ovulation induction for infertility supports Management -Fallopian tube may also be torsioned Signs & Symptoms -Surgical repair with ovarian conservation unless -Acute pelvic pain (although rarely can be mass or necrosis present chronic pelvic pain) -N/v -Low grade fever

106

CERVICAL DISORDERS Cervicitis Etiologies Signs & symptoms Workup -Infectious: chlamydia, gonorrhea, HSV, HPV, -Postcoital spotting -STI testing trichomoniasis, Mycoplasma genitalium, CMV, BV -Intermenstrual spotting -Wet prep -Noninfectious: cervical cap, pessary or diaphragm use, -Dyspareunia -Pap & pelvic chemical or latex allergy, cervical trauma -Unusual vaginal discharge -If chronic  cervical stenosis, leukorrhea, granular redness, Treatment erythema, vulvar irritation -Chlamydia  single azithromycin dose, or doxycycline -Salpingitis -Gonorrhea  ceftriaxone IM or single cefixime oral dose -Edematous or friable cervix -HSV  acyclovir -Trichomoniasis  single metronidazole Incompetent Cervix (Cervical Insufficiency) -Painless cervical changes in the 2nd trimester leading to recurrent Screening Workup Management pregnancy loss, stillbirth, or preterm delivery -UpToDate recommends routine TVUS screening for -Transvaginal US showing -No evidence for bedrest -Short cervix is defined as < 25 cm from external to internal os short cervix in singleton pregnancies at 16-28 weeks shortened endocervical -Progesterone supplements canal and funneling of -Indomethacin Causes Signs & Symptoms fetal membranes into -Prophylactic cerclage or -Congenital: short cervix, Mullerian abnormalities, collagen abnormalities, -Vaginal fullness or pressure endocervix pessary FH -Spotting or watery or brown discharge -Early US surveillance for -Trauma: cervical laceration, instrument dilation, cone biopsy, LEEP -Vague abdominal and back pain women not meeting criteria -Elevated serum relaxins (higher in multiple gestations) -Premature cervical effacement and dilation for cerclage Cervical Dysplasia

107

-After HPV infection, epithelia can develop active or latent infection or undergo neoplastic transformation -HPV types 16 and 18 are more likely to undergo malignant transformation -Most women will clear HPV infection within 2 years -CIN = cervical intraepithelial neoplasia (aka cervical dysplasia); premalignant squamous transformation cells (not glandular) -Bethesda system: ASCUS, LSIL, or HSIL -Women over 30 who are being screened no more frequently than every 3 years will have HPV testing done automatically with their cytology

Screening -Begin at age 21 or within 3 years of first sexual contact -Every 3 years if low risk with 3 consecutive normal paps -D/c after age 65 if last 3 paps were normal -Women who have had a total hysterectomy for benign reasons do not need paps

Management -Differs based on age of patient, as younger women tend to clear the infection before progression to dysplasia -If ASCUS is detected, specimen will be tested for HPV as well if woman is over 21, or repeat cytology in 6 months if woman is under 21 -LSIL or HSIL or AGC will need referral for colposcopy, where biopsies will be taken -Biopsies give corresponding histology grade of CIN 1, 2, or 3 -CIN 1 generally observed or may be ablated (cryotherapy or laser) -CIN 2 or 3 have 5-15% chance of progressing to cervical cancer and these lesions need to be excised via LEEP or conization procedure (↑ risk of preterm labor in future pregnancies)

108

Cervical Cancer -80% of women will be infected with a strain of HPV by age 50 Screening Workup Prognosis -Average age of cervical cancer dx is 51 -Has resulted in 75% ↓ in cervical cancers -Biopsy for grossly visible invasive -Depends on stage, -Pap cytology disease mets, tumor volume, Cervical carcinoma in situ -Imaging for mets invasion, and -Most common in 25-35 year olds Signs & symptoms histology -Early disease is asymptomatic Management -Good prognosis for Cervical squamous cell carcinoma -Abnormal vaginal bleeding or postcoital spotting -Carcinoma in situ: LEEP, CKC, simple early disease -The most common form of cervical cancer, accounts for 75% of all cases -Vaginal discharge hysterectomy responsive to -Usually from HPV 16 or 18 -Foul odor -Early stage: radical hysterectomy + treatment -Usually located within 1 cm of the squamocolumnar junction -Pelvic or flank pain pelvic lymphadenectomy, primary -Nontreatment or -Vesicovaginal or rectovaginal fistula radiation with concurrent chemo nonresponse yields Cervical adenocarcinoma -Weakness -Locally advanced: primary radiation 5% 2 year survival -Derived from glandular elements -Weight loss with chemo -Subtypes are mucinous, endometrioid, clear, or serous cell -Anemia -Mets or persistent or recurrent cancer: -Usually from HPV 18, though clear type associated with DES exposure -Speculum exam shows cervical lesion, enlarged chemo, palliative radiation, -More common in women under 35 cervix, nodularity, friable tissue, or decreased -Central pelvic recurrence: total pelvic mobility exenteration

VAGINAL & VULVAR DISORDERS Vulvar Neoplasms -Vulvar intraepithelial neoplasia (VIN) is a Differential Workup premalignant lesion that is difficult to distinguish or -Flesh-colored lesion: sebaceous gland, vestibular papillae, -Any lesion not previously known on the vulva warrants biopsy via physical may exist in association with invasive squamous skin tag, cyst, wart, molluscum contagiosum exam or colposcopy cell carcinoma, lichen sclerosus, or lichen planus -White lesion: lichen sclerosus, lichen simplex chronicus, -Malignant lesions include squamous cell vitiligo Management carcinoma (90% of vulvar cancers), melanoma, and -Brown, red, or black lesion: could be anything, need to -Wide local excision of VIN if high risk based on lesion characteristics and pt age basal cell carcinoma biopsy -Laser ablation or topical therapy with imiquimod for VIN lesions that would cause significant vulvar mutilation if excised Risk Factors Signs & Symptoms -Excision of malignant lesions with inguinofemoral lymph node evaluation ± -HPV infection -Vulvar pruritus and pain chemo or radiation -Immunosuppression -Visible or palpable abnormality, may be in multiple -Cigarette smoking locations Prognosis -Lichen sclerosus (can transform to SCC) -Dysuria -VIN recurs in 30% of women and 4-8% will go on to develop locally invasive vulvar cancer Pediatric Vaginitis Etiologies Urethral prolapse: treat with topical estrogen Management -No bubble baths or perfumed soaps -STI cream for 2 weeks -Treat underlying cause -Cool compresses -Vaginal polyp or tumor -Lichen sclerosus: treat with topical steroids -Wear nightgowns to allow air circulation -Use wet wipes instead of toilet paper -Atrophic prepubertal tissue (more susceptible to -Labial adhesions: treat with topical estrogen -Cotton underwear -Avoid sitting around in wet swim suits irritants) cream -Avoid tights, leotards, and leggings -Antibiotic therapy for purulent discharge that -Strep pyogenes and other respiratory pathogens -Systemic illness: measles, varicella, scarlet -Bathe in water only for 15 minutes and limit does not respond to hygiene measures -Foreign body fever, EBV, Crohn’s, Kawasaki disease soap to non-genital areas -Pinworms -Nonsexually transmitted vulvar ulcers -Dry genital area well after bathes, can use a -Urethral prolapse cool hair dryer -Ectopic ureter

109

Adult Vaginitis Etiology Bacterial vaginosis Trichomoniasis Yeast vaginitis Atrophic vaginitis Info -Polymicrobial overgrowth of normal flora -Usually sexually transmitted -Agent is Candida albicans -Inflammation of the vagina due to -May be ppt by hormonal changes, thinning and shrinking of tissues and oral steroids or abx, nylon panties, decreased lubrication hot weather, obesity -Seen in women with decreased estrogen Signs & -Fish odor -Copious vaginal discharge -Pruritus -Pruritus symptoms -Heavy bubbly discharge that is white or gray -Pruritus -Burning -Burning -Dysuria -Nonfoul cottage cheese discharge -Vaginal dryness -Dyspareunia -Dyspareunia -Dyspareunia -Abdominal pain -Vaginal or vulvar erythema -Spotting -Vaginal and cervical inflammation -May be asymptomatic -Pale, thin vaginal mucosa with punctate hemorrhages = strawberry cervix Workup -Wet prep: clue cells, alkaline pH -Wet prep: look for motile -KOH wet prep -Must r/o infectious cause -Whiff test trichomonads -Negative wet prep Treatment -1st line is metronidazole or clindamycin cream -Single dose of metronidazole -Single dose of butoconazole or -Estrogen replacement therapy fluconazole -Regular sexual activity -1st trimester pregnancy use -Lubricants and vaginal moisturizers itraconazole -OTC Monistat only treats Candida albicans and not other 2 species Picture

MENSTRUAL DISORDERS Premenstrual Syndrome -More severe form is premenstrual dysphoric disorder Workup Management -PMDD defined by 5+of the following: sadness, despair, suicidal -Exercise Signs & Symptoms ideation, tension, anxiety, panic attacks, irritability affecting others, -Regular sleep -Symptoms begin with ovulation and last 2 weeks until menses mood swings, crying, disinterest in daily activities, binge eating, -Stress management -Acne, breast swelling, fatigue, GI upset, insomnia, bloating, cravings -Healthy eating habits HA, food cravings, depression, anxiety, irritability -Avoiding caffeine, sugar, and salt -OCPs for severe symptoms -Consider antidepressants or counseling for PMDD

110

Amenorrhea Primary Amenorrhea Secondary Amenorrhea -Failure to menstruate by age 16 in presence of Workup -Cessation of menses for a period of time = to Workup 2˚ sex characteristics or failure to menstruate -Physical exam for sex characteristics and 3 cycles or 6 months in a woman who -Physical exam for hirsutism, acanthosis by age 14 in absence of 2˚ sex characteristics normal anatomy (breast development indicates previously had menses nigricans, vitiligo, galactorrhea, and signs of estrogen effects and functioning ovaries) estrogen deficiency or eating disorder Etiologies -US to look for presence of uterus Etiologies -Serum hCG, FSH, LH, PRL, TSH, -Chromosomal abnormality  gonadal -FSH level to determine whether cause is -Pregnancy progesterone, ?DHEAS (false negs) dysgenesis central or ovarian -Functional hypothalamic amenorrhea: -Serum total testosterone with signs of -Central: tumors, infiltration of hypothalamus -Karyotype if breast development not present excessive exercise, eating disorder, systemic hyperandrogenism or pituitary, congenital GnRH deficiency, -Normal FSH, signs of breast development, illness, psychological stress -Other workup based on clinical findings hypoprolactinemia, disrupted GnRH pulsations and presence of uterus indicate further workup -Hyperprolactinemia: pituitary tumor, -PCOS for secondary causes of amenorrhea medications, hypothyroidism Management -Anatomic abnormality or absence of uterus, -PCOS -All depend on desire for fertility cervix, or vagina Management -Premature ovarian failure -Hypothalamic amenorrhea: sufficient calorie -Treat underlying pathology -Endometrial scarring (Asherman’s) intake, CBT, leptin administration -Achieve fertility if desired -Hyperprolactinemia: dopamine agonist or -Prevent complications of disease process surgical treatment -Premature ovarian failure: OCPs to prevent bone loss -PCOS treatment -Hysteroscopic lysis of intrauterine adhesions Dysmenorrhea Primary = painful menses with normal anatomy Secondary = a result of disease or pathology -Leading cause of school absences Causes -Incidence decreases after age 20 -GYN: endometriosis, uterine fibroids, adenomyosis, STIs, endometrial polyps, ovarian cysts, pelvic adhesions, chronic PID, cervical stenosis Cause -Non-GYN: IBD, IBS, uteropelvic junction obstruction, psychogenic -Usually prostaglandins and uterine vasoconstriction Signs & Symptoms Signs & Symptoms -Usually begins well after menarche -Cramping pain radiating to back or inner thighs -May have associated heavy flow, n/v/d, HA, dizziness Workup -Refer for laparoscopy Management -NSAIDs beginning 1-2 days before expected menses Management -OCPs -Treat underlying cause -Progesterone -NSAIDs -Mirena IUD -OCPs -Acupuncture -IUD -Thiamine supplementation -Refer to OB-GYN for uterine artery embolization and evaluation for hysterectomy

111

BREAST DISORDERS Breast Abscess -Can be lactational or nonlactational Management -Etiologist is usually Staph aureus, with MRSA becoming increasingly more common -Needle aspiration if overlying skin is intact -I&D for compromised overlying skin or failed needle aspiration Risk Factors -Antibiotics: dicloxacillin, cephalexin, or clindamycin -Obesity -Bactrim, clindamycin, or linezolid if suspecting MRSA -Smoking -Continue breastfeeding -Black

Workup -Wound culture Breast Mass Screening Workup -Can assess risk for breast cancer using Gail Model -Screening mammo for ages 40-49 is grade C -Screening mammo recommended for women 50-74 every 2 years -Breast self-exam is grade D -Dedicated breast MRI for select high risk patients

Signs & symptoms -Mastalgia is rarely associated with breast cancer -Nipple discharge: only 2-3% will have malignancy -Concerning: single, nontender firm mass with ill-defined margins, skin or nipple retractions, axillary adenopathy, breast enlargement, erythema, peau d’orange, edema, pain, fixation of mass to chest wall

Differential -Fibrocystic changes -Fibroadenoma -Intraductal papilloma -Fat necrosis -Abscess -Malignancy

112

Breast Cancer -Usually arises from ducts or lobules Prevention Signs & Symptoms Workup -Most commonly diagnosed female -Women with high risk -Most commonly found on the upper outer quadrants -Biopsy of suspicious lesion cancer can consider -Early: single, painless firm mass with ill-defined margins or -Pathology and genomic marker assay -Only 5-10% are due to genetic mutations chemoprevention with possibly no palpable mass but an abnormality is detected on tamoxifen or raloxifene mammogram Management Risk Factors -Later: skin or nipple retractions, axillary adenopathy, breast -TNM classification -Obesity or inactivity Screening enlargement, erythema, peau d’orange, edema, pain, fixation -Tumor marker profiling -Use of hormone therapy -Mammography is of mass to chest wall -Surgical management: lumpectomy, sentinel node biopsy, -Nulliparity USPSTF grade C for -Very late: ulceration, supraclavicular adenopathy, arm edema, or mastectomy -First birth after age 30 women 40-49, grade B mets to bone, liver, lung, brain, or adrenal glands -Chemo is typically 3-6 months and is initiated for visceral ->1 alcoholic drink per day for women 50-74 every 2 mets, failed endocrine therapy, or ER-/PR- tumors -Not breastfeeding years Differential -Endocrine therapy with tamoxifen (premenopausal)or -Increasing age -Clinical breast exam -Fibrocystic disease aromatase inhibitors (postmenopausal) -White -Breast self-exam is -Fibroadenoma -Radiation therapy as an adjuvant -Hx of chest irradiation USPSTF grade D -Intraductal papilloma -Hx of atypical hyperplasia on previous -Dedicated breast MRI -Lipoma Prognosis biopsy for high risk populations -Abscess -Surgical complications: long thoracic nerve injury, -FH of breast cancer or inherited -Fat necrosis lymphedema mutations -Phyllodes tumor Breast Cancer Info S/S Management Type Ductal Carcinoma -Some consider this to be a pre-malignant lesion -Typically asymptomatic and discovered on screening -Lumpectomy followed by radiation is most in Situ -Arises from ductal hyperplasia and fills ductal lumen mammogram as calcifications common -Very early malignancy without basement membrane penetration -Usually not palpable on PE -Tamoxifen or aromatase inhibitor therapy for -Less than 30% recurrence rate following lumpectomy 5 years if receptor+ tumor Invasive Ductal -The most common breast cancer -Pt is typically postmenopausal -Chemo with Herceptin and Tykerb for Carcinoma -Worst and most invasive -Mammogram detects spiculated margins HER2+ tumors -Firm, fibrous, rock-hard mass with sharp margins and small, glandular, duct-like cells -Likes to metastasize Lobular -Some consider this to be a pre-malignant lesion -Usually not palpable and hard to detect on mammo Carcinoma in Situ -Contains signet ring cells -Often bilateral -Will progress to invasive lobular carcinoma in 10% Invasive Lobular -2nd most common breast cancer -Orderly row of cells in stroma that are fluid and mobile -Assessment with US preferred over Carcinoma -Often bilateral mammography Medullary -Fleshy, cellular, lymphocytic infiltrate -Mammogram detects linear crystallization pattern Carcinoma -Good prognosis although it is a rare subtype of invasive ductal carcinoma Comedocarcinoma -Subtype of DCIS -Ductal caseating necrosis Paget’s Disease of -Subtype of ductal carcinoma -Presents as eczematous lesions on the nipple the Breast -May also be seen on the vulva

113

Fibroadenoma -Common benign neoplasm in Signs & Symptoms Workup Management young women composed of -Pt is usually in teens to 30s -Consider malignancy or fibrocystic changes in -Observation if malignancy has been ruled out fibrous and glandular tissue -Firm, round, nontender, mobile 1-5 cm nodule women > 30 -Surgical excision if unable to r/o malignancy or if large that is solitary and unilateral -US for younger women -Growth is hormonally affected and can be -FNA or needle biopsy for confirmatory diagnosis Prognosis rapid during pregnancy -Can recur after excision Fibrocystic Breasts -The most common benign Signs & Symptoms Workup Management condition of the breast -Patients are usually ages 30-50 -Differentiate from fibroadenoma or malignancy by -Counseling to wear supportive bra -Uncommon in -Pain or tenderness in the breasts the presence of multiple transient lesions -Avoiding trauma and caffeine postmenopausal women unless -Cysts or multiple transient lumps that are firm, mobile, and tender -Further workup via US or mammogram indicated -Danazol for severe persistent pain on HRT -Changes are related to menstrual cycle and can be worsened by for lesions that persist throughout menstrual cycle -Evening primrose oil caffeine Gynecomastia -Benign proliferation of glandular tissue of the male breast Signs & symptoms Workup -Imbalance between stimulatory effect of estrogen and inhibitor -Rubbery or firm mass -Indicated for recent-onset or painful effects of androgens extending concentrically from gynecomastia the nipple -Not necessary for pts with underlying Etiologies contributing illness or use of drugs associated -Physiologic: common and self-limiting in infant and adolescent Differential with gynecomastia males -Male breast cancer -Serum hCG -Drugs: finasteride, spironolactone, ketoconazole, -Pseudogynecomastia: fat -LH metronidazole, omeprazole, ACEI, amiodarone, digoxin, deposition without glandular -Testosterone methyldopa, HAART, street drugs, anabolic steroids, diazepam, proliferation, seen in obese -Estradiol TCAs men -Cirrhosis or malnutrition Management -Male hypogonadism -Observation with reevaluation in 3-6 months -Testicular tumor or brief SERM trial if tender or cosmetic -Cryptorchidism concern -Hyperthyroidism -CKD -Idiopathic Galactorrhea -Non-pathologic physiologic nipple discharge in the absence of childbirth or nursing Signs & symptoms Workup Management -Considered normal when secreted for a short period of time from newborns -Usually bilateral -Breast exam -Reassurance if cause is -Can appear in a variety of colors -Hemoccult testing of discharge benign Etiologies -Visual field exam -Surgical terminal duct -Excessive nipple stimulation Differential -Mammogram for women > 30 excision if concern for -Pituitary adenoma -Non-physiologic nipple discharge such as -US if concern for pathologic pathologic discharge -Acromegaly breast cancer discharge -Other causes of hyperprolactinemia -Pregnancy -Urine bHCG -Hyperthyroidism -Bilateral discharge: prolactin, -Meds: methyldopa, opiates, SSRIs, antipsychotics BMP, TSH -Licorice -Dopamine release from surgery, trauma, or anesthesia

114

Mastitis Agents Workup Management -Staph aureus, increasingly MRSA -Malignancy workup if occurring in a non-lactating -Continue breastfeeding or pumping woman -Ibuprofen Signs & Symptoms -US to differentiate from abcess if needed -Cold compresses -Usually unilateral -Oral dicloxacillin, 1st generation cephalosporin, or erythromycin if not -Fever and flulike symptoms suspecting MRSA -Erythema, warmth, tenderness, and hardness of affected -Severe infection with MRSA risk  Bactrim, clindamycin, or linezolid breast

CONTRACEPTIVE METHODS Misc. Methods Info Failure Rate Cost Withdrawal 4-27% Fertility -Includes rhythm method, natural -Inexpensive 9-25% Awareness family planning, and symptothermal -Accepted by religious organizations method -Use alternate methods or alternate forms -Based on consistent symptoms of of pleasure during ‘unsafe’ days ovulation -Decreases spontaneity -Effective if regular cycles -Unreliable if irregular cycles -Must be committed, motivated, -Perimenopausal years more difficult vigilant -Control of fertility -No chemicals, hormones or foreign objects Barrier Methods Info Failure Rate Cost Spermicide -Only kind available in US is nonoxyl-9 10-29% $0.50-$1.50 per application -Natural alternatives: lemon juice, vinegar, neem oil -Comes as a vaginal film, suppository, cream, gel, or lubricant -No STI protection, can acually cause allergies an irritation  ↑ risk of STIs Cervical Cap -Silicone cap filled with spermicide -Can be inserted up to 24 hours before 7.6-14% $89 + exam & fitting -Only kind approved in US is the sex and worn for up to 48 hours Free for insured under new ACA FemCap (others associated with abnormal -No STI protection legislation paps) -Increased risk of nonmenstrual toxic -Requires prescription and fitting shock Diaphragm -Rubber that is filled with spermicide 10-20% $15-$75 + exam & fitting -Requires prescription and fitting Free for insured under new ACA -No STI protection legislation -Increased risk of UTIs, vaginitis, and nonmenstrual toxic shock Female Condom -Synthetic nitrile 5-20% $2-$4 each Male Condom -Latex, polyurethane, natural, or “spray on” 3-15% $0.25-$2 each -Often prelubricated with spermicide -Can cause UTIs in female partners -No STI protection with natural condoms

Sponge -Polyurethane with spermicide $13-$19 for 3 -Does not prevent STIs 115

Hormonal Methods -Absolute contraindications to all estrogen-containing BC (per CDC): -Relative contraindications to all estrogen-containing BC: • Age > 35 and smoking > 15 cigs/day • Gall bladder disease • Known CAD • H/o cholestatic jaundice in pregnancy • Multiple risk factors for CAD: DM, HTN, smoking • Epilepsy • HTN • Clot risks: leg injury or cast, elective surgery, sickle cell disease • H/o DVT, PE, stroke, or migraine with aura • Obesity • Known coagulopathy • Complicated valvular heart disease: pulm HTN, afib, h/o bacterial endocarditis • SLE • Breast cancer • Cirrhosis, hepatocellular adenoma, or malignant hepatoma Info Failure Rate Cost Combined OCPs -Estrogen portion suppresses the FSH surge by negative feedback  ovulation inhibition, also 3-9% $15-$30 per month alters endometrium and causes degeneration of the corpus luteum Free for insured under new ACA -Progestin portion suppresses LH surge  inhibited ovulation, also thickens cervical mucus to legislation inhibit implantation -Benefits: improvement of acne, DUB, mittelschmerz pain, endometriosis, ovarial failure, ovarian cysts, uterine fibroids, fibroadenomas or fibrocystic breasts, iron deficiency anemia; decreases risk of ovarian and endometrial cancers, ectopic pregnancy, and acute PID -Adverse effects: nausea, vomiting, weight changes, spotting, migraines, edema, rash, depression, decreased libido, ? ↑ risk of breast cancer, ↑ risk benign liver tumors, worsening gallbladder problems, blood clots, stroke -Need to adjust strength and estrogen/progesterone formulation if adverse effects are present -Most to least androgenic progestins: norgestrel, levonorgestrel, norethindrone, norethindrone acetate, ethynodiol, norgestimate, desogestrel, drospirenone -Ethynodiol is the only highly estrogenic estrogen, all others have lower estrogenic effects -No protection against STIs Adverse Effect Causes Management Breakthrough Need higher progestin content to - Monophasic formulation with a higher progestin dose bleeding increase endometrial support - Triphasic formulation with increasing dose of progestin - higher dose of estrogen Acne, oily skin, and Side effects from progestins Product with lower risk of androgenic effects hirsutism GI complaints Estrogen and progesterone - Estrogen – induces nausea and vomiting via the CNS - Progesterone – slows peristalsis, causing constipation and feelings of bloating and distention Headaches - discontinue the oral contraception - lower the dose of estrogen - lower the dose of progestin - eliminate the pill-free interval for 2-3 consecutive cycles Decreased libido and Low levels of estrogen ↓ vaginal Use of the NuvaRing may help with lubrication disorders depression lubrication Dyslipidemias Estrogen Replace an androgenic progestin with a more estrogenic progestin Mastalgia Estrogen component - lower-dose estrogen pills - if tenderness occurs prior to menses, switch to a contraceptive that offers extended cycle length Weight gain High estrogen content Switch to an estrogen product with <35 mcg of ethinyl estradiol Visual changes/ Estrogen stimulation of - progestin-only products contact lens melanocyte production - use sunscreen - refer to ophthalmologist if normal saline eye drops do not help disturbances 116

Info Failure Rate Cost Progestin-Only Pill -Must be taken with obessive regularity 1-13% Free for insured under new ACA legislation -Can have irregular bleeding -A good option for breastfeeding women, smokers > 35, or those who can’t tolerate estrogen Vaginal Ring -May be removed for up to 3 hours during intercourse without backup 1-2% $15-$70 per month protection Free for insured under new ACA legislation -Adverse effects: vaginitis, HA, leukorrhea, FB sensation, device expulsion, feeling it during sex Transdermal Patch -Can bathe, swim, or exercise with patch in place 0.3-8% Free for insured under new ACA legislation -Must use back-up if patch falls off > 1 day Medroxyprogesteron -IM injection q 3 months 1-2% $35-$75 per injection e Injection -Results in amenorrhea after a year or so of use Free for insured under new ACA legislation -Can use if smoker or nursing -Decreased risk of PID and endometrial cancer -AEs: bleeding abnormalities, weight gain, lipid changes, depression, acne, HA, delay in return to fertility -Black box warning for ↑ risk osteoporosis related to duration of use = should only use < 2 years -No protection against STIs Progesterone -Must be trained by company-approved provider to insert and remove 1-4% $400-$800 for insertion Implantable Rod -Good option for smokers or those who have contraindications to $75-$150 for removal estrogen Free for insured under new ACA legislation -May be less effective in obese patients -AEs: menstrual irregularity, amenorrhea, weight gain, acne, depression Mirena IUD -Changes mucus and sets up hostile environment for sperm 0.2% -Questionable use in individuals at risk for STIs -Often used in later reproductive years before menopause -Decreased risk of endometrial cancer -Can be in place up to 5 years -Women may become amenorrheic after a year of use -Less bleeding and cramping than with copper IUD -Increased risk of ovarian cysts -May want to culture IUD for Actinomyces after removal Surgical Methods Info Failure Rate Cost Vasectomy -Cutting and sealing the vasa deferentia 0.15% $350-$1000 -Clinic procedure under local anesthesia -Recovery period of 2-3 days -Men will still be fertile for several ejaculations afterwards, need to have semen analysis in 1 month to confirm sterility Tubal Ligation -An outpatient surgery under general anesthesia 0.5% $1500-$6000 -Recovery period of 1 week -Benefits: ↓ risk ovarian cancer and possibly breast cancer, can be done immediately postpartum -Increased risk of ectopic pregnancy -Need to confirm blockage with hysterosalpingogram

117

Other Methods Other Methods Info Failure Rate Cost Paragard IUD -Changes mucus and sets up hostile environment for sperm 0.6-1.0% -Questionable use in individuals at risk for STIs -Often used in later reproductive years before menopause -Decreased risk of endometrial cancer -Can be in place for up to 10 years -Can cause heavy bleeding and cramping -May want to culture IUD for Actinomyces after removal Lactation -Most effective if infant is not taking any supplemental formula and 10% mother is nursing at least every 4 hours Emergency Methods Info Failure Rate Cost Morning After Pill -Not an abortifacient = won’t work if already implanted (Plan B One-Step, -No evidence of teratogenic effects Next Choice) -Best if initiated within 72 hours of unprotected sex but can be taken for up to 5 days afterward -Rare risks or AEs, but may need prophylactic antiemetics before taking -Available without a prescription for ages 17+ Ulipristal acetate -Selective progesterone receptor modulator (Ella) -Rx only Mifepristone -Use within 72 hours of unprotected sex 15% (RU486) -An abortifacient = will dislodge implanted embryo -Also inhibits ovulation and changes endometrium

UNCOMPLICATED PREGNANCY Normal Labor & Delivery Stages of Labor Signs & Symptoms Management -1st stage: onset of labor to full dilation of 10 cm -Sequential changes within the -Measurement of uterine contractions via tocodynamometer -2nd stage: interval between full dilation and delivery of fetus myometrium and cervix take place over -Continuous fetal HR monitoring -3rd stage: time from fetal delivery to expulsion of placenta days to weeks -Adequate labor for delivery is 3-5 contractions in a 10 minute period -Loss of mucus plug  “bloody show” -IV placement Factors Influencing Course of Labor -Progressive cervical dilation and -Pain management: parenteral analgesics vs epidural anesthesia -Powers: uterine contractions effacement: should dilate at > 1.2 cm/hr -Clear liquid diet during labor -Passenger: fetal size and number, lie, presentation, station, for nulliparous women and > 1.5 cm/hr -Consider C-section for labor dystocia (“failure to progress”) presence of any fetal anomalies (ideally fetus is small and in for multiparous women -Consider operative vaginal delivery (use of forceps or vacuum assistance) for fetal vertex position, longitudinal lie, with head flexed and in -Fetal head can be observed to rotate as it distress, maternal exhaustion, or prolonged 2nd stage of labor anterior position and passing through pelvic inlet) navigates the birth canal (“cardinal -Routine episiotomy not recommended, instead repair lacerations if they present -Passage: pelvis and surrounding soft tissues movements of labor”) -Deliver placenta within 30 minutes of birth of fetus and examined to be sure it is intact

118

Prenatal Care Week(s) Initial visit: 8-12 16 20 24 28 30 32 & 34 36 37 38 & 39 40+ Postpartum Discussion -History -Begin fetal -Importance -Pregnancy ROS: -Signs of -Pregnancy -Postterm -1 week incision check for C-sections highlights -Counseling movements of daily fetal cramping, bleeding, n/v, true ROS once > 42 -2 week check for vaginal deliveries -Anticipatory guidance -Round ligament movements constipation, fetal contractions weeks, -4-6 week f/u for everyone -Genetic screening options spasms  flank pain from here on movement, leakage, -Loss of discuss -Adjustment, breastfeeding, -Discuss contractions, preeclampsia mucus plug induction postpartum depression, return to sex, preterm sx (HA, vision ∆, edema, -Pregnancy contraception, bowel movements, labor RUQ pain, ↓ urine output) ROS lochia Complete PE  Pap & pelvic   Weight, BP check, fetal heart tones Measure Follow up with US for height > 3 cm discrepancy from gestational age fundal height Leopold’s maneuvers Cervical checks Imaging TVUS for dating 20 week US to assess Consider additional US for select high risk pregnancies or inability to measure accurate fundal fetal anatomy and size heights: h/o preterm labor (cervical length), obesity, DM, multiple gestation NST Consider for high risk women: IDDM, AMA, maternal heart defect, intrauterine  growth restriction, multiple gestation Biweekly Genetic 10-13 weeks: CVS 15-22 weeks: window for quad screen and screen 11-14 weeks: PAPP-A, NT amniocentesis bHCG  CBC   T&S  GC/C   RPR   HIV   Hep B surface   antigen Varicella &  rubella titers Vit D level  Glucose Consider for select high  tolerance test risk individuals HSV, TB, If hypothyroid need to follow TSH q 8 weeks with goal TSH 2-3 TSH, urine drug screen Urine dip  Consider repeat or frequent UAs for certain high risk individuals: UTI at initial visit, h/o pyelonephritis or kidney problem, symptoms of preeclampsia or diabetes  culture if + and f/u with test of cure F/u proteinuria with preeclampsia labs: 24 hour urine, CMP, PT/PTT, uric acid Rhogam Only give for abnormal  Before leaving hospital administration bleeding during this time GBS swab  119

COMPLICATED PREGNANCY Induced Abortion -98% of unsafe induced abortions occur in the developing world Management -Many US states have limits on abortions after 20 weeks -Antibiotic prophylaxis: doxycycline -Rhogam if indicated Methods -Surgical: D&C, vacuum Prognosis -Medical: for women < 63 days since LMP -Surgical complications: cervical laceration, hemorrhage, uterine perforation, incomplete abortion, Workup sepsis -Confirm pregnancy and gestational age -Psychological complications? Studies show women post-abortion have no higher incidence of -CBC and T&S mental health disorders Spontaneous Abortion -Pregnancy that ends spontaneously before fetus has reached age of viability ( = Signs & Symptoms Workup before 22 weeks) -Vaginal bleeding -US: no cardiac activity in a fetus with CRL > 6 mm or no growth of -80% occur in the first trimester -Abdominal pain or cramping pregnancy over one week are diagnostic for miscarriage; bad signs -Occurs in up to half of all pregnancies, although only half of these are diagnosed -Open cervical os indicated miscarriage include yolk sac abnormalities, fetal HR < 100, -Products of conception visualized in and large subchorionic hematoma Causes the vagina or cervical os -Serial quantitative hCGs: normal doubling is reassuring -Chromosomal abnormalities, esp trisomy 16 -Signs of hemodynamic instability and -Fibroids, polyps, or scarring fever if septic Management -Thrombosis or other placental complication -Follow quantitative hCG to zero -Infection Differential -May need surgical intervention: D&C -Fetal exposure -Physiologic bleeding from -Medical management (90% efficacy): mifepristone or misoprostol implantation -Expectant management is an option as long as there is minimal Risk Factors -Ectopic pregnancy bleeding or discomfort, pt is < 13 weeks, stable VS, and no evidence -Maternal or paternal age -Cervical polyp of infection (80% efficacy but can take days to weeks) -Increasing parity -Cervical infection or neoplasia -Administer Rhogam if mother is Rh- -Smoking -Recent sex -Methylergonovine maleate to control bleeding -Cocaine or caffeine -Broad spectrum abx if septic abortion (clindamycin + gentamicin or -High BMI Zosyn) -Submucosal fibroids or other uterine abnormality -Grief counseling -Asherman’s syndrome -Pelvic rest for 2 weeks -DM or PCOS -No evidence for avoiding pregnancy for 2-3 cycles -Thyroid disease -Contraception if desired -H/o spontaneous abortion Cesarean Section Indications Scheduled C sections Technique Post-operative risks -Indicated when clinician and patient feel that abdominal -Should be done at 39-40 weeks, or -Pre-op abx: cefazolin -Infection: increased over vaginal delivery is likely to provide a better maternal or fetal at 39 weeks if prior C section -SCDs for DVT prophylaxis -Hemorrhage outcome vs vaginal delivery -Higher risk of increased hospital -Transverse abdominal incision -Injury to pelvic organs: urinary, GI -Failure to progress stay, neonatal respiratory preferred over vertical (less post-op -Ileus -Nonreassuring fetal status problems, abnormal placentation in pain, greater wound strength, better -Thromboembolism -Fetal malpresentation future pregnancies cosmetic results) -Future abnormal placentation -Maternal infection -Lower risk of fetal injury than -Transverse hysterotomy rather than -Abdominal adhesions -Multiple gestation planned vaginal deliveries vertical (less blood loss, lower risk of -Numbness or pain from ilioinguinal nerve laceration -Fetal bleeding diathesis rupture) -Increased risk of uterine rupture in subsequent -Cord prolapse -Spontaneous placental extraction pregnancies -Suspected macrosomia preferred -Wound dehiscence 120

Abruptio Placentae (Placental Abruption) -Partial or complete separation of the Risk Factors Management placenta from the uterine wall prior to -High: cocaine use, trauma, polyhydramnios, eclampsia, prior abruption, chronic -Fetal HR abnormality on NST suggests impending distress and delivery of the fetus HTN, PROM, chorioamnionitis, fetal growth restriction, smoking emergency management -Moderate: AMA, multiparity, male fetus -Stabilization of maternal hypovolemia with large bore IV access with blood replacement Signs & Symptoms -Expeditious delivery for nonreassuring fetal HR, maternal -Painful vaginal bleeding instability, or gestational age > 36 weeks (should be C-section if -Tender uterine fundus unstable or with malpresentation) -Contractions -Expectant management of select cases in pregnancies < 36 -Abdominal pain weeks with administration of glucocorticoids in fetuses 23-34 -Can be asymptomatic weeks -Can be chronic: light, intermittent vaginal bleeding, oligohydramnios, fetal growth restriction, and preeclampsia Prognosis -Separation > 50% usually leads to acute DIC and fetal death Workup -Increased risk of abruption in all future pregnancies -US to eval for retroplacental hematoma but sensitivity is only 25-50% = diagnosis is clinical -Blood type and Rh status -NST Cesarean Section Indications Scheduled C sections Technique Post-operative risks -Indicated when clinician and patient feel that abdominal -Should be done at 39-40 weeks, or -Pre-op abx: cefazolin -Infection: increased over vaginal delivery is likely to provide a better maternal or fetal at 39 weeks if prior C section -SCDs for DVT prophylaxis -Hemorrhage outcome vs vaginal delivery -Higher risk of increased hospital -Transverse abdominal incision -Injury to pelvic organs: urinary, GI -Failure to progress stay, neonatal respiratory preferred over vertical (less post-op -Ileus -Nonreassuring fetal status problems, abnormal placentation in pain, greater wound strength, better -Thromboembolism -Fetal malpresentation future pregnancies cosmetic results) -Future abnormal placentation -Maternal infection -Lower risk of fetal injury than -Transverse hysterotomy rather than -Abdominal adhesions -Multiple gestation planned vaginal deliveries vertical (less blood loss, lower risk of -Numbness or pain from ilioinguinal nerve laceration -Fetal bleeding diathesis rupture) -Increased risk of uterine rupture in subsequent -Cord prolapse -Spontaneous placental extraction pregnancies -Suspected macrosomia preferred -Wound dehiscence Shoulder Dystocia -When shoulders of infant can’t fit Prevention Management through pubic symphysis because -Routine prophylactic C-section not indicated for -Get help they are wider than the pelvic outlet suspected macrosomia but can be considered in mothers -Episiotomy with h/o shoulder dystocia and brachial plexus injury -McRobert’s maneuver Risk Factors -Drain bladder and disimpact bowel -Maternal obesity Signs & Symptoms -DM -Prolonged 2nd stage of labor Prognosis -H/o or current macrosomic infant -Recoil of infant head on perineum (“turtle sign”) -Fetal complications: brachial plexus injury, clavicular or -H/o shoulder dystocia -Lack of spontaneous restitution (translation: no natural humeral fracture, increased risk of asphyxia head turning) -Maternal complications: hemorrhage, 4th degree tear

121

Labor Dystocia -Failure of labor to progress as Signs & Symptoms Management anticipated -Labor not following the norms of the Friedman -Administer oxytocin and monitor for 4- curve (although these values are now debated) 6 hours before considering operative Causes and Risk Factors -Uterine contractions < 200 Montevideo units delivery -Hypocontractile uterine activity -Intervention not indicated as long as -Inadequate pelvis labor is progressing and fetal HR -Fetal malpresentation or macrosomia reassuring -AMA -Maternal medical issues: DM, HTN, obesity -Prolonged rupture of membranes -Chorioamnionitis -Short maternal stature -High station at complete dilation

Ectopic Pregnancy -Most occur in the fallopian tube Risk Factors Workup -Others are cornual (interstitial), cervical, fimbrial, ovarian, -High: tubal obstruction or injury (PID, tubal ligation), -Quantitative serum hCG abdominal previous ectopic, DES use, current IUD use -Transvaginal US to examine uterine contents: diagnostic if true -Rarely heterotrophic (intrauterine and ectopic at the same time) -Moderate: infertility, smoking, older age, non-white gestational sac, yolk sac, or embryo is detected inside or outside of -The leading cause of pregnancy-related deaths in the 1st trimester ethnicities, previous cervicitis the uterus (should be able to visualize if hCG > 1500 which is the limit of US detection) Signs & Symptoms -Abdominal or pelvic pain Management -Amenorrhea or vaginal bleeding -If hCG is < 1500 and US is nondiagnostic, need to repeat US and -Usual pregnancy symptoms hCG in 3 days or when hCG level reaches US limit -Shoulder pain from blood pooling under diaphragm -Surgical if unable to comply with nonsurgical management, -Urge to defecate from blood pooling in cul-de-sac ruptured, or hCG > 5000: best outcome with salpingostomy, but will -Orthostatic BP need salpingectomy if ruptured -Fever -Medical management is the treatment of choice for women who are -Rebound tenderness hemodynamically stable with hCG < 5000 and tubal size < 3-4 cm: -Adnexal pain on bimanual exam methotrexate IM followed by serial hCG measurements -Cervical motion tenderness -Expectant management only for asymptomatic women with small tubal pregnancy and low hCG levels who are willing to accept the risk of rupture or hemorrhage

Fetal Distress Causes Prevention Signs & Symptoms Management -Cord compression -Continuous fetal HR monitoring vs -Prolonged abnormalities on fetal HR monitoring -Correct underlying abnormality -Placental abruption intermittent auscultation during labor: -Outpatient: monitor with repeat NSTs -Cord prolapse no evidence one is better than the other Workup -Improve fetal oxygenation -Maternal medication -High risk women should have -Outpatient: NST -Rapid operative intervention if needed -Rapid descent of fetal head continuous fetal monitoring during labor -Inpatient: fetal scalp stimulation (FHR acceleration in response is reassuring), fetal ST analysis, fetal scalp blood sampling

122

Gestational Diabetes -Carb intolerance induced by human placental lactogen Management Prognosis -Occurs in 3-5% of all pregnancies -Diet + exercise, insulin if needed (preferred over orals) -Increased risk of having DM postpartum, -Classification via White’s classification (A1 = nutritional -BG monitoring 4x daily, with goal FBG < 95 and 1 hour postprandial BG < 130 as well as preeclampsia, bacterial infection, controlled; A2 = insulin requiring -Early NSTs with amniotic fluid index for fetal monitoring of insulin-requiring macrosomia, neonatal complications, mothers d/t higher rates of placental insufficiency, with biweekly NSTs after 32 polyhydramnios, preterm labor, and Screening weeks for type A2 ketoacidosis -Oral glucose tolerance test administered at 28 weeks, when -Single 3rd trimester US to screen for macrosomia -Child will be predisposed to developing HPL begins to have most effect -Deliver by 40 weeks or earlier if fetus is nearing 8.8 lb DM later in life -Positive results followed up with 3 hour glucose tolerance test -Rescreen mother at 6 weeks postpartum for glucose intolerance Intrauterine Growth Restriction -Fetal growth < 10%ile for gestational age and gender Risk Factors Signs & Symptoms Management -Multiples share the same growth curve as singletons up -Chronic maternal vascular disease -Fundal height consecutively < 2 than expected -Delivery with maturity or by 37 to 22-24 weeks -Smoking weeks if evidence of compromise or -Fetal abnormalities Workup poor growth Causes -Poor maternal weight gain or malnutrition -US to evaluate fetal growth and %ile, with Doppler of -Congenital malformations -Vaginal bleeding during pregnancy umbilical cord to assess blood flow Prognosis -Chromosomal abnormalities -Low pre-pregnancy weight -Symmetrically small growth may just indicate small -High infant mortality within first 2 -Damage during organogenesis -Prior fetal growth restriction baby years of life -Infection: rubella, CMV -Prior stillbirth -Asymmetrically small growth indicates placental -Risk of intellectual deficits -Placenta previa -Alcohol, cocaine, or heroin use insufficiency (brain will be larger than body) -Placental infarction or single umbilical artery -Elevated AFP during 2nd trimester screen -NST and biophysical profile -Small placenta -Fetal karyotyping if polyhydramnios present -Multiple gestation Screening -Fundal height measurement Molar Pregnancy (Hydatiform Mole) and Gestational Trophoblastic Disease -Occurs when an extra set of paternal chromosomes is Signs & Symptoms Management incorporated into a fertilized egg, transforming the placenta -Vaginal bleeding -Suction uterine curettage with testing of tissue by a into a growing mass of cysts -Enlarged uterus excessive for gestational age pathologist -A complete molar pregnancy means there is no embryo or -Pelvic pressure or pain -Weekly hCG levels until normal normal placental tissue -Theca lutein cysts -May need prophylactic chemotherapy for high risk disease -A partial molar pregnancy means there is an abnormal -Anemia nonviable embryo and possible some normal placental tissue -Hyperemesis gravidarum Prognosis -Can coexist with a viable fetus -Hyperthyroidism -Risk of developing malignancy with uterine invasion or -Preeclampsia before 20 weeks’ gestation metastatic disease if tissue is retained: persistent or invasive Risk Factors -Vaginal passage of hydropic vesicles gestational trophoblastic neoplasia, choriocarcinoma, or -Extremes of age placental site trophoblastic tumor -Prior molar pregnancy Workup -Quantitative hCG: will be higher than expected -Pelvic US showing “snowstorm pattern” Pregnancy-Induced Hypertension Signs & Symptoms Workup Management Prognosis -Defined as BP > 140/90 after 20th week of -Differentiate from preeclampsia: urine dip for -Weekly prenatal visits -Generally favorable, not associated with pregnancy WITHOUT proteinuria in a protein may not be 100% reliable, so need to -Delivery at 37-39 weeks for frequent morbidity and mortality of preeclampsia, previously normotensive woman do 24 hour urine or spot urine:creatinine mildly elevated BPs, or earlier for severe however women with gestational HTN are at -No symptoms of preeclampsia, such as HA, -Assess fetal wellbeing with biophysical HTN increased risk of developing preeclampsia vision changes, RUQ pain profile or NST with amniotic fluid estimation -Antihypertensives if severe to reduce risk -Associated with development of HTN later in of maternal stroke life 123

Preeclampsia (Toxemia) & Eclampsia -Pregnancy-induced Screening Differential HTN with significant -Urine dip for symptomatic women -Exacerbation of underlying renal disease proteinuria ± -Acute fatty liver of pregnancy pathologic edema Signs & Symptoms -TTP/HUS -Can also have -Lies on a spectrum from mild & -Exacerbation of lupus preeclampsia asymptomatic to severe superimposed on -Only appears after 20 weeks, with Workup chronic HTN majority of cases after 28 weeks -24 hour urine -Irritability -CBC Risk Factors -Hyperreflexia -CMP -Multiple gestation -End-organ damage: frontal HA, -Uric acid -Obesity photophobia and visual changes, -Coags: PT, aPTT -Chromosomal or epigastric pain, oliguria, -NST congenital fetal nondependent edema -Diagnose with BP > 140/90 + proteinuria > 0.3 g in a 24 hour urine anomalies -Eclampsia: all s/s of preeclampsia + specimen -Pregestational DM seizures due to neurologic irritability -First pregnancy -HELLP syndrome: preeclampsia + Management -Age < 20 or > 40 signs of hemolysis, elevated liver -Deliver if severe preeclampsia or eclampsia enzymes, and low platelets -Expectant management with frequent monitoring with delivery at 37 weeks if mild -Seizure prophylaxis with mag sulfate if severe -Labetalol or hydralazine only for BPs > 150/100 to reduce risk of stroke

Multiple Gestation -More common developed Risks of multiple gestation Management Prognosis countries secondary to use of -Increased preterm delivery -Weight gain for twins should be 35-45 lbs -Single fetal death after 20 weeks gestation fertility drugs -Intrauterine growth restriction -No intervention proven to reduce risk of preterm occurs in 5% of twin pregnancies -Natural incidence of triplet -Discordant growth labor -Average duration of gestation for twins is pregnancy is 1/6000 births -Twin-twin transfusion -Frequent US to follow fetal growth 35 weeks, 32 weeks for triplets -Increased risk of cerebral palsy -Weekly NSTs beginning at 32 weeks for triplets Screening  Higher infant mortality rates than in singleton pregnancies -Suggested delivery for twins is 36-37 weeks, 35 -First trimester US weeks for triplets Workup -Determine chorionicity with US Placenta Previa -When placenta implants Risk Factors Signs & Symptoms abnormally in the lower -Multiparity -Painless vaginal bleeding uterine segment  partial or -AMA total blockage of cervical os -Asian Workup -Prior placenta previa -Avoid pelvic exam which can rupture the placenta -Smoking -Transvaginal US to assess placental location -H/o C-section -Multiple gestation Management -Total placenta previa  refer to high risk OB Screening -Marginal previa  f/u with serial US, avoid cervical US and sex, activity restrictions, -Usually detected in 1st or 2nd deliver via C-section at 36-37 weeks trimester US -Active bleeding  hospitalization with close monitoring, may need emergency C-section

124

Postpartum Hemorrhage -Defined as blood loss > 1000 mL (or > 1500 Risk Factors Signs & Symptoms Management for C-section) -Chorioamnionitis -Signs of shock and hypovolemia -Treat underlying cause -Avg vaginal delivery EBL is 500 mL (or 1000 -Uterine distension -Delivery of placenta > 30 min after infant -Uterine atony  uterine mL if section) -Prolonged or induced labor -Uterine atony massage, oxygen, large-bore IV -Occurs in 5% of deliveries -Use of mag sulfate -Signs of uterine rupture: hypotension greater than access, oxytocin, -Can be early (within 24 hours of delivery) or -General anesthesia expected for EBL, increasing abdominal girth methylergonovine late (up to 6 weeks after delivery) -Multiparity -Uterine inversion  manual -Previous hemorrhage Workup reduction of uterus, laparotomy Causes -Placenta previa or abruption -Check for retained placenta: inspect delivered -Uterine rupture  surgical -Uterine atony (causes 70% of cases) -Operative delivery placenta for missing parts, explore uterus intervention -Retained placental tissue -Look for traumatic cause of hemorrhage: tear, -Embolization of uterine or -Infection Prevention hematoma, uterine inversion hypogastric arteries -Blood vessel damage during C-section -Active management of 3rd stage of labor -Coagulopathy workup: PT/aPTT, fibrinogen, -Hysterectomy is last resort -Congenital coagulopathy -Use of oxytocin after delivery of the anterior shoulder antithrombin III Preterm Labor -Regular, painful uterine contractions with Prevention Workup Management cervical dilation or effacement before 37 weeks -Treating infections has not been shown to improve -Check fetal fibronectin, has good -Progesterone: maintains cervical integrity, outcomes NEGATIVE predictive value for opposes oxytocin, and is anti-inflammatory Possible Etiologies -ID of high risk women with early care and enhanced assessing risk of delivering in next -Tocolytics (anti-contractants like terbultaline, -Dental disease prenatal services also has failed to improve outcomes 7-14 days (can be inaccurate with mag sulfate, CCBs, indomethacin): no -Bacterial vaginosis -Can follow women with h/o preterm labor with frequent recent cervical disruption like sex or evidence that they improve outcomes but they -Inflammatory response US to assess cervical length TVUS) do buy time to administer steroids or transport -ACOG recommends offering progesterone to women -US measurement of cervical to NICU facility Risk Factors with cervical length < 15 mm or with h/o preterm delivery length; preterm labor likely if < 20 -Steroids to mature fetal lungs -Smoking -Cervical cerclage or pessary an option mm -GBS prophylaxis if needed or if culture not -Black -Evaluation of fetal lung maturity recently done -Extremes of age Signs & Symptoms (amniotic fluid specimen): -Bed rest, pelvic rest, and hydration have no -Low SES, poor housing, or other social stress -Contractions: back pain, abdominal pain, cramping lecithin/sphingomyelin ratio, foam evidence to back them up -Multiple gestation -Diarrhea stability index, -Avoid sex and strenuous physical activity -Intergestational period < 6 mos -Leaking fluid phosphatidylglycerol, or -Outpatient follow-up feasible for reliable -H/o cervical surgery or short cervix fluorescence polarization patients -Infection: bacteriuria or UTI, BV Premature Rupture of Membranes (PROM) & Preterm Premature Rupture of Membrans (PPROM) PROM Signs & Symptoms Management -Rupture of membranes at full term but before onset of labor (normally amniotic -Feeling “leaking urine” or increased vaginal secretions -If term, good evidence that labor should be induced sac ruptures well into labor) -Sx of chorioamnionitis: odor, fundal tenderness, low after this in order to prevent NICU placement; GBS -Occurs in 10% of normal pregnancies grade fever, fetal tachycardia prophylaxis if > 18 hours since rupture, or with colonization or fever PPROM Workup -If preterm, need inpatient monitoring, treatment of -Refers to rupture of membranes before 37 weeks -Visual exam for pooling of amniotic fluid in vagina infection if present, deliver if > 34 weeks or with fetal -Usually caused by maternal infection with test for ferning of sampled fluid distress, otherwise expectant management with steroids -Risk factors: intra-amniotic infection, prior h/o PPROM, lower SES, teen mom, -GC/Chlamydia testing if needed for fetal lung maturation smoker, h/o STD, h/o cervical cerclage, multiple gestation, polyhydramnios

125

Rh Incompatibility -Maternal immunization Screening Differential Management can occur as a result of -Maternal Rh status and antibody screening done at first prenatal -RBC membrane defects: hereditary spherocytosis -Emergent neonatal transplacental visit and at delivery -RBC enzyme defects: G6PD deficiency, pyruvate kinase transfusion at delivery for fetomaternal hemorrhage deficiency infants with signs of shock or blood transfusion with Prevention -Gilbert’s syndrome -Later transfusions for Rh+ blood -Rhogam given to all Rh- mothers at 28 weeks, again just after symptomatic anemia delivery if neonate is determined to be Rh+, and anytime during Workup -EPO and iron for mild pregnancy when there is risk of fetomaternal hemorrhage -Maternal and infant blood T&S anemia -Coombs test Signs & Symptoms -Infant peripheral smear -Rh incompatibility causes a spectrum of disease from -Antibody titers during pregnancy for mothers with known Rh hyperbilirubinemia to hydrops fetalis sensitization and Rh+ fetus

OTHER REPRODUCTIVE SYSTEM TOPICS Menopause Climacteric = a phase in women transitioning from a reproductive state Signs & Symptoms Workup Management to a non-reproductive state; includes perimenopause as well as a time -Begin up to several years before cessation of menses and -Diagnosis is usually -Dressing in layers, avoiding before and after can last for 2-9 years after menopause clinical food triggers, regular exercise -Dry hair and mouth, facial hirsutism -FSH > 35 is diagnostic -Estrogen replacement therapy Perimenopause = ~4 years before menopause when cycles become -Menstrual irregularlity, postcoital bleeding, (FSH is ↑in response to low for moderate to severe irregular and there are increased climacteric symptoms intermenstrual spotting estrogen) symptoms of vasomotor -Loss of adiposity and collagen in vulva, loss of -Check TSH if there are instability: use lowest dose for Menopause = time during cessation of menses for 1 year; can be protective covering of clitoris, thinner vaginal surface, symptoms of shortest amount of time natural due to loss of ovarian estrogen activity, induced via surgery or vaginal dryness and atrophy, genital itching or burning, hyperthyroidism possible radiation, temporary due to diet or GnRH therapy, premature if before dyspareunia, pale or shiny vaginal epithelium with loss of -For women under 45, do -Vaginal moisturizers and age 40, or late if after age 55 rugae, spare pubic hair, introital stenosis, fusion of labia oligo/amenorrhea workup: lubricants for vaginal atrophy minora, pelvic organ prolapse, vulvar dermatoses, stress hCG, prolactin, TSH, FSH symptoms, may need vaginal Postmenopause = time following cessation of menses for 1 year incontinence, urinary frequency, decreased libido -Women under 40 need estrogen -Hot flashes, vasomotor instability, sleep and mood comple premature ovarian -SSRIs -Average age of natural menopause in US is 51.4 years disruptions failure workup -Biofeedback -Reduced breast size and loss of ligamentous supports -Acupuncture Pelvic Organ Prolapse -Herniation of pelvic organs to or beyond the vaginal walls Signs & Symptoms Workup Management -Can be a cystocele, rectocele, enterocele, uterus, vaginal vault, fibroid -Feeling a bulge or something falling out -Pelvic and rectovaginal exam to -No treatment needed if -Classified via the Pelvic Organ Prolapse Quantitation system of vagina determine location and source of asymptomatic -Urinary, defecatory, or sexual dysfunction prolapse -Symptomatic prolapse may be Risk Factors -Neuromuscular exam: treated conservatively (pessaries or -Multiparity bulbocavernosus and anocutaneous Kegels) or surgically -Advancing age reflexes, sharp/dull touch, strength -Obesity -Hysterectomy -Chronic constipation -Heavy lifting

126

Pelvic Inflammatory Disease -Inflammation of the uterus, Signs & Symptoms Workup Management fallopian tubes, and/or ovaries, and -Pelvic or abdominal pain -Testing for GC, Chlamydia, -If no other cause of pelvic or abdominal pain can be found in a sexually active possibly surrounding pelvic organs -Painful defecation HIV, hep B, syphilis woman at risk for STIs, always treat for PID -Usually polymicrobial, with STIs + -Abnormal vaginal bleeding -Cervical cultures -Begin antibiotic before cultures come back endogenous organisms -Dyspareunia -hCG -Admit for inpatient management if there is pregnancy, nonresponse to oral -Uterine, adnexal, or cervical motion -Pelvic US if concern for antibiotics, inability to take PO, severe illness, or tubo-ovarian abscess Risk Factors tenderness abscess -Outpatient treatment of mild-mod PID: ceftriaxone IM + doxycycline -Multiple sex partners -RUQ pain (from perihepatitis) -CBC -Inpatient treatment of severe or complicated PID: IV cefoxitin + PO doxycycline -Douching -Signs of STI infection -UA -Treat partners -Smoking Prognosis -Risk for infertility increases with each episode Infertility -Failure to achieve pregnancy Signs & Symptoms Management within one year of frequent, -Men: genital infection, hernia, absence of vas deferens, signs -Treat underlying problem unprotected sex if < 35 or of androgen deficiency, testicular mass, varicocele -Bromocriptine for hyperprolactinemia within 6 months if > 35 -Women: breast formation, galactorrhea, genitalia, signs of -Treat ED hyperandrogenism -Varicocele repair Etiologies -Referral to fertility specialist for semen abnormality -Male issues: 1° Workup -Ovulatory dysfunction treatment: ovulation-inducing meds or hormone injections hypogonadism (androgen -CBC and CMP for both partners -Tubal repair insensitivity, cryptorchidism, -GC/Chlamydia -Laparoscopic ablation of endometriosis meds, varicocele, genetic -UA -Fertility monitoring: timed intercourse with fertility awareness methods will result in pregnancy in defect), 2° hypogonadism -Men: consider post-ejaculatory UA for retrograde ejaculation, 90% of couples (androgen excess, infiltrative scrotal US, FSH and testosterone levels, sperm studies, -For unexplained infertility, 3-4 cycles of clomiphene followed by intrauterine insemination is disorder, meds, pituitary transrectal US recommended adenoma) -Women: consider FSH, prolactin, TSH levels, antral follicular -IVF results in the highest per cycle pregnancy rate in the shortest time interval but is most costly -Female issues: ovulatory count via US, hysterosalpingography, pelvic US, and has a high rate of high order multiple pregnancy dysfunction, tubal damage, hysteroscopy, laparoscopy endometriosis, cervical factor Prognosis -Overall likelihood of successful treatment is 50%

127

ENDOCRINE SYSTEM DISEASES OF THE THYROID GLAND Hyperparathyroidism Workup

Etiologies -Primary hyperparathyroidsm: adenoma or other hyperplasia -Secondary hyperparathyroidism as a response to low Ca levels (vit D deficiency  impaired abs of Ca, or CKD  failure to activate vit D)

Signs & symptoms -Incidental hypercalcemia finding on blood test -Low bone mineral density screen -Weakness and fatigue, depression, bone pain, myalgias, decreased appetite, n/v, constipation, polyuria, polydipsia, cognitive impairment, kidney stones and osteoporosis -Rickets -Osteomalacia

Differential -Malignancy

Management -Primary hyperparathyroidism  surgical parathyroidectomy if symptomatic, otherwise monitor Ca and Cr annually and DEXA every 2 years -Calcium mimics if unable to undergo surgery

128

Hypoparathyroidism Etiologies Signs & symptoms Differential -Genetic -Paresthesias in the mouth, hands, and feet -Pseudohypoparathyroidism -Parathyroidectomy -Muscle cramps in the hands and feet -Vit D deficiency -Autoimmune -Fatigue -Meds -Hemochromatosis -Headaches -Kidney disease -Mg deficiency -Bone pain -Malabsorption -Insomnia -Abdominal pain Workup -Chvostek’s sign -Labs: low Ca, PTH, albumin to correct -Trosseau’s sign -Seizures Management -Arrhythmias -IV Ca gluconate if severe -Respiratory failure -PTH supplementation

Hyperthyroidism Signs & symptoms: weight loss, Differential Workup Management hyperphagia, heat intolerance, -Grave’s disease -Depressed TSH -Referral for endocrine increased sweating, frequent stools, -Toxic multinodular goiter with elevated free workup and imaging, oily hair or skin, exercise intolerance -Single toxic nodule (Plummer disease) T4 possible ablation due to heart changes, proximal -Subacute (de Quervain) thyroiditis: a result of viral infection, eventually leads to -↑ LFTs and Ca -Antiadrenergics: muscle weakness, nervousness, hypothyroid -↓ Lipids propranolol or diltiazem irritability, sleep disturbance, tremor, -Initial destructive phase of Hashimoto thyroiditis -Antithyroids: palpitations, decreased menstrual -Postpartum thyroiditis propylthiouracil, flow, onycholysis, exophthalmos, lid -Amiodarone-induced thyrotoxicosis methimazole lag, goiter, thyroid bruits, brisk -Rare: gestational trophoblastic disease, increased iodine intake, thyrotoxicosis factitia -Untreated thyrotoxicosis DTRs, muscle cramps, osteoporosis, pretibial (consumption of TH), ovarian tumor secreting TH, pituitary tumor secreting TSH, can progress to a thyroid myxedema thyroid carcinoma storm that can be fatal!

Hypothyroidism -TSH screen recommended for all elderly with depression and Differential Workup Initial therapy all elderly entering long-term care -Post-Hashimoto thyroiditis (most common cause) -Elevated TSH -Thyroxine with recheck of TSH in 6 weeks -Congenital hypothyroidism -↑ LDL, TG, -For patients still having symptoms 2-3 weeks Signs & symptoms: cold intolerance, fatigue, heavy menstrual -Post thyroid ablation or neck radiation LFTs, CK into therapy, recheck TSH and free T4 bleeding, weight gain, dry skin, constipation, bradycardia, -Transient causes: post-postpartum thyroiditis, post-de delayed DTRs, hoarseness, coarse hair, hair loss, myalgia, Quervain thyroiditis Maintenance therapy cognitive impairment, depression, decreased concentration, -Goiterous hypothyroidism: due to impairment of TH -Once TSH reaches reference range, recheck decreased hearing, periorbital or facial edema, non-pitting ankle synthesis (lack of iodine or genetic) annually edema, ± goiter -Lithium or other drug therapy -Dose needs to be increased in pregnancy and -Central hypothyroidism: not enough TSH being made decreased with aging

129

Myxedema Coma -Severe hypothyroidism Signs & Symptoms Workup Management -AMS -TSH and free -If clinical suspicion, begin treatment without waiting for labs Causes -Hypothermia T4 -Stress dose steroids until adrenal insufficiency has been excluded -Severe longstanding -Hypotension, bradycardia, hyponatremia, -Cortisol level -Administer loading dose T4 followed by daily dose hypothyroidism hypoglycemia, hypoventilation -Also give T3 (more rapid onset) until pt is stabilized with clinical improvement -Adrenal insufficiency -Edema of hands and face -ICU admission -Precipitation by acute -Thickened nares, swollen lips, or enlarged -Supportive care: passive rewarming (don’t use active rewarming device), mechanical ventilation for infection, MI, cold, or tongue respiratory distress, fluid replacement, correction of chemistry abnormalities sedatives Prognosis -Mortality 40% Thyroid Neoplasms and Thyroid Nodules -More common in women Management -Not associated with hypo or -Follow low-risk nodules every 6 months with hyperthyroidism palpation and US -Characteristics suggesting -Benign nodules may disappear over time malignancy: age < 20 or > 70, solid or Surgical removal if concern for malignancy complex, cold nodules, single nodule, -Refer for neck US to assess for size and shape nodule that grows with TH -Low TSH  radionuclide scan to check replacement, hoarseness or hot/coldness of nodule obstruction symptoms, hx of neck or -Refer for FNA if US results show risk of head radiation malignancy -Refer for resection if FNA cytology is suspicious Workup -Check TSH for all patients Prognosis -Can check autoantibodies -10% of palpable nodules will be malignant -Surgical complications: recurrent laryngeal nerve damage, parathyroid damage

Benign Thyroid Malignant Thyroid Neoplasms Neoplasms Follicular cell Papillary adenocarcinoma Medullary Adenocarcinoma adenoma -Most common type of thyroid cancer -Arises from C-cells of the thyroid -Age > 40 Follicular adenocarcinoma -Associated with MEN type 2 -Diagnosis usually occurs during evaluation of a cold thyroid nodule -Regional lymph node involvement -Treatment is through radioactive iodine ablation with hormone replacement to suppress TSH with mets to the lung, bone, and liver -Evaluate serum calcitonin, CEA, Hurthle cell thyroid cancer Ca, and plasma fractionated metanephrines -Very deadly

130

Thyroiditis Subacute Thyroiditis Painless Thyroiditis Acute (Suppurative) Riedel’s (Fibrous) Thyroiditis Thyroiditis -AKA de Quervain’s thyroiditis Causes -Rare! Signs & Symptoms -Usually autoimmune: Hashimoto’s (aka chronic lymphocytic thyroiditis) or a variant, can be postpartum -Painful, stony, hard Causes -From exposure to drugs like Li Cause thyroid -Radioiodine therapy -Precipitating factors: infection, stress, sex steroids, pregnancy, iodine intake, and radiation exposure -Suppurative bacteria -Dysphagia -Viral or infectious cause -Dyspnea -Trauma Signs & Symptoms Signs & Symptoms -Hoarseness -Painless thyroiditis; typically involves 2-6 weeks of hyperthyroidism followed by transient -Painful, red, tender Signs & Symptoms hypothyroidism, then euthyroid thyroid Management -Painful thyroid, neck pain, or -Postpartum presentation can occur up to 1 year after giving birth and has a longer course than typical -Short-term steroids goiter painless thyroiditis Management -Long-term tamoxifen -Hashimoto’s typically causes a goiter -Antibiotics therapy Workup -Surgical drainage if -TSH, free T3 & T4, ESR Workup needed -Radioiodine imaging (uptake -TSH, free T3 & T4 will be low) -Differentiate from Grave’s disease with technetium scan or radioiodine uptake scan

Management Management -Pain control with NSAIDs or -Monitor thyroid panel every 2-8 weeks to confirm resolution of thyroid imbalance and normalization of prednisone if refractory function -Monitor thyroid panel every 2-8 -β-blockers for palpitations weeks to confirm resolution of -Hyperthyroid phase is generally mildly symptomatic and does not require treatment thyroid imbalance and -Treatment of symptomatic hypothyroid phase with thyroxine normalization of function -β-blockers for palpitations Prognosis -May have recurrent episodes -20% develop permanent hypothyroidism Thyroid Storm -Rare, severe form of thyrotoxicosis Signs & symptoms Workup Management -Delirium -Thyroid panel -ICU admission Causes -Tachycardia -Diagnosis is clinical -β-blocker, thionamide to block new hormone synthesis (PTU -Stressful illness or surgery -Vomiting, diarrhea, dehydration or methimazole), iodine to block release of TH, iodinated -Thyroid surgery -Fever contrast and steroids to block peripheral conversion of T4  -Radioactive iodine T3 -Longstanding hyperthyroidism -Childbirth

131

DISEASES OF THE ADRENAL GLANDS Adrenal Insufficiency Signs & symptoms Workup -Chronic fatigue, lack of appetite, unintentional weight loss -Morning cortisol levels: levels > 18 high enough to rule out AI, levels ≤ 3 high enough to rule in -Joint pain AI -Abdominal pain, nausea, diarrhea -Synthetic ACTH simulation test -CV instability, hypoglycemia in times of stress, hyponatremia, hypotension unresponsive to -Lastly check the ACTH levels fluids or pressors Management -If acute crisis, treat with IV dexamethasone and IVF for hypotension -If chronic, glucocorticoid maintenance therapy is needed -Primary AI will also need mineralocorticoid replacement -MedicAlert bracelet and emergency steroid injections -Stress dose steroids needed for surgeries Primary Adrenal Insufficiency Secondary Adrenal Insufficiency: Tertiary Adrenal Insufficiency: Adrenal Crisis: acute, life- (Addison’s Disease): occurs when adrenal gland does not failure of pituitary to secrete ACTH failure of hypothalamus to secrete threatening low levels of cortisol respond to ACTH or make adrenal hormones (including CRH aldosterone) due to damage -Most causes are -Adrenal hemorrhage -Pituitary tumor -Usually due to suppression of CRH -Underlying adrenal condition Etiologies autoimmune -Drugs -Radiation and ACTH by exogenous cortisol use -Infection -Surgery -Long-term steroid therapy -Megace (appetite stimulant drug) -Sarcoidosis Signs & -Presentation can be slow or -Hyperkalemia -Slow onset -Vomiting -Fever symptoms abrupt -Vitiligo -No skin hyperpigmentation (because -Abdominal pain -Confusion -Skin hyperpigmentation -Pallor there is no excess ACTH) -Weakness -Syncope -Salt craving -Autoimmune thyroid disease -Intact RAAS -Hyponatremia Workup -Labs showing hypoglycemia, hyponatremia, hyperkalemia, -Insulin tolerance test -Labs showing low cortisol, low low aldosterone, and high renin due to increased renal sodium -Metyrapone test glucose, hyperkalemia, losses hyponatremia, elevated BUN

132

Cushing’s Syndrome -A general term for hypercortisolism at any Signs and symptoms Workup Management level, including adrenal, ectopic, or -Supraclavicular and dorsal fat pads -Always remember to check for exogenous -Surgical pituitary source (“buffalo hump”) glucocorticoid use resection is first -Central obesity -Always do labs first before any imaging to line Etiologies -Proximal muscle weakness avoid incidentalomas and false negative -Drugs to block -Exogenous steroid use -Thinning of the skin, purple striae, scans adrenal -Excess pituitary ACTH production: spontaneous ecchymoses, skin -Establish presence of cortisol excess: 24 response: includes Cushing’s disease (refers hyperpigmentation, papular acne hour urine, dexamethasone suppression test, somatostatin specifically to an ACTH-secreting pituitary -Osteopenia or saliva cortisol analogs, adrenal adenoma) -Hypertension -Establish ACTH dependence or steroid synthesis -Ectopic ACTH production: small cell lung -Early or delayed puberty independence to pinpoint problem to the inhibitors ca, carcinoid tumors, pheochromocytoma, -Growth retardation adrenals vs the pituitary gland thymoma, pancreatic cell tumors, -Glucose intolerance medullary carcinoma of the thyroid -Decreased libido, infertility, amenorrhea -Adrenal hypercortisolism: adrenal -Nephrolithiasis or polyuria adenoma or carcinoma or hyperplasia -Headaches

Adrenal Neoplasms -Majority are benign, nonfunctioning incidentalomas -Imaging characteristics suggesting benignity are homogeneity, smooth border, rapid contrast medium washout -Imaging characteristics suggesting malignancy are irregular shape, inhomogeneous density, delayed contrast washout, diameter > 4 cm, tumor calcification -Workup for all incidentalomas: 24 hour urine metanephrines and catecholamines, overnight dexamethasone suppression test, plasma aldosterone-to-plasmin renin activity ratio and plasma K+ if pt is hypertensive -Surgical resection of incidentalomas associated with pheochromocytoma findings, subclinical Cushing’s, > 4 cm, or if suspicious -Repeat imaging of incidentalomas 3-6 months after discovery, with removal of tumors growing > 1 cm during this time Benign Malignant Adrenal cortex adenoma Adrenal cortex carcinoma Pheochromocytoma -Cause Cushing’s syndrome, primary aldosteronism, virilization, or -Rare -Medullary neuroendocrine tumor feminization -Aggressive -10% will be malignant -Most are < 4 cm -May cause Cushing’s syndrome or virilization or can be nonfunctional -S/s: HA, swelling, tachycardia, HTN -Unilateral adrenalectomy if Cushing’s or hyperaldosteronism present -Present as abdominal mass or incidentally unresponsive to therapy -Most are > 4 cm -Surgical resection -Half will have advanced disease at initial presentation -Surgical resection -Adjuvant mitotane -Unfavorable prognosis due to presence of micromets in most disease

133

DISEASES OF THE PITUITARY GLAND Acromegaly (Gigantism) -Caused by GH-secreting pituitary tumor in adulthood Workup Management -If this occurs before puberty, it is called gigantism -Initial screen is IGF-1 test, will be high -Surgery -Most sensitive test is ↑ GH after glucose tolerance test -Somatostatin analogs (inhibit GH) Signs & symptoms -Labs: abnormal glucose, ↑P and PRL -GH receptor agonists -Enlarged soft tissue of the hands and feet -Pituitary MRI -Radiation -Teeth splaying, prominent brow -GHRH if MRI is negative -Hyperhidrosis Prognosis -Arthralgias -Bony abnormalities usually won’t regress -Headaches -Hypogonadal symptoms -Vision deficits -Fatigue, weight gain -Galactorrhea -CV disease such as HTN, LVH, or cardiomyopathy -Enlargement of thyroid, liver, kidneys, or prostate Diabetes Insipidus Central diabetes insipidus Signs & symptoms Workup -Lack of ADH production in the hypothalamus or insufficient -5-10 L per day of dilute polyuria -Direct serum ADH measurement is difficult as it is in release of ADH from the posterior pituitary -Polydipsia extremely low concentration -Etiologies: idiopathic, familial, panhypopituitarism, infiltrative -Hypernatremia -Pituitary imaging diseases, metastatic tumor, trauma or surgery, Wolfram -Normal glucose -Water deprivation test: restrict water and check Na, urine syndrome osmolality, urine output, weight, and orthostatic BPs/HRs every Differential 1-2 hours  + if body wt ↓ by > 5%, serum Na > 145, or > 2 Nephrogenic diabetes insipidus -Inpatient: mannitol, post-op diuresis, hyperglycemia, diuretics, urine osmolalities differ by < 10% -Kidney is resistant to ADH overzealous IVF, cured acromegaly -Differentiate central vs nephrogenic by giving desmopressin -Etiologies: amyloidosis, myeloma, Sjogren’s, sickle cell, -Outpatient: hyperglycemia, psychogenic polydipsia, osmotic (synthetic ADH)  central DI if urine vol ↓ or there is > 50% ↑ hypercalcemia, recovery from ATN, Li, foscarnet, methicillin, load in urine osmolality, nephrogenic if there is < 50% change in demeclocycline, colchicine urine osmolality Dwarfism Etiologies Workup Management -Achondroplasia: most common, genetic -Bone x-rays -Growth or thyroid hormone supplementation -Growth hormone deficiency: will see delayed puberty -Referral to endocrinology -Distraction osteogenesis -Others: congenital dysplasias, Noonan syndrome, Tuner syndrome, osteogenesis imperfecta, hypothyroidism Pituitary Neoplasms -Differential: physiologic pituitary enlargement of pregnancy, sellar cyst or abscess, lymphocytic infiltration of the pituitary -W/u: MRI, eval of hypothalamic-pituitary axis function Benign Malignant Pituitary adenoma Craniopharyngioma Germ cell tumor Primary CNS lymphoma -Only tumor that causes hormonal hypersecretion -May secrete gonadotropins, TSH Meningioma Chordoma Breast or lung mets -S/s: neuro sx, hormonal abnormalities, incidental -Resection if causing neuro sx -Annual monitoring of growth and HPA

134

DIABETES MELLITUS Type II Diabetes Mellitus Prevention Signs & symptoms Prevention of complications Glycemic control -7% TBW loss, food journals, and visiting dietician -Blurred vision -Diabetic retinopathy, cataracts, -If A1c 6.5-7.5  start on metformin unless regularly better than starting on metformin -Fatigue glaucoma  annual eye exams contraindicated -Polyuria & polydipsia -Nephropathy and ESRD  BP -If A1c is 7.6-9%  start on metformin + Screening -Weight loss and BG control, ACEI, annual additional oral -ADA says do for all patients who are overweight or -Hirsutism (DM2) urine microalbumin (should be < -If A1c is > 9%  start on insulin if who have risk factors, and for all patients over age 45 -Acanthosis nigrans (DM2) 30) symptomatic or metformin + 1-2 other orals if every 3 years -DKA (more likely DM1) -DM is a cardiac risk equivalent asymptomatic -Peds: universal not recommended by AAP nor ADA, -In kids often occurs at onset of puberty as this causes  cholesterol control (LDL < -Initiate insulin if 3+ orals are needed to rather screen at risk children with BMI > 85th increased insulin resistance 100, HDL > 50, TG < 150), baby control BG or if A1c remains > 8.5 even after percentile and 2+ additional risk factors; screen every aspirin (ADA rec) dual therapy 3 years Workup -Neuropathy is the most common -Rebound (Somogyi) hyperglycemia occurs in -FBG: prediabetes is 100-125, diabetic is 125+ -C-peptide: ↓ in DM1, normal or hi in DM2 complication: cardiac response to hypoglycemia -Oral glucose tolerance test: prediabetes is 140-199, -Fructosamine (glycated albumin): gives info about denervation, gastroparesis, -Dawn phenomenon is morning hyperglycemia diabetes is 200+ short-term BG in last few weeks neurogenic bladder, ED, etc  as a result glucagon response to waning insulin -Random glucose test: diabetic if 200+ -Differentiate from DM1 by presence of excess annual diabetic neuropathy levels around 3-5 am  change insulin or -Hb A1c: diabetic if > 6.5%, want DM to be < 7%, weight, acanthosis nigricans, HTN, dyslipidemia, screens move peak to a more physiologic time check this every 3 mo if it is not at goal or 1-2 times PCOS, FH, ethnic group risk factors per year for patients at goal Oral Pharmacologic Treatment: most are not for DM1, they have no insulin to secrete! Sulfonylureas Biguanides: metformin GLP-1 agonists: exenatide pen, long-acting exenatide, -1st gen: chlorpropamide, tolazamide, tolbutamide  more -Decrease hepatic glucose output 1st and then increases uptake liraglutide AEs by fat and muscle -Aid native GLP-1 naturally released by the gut to help insulin -2nd gen: glipizide, glyburide, glimepiride  don’t work better -May help with weight loss secretion while reducing glucagon but have less AEs and less drug interactions -Every DM2 should be put on this at time of diagnosis -Only respond when BG is high! -Hug pancreas all day long = tend to burn out pancreas after 3- -Must monitor creatinine @ baseline and annually to screen for -Need to be paired with a DPP-4 inhibitor like sitagliptin, 5 years of use kidney disease (from the DM, not from the metformin itself), saxagliptin, or linagliptin -Hypoglycemia risk stop using if Cr > 1.4 in women and 1.5 in men  risk of lactic -Need to decrease sulfonylurea dose to avoid hypoglycemia -Start on low dose and adjust over 3-4 weeks acidosis -Contraindications: pancreatitis, gastroparesis, GI issues, CrCl < -Begin combination therapy with another agent when -Contraindicated in kidney or liver disease, elderly, CHF, 30, thyroid cancer approaching max dose alcohol abuse or binge drinking, IV contrast -Many drug interactions, must take 1 hour prior Meglitinides: repaglinide, Glitazones: pioglitazone, rosiglitazone Amylin agonists: pramlintide α-glucosidase inhibitors: Bile acid Dopamine nateglinide -Increases glucose uptake in fat and -Aid amylin, which is co-secreted with acarbose, miglitol sequestrants: agonists: -Hug pancreas with one quick muscle 1st, then decreases hepatic insulin, to suppress glucagon secretion and -↓ Glucose abs in the colesevelam bromocriptine squeeze = take with a meal glucose output regulate gastric emptying intestine -Only used if -Only offer -Hypoglycemia risk -Current FDA restrictions due to risk of -Can be used in DM1 but at lower doses -Cause farts patient needs lipid modest -May cause weight gain = less bladder ca, fracture risk, CV side effects -Rarely used because it only serves to fine- -Contraindicated in bowel management as well decreases in favorable = try other meds first tune blood sugar control while doubling the disorders, liver or renal as DM and can’t A1c with -No renal or liver patients -Contraindicated in liver disease, heart number of injections needed impairment tolerate a statin significant GI -Pregnancy C failure -Contraindicated with GI disorders side effects -Pregnancy C

135

Insulin Formulations Initiating insulin in DM2 Initiating insulin in DM1 Switching from NPH to long-acting insulin: if -Rapid: aspart, glulisine, lispro  30 minute peak = before each meal -Avg size patient  begin with -Calculate total daily dose, which is 0.5- NPH was once daily, a unit-to-unit change is ok; -Short (may be purchased without rx): regular  2 hour peak = before each 10 U basal insulin once daily 0.7 U/kg/day in adults if NPH was 2x daily, ↓ total dose by 20% and meal -Obese patient  begin with 0.2 -Individual injections of basal and bolus give it as a once daily dose -Intermediate: NPH (may be purchased without rx)  6-10 hour peak = before breakfast + supper U/kg basal insulin once daily are best: 50% of TDD should be basal, Rule of 1800: 1800/TDD = how many mg/dL -Long-acting/basal: detemir, glargine, aspart protamine  no peak = 1-2x -Insulin mixes  begin with 0.6 50% should be bolus (and further divided that 1 U of insulin will change your patient’s BG daily U/kg total daily dose, 2/3 of this into 3 mealtime doses) -Mixes: 70% NPH/30% regular, 75% aspart protamine/30% rapid in the am and the remaining 1/3 -Can also use insulin mixes and Rule of 500: 500/TDD = how many g of CHO in the evening administer as in DM2 for patients who that 1 U of insulin will cover -Most syringes are U-100 but U-500’s are 5x as concentrated and are are unwilling to do multiple injections manufactured for patients needing > 200 U of insulin daily per day

Type I Diabetes Mellitus -Accounts for most cases of diabetes in kids under 19 Workup -Genetic and environmental influences -Differentiate from DM2 by islet autoantibody screen

Signs and symptoms Management -Polyuria -Formal training and education using a diabetes team -Polydipsia -Intensive insulin regimen -Weight loss -Address depression and anxiety -Lethargy -Annual urine microalbumin -DKA is often the initial presentation -Ophtho visits at age 10 or after 3-5 years of diagnosis -Lipid screens -Periodic autoimmune thyroid and celiac screening Diabetic Ketoacidosis -Typically occurs in DM1 but can happen in DM2 Signs & Symptoms Management -Usually present with early symptoms of -Treat precipitating cause Differential SOB and abdominal pain -Correct deficits gradually to avoid cerebral edema -Other things that cause anion gap metabolic -Later symptoms of hyperosmolarity: -Replace fluid deficits: give several L of NS, switching to ½ acidosis (lactic acidosis, aspirin, methanol, ethylene polyuria, polydipsia NS once hyponatremia corrects, and adding dextrose once glycol, etc) serum glucose ↓ to 200 Workup -Replace K+ deficits: initial hyperkalemia will rapidly Precipitating Factors -Serum glucose is usually < 800 become hypokalemia once insulin is started; may need to -UTI is #1 trigger -3 ketone bodies are produced: acetoacetic supplement K before starting insulin! -Pneumonia acid, β-OH-butyrate, and acetone, so check -Start insulin drip (preferred over SC insulin unless DKA is -Pancreatitis dipstick UA and serum ketones mild) ± bolus, continue with titration by protocol until BG is -MI -BMP shows anion gap metabolic acidosis, 200, then add on SQ therapy and wean off drip in ~1-2 hours -Stroke hyponatremia (intracellular shift), -Continue to monitor BMP and continue drip until gap -Trauma hyperkalemia (shift out of cells) closes -EtOH or drug abuse -VBG to determine severity of acidosis -Can monitor serum ketones, however they may persist up to -Missing insulin dose -May have elevated amylase or lipase even 36 hours after the gap closes as removal takes some time -Dehydration if pancreatitis is not present -Bicarb only given for severe acidosis Hyperosmolar Hyperglycemic Nonketotic Syndrome (Hyperosmolar Hyperglycemic State) -DKA without the Signs & Symptoms Workup Prognosis ketoacidosis -May see neurologic changes or coma -Differentiate from DKA by absence of -Associated with higher mortality than DKA ketones -Serum glucose is usually > 1000 136

LIPID DISORDERS Hyperlipidemia -Most LDLs > 190 have a genetic component Labs Cholesterol Goals Management -2° dyslipidemia can be a result of DM, -Lipid panel: TC, LDL, HDL, TG (TC and Known LDL goal < 100 1.) With abnormal lipids without hypothyroid, obstructive liver disease, chronic HDL can be measured even if patient was CAD CAD or CAD risk equivalents, set an renal failure, meds, diet, or sedentary lifestyle not fasting) Sometimes < 70 LDL goal first and initiate therapeutic -Direct LDL: for nonfasting patients or if TG lifestyle changes for 3-6 months -Treating known hyperlipidemia up to age 75 > 400 Risk MI, stroke, DM, LDL goal < 100 (unless TG > 500, then address this results in significant reductions in morbidity and -CAD  LDL goal > 100 or sometimes < 70 equival carotid artery first to prevent pancreatitis) mortality -CAD equivalents (MI, stroke, DM, carotid ents disease, PVD, AAA Sometimes < 70 2.) If lifestyle changes don’t work, artery disease, PVD, AAA)  LDL goal > consider meds 100 or sometimes < 70 3.) If meds don’t result in LDL goal Screening Risk Smoking 1 risk factor makes -Once between 2-10 years and q 3-5 years -Risks factors (smoking, HTN, HDL < 40, being met, add a higher dose statin or factors HTN an LDL goal < thereafter for pediatric patients with risks (obesity, FH premature CAD, men > 55, women > 65) another med HDL < 40 190 HTN, FH)  one risk factor makes an LDL goal < 190 4.) If TG remain elevated, set another FH premature CAD -Every 5 years for patients 20-35 with risk factors while two risk factors makes an LDL goal < goal for TG that is LDL goal + 30 Men > 55, Women > 2 risk factors (DM, FH, CV risk) 130  For CAD or CAD equivalent 65 makes an LDL -Otherwise begin at 35 in men and 45 in women patients, start meds and lifestyle goal < 130 changes right away

Drug Class Statins Fibric Acid Bile Acid Niacin Ezetimibe Other Derivatives Sequestrants Options Information -Inhibit HMG CoA reductase -Stimulate -Cause liver to break -Blocks VLDL -Inhibits cholesterol -Fish oil: ↓ -First-line medication to reduce LDL transcription factor to down more cholesterol synthesis  shift in absorption at the TG by 20- -Taken at bedtime, when cholesterol synthesis peaks promote lipid to make new bile LDL from small brush border 50%, minor ↑ -Check baseline LFTs before starting metabolism -Good addition to and dense to larger -Better tolerated LDL and -FDA recs rechecking LFTs into treatment only if patient is -Contraindicated in statin therapy to and more buoyant than a bile acid HDL, symptomatic and d/c of therapy if LFTs have increased to 3x severe renal or further ↓ LDL -SE of flushing, sequestrant increased risk ULN hepatic disease -Contraindicated with prevent with aspirin -Contraindicated in of bleeding -May also check TSH before starting statin b/c hypothyroidism TG > 400 before liver disease -Red rice predisposes to myalgias as well as dyslipidemia -Contraindicated yeast: has -If pt has myalgias, check CK but it isn’t always elevated even with liver disease, natural HMG with statin-induced myalgias; CK ↑ > 10x ULN is a reason for gout CoA d/c of statin reductase -Drug interactions activity -Contraindicated in chronic liver disease -Pregnancy category X Effect on ↓ LDL by 20-60% (doubling dose  additional 6% ↓) ↓ LDL by 5-10% ↓ LDL by 15-30% ↓ LDL by 5-25% ↓ LDL by 15-20% cholesterol ↓ TG by 7-30% ↓ TG by 20-50% No effect on TG ↓ TG by 20-50% ↓ TG by 5-10% ↑ HDL by 5-15% ↑ HDL by 10-20% ↑ HDL mildly ↑ HDL by 15-30% ↑ HDL mildly Specific -Atorvastatin: ok to use -Fluvastatin: less muscle toxicity, ok -Gemfibrozil -Cholestyramine Drugs in CKD to use in CKD -Fenofibrate -Lovastatin -Rosuvastatin -Pravastatin: less -Simvastatin: new FDA warning, muscle toxicity, good don’t exceed 20 mg simva with 2nd choice for pts with ↑ amiodarone, amlodipine, or LFTs on other statins ranolazine; don’t exceed 10 mg simva with diltiazem or verapamil

137

OTHER ENDOCRINE TOPICS Hypoglycemia Causes Signs & Symptoms Workup Management -Drugs: insulin or insulin secretagogue, other drugs -Autonomic response: diaphoresis, -Only indicated in nondiabetic patients who exhibit Whipple’s -Administer rapidly absorbed -Alcohol weakness, tachycardia, palpitations, triad: symptomatic hypoglycemia confirmed on lab testing with carb if able to eat or IV glucose, -Critical illness tremor, nervousness, hunger, relief of symptoms after plasma glucose is raised or glucagon SQ if no IV access -Malnutrition paresthesias -If BG is low in a nondiabetic, also draw insulin level, C-peptide, -Maintenance glucose as -Hormone deficiency: cortisol, glucagon, -Irritability, confusion, or seizure and oral hypoglycemic agent screen before treating needed with monitoring every epinephrine -Transient focal neuro deficits -Insulin and C-peptide elevated in insulinoma, oral agent use 10-15 minutes -Nonislet cell tumor -Visual disturbance -Exogenous insulin use will also cause elevated insulin levels but -Insulinoma C-peptide will be low -Post gastric bypass -Insulin autoimmune disorder -Factitious hypoglycemia Hypothermia Differential Signs & symptoms Workup Management -Environmental exposure -Mild: tachypnea, tachycardia, hyperventilation, -POC glucose: insulin does not -ABCs -Hypothyroidism or adrenal insufficiency ataxia, dysarthria, impaired judgment, function below 30° -Head to toe survey for frostbite, etc. -Sepsis shivering, cold diuresis -EKG -May need to check pulses with a Doppler -Neuromuscular disease -Moderate: bradycardia, hypoventilation, CNS -BMP -Chest compressions only if no signs of life -Malnutrition depression, hyporeflexia, loss of shivering, -CBC -Warmed crystalloid infusions -Thiamine deficiency paradoxical undressing, arrhythmias -Lactate -Avoid rough movement (heart sensitive to -Hypoglycemia -Severe: pulmonary edema, oliguria, areflexia, -CK arrhythmias during this time) -EtOH or CO intoxication coma, hypotension, bradycardia, arrhythmias, -Fibrinogen -Rewarming: passive if mild, active if mod to -Meds: anxiolytics, antidepressants, antimanics, asystole -ABG severe antipsychotics, opioids -Pt will be coagulopathic as factors are -CXR inhibited below 37° Polycystic Ovarian Syndrome -Highly genetic predisposition Workup Management -Diagnose with 2/3 Rotterdam -Weight loss and exercise Signs & Symptoms criteria: oligomenorrhea, -Follow fasting lipids and glucose regularly -Oligomenorrhea hyperandrogenism, polycystic -Assess for depression, eating disorders, and sleep apnea regularly (hi prevalence in this -Hyperandrogenism  acne, hirsutism, male-pattern hair ovaries on US population) loss, DUB due to endometrial hyperplasia -Also can check total testosterone -Fertility evaluation if desired -Obesity -Rule out other causes of irregular -Hirsutism and other androgenic symptoms  OCPs, adding spironolactone later if needed (has -Glucose intolerance menses: bHCG, prolactin, TSH, antiandrogenic effects) -Dyslipidemia FSH -Endometrial protection against hyperplasia  OCPs or intermittent progestins to induce bleeding -OSA -Glucose intolerance  metformin especially helpful if infertility also present -NASH -Infertility  weight loss, consider clomiphene

138

GENITOURINARY SYSTEM GENITOURINARY TRACT CONDITIONS Benign Prostatic Hypertrophy (Hyperplasia) -Risk increases with age, and prostate Workup Pharmacologic MOA Information undergoes growth spurt after age 40 -DRE +PSA (prostate size will not be correlated to symptoms, Therapy -Only some men are symptomatic rather you are trying to detect malignant cause) Alpha-1 -Decrease muscle -Do not decrease prostate size -Abdominal exam for bladder distension blockers tone for rapid -Nonselective (terazosin, doxazosin, Signs & symptoms -Neuro exam symptom relief prazosin) not recommended with -Irritative symptoms = bladder storage -Post-void residual/bladder scan concomitant HTN due to risk of first dose problems like urgency, frequency, -UA to exclude infection or hematuria syncope and orthostatic HTN, take 2-4 weeks nocturia, urge incontinence, stress -Rule out urethral stricture of bladder neck contracture (no for full effect incontinence instrumentation, urethritis, or trauma) -Selective (tamsulosin, alfuzosin) have no -Obstructive symptoms = bladder effect on BP and begin to work in days to 1 emptying problems like hesitancy, poor Management week, slight risk of ejaculatory dysfunction flow, intermittency, straining, dysuria, -Always based on symptoms (calculate AUA symptom index 5-alpha -Blocks conversion -Finasteride dribbling, incomplete bladder emptying score) reductase of testosterone  -For those who can’t tolerate alpha- blockers -Watchful waiting only if pt is not bothered inhibitors dihydrotestosterone -Shrink prostate -Natural saw palmetto supplement -Take 6-9 months -Refer to urology for surgical intervention with recurrent -Pregnancy category X UTIs, recurrent gross hematuria, bladder stones, CKD, urinary -AEs of ED and ejaculatory dysfunction retention Saw palmetto -Not shown to be helpful in clinical trials Congenital Urinary Tract and Kidney Abnormalities -Account for 25% of all anomalies identified in the prenatal period Postnatal workup -Caused by genetic and environmental factors -Voiding cystourethrography -Renal scan Types -DMSA scan to detect ectopic renal tissue -Renal dysplasia -Renal agenesis Management -Renal tubular dysgenesis -Serial US to monitor compensatory growth of unaffected kidneys -Polycystic renal disease -Collecting duct abnormalities  ureteropelvic junction obstruction, megaureter, ectopic ureter, vesicoureteral reflux, bladder exstrophy, posterior urethral valve -Horseshoe kidney -Pelvic kidney

Vesicoureteral Reflux -Currently this is treated as it is thought to promote renal Workup Management scarring and recurrent pyelonephritis -Renal US for infants diagnosed with prenatal hydronephrosis -Grades I and II can be managed with observation -Can occur prenatally and may be seen on prenatal US as -Contrasted voiding cystourethrogram -Kids with > grade III reflux are treated hydronephrosis -Radionuclide cystogram -Antibiotic prophylaxis: Septra, trimethoprim, nitrofurantoin -Graded I-V based on severity -Serum creatinine -Surgical correction -UA -Annual imaging for medical or observational therapy -Screen siblings for reflux -Annual growth checks, BP, and UA

139

Cryptorchidism -Most undescended testes will descend spontaneously by the time an infant is several months old but Management will rarely occur after 6 months -Refer for testes not descended by 6 months for surgical orchiopexy due to risk of malignant -Ectopic testes are descended but are in an aberrant position such as the inguinal pouch, suprapubic degeneration, subfertility, torsion, or inguinal hernia region, or perineum -Occasionally descended testes can ascend as child grows -Retractile testes and located suprascrotally but can descend to the scrotum and remain there as long as the cremasteric reflex is overcome Erectile Dysfunction Causes Workup Pharmacologic MOA Information -Usually neurovascular, and may be -Ask if pt is having normal erections, if not then it is Therapy an early marker of vascular disease an organic issue, if yes than it is a psychogenic issue Phosphodiesterase -Interfere with -Sildenafil, tadafil, vardenafil -HTN -Early morning testosterone with free testosterone type 5 inhibitors cGMP breakdown -First line agents -Dyslipidemia -Lipids  continued dilate -May need 6-8 tries before these meds work -Smoking -FBG of inflowing blood -AEs: loss of blue-green color vision, hypotension -Hyperglycemia -Prolactin if there is nipple discharge vessels -Contraindications: concomitant nitrates, severe CV -Penis curvature disease -Diabetic neuropathy Management Yohimbe -Natural alpha-2 -Safe and low cost -Not currently -MS -Helpful book “The New Male Sexuality” adrenergic-R recommended by AUA -Low testosterone -Refer for surgical options: inflatable prosthesis, blocker derived  It is normal for men to have balloon dilation, vein ligation from African tree slowly decreased erection hardness -Smoking cessation has greatest effect when patient is Alprostadil -Prostaglandin -Not a good initial -Painful and longer refractory periods in younger with mild symptoms injection penile injection treatment -Contraindicated in sickle between orgasms as they age -Issues of fibrosis cell

Prostaglandin -Expensive, does not work for many intraurethral pellet

Hydrocele -Collection of peritoneal fluid between the Signs & symptoms Workup parietal and visceral layers of the tunica -Soft, cystic scrotal mass -US vaginalis in the scrotum -Usually painless -Mass transilluminates Management Etiologies -May be bilateral -Treatment not necessary unless symptomatic -Fluid imbalance between secretion/absorption -Surgical excision in the tunica vaginalis -Simple aspiration has high recurrence rate -Injury or inflammation -Surgical repair of patent processus vaginalis -Neoplasm -Torsion

140

Varicocele -Dilation of pampiniform vein plexus Signs & symptoms Management -May be due to valve insufficiency in gonadal -More common on the left side -Scrotal support veins -“Bowl of spaghetti” or “bag of worms” appearance that -Analgesics ↑ with valsalva -Surgical repair -Oligospermia or asthenospermia -Painful, dull, or heavy sensation in scrotum

Incontinence Type Stress Incontinence Urge Incontinence Overflow Incontinence Functional Incontinence Signs & -Leakage with coughing, sneezing, standing -Involuntary loss of large amount of -Continuous or persistent urine loss -Inability to toilet due to cognitive symptoms -No leakage when supine urine preceded by intense feeling of through day and night due to chronic impairment, physical disabilities, having to void but without sufficient urine retention psychological problem, or warning -Painful abdomen environmental barriers -Loss of urine with sound of running -Large PVR water or waiting to use toilet -Urine dribbling -Unrelated to position -Unawareness of urine loss -Normal PVR Management -Topical estrogens if due to atrophic vaginitis -Fluid restriction after 6pm -Alpha blockers to relax bladder neck -Decrease use of sedatives, alcohol, -Kegel exercises -Frequent, scheduled voids -Treat BPH if present and anticholinergics -Weight loss -Relaxation techniques -Refer to urology for prostate -Reschedule meds to not act during -Refer to urology/urogyn for sling procedures, vaginal -Bladder antispasmodics: tolterodine, resection sleeping hours repair, injection of bulking agents, or pessary fitting oxybutynin -Indwelling, intermittent, or -Easy access to commode or urinal -Anticholinergics suprapubic catheterization -Easy-to-remove clothing -Refer to urology for nerve stimulator, -Scheduled toileting -Scheduled or prompted toileting Botox injections Paraphimosis & Phimosis Paraphimosis Phimosis -Retracted foreskin in uncircumcised male that Management -Foreskin of Management can’t be returned to normal position -Immediate urologic consult if uncircumcised male can’t -Steroid cream ischemic be retracted -Manual stretching Causes -Pain control, may need local -Circumcision -Over-retraction of foreskin while cleaning anesthesia Causes -Sexual intercourse -Reduce swelling -Lichen sclerosus Prognosis -Iatrogenic: cystoscopy, bladder catheterization -Reduce foreskin back manually -Balanitis -Untreated phimosis is a risk -Repeated episodes of balanitis -May need surgical techniques for factor for urinary retention and failed manual reduction penile carcinoma Signs & Symptoms -Penile swelling and pain -Blue or black discoloration of glans if ischemic

141

Nephrolithiasis/Urolithiasis -Southern “stone belt” Differential: AAA, appendicitis, tubo-ovarian abscess, Management -FH incurs 3x greater risk ectopic pregnancy, renal cell carcinoma, intestinal -Asymptomatic stones found incidentally are usually left obstruction alone unless pt is a pilot, frequent flyer, or only has one Types of stones kidney -Ca oxalate Workup -Send to ED if septic, intractable n/v, solitary kidney, large or -Ca phosphate -Spiral CT (no contrast) for evaluation of flank pain in proximal stones -Struvite: may be secondary to recurrent infection first-time stoners, initial imaging of choice -If tolerating PO meds and stone is < 5 mm, can do home -Uric acid: h/o gout -US (less sensitive but good for eval of secondary signs of management with oral fluids, pain control, tamsulosin, urine -Cystine obstruction) straining for 48-72 hours, nephrology referral if no -Medication crystallization -KUB will show 85% of stones since most are made of Ca improvement -Staghorn calculi associated with Proteus -UA usually shows microscopic or gross hematuria -Stones > 5 mm may need urology eval for shock wave -Urine culture to look for Proteus lithotripsy or other method of stone removal Signs & symptoms -Urine pH persistently < 5.5 suggests uric acid or cystine -Admit for pts with intractable pain, inability to take PO meds -Abrupt flank pain that is severe, colicky, may radiate to scrotum/labia stones and fluids, or fever or groin -Urine pH persistently > 7.2 suggests struvite stones -Nausea and vomiting -Urine pH between 5.5-6.8 suggests Ca-based stones Prevention -Hematuria -CBC to look for infectious cause -Limit sodium intake -Prior episodes -First-time stoners with uncomplicated stone need BMP -Don’t limit Ca supplements or intake unless stones are Ca -CVA tenderness and uric acid levels -Potassium citrate: regulates urine pH and binds Ca -LQ pain on palpation -Recurrent stoners or FH need extensive workup, do 2 x 24 -Thiazide diuretics reduce Ca -Pts will want to constantly move hour urines and refer to nephrology -Allopurinol lowers urinary uric acid -Urinary frequency and urgency with stone lodgement at -Tiopronin/D-penicillamine reduces cystine levels ureterovesicular junction -Acetohydroxamic acid with antibiotics reduces struvite -Less pain after stone passes into the bladder and through the urethra formation -Size of stone does not correlate to severity of symptoms Testicular Torsion -Twisting of the spermatic cord within a Signs & Symptoms Management testicle, cutting off blood supply -Scrotal pain and swelling -Surgical emergency, must be treated -A result of inadequate fixation to the tunica -N/v within 4-6 hours with irreversible damage vaginalis -Abdominal pain and possible infertility after 12 hours -Can be spontaneous or post trauma -May wake child up in the middle of -Manual detorsion if surgery unavailable -More common in neonates or postpubertal the night males -Tender epididymis, elevated testis, and scrotal discoloration -Absent cremasteric reflex

Management -Color Doppler US

142

INFECTIOUS & INFLAMMATORY CONDITIONS Cystitis Group Women Men Pediatrics Info -Risk factors: sex, spermicide, diaphragms, DM, h/o recurrent UTIs, recent abx -Rare in men under -Agents: 90% are E. coli, also Staph saprophyticus, Enterococcus, enterics -Agents: usually E. coli or Staph saprophyticus, also Proteus, Klebsiella, age 50 -Uncomplicated = limited to lower urinary tract, child > 2 years, no underlying enterococci -Risk factors: medical problems, no underlying anatomic or physiologic abnormalities -Uncomplicated = healthy young nonpregnant female MSM, -Complicated = upper tract disease, MDR pathogen, host with malignancy, -Complicated is anything else! uncircumcised, DM, or anatomic or physiologic abnormality, indwelling catheter low CD4 count -Risk factors: female, sexual activity, vesicoureteral reflux, polycystic kidneys, dysfunctional elimination syndrome, fecal impaction, paraplegia, sickle cell anemia, kidney transplant, DM, bladder stones, immunodeficiency, recent instrumentation Signs & -Lower UTI: dysuria with muscle spasm, frequency with small vol, urgency, -Elderly may have -Infants < 1 month: may only have fever symptoms suprapubic pain, ± hematuria AMS only -Older kids: dysuria, frequency, urgency, enuresis, abdominal or suprapubic -Upper UTI: lower symptoms + fever > 100.4, flank pain, CVA tenderness, pain, hematuria (fever, chills, flank pain suggest upper tract infection) nausea, vomiting -Elderly may have AMS only Differential -Urethritis with STI -Cervicitis -Urethritis -Chemical cystitis -Prostatitis or epididymo-orchitis -Vaginitis -FB -Prostatitis -Autoimmune cystitis -Nephrolithiasis -PID -HSV -HSV -Drugs -Urethral stricture -Bladder dysfunction -Neoplasm -Vulvovaginitis, cervicitis, or -Vaginal foreign body urethritis Workup -Uncomplicated  proceed to empiric treatment -UA and always -UA with culture (catheterized specimen for non-toilet trained children) -Complicated = UA with microscopy (look for pyuria, bacteriuria, varying culture -GC/chlamydia if sexually active hematuria), culture, wet prep -DRE -Renal bladder US indicated for first febrile UTI in kids under 2 who did not -Hematuria will not be present in cervicitis or urethritis but is common in UTI! have normal prenatal screening US, for kids of any age with recurrent UTIs, and kids of any age with UTI, poor growth, HTN, or FH of renal disease -VCUG indicated for evaluation of possible reflux in kids of any age with > 2 febrile UTIs Treatment -Uncomplicated: 3-5 days of nitrofurantoin (DOC) or FQ if more severe -7 days of Septra -Admit for infants < 2 months, immunocompromised, vomiting, inability to symptoms, Septra alternative if local E. coli resistance is not > 20% or FQ tolerate orals, lack of outpatient f/u, and failure of outpatient therapy -Complicated: home treatment with 7-14 days of FQ or Septra OK as long as -Ages 2-13 years  2nd or 3rd generation cephalosporin, add amoxicillin if there is no n/v, otherwise send to ED for inpatient treatment suspecting enterococcal infection -Preventive cranberry juice acidifies urine and prevents pathogens from -Age > 13  Septra or cephalosporin binding to urinary epithelia -In general, treat for 3-5 days if afebrile, 10 days if febrile -Short-term phenazopyridine for dysuria -First episode in uncomplicated female should be treated 5-7 days -Consider prophylaxis for women with > 2 UTIs in last 6 mo or > 3 UTIs in -Young children, male adolescents, and children with recurrent, febrile, or last year: clean UA followed by 6 months of Septra, nitrofurantoin, cefaclor, complicated cystitis should be treated for 7-14 days cephalexin, or FQ -Postmenopausal women may benefit from vaginal estrogen cream

143

Pyelonephritis -Agent is usually uropathic strain of E. coli Workup -UA with microscopy & culture Signs & symptoms -Blood cultures if hospitalized -Same manifestations as lower UTI + fever > 100.4, flank pain, CVA tenderness, n/v, rigors -Sepsis Management -Multi-organ dysfunction -If mild with no n/v  outpatient treatment with cipro -ARF -Mod-severe  inpatient treatment with initial empiric IV ceftriaxone, cipro, or imipenem, f/u -Alternative presentation of weeks to months of insidious, nonspecific symptoms such as malaise, culture 2 weeks after therapy fatigue, nausea, or abdominal pain Epididymitis Etiologies Differential: scrotal tumor (would be painless), testicular Management -Men under 40  STI torsion, epididymis or appendage torsion -Bed rest with scrotal elevation -Men over 40  seeding UTI or prostatitis, gram negs -Treatment of pathogen: 10-21 days of abx if Workup STI, 21-28 days of abx if not STI Signs & symptoms -CBC -Fever -GC/chlamydia, followed by urethral swab with gram stain if -Irritative voiding symptoms negative -Painful enlargement of epididymis with scrotal swelling -UA will show pyuria, bacteriuria, and varying hematuria if agent -Urethritis is not an STI -May have tender prostate -Prehn sign: relief with elevation of scrotum above pubic symphysis, not reliable Orchitis -Usually viral: mumps, rubella, coxsackie, echovirus, Differential Management parvovirus -Epididymitis -NSAIDs -May be STI if sexually active -Testicular torsion: absent cremasteric reflex -ABs if suspecting STI cause -Appendix testis or appendix epididymis torsion -Scrotal support Signs & symptoms -Trauma -Ice packs -Scrotal swelling -Incarcerated inguinal hernia -Pain and tenderness with erythema and shininess of the overlying scrotal skin -May also have epididymis involvement with STI orchitis Urethritis Differential -With discharge  think gonorrhea or chlamydia first, others include Mycoplasma, Ureaplasma, -Urethral carcinoma Trichomonas -Men: balanitis -Reactive arthritis with associated urethritis -Women: candidiasis, cystitis

144

Prostatitis Acute Bacterial Prostatitis Chronic Bacterial Prostatitis Chronic Nonbacterial Prostatitis and Chronic Pelvic Pain Syndrome -Least common form of prostatitis Differential: acute Etiologies -Urinary or genital pain Differential: infection, GU -Can be life-threatening pyelonephritis, acute -May evolve from untreated acute bacterial prostatitis with no evidence of cancer, urinary tract disease, epididymitis, acute infection, with symptoms urethral stricture, neurologic -Organisms: mostly gram negs, also diverticulitis, BPH, Signs & symptoms for 3 of the last 6 months disease, emotional disorder gonorrhea or chlamydia in sexually malignancy -Wax and wane -Affects all ages of men active younger men -Irritative voiding symptoms -The most common form of Workup Workup -Dull suprapubic or perineal discomfort prostatitis -May have WBCs in prostatic Etiologies -UA with culture: should -Recurrent UTIs with same organism and no explanation secretions -STI be + -DRE may appear normal Etiologies -Urine reflux seeding -Urethral culture if -Not well understood Management -Spread from distant source or adjacent discharge present Workup -Nanobacteria? -Refer for cystoscopy if at risk infection -GC/chlamydia -UA using Meares-Stamey 4 glass method: 1st 10 mL is -Voiding dysfunction for bladder cancer from the urethra, midstream void from the bladder, -Pelvic floor myalgia -NSAIDs for pain control Signs & sympoms Management prostatic massage for 3rd sample (this should be +), next 10 -Alpha blockers for urinary -Frequency, urgency, dysuria, nocturia, -Send to ED for IV abx mL will contain urethral and prostatic fluid Signs & symptoms symptoms change in urine stream -Wax and wane -Muscle relaxants for painful -Low back pain Management ejaculations -Genital pain -Prolonged course of FQ or Septra -Finasteride to shrink prostate -Abdominal pain -NSAIDs -Sitz baths -Fever and chills -Sitz baths -Pelvic floor PT -Nausea/vomiting -Consider suppressive abx treatment if 3+ recurrences per -Hypotension year -Tender prostate on DRE, may be enlarged or fluctuant, be careful to not cause bacteremia

GENITOURINARY NEOPLASMS Bladder Neoplasms -More common in men than women Workup Management -Risk factors: smoking, exposure to dyes and solvents -Cystoscopy is initial test of choice -Neoadjuvant chemo -Repeat urine cytologies (low sensitivity) -If superficial, resection (usually total cystectomy with urinary Signs & symptoms -CT to assess local extent of disease diversion) ± intravesicular chemo -Painless hematuria -Staging based on biopsy results and imaging -If advanced, combo chemo ± radiation -Urinary frequency or urgency -May be asymptomatic Prognosis -Early disease has > 80% survival Benign Bladder Neoplasms Malignant Bladder Neoplasms Low-Grade Intraurothelial Neoplasia Carcinoma In Situ Adenocarcinoma Metastatic Disease -Aggressive -Commonly from the colon or rectum, Urothelial Papilloma Squamous Cell Carcinoma prostate, or cervix -Can have malignant potential -More common in areas of the world with Small Cell Carcinoma schistosomal infections -Neuroendocrine in origin Invasive Urothelial Cell Carcinoma Inverted Papilloma -Aggressive -Aggressive clinical course with poor -AKA transitional cell carcinoma -Can have malignant potential prognosis -Most common form of bladder cancer in US

145

Prostate Cancer -Usually adenocarcinoma Signs & symptoms Management -The most commonly diagnosed male cancer and 2nd leading -Asymptomatic early in disease -Treatment based on life expectancy, general health, tumor cause of male cancer deaths -Later disease: obstructive urinary symptoms, hematuria, characteristics -Risk factors: age, black, high fat diet, FH, obesity hematospermia -Treatment is controversial for localize disease -No association with smoking, sexual activity, prior infections, -Bone pain with mets -Radical prostatectomy or BPH -Radiation Workup -Hormone therapy for advanced or metastatic disease Screening -Prostate biopsy guided by transurethral US, with scoring by -USPSTF grade I for men up to age 75 and grade D after 75 Gleason system -If patient elects, DRE and PSA should be done every 2 years -MRI -PSA will be elevated in cancer, inflammation, or BPH, and will -PET if suspected mets naturally rise as men age -CXR, LFTs for mets Renal Neoplasms -“Small renal mass” is often detected incidentally and defined as Differential Management a contrast-enhancing mass < 4 cm; most are renal cell -Polycystic kidney disease -Active surveillance if < 1 cm carcinomas Workup Prognosis Signs & Symptoms -Imaging can’t reliably differentiate a benign tumor from RCC -Neither tumor size at diagnosis nor growth rate are accurate -Most are asymptomatic -Dedicated renal CT or MRI for incidental lesions predictor of malignancy status -Hematuria -Surgical resection for masses 1-4 cm -Paraneoplastic syndrome -Percutaneous biopsy for low malignancy suspicion or for -Abdominal or flank mass nonsurgical candidates -Abdominal pain Benign Renal Neoplasms Malignant Renal Neoplasms Simple Renal Cyst Angiomyolipoma Renal Cell Carcinoma Prognosis -Accounts for 80% of renal cancers -Good for cancers confined to renal capsule Renal Oncocytoma Metanephric Adenoma -More common in men than women -50-60% for tumors extending beyond capsule -Risk factors: smoking, obesity, HTN, polycystic kidney disease, occupational exposures, -0-15% for node positive tumors Cystic Nephroma Renal Medullary Fibroma prolonged NSAID use, chronic hep C, sickle cell disease -Signs & symptoms: hematuria, flank pain or abdominal mass, cough, bone pain with mets, paraneoplastic syndromes -Nephrectomy needed Testicular Cancer -Risk factors: cryptorchidism, abnormalities in spermatogenesis, FH Investigation -Chest, abdomen, and pelvis CT -Most commonly germ cell tumor, but cana lso be stromal tumor -Scrotal US: distinguishes benign vs malignant and intra vs extratesticular Management Screening -Excisional biopsy -Inguinal orchiectomy with f/u of tumor -USPSTF grade D in asymptomatic adolescents and adult males -β-hCG levels: will be elevated in some carcinomas and markers seminomas -May need chemo Signs & symptoms -AFP: elevation excludes diagnosis of seminoma -Firm, painless mass arising from the testis Prognosis -Scrotal pain -High survival rate if caught early -Affected area is usually unilateral -Signs of mets: cough, GI, back pain, neuro signs, supraclavicular lymphadenopathy

146

Wilms Tumor -A renal cancer that is the 4th most common Signs & symptoms Management Prognosis childhood cancer -Abdominal mass or swelling -Refer to surgery and pediatric cancer center -Good with early disease -Most diagnosed before age 10 -Abdominal pain -Abdominal US or contrasted CT to -Lung is most frequent first site of recurrence -Hematuria differentiate from other masses -HTN

RENAL DISEASES Chronic Kidney Disease -Defined as GFR < 60 for at least 3 months or presence of kidney Signs & symptoms Management damage irrespective of GFR -Salt and water imbalance  fluid accumulation, -BP control using ACEIs -Blacks have 3.8x greater risk, native Americans have 2x greater HTN, peripheral edema, hypo or hypernatremia, -Smoking cessation risk, and Latinos have 1.5x greater risk neuro effects -Lipid management -Cardiac conduction errors due to potassium -Tight control of A1c in diabetics Stages imbalance -Avoid nephrotoxins: contrast, NSAIDs -Stage I = GFR > 90 with kidney damage but asymptomatic -Imbalance of Ca and P affects bone metabolism and -Monitor for anemia and treat with folate, B12, or Fe if needed, then -Stage II = GFR 60-89 and kidney damage but asymptomatic cell membrane activity  loss of bone Ca with consider EPO -Stage III = GFR 30-59, mild anemia, ↑ BUN and Cr, but metastatic deposition, osteoporosis, fx -Check GFR 1-2 times per year asymptomatic -Acid/base imbalance affects functioning of cells and -Every 3 months monitor PTH, P, Ca, bicarb, and vitamin D if stage 3 -Stage IV = GFR 15-29, symptoms of fatigue, electrolyte enzymes or above imbalance, acidosis, anemia -Buildup of uremic toxins  nausea, anorexia, -Diet: good Ca intake, minimize P, low salt, fluid restriction to 2 L -Stage 5 = GFR < 15 or dialysis-dependent abnormal metallic taste in mouth, insomnia, seizures, -Phosphate binders if needed: Ca acetate, sevelamer, lanthanum coma, bleeding, immune dysfunction, arrhythmias, -Treat hyperkalemia: usually avoided as long as GFR > 10, prevent by Screening accelerated atherosclerosis, cardiomyopathy, pruritus avoiding K-sparing diuretics and cautious use of ACEI in ESRD -Regularly screen those with risk factors for DM, glomerular -No erythropoietin  anemia -Treat hypokalemia diseases, vascular diseases, cystic diseases, transplant -No activation of vit D  hyperparathyroidism, renal -Treat vol overload: thiazides for stages 1-2, loop for stages 4-5 (greater complications, FH of severe kidney disease, CV disease, etc, as osteodystrophy, fx diuresis) early diagnosis can add 2+ ESRD-free years to a patient’s lifespan -Heart disease: CAD lesions, CHF, acute MI (most -Daily baby aspirin -Use spot urine:creatinine test in diabetics patients will die of a cardiac-related cause before -Refer to nephrologist when GFR < 30 -Use spot test or urine dipstick in all other populations ESRD develops) Hydronephrosis -Distension and dilation of the renal pelvis and calyces Signs & symptoms Workup Prognosis -Obstruction can occur anywhere along the urinary tract -Depends on site of obstruction, degree, and rapidity of development -US is imaging of choice, -Return of renal function -Pain will show dilation of depends on severity and duration Etiologies -Change in urine output collecting system of obstruction -BPH -Hematuria -Prostate cancer -Increasing serum creatinine Management Sequelae -Nephrolithiasis -HTN -Relief of obstruction -Risk of UTI, urosepsis or ESRD -Structural abnormalities -Distended abdomen or abdominal mass -Bladder catheterization if untreated -Nephrostomy tube Polycystic Kidney Disease -Common after age 50 Signs & symptoms Workup -Usually asymptomatic -UA for hematuria or proteinuria Etiologies -If inherited form, may have abdominal or flank pain, -Renal US: diagnosis depends on age and # of cysts present -Genetic form characterized by multiple cysts in both kidneys, hematuria, history of UTIs, history of stones, HTN, abdominal with increased risk of UTIs, renal cell carcinoma, ESRD mass Management -Usually requires kidney transplant

147

Acute Kidney Injury (Acute Renal Failure) • Azotemia: abnormally high levels of nitrogen- Signs & symptoms Workup containing compounds, such as urea, creatinine, -Symptoms of uremia: DOE, pericarditis, fatigue, loss of appetite, headache, nausea, -Definition of AKI: acute ↓ in renal function with various body waste compounds, and other vomiting, nocturia, AMS, SOB, pruritus, easy bruising, asterixis Cr ↑ of 0.5 (or 50%) above baseline, or CrCl ↓ by nitrogen-rich compounds in the blood  can -Flank pain from stretching of fibrous capsule surrounding kidney during blockage 50% result from many disorders including renal failure -Irregular heartbeat from hyperkalemia -Assess degree of renal dysfunction: estimate • Acute renal failure: an abrupt decrease in GFR -Dehydration GFR sufficient to result in azotemia and perturbation of -Decreased UOP  vol overload, peripheral edema, pulmonary edema, pulmonary rales, -UA ECF volume, electrolyte, and acid-base balance = elevated JVP, cardiac tamponade, pulsus paradoxus (drop in BP during inspiration) -Labs: urine Na, urine Cr, compare with serum kidney is not removing proteins that should -Seizures Na, Cr, and BUN normally be removed from the blood -Kussmaul respirations from acidosis -Renal US to rule out postrenal causes Prerenal AKI -A result of interrupted blood flow to the kidneys  ischemia of the proximal tubular cells Signs & symptoms -Decreased renal perfusion means renin is released  maximal retention of Na and water in an -Hypotension effort to ↑ circulating vol -Decreased UOP with concentrated urine

Etiologies Management -Decreased effective circulating vol: HF, cirrhosis, nephrotic syndrome, excessive diuretic use, -IV NS vomiting, NGT, diarrhea, burns, DKA, hypercalcemia, Addison’s, shock, pancreatitis, 3rd spacing -Treat underlying illness -Decreased renal perfusion: severe hypoalbuminemia, sepsis, psychotropic drug overdose, -Stop antihypertensives or diuretics excessive antihypertensives, NSAIDs, renal artery stenosis or renal vein thrombosis -Octreotide in patients with cirrhosis (helps increase blood flow to kidneys when sick liver is -Cardiac arrest shutting it down) -Low BP: anorexia, GIB, cardiac surgery Intrinsic Renal AKI Acute Tubular Necrosis Acute Interstitial Nephritis Glomerulonephritis -The most common cause of AKI where there is abrupt and sustained -Results in abrupt deterioration in renal Focal disease decline in GFR occurring within minutes to days in response to an acute function with inflammation and edema of the -IgA nephropathy ischemic or nephrotoxic insult renal interstitium -Membranoproliferative glomerulonephropathy -SLE Signs & symptoms Etiologies -Volume overload -Allergic: cephalosporins, diuretics, NSAIDs, Diffuse disease -Oliguria or anuria penicillin, rifampin, sulfa -Goodpasture’s disease -Infectious: hantavirus, HIV, Legionnaire’s, -Churg-Strauss Workup leptospirosis, pyelonephritis -Cryoglobulinemia -Rise in Cr and BUN in = proportions -Autoimmune: cryoglobulinemia, Sjogren’s, -Membranoproliferative glomerulonephropathy -Check urine sodium (should be > 40) to distinguish from volume SLE -SLE depletion (Na < 20) -Infiltrative: amyloidosis, multiple myeloma, -Vasculitis -FENa increased at > 2-3% as either excess sodium is lost due to tubular sarcoidosis -Wegener’s damage, or the damaged glomeruli result in hypervolemia resulting in the -Microscopic polyangiitis normal response of sodium wasting Workup -Postinfectious glomerulonephritis: usually Strep but can also be TB, -Will see metabolic acidosis with hyperkalemia -UA showing proteinuria, pyuria, hematuria, HIV, hepatitis, MRSA, or meningococcal infection-induced, with -UA shows multiple granular and epithelial cell casts with free epithelial renal tubular epithelial cells or casts, onset 1-3 weeks after infection cells eosinophiluria -Increased BUN and Cr, hyper or hypokalemia Workup Management -Hyperchloremic metabolic acidosis -UA showing red cell casts -Typically conservative -Elevated LFTs -Consider dialysis if severe

148

Post-Renal AKI -A result of obstruction of urine flow

Etiologies -Enlarged prostate -Kidney stones -Bladder tumor or injury -Obstructed urinary catheter -GYN cancers -Retroperitoneal fibrosis -Neurogenic bladder or spinal cord injury -Retention secondary to meds: anticholinergics, opioids, amphetamines Working Up Cause of AKI Intrinsic Renal Disease Prerenal Azotemia Postrenal Azotemia Acute Tubular Necrosis Acute Interstitial Nephritis Acute Glomerulonephritis -BUN:Cr ratio > 20:1 -BUN:Cr ratio > 20:1 -BUN:Cr ratio < 10:1 because there is renal damage -BUN:Cr ratio > 20:1 -Rise in BUN out of proportion to rise in Cr because back-up into the causing reduced reabsorption of BUN (may not be reliable) Serum BUN:Cr Ratio because decreased flow to kidney means kidney allows for increased back-up of urea and Cr in the blood, and urea reabsorption while urea is reabsorbed into the blood after entering the kidney (and has greater time to do so due to the reduced flow) Cr can’t be and is excreted Urinary Indices -UA will be normal or near-normal

Urine Na < 20 Variable > 20 Variable < 20 FENa -FENa will be < 1% (salt conserving, Variable, usually normal if > 1% if oliguric < 1% -Variable indicating a functioning kidney with normal injury is acute and there is physiologic response to volume depletion) still tubular functioning Urine Osmolality > 500 < 400 250-300 Variable Variable Urinary Sediment -Benign or hyaline casts -Usually normal -Muddy br -White cells, white cell -Red cells, dysmorphic red -May see RBCs, WBCs, or own casts, renal tubular casts, ± eosinophils cells and red cell casts crystals casts Nephrotic Syndrome -Occurs when kidney damage causing pores in the podocytes allows large amounts of protein Signs & symptoms Workup leakage, but not large enough for RBCs to pass -Generalized edema: periorbital, pitting edema of -Proteinuria ≥ 3.5 g/day the legs, pleural effusions, ascites -Hypoalbuminemia Etiologies -Anemia due to transferrin loss -Hyperlipidemia -DM -Foamy urine -Granular or fatty urine casts with oval fat -Focal glomerulosclerosis bodies and Maltese cross crystals -Membranous nephropathy -Must get biopsy -Amyloidosis -Minimal change disease -SLE -HIV, hep B or C

149

Renal Vascular Disease -S/s: -Testing indicated in patients with clinical evidence of secondary hypertension without a cause such as primary kidney disease, primary aldosteronism, or pheochromocytoma -Intervention is planned if a significant stenotic lesion is found Hypertensive Nephrosclerosis Renal Artery Stenosis -Renal disorder associated with chronic HTN  presence of vascular, -Narrowed renal arteries renal hypoperfusion  activation of RAAS  adequate renal perfusion but peripheral HTN glomerular, and tubulointerstitial lesions -Disease is usually unilateral and may be due to atherosclerosis or fibromuscular dysplasia -Risk factors: black, underlying CKD with slow deterioration of renal -S/s: refractory HTN, rapid deterioration of renal function function, severe HTN -W/u: only test pts with high suspicion as procedures can be invasive (begin with US but may need arteriography) -S/s: CKD, h/o HTN, LVH, proteinuria, hyperuricemia -Tx: ACEI or ARB (risk of long-term ischemic damage to stenotic kidney), revascularization procedures, considered to be a CAD equivalent = treat CV risk

FLUID AND ELECTROLYTE DISORDERS Hypermagnesemia -Normally 1.5-2.5 Signs & Symptoms Management -CNS depression -Similar to hyperkalemia treatment Causes -↓ DTRs -IV calcium gluconate -TPN -Respiratory failure -Insulin + glucose -Renal failure -Furosemide -Iatrogenic oversupplementation -Dialysis Hypomagnesemia Causes Signs & Symptoms Management -TPN -Asterixis, tremor, Chovstek’s sign -Fix any hypokalemia first and Mg may correct itself! -Hypocalcemia -Ventricular ectopy and other dysrhythmias -IV MgSO4 -Gastric suctioning -Vertigo -PO Mg oxide if chronic -Aminoglycoside abx -↑ DTRs -Renal failure -Tachycardia -Diarrhea -Vomiting Hypophosphatemia -Normally 2.5-4.5 Signs & Symptoms Management -Weakness -Supplement with IV K3PO4 or Na3PO4 Causes -Cardiomyopathy -GI losses -Neurologic dysfunction -Meds -Rhabdomyolysis -Sepsis -Hemolysis -EtOH abuse -Poor pressor response -Renal loss -Respiratory failure if severe

Hyperphosphatemia Causes Signs & Symptoms Management -Renal failure -Ectopic calcification -AlOH3 to bind phosphate -Sepsis -Heart block -Chemotherapy -Hyperthyroidism

150

Hyponatremia Management of hyponatremia Signs & symptoms -Treat to magic number 125 -Seizures, coma, lethargy -Don’t exceed replacement of sodium by more than 12 mEq/L due to risk of causing a -Nausea/vomiting demyelination syndrome  confusion, spastic quadriplegia, horizontal gaze paralysis -Weakness -Definitive treatment is based on underlying cause of impaired renal water excretion -Ileus

Isotonic Hyponatremia Hypertonic Hyponatremia Hypotonic Hyponatremia Low serum Na Low serum Na Occurs when ↑ ADH  water and Na loss in urine  low serum osmolality Normal ECF osmolality (total High ECF osmolality and tonicity True hyponatremia! solutes) and tonicity (no oncotic Further characterized based on volume status pressure generated) -A kind of pseudohyponatremia -A kind of pseudohyponatremia Hypervolemic hypotonic Euvolemic hypotonic hyponatremia Hypovolemic hypotonic -Probably due to high levels of TG -Caused by a highly osmotic hyponatremia hyponatremia or proteins (multiple myeloma) that molecule with glucose or mannitol push Na intracellularly to prevent in the ECF, which draws water out -3rd spacing of fluids  reduced -Due to excessive ADH release -Occurs when water loss causes salt increased serum osmolality of cells and dilutes the Na circulating vol (even though TBW -Occurs in SIADH (sodium is always loss -No treatment needed concentration will be hypervolemic)  activation down here) & paraneoplastic -An extrarenal cause when kidneys -Treat with NS until of ADH  serum that is hypotonic syndromes, postoperative are attempting to resuscitate volume hemodynamically stable, then ½ NS because too much Na is retained in hyponatremia, hypothyroidism, by saving Na and water, such as the urine psychogenic polydipsia, excess beer dehydration, diarrhea, vomiting, -Occurs in CHF, liver failure, drinking burns, NGT suctioning, diaphoresis, nephrotic syndrome, ESRD, and -Management is water restriction with or pancreatitis iatrogenic fluid overload (the most hypertonic NaCl infusion -A renal cause when kidneys allow common cause of mild postop high Na losses despite ↓ circulating hyponatremia) vol, such as diuretics, ACEIs, -Management is water and Na nephropathies, or mineralocorticoid restriction, diuretics, treat underlying deficiencies cause of 3rd spacing -Manage with NS IV and correction of underlying cause

Hypernatremia Signs & symptoms -Seizures -Lethargy -Coma -Weakness from brain cell shrinkage -Focal intracerebral and subarachnoid hemorrhages -Irritability -Peripheral edema -Twitching Hypovolemic Hypernatremia Euvolemic Hypernatremia Hypervolemic Hypernatremia Chronic Hypernatremia -Non-renal causes: excess water loss from skin -Non-renal causes: excessive sweating from -Non-renal causes: treatment of previous -Must correct especially slowly to prevent or diarrhea or dehydration  concentrated skin or respiratory system water loss hypotonic fluid loss with higher sodium fluids, cerebral edema urine with low Na -Renal cause: due to diabetes insipidus sea water ingestion, or overuse of NaHCO3 in -Calculate water deficiency and accomplish -Renal causes: osmotic diuresis due to (inadequate ADH or kidney nonresponse to CPR correction over 36-72 hours (normal TBW = mannitol, glycosuria, or diuretics  salt as ADH) -Management is to give D5W to reduce present TBW x (present serum Na/140) well as free water loss  high urine Na with -Management is to increase PO water or use IV hyperosmolality, may need dialysis if pt has normal urine osmolarity D5W renal failure

151

Hypokalemia Etiologies Signs and symptoms Management -Decreased K+ intake -Weakness -Mild hypokalemia can be treated -Increased K+ entry into cells: alkaline pH, insulin, stress, epinephrine, β-agonists, ↑ RBC -Paresthesia orally with KCl supplements production, hypothermia, chloroquine toxicity -Tetany -KCl IV supplementation if severe -Increase GI losses: vomiting, diarrhea, NGT drainage, laxative abuse, intestinal fistula -Nausea/vomiting (prefer central line as K is corrosive to -Increased urinary losses: diuretics, mineralocorticoid excess, loss of gastric secretions, non- -Ileus (very commonly seen in surgical pts) vasculature) reabsorbable anions, alkalosis (causes intracellular K+ shift and increased urinary excretion of -Exacerbation of digoxin toxicity -K sparing diuretic if resistant HCO3- coupled to K+), hypomagnesemia, amphotericin B, salt-wasting nephropathies, polyuria Workup -Increased sweat losses -EKG may show U wave, flattening of T waves, PACs, -Dialysis PVCs, afib -Plasmapheresis -Blood tube metabolism of K+ -Low Mg (inhibits K+ reabsorption from renal tubules)

Hyperkalemia -Normal K+ 3.5-5.0; critical value if > 6.5 Signs & symptoms Management -Malaise -Depends on EKG findings; if there is a change treat it Etiologies -Palpitations & arrhythmias -Ca gluconate or Ca chloride IV to stabilize the myocardium -Renal insufficiency -Muscle weakness (give first!) -Meds: ACEI, ARBS, K-sparing diuretics, NSAIDS -↓DTRs -Bicarb IV to cause alkalosis  intracellular K+ shift -Mineralocorticoid deficiency -Paresthesias -Insulin with glucose to increase K+ cellular uptake -Excessive release from cells: rhabdo, burns, tumor lysis, blood -Respiratory failure -K+ binder (Kayexalate) transfusion, hemolysis, acidosis, low insulin, β-blockers -Furosemide to renally excrete K+ -Excess intake: salt substitute, KCl infusion Workup -Dialysis if severe -Lysis from cells after blood draw (“pseudohyperkalemia”) -EKG showing peaked T waves, can go into vfib if severe Hypocalcemia Etiologies Signs & symptoms Workup -If PTH is low  hypoparathyroidism -Early: perioral and extremity paresthesias (areas -Check Mg level, phosphate, vitamin D -If PTH is high  vit D deficiency, chronic renal failure, with high innervation affected most quickly) -Need to correct Ca in face of low albumin or check ionized Ca inadequate production of PTH or resistance, CKD, -Late: ↓ DTRs, AMS, hallucinations, psychosis, hepatic disease, osteoblastic mets, hypomagnesemia, Chvostek’s and Trousseau’s signs (but don’t do BP Management large blood transfusion, acute pancreatitis, severe sepsis cuff in real life b/c it is extremely painful), -If severe or symptomatic, give Ca gluconate or CaCl3 (but beware tissue necrosis or illness, meds, pseudohypocalcemia (gadolinium laryngospasm, bronchospasm, prolonged QT, d/t Ca if IV infiltrates!), treat low Mg if present interference with assay) hypotension, HF, arrhythmia, papilledema -If asymptomatic, give oral Ca -Surgical: short bowel syndrome -If due to vit D deficiency, give calcitriol (active vit D) -Can add Ca to dialysis fluid if on HD

152

Hypercalcemia Etiologies -Increased bone resorption: malignancy/paraneoplastic effects, hyperparathyroidism, CKD, thyrotoxicosis, immobilization, Paget’s disease of bone -Increased Ca absorption: increased intake, lots of milk & Tums, elevated vit D, genetic, meds, pheochromocytoma, adrenal insufficiency, rhabdo, ARF

Signs & symptoms -“Stones, bones, abdominal groans, and psychiatric overtones” -Anxiety, depression, lethargy, confusion, psychosis, coma -Constipation, anorexia, pancreatitis, PUD, abdominal pain, n/v -EKG changes: prolonged PR and QRS, AV block, cardiac arrest -Volume depletion, renal insufficiency, nephrolithiasis, nephrogenic diabetes insipidus, distal RTA  polydipsia and polyuria -Muscle weakness, fatigue, bone pain, fractures

Management -Treat if symptomatic or serum Ca is > 14 -IVF as pts tend to be volume depleted, followed by diuresis with furosemide -Calcitonin if severe -Bisphosphonates -Steroids -Dialysis is last resort

Hypomagnesemia Etiologies Signs & symptoms Management -GI loss: diarrhea, short gut syndrome, TPN -Secondary electrolyte disturbances like hypoK, hypoCa, vit D -Oral replacement with MgO2 -Alcoholics deficiency -Renal loss: diuretics, EtOH, nephrotoxic drugs, primary aldosteronism, post ATN -Weakness, anorexia, tetany, convulsions -Pancreatitis -Widened QRS, peaked or inverted T waves, QT or PR prolongation, -DM VT, torsades -Hypercalcemia or hypophosphatemia -Hungry bone syndrome

ACID/BASE DISORDERS Respiratory Acidosis -Primary problem is that there is ↑ CO2 due to hypoventilation -Kidneys respond by secreting H+, generating new bicarb and other H+ buffers, and excreting NH4+  acidic urine, ↑serum bicarb Respiratory Alkalosis -Primary problem is that there is ↓ CO2 due to hyperventilation (lungs will override -May also have hyponatremia and hypochloremia chemoreceptors in other parts of the body) -Kidneys compensate by increasing bicarb excretion in urine and decreased NH4+ excretion in -Hypocapnea causes intracellar shift of K and PO4  ↓K, ↓PO4 urine  alkaline urine, ↓serum bicarb -Alkaline pH causes more binding of Ca to albumin  ↓Ca -Seen in early asthma exacerbation, PE 153

Metabolic Alkalosis -Primary problem is that there is too much bicarb (no H+ left to react with) -Body will eventually compensate by hypoventilation to ↑ CO2, and kidneys can compensate by increasing bicarb excretion Chloride-Responsive (urine Cl < 10 mEq/L) Chloride-Resistant (urine Cl > 20 mEq/L) Vomiting Hyperaldosteronism -Occurs when vomiting/NGT  loss of HCl and vol depletion -Occurs when adrenals make too much aldosterone  ↑↑ Na and bicarb reabsorption with urinary -Vol depletion stimulates renin & aldosterone  ↑ Na reabsorption with loss of K+ and H+ loss of K+/H+ (countertransporter pumps) (countertransporters)  low urine Na, serum hypokalemia, and alkalosis Other etiologies Loop or thiazide diuretics -Bicarb retention -Cause loss of Cl- with retention of bicarb -Hypokalemia  H+ shift into cells -Excess administration of antacids -Weird syndromes Metabolic Acidosis -Initially kidney ramps up production of Winter’s formula: Expected PCO2 = (1.5 x bicarb) + 8 ± 2 Urine anion gap bicarb but then the primary problem is -If pt’s PCO2 corresponds, they are compensating adequately via respiration -Only calculated in NAGMA 2- 3- that bicarb is used up or there is not changes -Main anion is Cl-, unmeasured anions are bicarb, PO4 , SO4 , enough to offset the increased acid -If measured PCO2 is higher than expected, there is also a primary respiratory lactate -There is respiratory compensation by acidosis -Main cations are Na+ and K+, unmeasured cations are Li, Ca, Mg, talking rapid, deep breaths (Kussmaul -If the measured PCO2 is lower than expected, there is also a primary respiratory and NH4+ respirations); check to see if the pt is alkalosis -Normally urine is electrically neutral (UAG = 0) or slightly adequately compensating using Winter’s positive/acidic formula Serum anion gap = diff bet + and – ions = Na+ – (Cl- + bicarb) UCl- + UA = UNa+ + UK+ + + UC -Normally 8-12 (UA –UC) = UNa+ + UK - UCl- Etiologies -Used to estimate NH4+ levels if you don’t have a direct test -Most common cause is decreased tissue Types of Metabolic Acidosis -Decreased UAG (abnormally negative) when there is loss of bicarb perfusion/ischemia (exercise, sepsis, 1.) Normal anion gap (hyperchloremic anion gap) via the bowel and kidneys respond by increasing excretion of NH4+ shock, seizures, neuroleptic malignant -Occurs when Cl- replaces lost bicarb as the H+ buffer (= high amounts of an unmeasured anion), or after eating a protein- syndrome)  production of lactic acid -Causes (HARDUP): hyperalimentation or hyperventilation, acetazolamide, rich meal from anaerobic metabolism RTA (loss of bicarb from kidneys), diarrhea, ureterosigmoidostomy (loss of -Increased UAG (higher than normal positive) when there is a renal -Ketone bodies bicarb through colon), pancreatic fistula (loss of bicarb through colon) cause blocking urinary acid excretion such as RTA because kidney is -Decreased renal excretion of H+ 2.) Increased anion gap not getting rid of enough NH4+ -Bicarb loss from kidney -Occurs when anion replacing bicarb is not one that is routinely measured, such 2- 3- -Diarrhea  loss of bicarb as albumin, PO4 , SO4 , lactate Management -Causes (MUDPILES): methanol, uremia, DKA, paraldehyde, iron -Give NaHCO3- ingestion/INH, lactic acidosis, ethanol (makes acetic acid), salicylates -Intubate for respiratory distress/can’t keep up with breathing quickly

OTHER GENITOURINARY TOPICS Enuresis -Not clinically significant until child is > 5 years of age Differential Management -Contributing factors: nocturnal polyuria, detrusor -Kidney disease -High rate of spontaneous resolution by 15 years of age overactivity, disturbed sleep, maturational delay, -Daytime incontinence -Behavioral changes: regular voiding and emptying bladder before bedtime, no fluids after 6pm genetics, abnormal ADH secretion -Constipation -Rewards for voiding before bedtime, working up to rewards for staying dry overnight -Pinworms -More active interventions needed as child gets older, social pressures increase, and self-esteem is affected Workup -Spinal dysraphism or -Enuresis alarms for wetting > twice per week -Voiding diary abnormality -Desmopressin for children with nocturnal polyuria and normal bladder capacity who have failed alarm -UA -Urologic anatomic abnormality trials 154

Male Circumcision -Currently promoted as the health benefits outweigh the risks: reduced UTIs, reduced STI -Not covered by Medicaid and typically costs $200 out of pocket transmission, reduced penile inflammatory and retractile disorders, easier hygiene -Uncircumcised infants will need parent education on how to care for and clean the penis -Procedural risks are rare regularly to prevent phimosis

NEUROLOGIC SYSTEM DISEASES OF PERIPHERAL NERVES Bell’s Palsy Etiologies Workup Management -Usually idiopathic -Must differentiate peripheral cause from central -Controversial! -Virus (stroke)  central cause will result in partial sparing -Prednisone taper -Lyme of the frontalis muscle because there is bicortical -Artificial tears -Sarcoid input from the brain, so half the input is still -Acyclovir -HIV functioning -Tumors -If uncertain refer for head CT to r/o stroke or TIA Prognosis -HSV reactivation -Lyme titer -60% recover completely -EMG/NCS will indicate severity but won’t guide -10% have permanent Signs & symptoms treatment dysfunction -Abrupt onset that may progress over days -Motor deficits: facial nerve paralysis, ptosis -Sensory deficits: ear pain, taste differences, hyperacusis Complex Regional Pain Syndrome -Pathophysiology not well understood Prevention Differential Workup -Early mobilization following stroke or fracture -Spinal nerve root impingement -Diagnosis is clinical Reflex sympathetic dystrophy = old term for -Supplemental vit C for wrist fractures -Pancoast syndrome -Radionuclide bone scan shows ↑ uptake CRPS where there is no definable nerve lesion, -Thoracic outlet syndrome -MRI now called CRPS type II Signs & symptoms -Vasculitis -Sx will show good response to sympathetic -Severe burning or throbbing pain with low -RA nerve block Causalgia = named peripheral nerve injury is threshold or normal stimuli -Peripheral neuropathy present, now called CRPS -Swelling and limited ROM -Migratory osteolysis Management -Vasomotor instability  altered skin -Venous thrombosis -Stage 1 disease: topical capsaicin, TCA, Etiologies temperature, can be warm or cold -AV fistula calcitonin, NSAID daily; refractory pain  -Usually follows a minor or major injury such -Diaphoresis -Systemic sclerosis trigger point injections with local anesthetic as soft tissue injury or fracture -Skin changes, thickening -Diffuse atrophy and steroids -Post-surgical -Loss of muscle strength -Angioedema -Stage 2 disease: oral steroids -Post-stroke or MI -Patchy bone demineralization -Stage 3 disease  pain center referral -May have no precipitating event -Urinary problems -Emotional stress can contribute

155

Peripheral Neuropathy Common signs & symptoms Workup Management -Weakness, incoordination, ataxia, muscle wasting, -Try to localize the lesion: unilateral extremity affected  brain or entire nerve plexus; -Acute focal neuro deficit  send to ED for numbness, tingling, loss of sensation, pain, ataxia, symmetric disease  bicortical, brainstem, cord lesion, or peripheral neuropathy; portion of neuro consult dizziness, loss of consciousness, exercise intolerance, limb or trunk affected  brain, spinal cord, plexus, or peripheral nerve; dermatome or -Subacute or chronic focal neuro deficit  difficulty digesting foods, constipation, urinary myotome affected  specific spinal cord segment refer for EMG/NCS (and neuro consult if at symptoms, sexual dysfunction, visual symptoms -EMG/NCS an unusual site) -Axonal disease: sensory symptoms > motor -B12, CBC, glucose tolerance, RPR, CMP, serum protein electrophoresis, TSH -Multifocal deficit  refer to neuro for symptoms, greater distal weakness, ↓ DTRs -Select patients: anti-Hu, ESR, ANA, RF, SS-A, SS-B, HMSN, HIV, Lyme, phytanic acid, 24 EMG/NCS -Demyelinating disease: motor symptoms > sensory hour urine for heavy metals, referral for CSF sample or nerve biopsy -Symmetric deficit  refer to neuro for symptoms, greater proximal weakness, ↓ DTRs EMG/NCS Single Peripheral Neuropathies Multiple Peripheral Mononeuropathies Peripheral Polyneuropathies Carpal tunnel: + Phalen’s, + Tinel’s -Late thenar atrophy Discogenic -Motor, sensory, and Hereditary -Charcot-Marie-Tooth disease types median nerve -Worse at night -Wrist splint neuropathies: autonomic dysfunction compression impingement of -MRI, CT myelogram spinal nerve by -EMG/NCS lateral disc -Rest, immobilization, protrusion or PT, surgical arthropathy decompression Ulnar neuropathy -Worsened by elbow flexion or wrist extension Plexopathies: -Risk: trauma, DM, Endocrine -Diabetic peripheral neuropathy and -Modify elbow or wrist activities cervical, brachial, radiation related Charcot arthropathy -Extensor splint at nighttime lumbar, sacral -Motor, sensory, and -Uremic peripheral neuropathy autonomic dysfunction -Alcohol and nutrition deficiency -EMG/NCS peripheral neuropathy -Infectious peripheral neuropathy -HIV-related Radial neuropathy -Axilla crutch injury -Motor deficits > sensory Mononeuritis Inflammatory -Saturday night palsy -Splints, PT, OT multiplex -Humeral fx Meralgia -Risk: obesity, tight -Pain and numbness on Toxic -From exposure to neurotoxins like paresthetica: clothes, pregnancy, lumbar outer thigh pesticides, heavy metals, mds compression of lordosis, DM lateral femoral -No motor symptoms cutaneous nerve Femoral neuropathy -Risk: lithotomy position, femoral artery cath, DM Metastatic -Invasion of plexus or peripheral neuropathy polyneuropathies nerves by malignant cells -Sensory deficits over thigh and leg to medial malleolus -Treat with radiation -Weakness and atrophy of quads -Knee buckling -Depressed patellar DTRs Sciatic nerve -Risk: DJD, hip replacement, spinal stenosis, lumbar disc Paraneoplastic -Immune response to neoplasm palsy/sciatica herniation peripheral -Weakness with leg flexion, foot dorsiflexion, foot neuropathies eversion -Depressed ankle DTRs -Sensory deficits over posterior thigh, leg, and foot -EMG to distinguish from peroneal neuropathy -X-rays

156

Peroneal (common -Risk: leg crossing, trauma to knee, fx of fibula, tight Critical illness- -Associated with ICU admission, fibular) nerve palsy casts, high boots related sepsis, multi-organ dysfunction, -Weakness on dorsiflexion and foot eversion neuropathies difficulty weaning from ventilator -Paresthesias over anterolateral calf and top of foot -EMG to distinguish from sciatic nerve palsy -Splints, PT Tibial neuropathy -AKA tarsal tunnel syndrome -Rare

HEADACHES Pediatric Headaches Etiologies Workup Management of chronic When to refer -Acute & localized: URI, other viral infection, post-traumatic, -CT without contrast or MRI indicated for kids with headaches and headaches -Headaches associated with dental abscess, TMJ dysfunction, brain abscess, first migraine neuro signs or symptoms suggestive of intracranial pathology = -Provide realistic mood disturbance or -Acute & generalized: fever, systemic infection, CNS infection, headache that awakens child during night or occurs upon waking, expectations for medical anxiety HTN, CH, exertional, first migraine, trauma, toxins, meds sudden severe headache, persistent nausea or vomiting, AMS, interventions -Uncertain diagnosis -Acute & recurrent: migraine, cluster headache ataxia, headache worsened by cough, urination, or defecation, -Plan for return to school -Headaches refractory to -Chronic & nonprogressive: tension headache, psychiatric issue, absence of aura, chronic and progressive headaches, change in -Avoid triggers: lack of primary care management post-traumatic, postconcussive, medication overuse headache quality, severity, or frequency, occipital headache, sleep, dehydration -Chronic daily headaches -Chronic & progressive: idiopathic intracranial HTN, space- recurrent localized headache, lack of response to medical therapy, -Address comorbid occupying lesion, post-traumatic, postconcussive cranial bruits, growth abnormalities, papilledema or retinal problems: insomnia, mood hemorrhages, age < 3 problems, anxiety Pediatric Migraine Headaches Signs & symptoms Workup Management -May be shorter in kids, as short as 1 hour -Imaging to rule out -Initial abortive therapy with acetaminophen or ibuprofen -Toddlers: pallor, decreased activity, vomiting, sensitivity to light more serious causes is -Antiemetic like promethazine for nausea & vomiting and noise indicated for -2nd line is abortive therapy with triptans -Nausea, vomiting, abdominal pain, desire to sleep complicated-type -Begin prophylactic treatment if > 4-5 migraines per week  cyproheptadine for kids under 6, -Complicated migraine symptoms: hemiplegia, ophthalmoplegia, symptoms and for propranolol for older kids, amitriptyline for concomitant depression tinnitus, vertigo, ataxia, weakness, confusion, paresthesias occipital location -For menstrual migraines, naproxen BID just before beginning period has been shown to be beneficial Pediatric Tension Headaches Signs & symptoms Differential Workup Management -Bilateral pressing tightness -Migraine without aura -Imaging has a low yield but may be needed to -Minimize stress -Non-throbbing -Increased ICP relieve parental apprehension or with sleep- -Acetaminophen or NSAIDs for infrequent use -Lasts hours to days -Tumor related headache, no FH of migraine, presence -If prophylactic therapy is needed, can use amitriptyline -May have sensitivity to light and noise -Infection of vomiting, absence of visual symptoms, -Psychotherapy, relaxation techniques, and biofeedback -Daily activities typically not affected headache of < 6 months duration, confusion, techniques about as successful as amitriptyline -Not aggravated by walking stairs or similar routine activity or abnormal neuro exam

157

HEADACHES Tension Headache -Most common type of headache Management -DOC are OTC analgesics: ibuprofen, naproxen, aspirin, acetaminophen, Excedrin tension Signs & symptoms headache -No aura, nausea, or vomiting -Consider prophylaxis if > 2 days/week with TCAs (amitriptyline or nortriptyline) or duloxetine if ± Photophobia and phonophobia there is comorbid depression (at least 8 weeks) -May have muscle tender points -Muscle relaxants should be taken at first sign of headache: cyclobenzaprine, methocarbamol, -Bilateral head pain “like a tight hat” tizanidine -Steady, non-pulsating pain that is dull, tight, or vise-like -Trigger point Botox injections -Mild to moderate pain intensity -Normal physical activity does not aggravate With tension and migraine headaches, watch for signs of hemicrania continua (daily unilateral headache with miosis, ptosis, eyelid edema, lacrimation, nasal congestion, rhinorrhea), which can transform from migraine or tension headaches and is prompted by medication overuse; responds only to indomethacin! Migraine Headache -Highest prevalence in 25-45 year olds with decreased Pharmacologic Therapy incidence during childbearing years Abortive Non-opioids: NSAIDs, acetaminophen, rectal indomethacin, IM ketorolac, Ergots: direct smooth muscle -May have genetic component incurring Excedrin migraine vasoconstrictors, non-selective 5-HT1-R hypercoagulability agonists -High incidence of comorbid depression Triptans: constrict intracranial bood vessels, interrupt pain transmission -Ergotamine -Precipitators: stress, hormones, hunger, sleep centrally -Dihydroergotamine: available as deprivation, odors, smoke, alcohol, meds, high -Never use during an aura due to risk of stroke injection, nasal, rectal, SL tyramine foods -Sumatriptan, zolmitriptan (wafer avail), etc. -AEs: fibrosis with long-term use, -High incidence of PFO with migraines with aura -AEs: paresthesias, dizziness, flushing, somnolence, rebound HA with overuse rebound headache, paresthesias Prophylactic -Consider with migraines > 2 per week, incomplete response to acute therapies, SSRIs Signs & symptoms or patients with rebound headaches using acute treatments -Fluoxetine, fluvoxamine, sertraline -May have prodrome of sensitivity to touch or -Must give 6-8 week trial for each therapy -Not as much data combing hair -Prolonged headache-free intervals can signal time for dose reduction or d/c -May have aura up to 1 hour before, most commonly with slow taper Atypical antidepressants scintillating scotoma, followed up visual, -Bupropion sensorimotor, speech, or brainstem disturbances β-blockers -Venlafaxine -Nausea, vomiting, photo and phonophobia, unilateral -Propranolol, timolol, metoprolol, nadolol, atenolol -Not much data pulsating frontotemporal pain -Helpful in patients with comorbid anxiety, HTN, or angina -May correlate with menstrual cycle Anticonvulsants CCB: prevent vascular spasm -Carbamazepine, gabapentin, tiagabine, Management -Verapamil topiramate, valproate, oxcarbazepine, -If related to menstrual cycle, prophylax with 2-7 days -Take up to 8 weeks to work lamotrigine, vigabatrin, zonisamide of NSAIDS prior to menses and continue through last -β-blockers work better day of flow, consider OCPs as long as there is no aura Botox -Exercise shown to be just as good as meds TCAs -Refer when symptoms are refractory to treatment, -Amitriptyline (best evidence), imipramine, doxepin, nortriptyline worsening disability, comorbid conditions requiring -AEs: tremor, weight gain, anticholinergic polypharmacy, rebound headaches, symptoms no longer fitting diagnostic criteria

158

Cluster Headache -More common in men Management -Abortive therapy: 100% O2 on a non-rebreather @ 6-12 L/min for 15 min, SQ or nasal Signs & symptoms sumatriptan, octreotide, nasal lidocaine -Unilateral, excruciating, steady pain in the eye, periorbital region, or temple -Prophylaxis: DOC is verapamil (takes 8 weeks to work), Li, ergotamine, prednisone taper, nerve -Increased swelling on ipsilateral side of face and eyelid block -Ipsilateral miosis or ptosis -Ipsilateral nasal congestion or rhinorrhea -Lasts 15-180 minutes untreated -Occurs in bouts up to every other day or up to 8 attacks daily for weeks at a time followed by remission for months or years -Can be precipitated by sleep, occurring 90 minutes after falling asleep -Patient may complain of “worst headache of life” Thunderclap Headache -Refers to sudden onset headache that is severe -If features do not fit cluster headache, must send to ED to r/o ICH -Patients may also complain of “worst headache of life”

INFECTIOUS DISORDERS Encephalitis -Represents an infection Agents Workup of the brain itself -HSV -CT or MRI to investigate space-occupying lesions, brain abscess, demyelination, areas of -May be primary or -Rabies virus abnormalities postinfectious -WNV -CSF shows lymphocytic predominance -PCR for HSV Differential Signs & Symptoms -Serum for WNV -Meningitis -Fever -Test for other agents depending on travel or exposure history -Meningoencephalitis -Headache -Stroke -Lethargy, confusion, AMS (what usually differentiates it from meningitis) Management -Seizures -Empiric treatment with acyclovir until results come back -Can have focal neuro abnormalities -No meningeal signs like photophobia or nuchal rigidity Meningitis -Represents an infection of the arachnoid mater and CSF Signs & Symptoms Group Agents Empiric Drugs -Bacterial: fever, nuchal rigidity, AMS, severe HA Under 1 mo GBS, E. coli, Listeria, Klebsiella, -First 3-6 days of life: ampicillin (cover Listeria) + -Listeria: more likely to have seizures and focal neuro deficits Enterobacter gentamicin (together cover enterococci) -Viral: severe HA, fever, photophobia, no focal neurologic 1 mo to 50 years -#1 cause is Strep pneumo -Cefotaxime (cover normal Strep pneumo, H.flu, M. cat, signs -Greater prevalence of Neisseria Neisseria) + vanco (cover MSSA/MRSA, PRSP) -Meningococcal: petechial rash, palpable purpura, DIC, meningitidis in ages 0-5 and 14-21 -Can sub ceftriaxone for cefotaxime arthritis Over 50 -Strep pneumo still #1 but also consider -Cefotaxime (cover normal Strep pneumo, H.flu, M. cat, -TB: gradual onset with listlessness and irritability, CN palsies Listeria Neisseria) + vanco (cover MSSA/MRSA, PRSP) + -Brudzinski’s and Kernig’s signs have low sensitivity but high ampicillin (cover Listeria) spec -Can sub ceftriaxone for cefotaxime Impaired cellular -Vanco + ampicillin + cefepime (to cover Pseudomonas Workup immunity (lymphoma, and other gram negs) -CT before LP to assess for ↑ICP ONLY if cytotoxic chemo, steroids) -Can sub meropenem for cefepime immunocompromised, h/o CNS disease, new seizure, papilledema, altered LOC, focal neuro deficit 159

Any; typically younger -Viral or “aseptic meningitis” Acyclovir or valacyclovir if suspecting HSV -Otherwise don’t delay LP -Usually enteroviruses -Gram stain of CSF -Also Coxsackie, ECHO, mumps, HSV, -CBC shows leukocytosis or leukopenia, poss HIV thrombocytopenia -Rodent exposure: LCMV -Get 2 sets of blood cx before starting abx -Tick exposure: Lyme, RMSF, ehrlichia -HIV if suspecting viral cause -Mosquitoes: WNV, St. Louis -Remember that a patient who has recently received abx and encephalitis has a bacterial meningitis may have milder lab findings more -Sex: syphilis suggestive of an aseptic meningitis Any -Subacute or chronic meningitis -Could be viral, bacterial, fungal, or Management parasitic -Begin empiric abx or acyclovir after LP if suspecting bacterial -HIV, TB, syphilis, late stage Lyme, meningitis or HSV meningitis Cryptococcus, Histoplasma, -Give dexamethasone with abx if bacterial meningitis Coccidioides, cysticercosis -Meningococcal contacts need prophylaxis -Tailor abx to results of gram stain and cultures

MOVEMENT DISORDERS Benign Essential Tremor -AKA familial tremor if there is a FH Workup Management -No other abnormalities -Propranolol if symptoms are disabling Signs & symptoms -Refer to neurology to r/o other causes -Primidone is 2nd line therapy -Involvement of one or both hands, the head, or hands + head -Other options: alprazolam, topiramate, gabapentin, mirtazapine -May affect manual skills or speech -Botox -Unusual to have in legs -May be enhancement with emotional stress -Often relieved with alcohol

160

Huntington’s Disease -Abnormal CAG repeats on chromosome 4  loss of caudate Signs and symptoms Management and squared-off lateral ventricles -Usually begins with a psychiatric disorder -Symptomatic -Onset will occur between ages 20-40, which can be affected by -Subcortical dementia, chorea, dystonia, motor impersistence, -Give dopamine-R blockers for chorea (like a DA-excess state) anticipation incoordination, gait instability, depression, anxiety, impulsivity, such as haloperidol or risperidone apathy, OCD, athetosis -SSRIs for depression and anxiety

Workup Prognosis -Diagnosis is clinical -Death within 10-15 years of onset of symptoms -CT or MRI will show cerebral atrophy and loss of caudate Parkinson’s Disease -Degeneration of CNS due to death of DA- Signs & symptoms Workup generating cells in the substantia nigra and -Most symptoms are unilateral (if bilateral the cause is more like to be an exposure) -Diagnosis is usually clinical accumulation of Lewy bodies in neurons -Pill-rolling resting tremor, cogwheel rigidity, shuffling steps, difficulty initiating movements with -Head MRI to rule out brain lesions -Risk factors: age, exposure to synthetic heroin festinating gait (unwanted acceleration after walking commences) -Genetic testing is available but should byproduct MPTP, manganese exposure, flu -Bradykinesia, fatigue, stooped posture, masked facies only be performed after careful epidemic -Memory loss consideration due to inconclusive testing -Small, cramped handwriting results and lack of specific treatment for Etiology the disease -Most cases are idiopathic Differential -5% of cases are hereditary -Lewy body dementia and other neurodegenerative disorders Management -Essential tremor -Treatment should begin when patient -Secondary parkinsonsism (usually a drug reaction) experiences functional impairment Pharmacologic Therapy Synthetic DA: DA precursor that can cross the BBB to DA Agonists: act directly on DA-R in COMT Inhibitors: prevent MAO-B Inhibitors: inhibit Others replace deficit the corpus striatum breakdown of levodopa by another metabolism of dopamine pathway -Levodopa -Bromocriptine, pramipexole, -Entacapone, tolcapone -Selegiline, rasagiline -Amantadine: -Must also give carbidopa to prevent peripheral conversion ropinirole, apomorphine -Allows for decreased levodopa -Can help delay need for antiviral that -Can be given to help diagnose Parkinson’s -First-line therapy for younger patients dosage levodopa increases DA release -First-line therapy in older patients (> 65) as effectiveness ↓ with milder disease -AEs: dyskinesias, nausea, -AEs: insomnia, nausea from nerve terminals over time -Can delay need for levodopa dizziness, hallucinations, abd pain, -Anticholinergics: -Won’t help with postural instability, dementia, autonomic -Can be add-on to levodopa therapy diarrhea, orthostasis, somnolence, block Ach dysfunction, or “freezing” -AEs: cardiac valve fibrosis, dizziness, HA -Vit E -Won’t stop disease progression HA, insomnia or somnolence, fonusion, supplementation not -AEs: n/v, anorexia, postural hypotension, arrhythmias, hallucinations, hypotension, syncope, shown to be mental disturbance, dyskinesias, overactivity, agitation impulsivity beneficial

VASCULAR DISORDERS Cerebral Aneurysm -Baseline prevalence of 0.2-6% in adults Screening Management -Generally low risk of rupture -Not indicated in the general population -Treatment depends on patient age, severity and progression of symptoms, and -Consider screening of individuals with 2+ first-degree available alternatives Risk factors relatives with known cerebral aneurysm with MRI every -Endovascular techniques associated with lower mortality than surgical clipping -Ehlers Danlos 3-5 years -Monitoring via CTA or MRA annually for 2-3 years, then every 2-5 years if stable -Polycystic kidney disease -Risk reduction: quit smoking, avoid heavy alcohol consumption, stimulants, illicit -Bicuspid aortic valve drugs, and heavy lifting and straining -Aldosteronism 161

Stroke -An acute neurological deficit of vascular etiology with symptoms lasting > 24 hours Differential: transverse myelitis, Bell’s palsy, Gullain-Barre, myasthenia gravis, TIA -More prevalent in the “stroke belt” in SE US Hemorrhagic Stroke (Intracerebral or Intracranial Hemorrhage) Ischemic Stroke Accounts for 15-20% of strokes -Accounts for 80-85% of strokes Workup -Head CT: warning! will look normal until Parenchymal ICH Atheroembolic several hours into stroke -Bleeding within the brain itself -Occlusion of artery supplying the brain due to -Primary if due to spontaneous rupture of small vessels damaged by chronic HTN or amyloid CAD, stenosis, or cholesterol embolus Management angiopathy -The most common kind of stroke -Start TPA if within 4.5 hours of symptom -Secondary if due to trauma, vascular abnormalities, tumors, impaired coagulation, or vasculitis -There will be warning signs with a stepwise onset (this is the cutoff point for -Presentation will be severe HTN, bad HA, n/v, focal neuro deficits progression to full stroke, such as transient language prevention of disability) after cleared by -If in thalamus or basal ganglia  contralateral motor and sensory deficit, aphasia, language or disturbances and weakness head CT spatial neglect, depressed LOC due to mass effect, intraventricular extension  hydrocephalus -Ophthalmic artery occlusion  amaurosis fugax -TPA relative contraindications, within 3 -If in the cerebellum  ipsilateral ataxia, -ACA occlusion  weakness and sensory loss in hours of symptom onset: recent head depressed LOC contralateral leg, may have mild weakness of the trauma or stroke, prior ICH, recent arterial -If in the pons  vertigo, diplopia, crossed arm puncture, active bleeding or acute trauma, signs, depressed LOC -MCA occlusion  contralateral hemiplegia, on oral anticoags with hi INR, low hemisensory loss, homonymous hemianopia with platelets, hypoglycemia, HTN > 185/110, Subarachnoid hemorrhage eyes deviating towards affected side, global aphasia CT with hypodensity in > 1/3 of cerebral -Bleeding outside the brain if in dominant hemisphere, hemisphere, rapidly improving symptoms, -Most common cause is a ruptured aneurysm, -Lacunar stroke: a kind of atheroembolic stroke seizure with postictal impairment, recent usually of the anterior communicating artery, where one of the penetrating arteries providing blood MI, recent major surgery but can also occur at the bifurcation of the to the brain’s deep structures is occluded  can -TPA further contraindications if > 3 hours carotid, PCCM, MCA, basilar tip artery, or appear as pure motor strokes, pure sensory strokes, after symptom onset: age over 80, oral PICA ataxic hemiparesis, or dysarthria + clumsy hand anticoags, h/o prior stroke + DM -Less common causes are vasculitis, -Dilated pupils with vertebrobasilar stroke -Consider endovascular repair or clot infection, neoplasms, or blood coagulopathies removal if TPA is not an option, and give -Risk factors: heavy alcohol, smoking, HTN, genetics Cardioembolic aspirin instead -Presentation will be with an abrupt, severe headache (“worst -Embolus thrown from the heart goes to the brain; -Avoid D5W as glucose crosses into the HA of life”), meningismus, may have rapid LOC can then break up into many clots and travel to brain and is quickly metabolized  edema -Neuro exam will be nonfocal because it is outside of the multiple vascular territories -Only lower BP if > 220/120, and only by brain -Can also cause a hemorrhagic infarction as the 15-20% the first day, otherwise avoid ischemic blood vessels die and split open (will want treating HTN for at least 2 weeks to avoid Workup to differentiate this from primary ICH, because if it’s further cerebral ischemia -CT will show white in area of bleed primary you can’t give blood thinners or lytics ever -Hypothermia induction therapy? -If SAH, LP will show xanthochromia again but if the hemorrhage is secondary to blood -Antithrombotic agents clot you want to put the pt on blood thinners to -Later PT, OT, speech therapy Management prevent future embolic strokes) -Parenchymal ICH is managed supportively, with surgical -Sources: afib, cardiomyopathy, acute MI, valvular Prognosis consult if there is mass effect heart disease -Everyone improves to some degree after a -Subarachnoid hemorrhage typically requires surgical intervention -Most commonly lodges in the middle cerebral stroke artery -Fastest period of recovery is the first 30- 60 days afterwards

162

Transient Ischemic Attack -An acute focal neurologic deficit as a result of Workup Management Prognosis ischemia that resolves within 24 hours -CBC, BMP to r/o metabolic causes -Risk reduction: smoking cessation, statins, BP -Incurs greater risk of stroke in the future, can -Can be caused by brain, spinal cord, or retinal -Lipids control use ABCD2 criteria (age, BP, clinical features, ischemia -Brain imaging to r/o hemorrhage or cerebral -Antiplatelet therapy indicated for duration of sx, DM) to estimate risk tumor noncardioembolic attacks: aspirin or Differential -Neurovascular imaging: carotid US, CTA if clopidogrel (only marginally better than ASA) -Seizure needed -Anticoagulant therapy indicated for pts with -Migraine with aura -Cardiac eval: EKG, echo if there is suspected concurrent afib -Syncope endocarditis -Carotid endarterectomy for select patients -Hypoglycemia -Encephalopathy -Multiple sclerosis Subdural Hematoma -Usually due to tearing of Signs & Symptoms Workup Management bridging veins on brain -HA, vomiting, dysphagia, anisocoria, CN -Noncontrast head CT will -ABCs and resuscitation surface to dorsal sinuses palsies, nuchal rigidity, ataxia show crescent-shaped -C-spine immobilization -Can also occur from -H/o acceleration/deceleration injury hematoma because it extends -Short-term hypoventilation in order to ↓pCO2  ↓ICP by cerebral vasoconstriction arterial rupture -Usually acute but can become chronic beyond the suture lines -Mannitol or hypertonic saline to ↓ICP -Behavioral changes such as apathy, -Seizure prophylaxis cognitive impairment with chronic SDH -May require surgery -ICU admission with serial head CTs

Prognosis -More severe injury than epidural; mortality of those requiring surgery is 40-60% ***The textbook presentation of an epidural hematoma is that there will be a lucid interval followed by a loss of consciousness, however in reality EITHER a subdural or an epidural can have a lucid interval  LOC; the only difference is that the decompensation to coma may be slower in a subdural Subdural Hematoma Epidural Hematoma Subarachnoid Hemorrhage (Stroke)

Epidural Hematoma -Due to tearing of middle meningeal Signs & Symptoms Workup Management artery -HA, vomiting, confusion/lethargy, aphasia, seizures, hemiparesis -Noncontrast head CT shows hematoma that does -Usually requires craniotomy -Rarely seen in kids < 2 and in the -Unconsciousness, abnormal pupil reactions to light, or abnormal not cross suture lines, brain parenchyma may be with evacuation of bleed elderly as the dura is firmly attached in posturing due to compression of CN by hematoma compressed to the midline these ages -Usually coexists with a skull fracture

163

OTHER NEUROLOGIC DISORDERS Cerebral Palsy -A group of nonprogressive Presentations Signs & symptoms Management clinical syndromes -Spastic CP: an UMN syndrome with slow effortful voluntary -FH of the disease -Multidisciplinary team characterized by motor and movements, impaired fine-motor function, difficulty in isolating -Loss of developmental milestones needed postural dysfunction individual movements, and fatigability -Ataxia, involuntary movements, oculomotor abnormalities, -Botox for joint contractures -Dyskinetic CP: usually a result of severe perinatal asphyxia; muscle atrophy, or sensory loss -Regular x-ray screenings for Etiologies encephalopathy characterized by lethargy, decreased spontaneous -Hypotonia associated with weakness hip dysplasia -Most cases are prenatal due movement, hypotonia, suppressed primitive reflexes, later -Rapid deterioration of neuro signs -Physical therapy to reduce to prematurity, intrauterine athetosis, chorea, and dystonia muscle tone growth restriction, -Ataxic CP: ataxic movements and speech, widespread disordered Workup -May need gastrostomy tube intrauterine infection, motor function; a diagnosis of exclusion -Glucose, ammonia, lactate, pyruvate, ABG, and other studies are antepartum hemorrhage,  Frequently accompanied by other disorders of cerebral function needed to exclude a metabolic disorder Prognosis placental pathology, or such as intellectual disability or learning disability, behavioral and -Requires a constellation of findings including motor delay, -CP lesion will be static but multiple pregnancy emotional disorders, seizures, impaired vision or speech neurologic signs, persistence of primitive reflexes, and abnormal clinical signs may evolve as -Perinatal hypoxia or  Also may have secondary consequences such as poor growth postural reactions the nervous system matures ischemia and nutrition, orthopedic problems, osteopenia, and urinary -Diagnosis may require serial exams and is not possible until -Perinatal stroke disorders later infancy; CP is a diagnosis of exclusion -Low birth weight -Brain MRI to determine site of lesion Concussion -Considered to be a mild TBI as a result of Signs & symptoms Management blunt force or acceleration/deceleration -Often involves brief loss of consciousness -Avoid further contact sports as repeat TBI injury -Confusion or amnesia soon after initial injury can lead to cerebral -HA edema -Dizziness -ED workup indicated for LOC > 1 min, -N/v concern for C spine injury, high risk for -Seizures within first week after injury intracranial bleed, possible skull fracture, or -Hours to days after: mood and cognitive worsening of patient’s condition disturbances, photo and phonophobia, sleep -NSAID analgesics (don’t want to affect disturbances cognition)

Sequelae -Post-concussive syndrome: HA, dizziness, neuropsychiatric symptoms, cognitive impairment; generally resolve within a few weeks to months -Epilepsy -Vertigo

164

Dementia -The most common cause of dementia is Differential Management Alzheimer’s disease -Delirium -Cholinesterase inhibitor for mild to -Other major types are Lewy body dementia, -Depression (“pseudodementia”) moderate dementia (MMSE 10-26): frontotemporal dementia (more prominent -Structural brain disease: Parkinson’s, Huntington’s, Down’s syndrome, head trauma, brain tumor, donepezil, rivastigmine, galantamine personality and behavioral changes), vascular normal pressure hydrocephalus, MS, subdural hematoma -Vitamin E 1000 IU BID if no CV dementia, and Parkinson-associated dementia -Metabolic: hypothyroid, hypoxia, B12 or folate or thiamine deficiency, Wilson’s disease, lead toxicity disease -Dementia is characterized by memory -Infectious: Lyme, HIV, Creutzfeldt-Jakob, neurosyphilis, meningitis, encephalitis -Add memantine for mod-advanced impairment and impairment in one other -Drugs dementia (MMSE < 17) cognitive domains: aphasia, apraxia (clock -Mild cognitive impairment: normal daily function with abnormal memory for age; most progress to -D/c treatment when MMSE < 10 unless drawing), agnosia (inability to interpret dementia pt worsens significantly when off of sensations correctly), executive function -Normal age-related cognitive decline: mild changes in memory and rate of info processing that are them (planning, organization, abstract thinking) NOT progressive and don’t affect daily function -For behavioral issues, first r/o superimposed delirium Screening Workup -Agitation and aggression are best -USPSTF grade I -15% of “demented” patients have a treatable and potentially reversible condition! managed by behavioral intervention -Full neuro exam rather than antipsychotics like Signs & symptoms -MMSE: scores < 24 suggestive of dementia or delirium haloperidol (evidence for aromatherapy -Insidious appearance and progression of -Clock drawing test appears to correlate with MMSE exists as well as exercise, music, pet cognitive deficits, usually beginning with -Labs: B-12, TSH therapy, and massage) language function and visuospatial skills -Depression screen -Depression: SSRIs may help, better -Pt will not usually report memory loss but -Noncontrast head CT or MRI (will easily diagnose vascular dementia) choice for the elderly is citalopram family members will -DSM-IV criteria for dementia: -Sleep disorders: small quantities of -Later deficits in executive function and behavior 1. Memory impairment trazodone if needed -Noncognitive neurologic deficits in late-stage 2. At least one of the following: apraxia, aphasia, agnosia, or disturbance in executive -Wandering disease: pyramidal and extrapyramidal motor functioning -Sexually inappropriate behavior: signs, myoclonus, and seizures 3. Disturbance significantly interferes with work, social activities, or relationships antidepressants, antipsychotics, -Stepwise loss of function in vascular dementia 4. Disturbance does not occur exclusively during delirium cholinesterase inhibitors, other agents as microinfarcts add up -Additional DSM-IV criteria for Alzheimer type dementia: -Delusions and hallucinations: atypical -Atypical presentations: visual variant, primary 1. Gradual onset and continuing cognitive decline antipsychotics incur increased risk of progressive aphasia 2. Not caused by identifiable medical, psychiatric, or neurologic condition mortality but may be necessary -May have psychotic symptoms or paranoia -Additional DSM-IV criteria for vascular dementia: -Personality changes and mood swings 1. Focal neuro signs or evidence of cerebrovascular condition Prognosis -Depression -Gross pathology will show diffuse atrophy with enlarged ventricles and flattened sulci -Average life expectancy of 3-8 years -Anger -Microscopic pathology (brain biopsy) will show senile plaques composed of amyloid, neurofibrillary tangles derived from Tau protein, and neuronal and synaptic loss (however senile plaques and neurofibrillary tangles are also seen in normal aging) Tourette Syndrome -A movement and Signs & symptoms Management neurobehavioral disorder in -Waxing and waning tics: eye blinking, facial grimacing, shoulder shrugging, head jerking -Pharmacotherapy indicated only when symptoms children characterized by multiple -Irresistible urge before a tic and sudden relief afterwards interfere with social interactions, school or job motor and vocal tics -Complex sequences of coordinated movements: bizarre gait, kicking, jumping, gyrations, scratching, performance, or ADLs -Possible genetic cause seductive or obscene gestures -Bothersome tics: fluphenazine or Botox injections -Utterances: simple noises, obscene words, echolalia, palilalia -Behavioral therapy with habit reversal training -May have bizarre thoughts and ideas, fixations, compulsive ruminations, perverse sexual fantasies -Stimulants for comorbid ADHD -Sleep complaints: restlessness, insomnia, nightmares, enuresis, teeth grinding, sleepwalking -Clonidine for impulse control and rage attacks -Onset usually between ages 2-15 -Comorbid ADHD, OCD, conduct disorder

165

Delirium -AKA acute dementia, acute Risk factors/possible etiologies Signs & symptoms Management confusional state, acute organic -Metabolic disorders -Untreated pain -Decreased consciousness -Thiamine supplementation brain syndrome -Infections -Withdrawal -Reduced focus and attention -Frequent reassurance, touch, -A severe neuropsychiatric -Anemia -Electrolyte imbalances -Changed cognition or perception and verbal orientation syndrome with core features of -Decreased cardiac output -Advanced age -Agitation or lethargy -Cautious trial of haloperidol acute onset and fluctuating course -Hypotension -Males -Not explained by underlying dementia for severe agitation or with attentional deficits and -Greater than 3 new meds -Deprivation: sensory stimuli, sleep -Rapid onset psychosis generalized disorganization of -Hypo or hyperthermia -Immobilization -Fluctuating behavior -Fecal impaction or urinary retention -Transfer to new environment Prognosis -Pathophysiology not well -Foley catheter -Severe illness Workup -May require weeks to understood -Dehydration -CMP months to resolve -Occurs in 30% of hospitalized -Ca -62% of patients will still patients -CBC have symptoms 6 months out -UA from discharge -Tox screen -ABG Guillain-Barre Syndrome -Refers to the acute immune-mediated Agents Signs & Symptoms Management polyneuropathies, of which there are several -EBV -Symmetric motor and sensory polyneuropathy that begins in an ascending fashion, -Supportive: mechanical ventilation variants -HIV from legs to trunk to arms -Plasma exchange -Thought to be an immune response to a -HSV -Progresses over a 2 week period -IV Ig preceding infection that cross-reacts with -CMV -Paresthesias peripheral nerve antigens -Campylobacter -Areflexia Prognosis -Recent viral illness -Gradual recovery of function 2-4 Differential -Absent or depressed DTRs weeks after progression stops -Other polyneuropathy -Dysautonomia: tachycardia, urinary retention, hypo/hypertension alternating, ileus, -Spinal cord disease loss of sweating -Brainstem stroke -Wernicke’s encephalopathy Workup -Brainstem encephalitis -Initial test is CSF: increased protein, normal WBCs (but not present in all pts) -EMG studies Multiple Sclerosis Etiology & natural history Forms Signs & symptoms Workup Management -Likely multifactorial, with infectious agents, 1.) Relapsing-remitting: partial -Optic neuritis -Can’t diagnose MS from -Refer to neurology for immunomodulators and genetic predisposition, and environmental recovery for disability between -Transverse myelitis just one attack, need to have immunosuppressants factors all playing a role in the abnormal relapses, accounts for most cases, -Paresthesias at least 2 -Large dose steroids for relapses or aggressive immune response all meds are for this variety! -Ataxias -Refer for brain MRI: disease -Inflammation  demyelination  axonal 2.) Secondary progressive: -Weakness T1/gadolinium contrast -Vitamin D supplementation loss Increasing disability with distinct -Incoordination imaging enhances active -More prevalent in individuals living further relapses -Spasticity lesions while T2 imaging Prognosis from the equator 3.) Primary progressive: Nearly -Cognitive impairment represents cumulative -If untreated brain atrophy will occur and half of -Demyelination continues to occur during the continuous worsening of -Episodes that come and go disease burden; white spots all MS patients will need an assistive device to clinically silent periods in between relapses disability must be in characteristic MS walk within 5 years and relapsing MS will -As disease progresses, MRI lesion burden locations progress to progressive MS within 10 years and disability increase as cognitive function decreases

166

Myasthenia Gravis Etiology Differential Workup Management -Production of ABs against Ach receptors -Botulism -Tensilon test: acts as an Ach -Thymectomy -Many cases are associated with a thymoma -ALS converting enzyme inhibitor to -Meds: Ach converting enzyme inhibitors like -Dermatomyositis or polymyositis temporarily overcome neuromuscular pyridostigmine, immunosuppressants Signs & symptoms -Lambert-Eaton myasthenic syndrome jcn Ach deficit -Intubation if needed (don’t use depolarizing or -Ocular: Ptosis, blurred vision, diplopia -MS -Labs: anti-Ach-R AB, MuSK AB non-depolarizing agents) -Bulbar/facial: difficulty chewing or swallowing, -MI -Repetitive nerve stimulation test -Plasmapheresis to remove pathologic AB dysarthria, tired facial appearance, difficulty smiling or -PE -Single fiber EMG is the most -Exacerbation can be precipitated by infection, whistling, difficulty keeping food in mouth -Sarcoidosis sensitive test for myasthenia gravis surgery, pregnancy, or certain meds = avoid -Asymmetric proximal or distal extremity weakness -Neuropathy -Chest CT to rule out thymoma aminoglycosides, azithromycin, quinolones, -SOB -Thyroid disease botox, CCB, Mg, IV contrast -Muscle weakness with repetitive use Seizure Disorders -Epilepsy = documented h/o at least 2 seizures Differential Workup Management not related to a metabolic or febrile cause -Hyperventilation -EEG to determine seizure -When to treat after a single seizure: patients with structural lesion, abnormal EEG, focal -Risk factors: head trauma, CNS infections, -Migraine type seizure cerebrovascular disease, alcohol, drug -Panic attack -Electrolytes, glucose, -When NOT to treat after a single seizure: EtOH, drug abuse, provoked seizure, head overdose or withdrawal, metabolic disorders, -Pseudoseizure anticonvulsant levels, alcohol injury with no structural abnormality genetics, malignancy -Syncope and tox screen, ABG -Drugs are selected based on type of seizure, AEs, toxicity, cost, and childbearing -Common provoking factors: sleep deprivation, -Transient global -LP to r/o meningitis potential excessive stimulants, withdrawal from ischemia -Head CT or MRI -Begin with monotherapy sedatives or alcohol, substance abuse, high -TIA -Consider 2nd agent if inadequate trial of 2 different single agents fever, hypoxia, hypoglycemia, electrolyte -Sleepwalking disturbance, estrogen (= more seizures during -Meningitis Prognosis ovulation and menses) -Most epileptics who go into remission do so within 3 years after their first seizure -Poor prognostic factors for remission: FH of epilepsy, psychiatric comorbidity, h/o febrile seizures, > 20 seizure history, adult age, failed monotherapy Partial Seizures Management -1st line: carbamazepine, phenytoin, lamotrigine, valproate, or oxcarbazepine -2nd line: gabapentin, topiramate, levetiracetam, zonisamide, tiagabine, phenobarbital, felbamate Simple Partial Seizures Complex Partial Seizures -No LOC -May experience unpleasant tastes or odors -The most common kind of seizure -Inability to carry out -Alternation contraction and relaxation of -Dizziness -Can occur after head trauma and many of simple commands or muscle groups -Autonomic symptoms: flushing, incontinence, nausea, vomiting, these pts will have abnormal tissue or execute willful movement -Eye movements and turning of head to the goose bumps, pupillary dilation, sweating, tachycardia lesions in their temporal lobe -Lack of awareness of same side -Psychiatric symptoms: detachment, memory distortion, time -Involves alteration of consciousness surrounding and events -Speech arrest or vocalization distortion, unprovoked emotion -Automatisms such as lip smacking, -Can become generalized -May see flashes of light or color or have -Can turn into a complex partial seizure or manifest in a continuous picking, patting, chewing, or swallowing tonic-clonic seizure hallucinations form (epilepsia partialis continue) -May hear humming, buzzing, or hissing

167

Generalized Seizures Absence (Petit Mal) Seizures Tonic-Clonic (Grand Mal) Seizures Myoclonic Seizures Other Seizures -5-10 recurrent episodes of staring -Tonic phase begins with LOC, tensing of -Caused by metabolic abnormalities such as Tonic seizures -May have minor motor automatisms muscles, and often a loud yell or moan hepatic or renal failure -Relatively rare to occur alone -Pts have no memory of incident but are completely -Clonic phase commences with convulsions, -Brief major motor seizure with quick, -Involve stiffening of the body, upward normal afterwards eyes rolling back, strong jaw contractions lightning-like jerking movements of the deviation of the eyes, dilation of the pupils, -Can be triggered by hyperventilation -May have aura trunk or extremities and altered respiratory patterns -Lasts 5-20 minutes -May occur throughout body or limited to Workup -May have incontinence certain muscle groups Atonic seizures -EEG abnormality will be present even when not -May have unconsciousness after seizure -Onset may be so sudden that pt falls to the -Sudden loss of muscle tone that may cause a seizing followed by post-ictal state ground but can also be so brief that fall consciousness is not lost -Last 1-4 secondes but without LOC Management Management -May affect one part of body to all body tone -1st-line: valproate, ethosuximide -1st line: phenytoin, carbamazepine, valproate Management -2nd line: lamotrigine, levetiracetam -2nd line: lamotrigine, levetiracetam, -1st line: clonazepam, valproate Management topiramate, phenobarbital, primidone, -2nd: lamotrigine, levetiracetam, topiramate, -1st line: valproate Prognosis oxcarbazepine felbamate, zonisamide -2nd line: lamotrigine, topiramate, zonisamide -Most cases resolve spontaneously Anticonvulsants Agent & MOA Info Risks & AEs Carbamazepine: inhibits -Also for treating bipolar disorder, trigeminal neuralgia, and -AEs: diplopia, dizziness, drowsiness, nausea, Stevens-Johnson (don’t use in Asians), hypoCa, voltage-gated Na channels glossopharyngeal neuralgia hypoNa, SIADH, hematologic, hepatitis  monitor CBC, LFTs, mental status, bone density, levels -Decreases effectiveness of OCPs and warfarin -Pregnancy D Oxcarbazepine: blocks voltage- -For partial seizures -AEs: sedation, dizziness, ataxia, nausea, Stevens-Johnson, hypoNa  monitor Na gated Na channels, modulates Ca -Decreases effectiveness of OCPs and phenytoin channels, increases K -Pregnancy C conductance Clonazepam: modulates GABA -Not a first-line choice transmission in the brain -Frequently added as a 2nd agent with levetiracetam Ethosuximide: increases seizure -For absence seizure -AEs: ataxia, drowsiness, GI, unsteadiness, hiccups, Stevens-Johnson, hematologic, SLE threshold, depresses nerve -Interactions with carbamazepine and valoproate transmission in the motor cortex -Pregnancy C Felbamate: glycine-R agonist -For partial and generalized seizures -AEs: anorexia, n.v, insomnia, HA, Stevens-Johnson, aplastic anemia, hepatic failure = weekly LFTs & last resort drug! -Must sign informed consent -Interacts with many other seizure meds -Pregnancy C Gabapentin or pregabalin: -Add-on therapy for seizures -Renal dosing needed modulate Ca channels -Also for neuropathic pain -AEs: dizziness, fatigue, ataxia, nystagmus, tremor, HA, peripheral edema, Stevens-Johnson -Pregnancy C Lamotrigine: blocks voltage- -Also for bipolar disorder -AEs: nausea, diplopia, dizziness, unsteadiness, HA, rash, Stevens-Johnson, hematologic, gated Na channels and inhibits liver failure glutamate release -Interaction with valproate -Pregnancy C

168

Levetiracetam: inhibits Ca -For partial, tonic-clonic, and myoclonic seizure -AEs: sedation, suicidal ideation, pancytopenia, liver failure channels, facilitates GABA, -Pregnancy C reduces K currents, modulates NT release Phenobarbital: decreases post- -Indicated for seizure and sedation -AEs: ataxia, hyperactivity, HA, unsteadiness, sedation, nausea, cognitive impairment, blood synaptic excitation dyscrasia, S-J, hepatic injury, osteopenia -Many drug interactions -Pregnancy D Phenytoin: stabilizes neuronal -May be given as fosphenytoin for faster effect -AEs: ataxia, nystagmus, behavior, dizziness, HA, sedation, lethargy, incoordination, blood membranes by altering Na efflux -For generalized and complex partial seizures dyscrasias, rash, hirsutism, peripheral neuropathy Tiagabine: inhibits GABA -Adjunct therapy for partial seizures -Pregnancy C reuptake Topiramate: modulates Na -For partial or generalized seizures -AEs: difficulty concentrating, psychomotor retardation (“dopamax”), speech or language channels, enhances GABA, -Also indicated for migraine prevention problems, fatigue, HA, metabolic acidosis, kidney stones  monitor BMP antagonizes glutamate-R -Interacts with OCPs -Pregnancy C Valproate: increases GABA -For absence, complex partial, or mixed-type seizures -AEs: GI upset, sedation, unsteadiness, tremor, thrombocytopenia, palpitations, immune -Also for bipolar disorder or migraine prophylaxis hypersensitivity, ototoxicity  monitor CBC and LFTs -Many drug interactions -Pregnancy D Vigabatrin: irreversibly inhibits -For refractory complex partial seizures or complex generalized -AEs: permanent visual loss, psychiatric disturbances = in a restricted dist program GABA transaminase seizures Zonisamide: MOA unknown -Adjunct for partial seizure -AEs: sedation, dizziness, cognitive impairment, nausea, kidney stones, S-J, schizophreniform disorder -Pregnancy C Status Epilepticus -Single unremitting seizure with duration > 5-10 minutes, or frequent Workup Management seizures w/o interictal return to baseline clinical state -Simultaneous assessment & treatment -Lorazepam, repeat as needed -Careful neuro exam for any focal deficits -Loading dose of phenytoin of fosphenytoin for maintenance Causes -EEG -Noncompliance with antiepileptic drug regimen Prognosis -Drug or EtOH withdrawal -May have increased risk of mortality and neurologic -Acute brain injury or infection sequelae -Metabolic disturbances

169

Febrile Seizures -Typically occur in the setting of systemic bacterial or viral infection, but patient/family may not be aware of infection until sudden fever -Genetic component Type Information Workup Management Prognosis Simple febrile -Less than 15 min (or total duration < 30 min if they -LP only indicated with meningeal -Treat any febrile seizure longer than -Recurrence rate is 30% or more seizures occur in a series) signs or suspected intracranial 5 minutes: lorazepam -Neurologic sequelae are rare -No focal features infection, with infants 6-12 months -Intubation if breathing becomes -Preventative acetaminophen -Usually generalized tonic clonic seizures but may be not immunized with HIB and PCV, compromised administered at the first sign of fever atonic when patient is on antibiotics -Electrolytes and glucose if > 5 may or may not prevent a febrile Complex febrile -Greater than 15 minutes (or total duration > 30 min if (masking of meningeal signs), and minutes seizure seizures they occur in a series) with seizures occurring after 2nd day -Parents may be taught how to give -Greater risk of later epilepsy, -Focal features of illness rectal lorazepam once for recurrent although prevention of febrile -Postictal paresis -Imaging for abnormal neuro exam febrile seizures seizures using antiepileptics does not -Generally preventative antiepileptic appear to reduce this risk drug therapy is not indicated in this population Febrile status -Lasts 30 minutes or longer -May need LP -Emergency management with epilepticus -Unlikely to stop if not treated with antiepileptics antiepileptics and lorazepam to end seizure, cooling blanket, antipyretics Syncope -True syncope is defined as a loss of consciousness followed by an immediate, spontaneous return to baseline without any new focal neurologic findings Differential Workup Cardiac causes Blood loss Other causes -EKG: check the PR and QT intervals -Arrhythmia (#1 cause!): slumping, brief loss of -Acute aortic dissection -Intoxication -BMP, BNP consciousness -Trauma -Pulmonary embolism -CXR -Aortic stenosis -GIB -Cataplexy -Autonomic testing: carotid sinus massage -Valvular disease -Ruptured ovarian cyst or ectopic -Drop attacks -Referral for electrophysiology studies -Acute MI or CAD: slumping pregnancy -Meds: causing orthostasis or -Referral for upright tilt table test for -Cardiac tamponade -Ruptured spleen cardiotoxicity suspected or to r/o vasovagal syncope -Pacemaker malfunction -Epilepsy -Obstructive cardiomyopathy: with exertion -Psychogenic syncope Management Neurologic causes -Pulmonary HTN -ER if a cardiac etiology is suspected or if Neurocardiac causes (“vasovagal syncope”) -Subarachnoid hemorrhage -Seizure there is recurrent syncope or if it related to -Micturition or defection syncope -TIA -Neural cause: specific trigger, no cardiac exertion -Situational syncope -Subclavian steal syndrome history, after meals, loss of consciousness -Admit for cardiac monitoring if syncope is -Cough-mediated syncope -Complex migraine headache > 5 minutes due to cardiac cause or pt is high risk -Carotid sinus hypersensitivity: occurs with neck rotation (abnormal EKG, h/o cardiac disease, or pressure Metabolic causes hypotension, anemic, older age with -Orthostatic syncope: usually a result of volume depletion -Hypoglycemia comorbidities, FH of sudden cardiac death) or autonomic instability -Hypoxia -After prolonged standing -Hyperventilation -Exertion in an athlete  Typically have a prodrome of nausea, chills, sweats

170

PSYCHIATRY PSYCHIATRIC PHARMACOLOGY Receptor type Effects of psychiatric drugs Receptor type

Dopamine (D2) Antagonists  antipsychotic effect, relief of + symptoms of schizophrenia, Serotonin 3 (5-HT3) ↑extrapyramidal symptoms, increased prolactin levels

Serotonin 1A (5-HT1A) Agonists  antidepressant & anxiolytic effects Alpha-1 adrenergic (α-1)

Serotonin 2A (5-HT2A) Antagonists  improvement in neg symptoms of schizophrenia and Histamine (H1) improved cognition

Serotonin 2C (5-HT2C) Antagonists  weight gain and associated risks Muscarinic (m1) Class & MOA Generic Agent Brand Info SSRIs: inhibit Fluoxetine Prozac -Longest half-life = highest risk for serotonin syndrome reuptake of serotonin -Many drug interactions as well as slight -Most stimulating SSRI effects on histamine- -Lowest weight gain = good for eating disorders R, α1-R, and Citalopram Celexa muscarinic-R -Low risk of sexual AEs Escitalopram Lexapro -AEs: GI, CNS, sexual, sedation, fatigue, dry mouth, hypotension, Fluvoxamine Luvox withdrawal if d/c abruptly, prolonged QT, rash, insomnia, asthenia, seizure, tremor, somnolence, mania, suicidal ideation, worsened depression -Risk of serotonin syndrome: shivering, hyperreflexia, myoclonus, ataxia, Sertraline Zoloft -Few drug interactions n/v/d -Highest risk of GI problems Paroxetine Paxil -Shortest half-life = highest risk of d/c symptoms -Most sedating SSRI and greatest weight gain and greatest sexual AEs -Greatest anticholinergic activity SNRIs: inhibits Venlafaxine (ER Effexor -HTN -Equally effective as SSRIs for treating major depression reuptake of both avail) -Sedating -May be more effective in the setting of diabetic neuropathy, fibromyalgia, serotonin and msk pain, stress incontinence, sedation, fatigue, and patients with comorbid norepinephrine Duloxetine Cymbalta -Less AEs than venlafaxine anxiety -Works well for fibromyalgia -AEs: GI, HTN, CNS, permanent sexual?, diaphoresis, dizziness, fatigue, -Good for sleep and pain insomnia, blurred vision, suicidal ideation, dysuria, worsened depression Desvenlafaxine Pristiq -Fewer drug interactions Atypical Bupropion Wellbutrin -May increase sexual function Antidepressants -Has stimulant effects = good for comorbid ADHD or for helping quit smoking but don’t use if comorbid anxiety or eating disorder -AEs: lower seizure threshold, insomnia, nervousness, agitation, anxiety, tremor, arrhythmias, HTN, tachycardia, S-J, weight loss, GI, arthralgia or myalgia, confusion, dizziness, HA, psychosis, suicidal ideation Mirtazapine Remeron -Less nausea and sexual AEs -Overdose is generally safe -AEs: the most sedating antidepressant (= good for insomnia!), weight gain, orthostatic hypotension, dizziness, dry mouth Nefazodone Serzone Trazodone Oleptro -AEs: arrhythmia, hyper or hypotension, diaphoresis, GI, hemolytic anemia, leukocytosis, dizziness, HA, insomnia, lethargy, memory impairment, seizure, somnolence, priapism, weight gain

171

Class & MOA Generic Agent Brand Info Class & MOA Tricyclic Amitriptyline Elavil -Good for sleep, pain, and depression Antidepressants: inhibits reuptake of -AEs: anticholinergic, CV, CNS, weight gain, sexual dysfunction, decreased both serotonin and Clomipramine Anafranil seizure threshold norepinephrine Desipramine Norpramin -Least sedating -CV effects: orthostatic hypotension, conduction disturbance, cardiotoxicity Doxepin Silenor  consider EKG prior to initiation Imipramine Tofranil -Overdose can be lethal Nortriptyline Pamelor MAOIs: block Phenelzine Nardil -Irreversible -MAO-A acts on norepinephrine and serotonin destruction of -MAO-B acts on phenylethylamine and DA monoamines Tranylcypromine Parnate -Irreversible -AEs: anticholinergic, lower seizure threshold, weight gain, rash, orthostasis, centrally and sexual dysfunction, insomnia or somnolence, HA, HTN crisis in presence of peripherally Selegiline Emsam -Reversible monoamines (transdermal) -Must be on tyramine-free diet = no wine, beer, cheese, aged food, or smoked meats -Overdose is lethal -2 week washout period of other antidepressants needed before starting in order to prevent serotonin syndrome Mood Stabilizers Carbamazepine Tegretol -MOA: antiepileptic; inhibits voltage-gated Na channels -AEs: diplopia, dizziness, drowsiness, nausea, Stevens-Johnson (don’t use in Asians), hypoCa, hypoNa, SIADH, hematologic, hepatitis  monitor CBC, LFTs, mental status, bone density, levels -Contraindicated with bone marrow depression -Decreases effectiveness of OCPs and warfarin -Pregnancy D Valproate Depakene -MOA: antiepileptic; increases GABA Depakote -AEs: GI upset, sedation, unsteadiness, tremor, thrombocytopenia, palpitations, immune hypersensitivity, ototoxicity  monitor CBC and LFTs and levels -Contraindicated with liver disease -Many drug interactions -Pregnancy D Lamotrigine Lamictal -MOA: blocks voltage-gated Na channels and inhibits glutamate release -AEs: nausea, diplopia, dizziness, unsteadiness, HA, rash, Stevens-Johnson, hematologic, liver failure -Overdose can be fatal -Interaction with valproate -Pregnancy C Lithium Eskalith -Inhibits adenylate cyclase Lithobid -AEs: diabetes insipidus, cognitive complaints, tremor, weight gain, sedation, diarrhea, nausea, hypothyroidism -Many drug interactions -Requires baseline BMP, TSH, EKG, Ca as well as monitoring of BMP and TSH q 6-12 mo -Monitoring for signs of toxicity: nausea, tremor, polyuria, thirst, weight gain, diarrhea, cognitive impairment -Need to monitor levels -Pregnancy D for neural tube defects Gabapentin Neurontin -AEs: somnolence, dizziness, ataxia, fatigue, leukopenia, weight gain, Stevens-Johnson

172

Class & MOA Generic Agent Brand Info Benzodiazepines: Chlordiazepoxide Librium -Long-acting GABA-R agonists  -Used often during EtOH withdrawal CNS inhibition Clorazepate Tranxene -Long-acting Diazepam Valium -Long-acting Flurazepam Dalmane -Long-acting Alprazolam Xanax -Intermediate acting -Approved for panic disorder Clonazepam Klonopin -Intermediate acting -Approved for panic disorder Lorazepam Ativan -Intermediate acting Temazepam Restoril -Intermediate acting Oxazepam Serax -Short acting Triazolam Halcion -Short acting Other Anxiolytics Buspirone BuSpar -5-HT partial agonist -Gradual onset in 2 weeks -Does not potentiate effects of alcohol = useful in alcohols -Low addiction potential = good for pts who were addicted to benzos or other drugs -AEs: sexual, dizziness, nausea, HA -Drug interactions Typical Haloperidol (inj Haldol -Good for acute agitation as onset is 30 min Antipsychotics: avail) nonselective DA-R Fluphenazine Prolixin antagonists Perphenazine Trilafon Thioridazine Mellaril -AE: retinitis pigmentosa -Less risk of EPSEs Chlorpromazine Thorazine -Less risk of EPSEs Atypical Aripiprazole Abilify Antipsychotics: Asenapine (SL Saphris -Costs $$$ block postsynaptic tablet avail) DA-R, block Olanzapine (inj Zyprexa -High risk of weight gain and metabolic syndrome serotonin-R, variable avail) Zyprexa -Injectable can cause post-injection delirium  must give at healthcare facility and monitor for 3 hours effect on histaminic Relprevv (inj) and cholinergic-R Quetiapine Seroquel -Need q 6 month eye exams due to risk of cataracts Risperidone Risperdal -Least amount of AEs Consta (inj) -Highest risk of hyperprolactinemia Ziprasidone Geodon -AE: dose-related QT prolongation -Less wt gain Clozapine Clozaril -The only atypical antipsychotic proven effective in treatment of schizophrenia -Use limited by AEs: high risk of weight gain and metabolic syndrome, seizures, agranulocytosis, myocarditis, lens opacities  need to monitor WBC and ANC frequently Iloperidone Fanapt -Costs $$$ -Not proven better than other atypical antipsychotics

Lurasidone Latuda -Best choice for reversing metabolic effects

Paliperidone (inj Invega avail) Invega Sustenna (inj) 173

Management of Psychiatric Drug Adverse Effects Dystonias Parkinsonianism -Benztropine -Amantadine -Biperiden -Levodopa -Diphenhydramine -Trihexyphenidyl Extrapyramidal Symptoms -Parkinsonian syndrome, acute dystonias, akathisia Akathisias = restlessness -Benztropine -Propranolol -Benadryl -Benzos

ANXIETY DISORDERS Generalized Anxiety Disorder -An inappropriate response to a perceived threat Workup Differential Management or no adequate source for fear -DSM-IV criteria: -Adjustment disorder -Combination of psychotherapy and pharmacotherapy is most -More common in women; 30% lifetime risk 1. Excessive anxiety and worry about with anxious mood: fits effective -More common in high SES lots of things out of proportion to pts who have had sx < 6 -First-line is CBT or an SSRI or SNRI with a 6-8 week trial, treat for the likelihood or impact of feared mo that are associated at least a year Etiology events, occurring more days than not with a particular stressor -Second-line: -Likely a combination of genetic factors and for at least 6 mo or event within 3 months • buspirone: reduced abuse potential, less weight gain and environment 2. Worry is pervasive and difficult to of onset of sxs sexual AEs (should NOT be taken PRN) control -CV problem • benzos Screening 3. Anxiety and worry are associated -Excess caffeine or MSG • TCA such as imipramine: limited use due to AEs -GAD-7 has been validated as a primary care with 3+ of these sx: restlessness or intake or other stimulants -Third-line: tool on edge, easily fatigued, difficulty -Vitamin deficiency • hydroxyzine: use limited by sedation and lack of efficacy concentrating, irritability, muscle -Anemia, adrenal in comorbid diseases Signs & symptoms tension, sleep disturbance disease, or secreting • pregabalin -Palpitations 4. Anxiety, worry, or physical tumor • quetiapine -Diaphoresis symptoms cause significant stress or -Hyperthyroidism -For remaining insomnia, add on a non-benzo hypnotic, benzo, -Dizziness impairment socially, at work, or in -B12 deficiency trazodone, mirtazapine, or sedating hypnotic -Trembling other areas of life -Hypoxia -For inadequate response, switch antidepressants or add atypical -SOB or choking sensation -Labs to consider: CBC, BMP, TSH, UA with -Drugs: antidepressants, antipsychotic, benzo, antihistamine, or buspirone -Tingling of extremities tox screen, EKG in pts over 40 with associated penicillin, amphetamines -Somatic complaints such as muscle tension chest pain or palps -EtOH withdrawal Prognosis -Common comorbidity is major depression -Half will fully recover within a few years of therapy while half will continue to struggle with lifelong fluctuating symptoms

174

Panic Disorder -Periods of intense fear or apprehension that are of sudden onset and Signs & Symptoms Workup Management relatively brief duration -May not have inciting factor -DSM-IV criteria for panic attack: 4+ -Acute: benzodiazepines -Further specified as being with or without agoraphobia -Shaking and trembling symptoms that develop abruptly and -CBT ± pharmacotherapy -Choking sensation reach a peak within 10 minutes -1st line long-term management Etiologies -SOB -DSM-IV criteria for panic disorder: h/o SSRI or SNRI, 60-80% of pts -Caused by an overreaction to stimulation of the amygdala and -Diaphoresis, hot flashes, or chills panic attack + intense fear of having will be panic free after > 4 adrenal gland -Derealization and depersonalization another weeks -Common inciting factors: hyperventilation, breathing in and out of a -Chest pain and palpitations -BMP -2nd line therapy with paper bag, caffeine, nicotine -Persistent concern about having another -Cardiac enzymes for suspected ACS benzodiazepines, studies show attack -TSH low risk of misuse in panic Differential -Fear of dying or losing control -CBC disorder pts, alprazolam has the -SVT -Abdominal pain -Urine tox screen most data with 55-75% of pts -Angina pectoris -Paresthesias -D-dimer panic free after 1 week -CHF -Can occur up to several times per week, with -ABG or VBG shows primary respiratory -Hyperthyroid waxing and waning of frequency alkalosis from hyperventilation Prognosis -Pheochromocytoma -Medical comorbidities often present: HTN, -EKG -Untreated attack usually -Caffeine, nicotine, cocaine, amphetamines, pseudoephedrine asthma, mitral valve prolapse, IBS, interstitial subsides in 25 minutes -Other anxiety disorder: OCD, PTSD cystitis, migraines -Somatization Posttraumatic Stress Disorder -A response to a catastrophic life Signs & Symptoms Workup Management experience in which the pt re- -Marked cognitive, affective, and -Diagnosed clinically by presence of criteria at least one month after traumatic -CBT is first-line experiences the trauma, avoids behavioral responses to stimuli reminding event has passed -SSRI or SNRI 2nd line reminders of the event, and experiences them of the experienced trauma -DSM-IV criteria: -Atypical antipsychotics emotional numbing or hyperarousal -Avoidance, emotional numbing, and 1. Experienced a traumatic event that was potentially harmful or fatal and for refractory symptoms diminished interest in people and activities initial reaction was intense fear or horror -Prazosin for sleep Causes -Comorbid depression, substance use, and 2. Persistent re-experiencing of the event through dreams, flashbacks, or disruption or nightmares -Military combat somatization recollections -Sexual or physical assault 3. Avoidance of stimuli associated with the trauma Prognosis -Disasters Differential 4. Numbing of responsiveness: limited affect, feelings of detachment or -Half of pts will remain -Childhood sexual abuse -Acute stress disorder: meets criteria for estrangement from others symptom-free after 3 -Severe physical injury PTSD but has had symptoms < 1 month 5. Persistent symptoms of increased arousal: difficulty sleeping, anger months of treatment -ICU hospitalization outbursts, exaggerated startle response, difficulty concentrating Obsessive-Compulsive Disorder -Obsession = recurrent and intrusive thought, Signs & Symptoms Workup Management feeling, or idea -Common obsessions: -DSM-IV criteria: -1st line: CBT using exposure and response prevention and/or -Compulsion = a conscious repetitive behavior contamination, doubt/repeated 1. Obsessions and/or compulsions SSRI (may need HIGH doses!) linked to an obsession that functions to relieve checking of objects for safety, 2. Person is aware that the -Augment nonresponders with an antipsychotic or TCA anxiety caused by the obsession symmetry, intrusive sexual or obsessions and compulsions are -Deep brain stimulation for refractory cases is showing -Onset is usually in adulthood violent thoughts unreasonable and excessive promising results -Common psych comorbidities 3. Obsessions cause marked distress, Causes include MDD, eating disorders, are time-consuming, or Prognosis -Genetic predisposition other anxiety disorders, and significantly interfere with daily -20-40% of pts will remain significantly impaired from their -Stressful life event triggers many cases obsessive-compulsive personality functioning symptoms -Abnormal serotonin levels disorder

175

Phobias -The most common mental disorders in the US Workup Management -An irrational fear that leads to avoidance of feared object or -DMS-IV criteria for phobia: -Pharmacologic treatment of specific phobias is situation 1. Persistent, excessive fear brought on by a specific situation or not effective -Specific phobia = fear of specific object or situation object -Systemic desensitization ± benzos during -Social phobia (social anxiety disorder) = fear of social situations 2. Exposure to the situation brings about an immediate anxiety session in which embarrassment can occur response -Supportive psychotherapy 3. Pt recognizes that this fear is excessive -Paroxetine effective for social anxiety disorder Causes 4. Situation is avoided when possible or tolerate diwth intense anxiety -β-blockers for performance anxiety -Likely a combination of genetic, behavioral, and neurochemical 5. Duration must be > 6 mos of < 18 years old factors

EATING DISORDERS -Anorexia and bulimia are more common in middle and Screening Management upper-class families -SCOFF screen useful in -Intervention designed to decrease shame, validate patient feelings, assess social supports, encouragement primary care, considered to of patient honesty and openness, inform about available resources, and affirm provider willingness to Etiology: combination of psychological, social, and be 100% sensitive: sick, provide ongoing support biologic factors control, “one stone”, fat, -Psychotherapy: individual and family -Psych: perfectionism, high expectations, need for food  2+ points suggest -Regular medical visits control, people pleasing, hypersensitivity to real or eating disorder -Admit for: weight loss > 20% ideal, unresponsiveness to outpatient therapy, rapid weight loss, perceived rejection hypovolemia, electrolyte abnormalities, malnutrition, severe depression or suicidality -Social: over-valuing thinness, sexualization of women, Workup restricted expression of emotion, familial emphasis on -EKG Prognosis weight control, high amount of life stressors -BMP, TSH, vitamin levels -Complication of refeeding syndrome, when shift from fat to CHO metabolism causes ↓ P  depletion of -Biologic: genetic influences, serotonin imbalance in -DEXA intracellular ATP and tissue hypoxia  impairment of myocardial contractility  CV collapse, seizures, bulimia, comorbid major depression or bipolar disorder in delirium, or rhabdomyolysis bulimia -Complication of Wernicke’s encephalopathy, prevented with thiamine supplementation Anorexia Nervosa -Far more common in women, in developed countries, and Other signs & symptoms Differential Management in professions requiring a thin physique -Low body weight -Malignancy or other medical condition causing -Inpatient needed if < 20 below ideal -Preoccupation with food cachexia body weight Restrictive type anorexia nervosa -Social withdrawal -Major depression -Refer for psychotherapy: family -Eat very little -Frequent weighing -Bulimia therapy -Exercise vigorously -Fatigue -Somatization disorder -Meds only after weight is restored: -More often withdrawn with obsessive-compulsive traits -Hair loss -Schizophrenia atypical antipsychotics, tricyclics, -Cessation of menses SSRIs, Li, anxiolytics before eating Binge eating & purging type anorexia nervosa -Sensitivity to cold Workup -Eat in binges followed by purging, laxatives, excessive -Serious: arrhythmia, dehydration, -DSM-IV criteria: Prognosis exercise, or diuretics malnutrition, hypotension, bradycardia, 1. Body weight at least 15% below normal -Mortality of 5-20% -Melanosis coli: darkening of colon secondary to laxative reduced bone density, heart failure, 2. Intense fear of gaining weight or becoming -50% will have good results, 25% abuse dental problems, hypothermia, fainting, fat intermediate, 25% poor -Comorbid major depression or substance abuse lanugo 3. Disturbed body image -Average duration of illness is 5.9 4. Amenorrhea years

176

Bulimia Nervosa Purging type bulimia nervosa Signs & symptoms Workup Management -Use vomiting, laxatives, or -Normal weight or overweight -DSM-IV criteria: -Psychotherapy diuretics to counteract binge -Patients are embarrassed by bingeing 1. Recurrent episodes of binge eating -SSRIs or TCAs eating -Over-concern with body weight 2. Recurrent, inappropriate attempts to compensate for -Esophagitis overeating and prevent weight gain Prognosis Nonpurging type bulimia -Dental erosion 3. Binge eating and compensatory behaviors occur at least twice -Better than anorexia nervosa nervosa -Callused knuckles a week for 3 months -May have relapse in times of -Use excessive exercise or -Salivary gland hypertrophy 4. Perception of self worth is excessively influenced by body stress fasting to counteract binge -Comorbid mood disorder, impulse control weight and shape -Half will recover fully and half eating disorder, or alcohol abuse -Chem panel shows hypochloremic hypokalemic alkalosis will be chronic with fluctuating symptoms

Binge-Eating Disorder Binge-eating = eating an excessive Workup amount of food in a 2 hour period with -DSM-IV criteria: associated lack of emotional control 1. Recurrent episodes of binge eating 2. Severe distress over binge eating Signs & symptoms 3. Bingeing occurs at least 2 days a week for 6 months and is not associated with compensatory behaviors -Emotional distress associated with 4. 3+ of the following are present: eating very rapidly, eating until uncomfortably full, eating large amounts when not hungry, eating alone due to overeating embarrassment over eating habits, feeling disgusted, depressed, or guilty after overeating -No use of laxatives or vomiting Management -Often obese -Psychotherapy -Comorbid mood or anxiety disorder -Strict diet and exercise program -Pharmacotherapy to support weight loss: stimulants, orlistat, or sibutramine (Meridia)

Obesity -Overweight = BMI 25-29.9 Bariatric surgery -Obesity = BMI > 30  greater risk of DM, stroke, CAD, early death -NIH recommends limiting to patients with BMI > 40, or > 35 if obesity complications are present Pharmacologic options -Results in significant reduction in deaths from obesity -Catecholaminergics (phentermine, diethylpropion, mazindol): short-term use only -Options: adjustable “lap band”, sleeve gastrectomy, Roux-en-Y bypass -Orlistat: inhibits lipase

177

MOOD DISORDERS -Mood disorders can be further classified with specifiers -“Catatonic features” = prominent psychomotor disturbances -“Psychotic features” = delusions or hallucinations -“Postpartum onset” = onset of mood episodes within 4 weeks of childbirth Major Depressive Disorder -“Episodes” are classified as mild, moderate, or severe and Screening Workup Management are defined by presence of symptoms for at least 2 weeks -USPSTF grade B for -MMSE -Referral to psychiatry indicated for pts with -A pt has a “disorder” when there is a pattern of episodes nonpregnant adults as long as -TSH, CMP and Ca to r/o metabolic disturbance, severe depression, depression unresponsive -Half of cases are missed by PCPs there are supports in place for RPR, CBC to r/o systemic infection, folate, vit D, to initial treatment, psychotic depression, -First episode most common in ages 30-40, with smaller peak effective treatment vit B12, drug screen depression with other psychiatric diagnosis at 50-60 -PHQ-2 or PHQ-9 -DSMI-IV diagnostic criteria for major depressive -Psychotherapy recommended in -Geriatric depression scale episode is 5+ of the following symptoms present combination with pharmacotherapy for Subtypes nearly every day for a minimum of 2 weeks (at patients with severe chronic or recurrent -Melancholic depression: loss of pleasure in most activities, Differential least 1 symptom must be depressed mood or loss depression nonreaction to pleasurable stimuli, worsening of sx in early -Dysthymia of interest or pleasure) -Exercise morning hours, psychomotor retardation, excessive weight -Adjustment disorder with • Depressed mood -Melancholic subtype responds best to loss or guilt depressed mood • Loss of interest or pleasure in most or TCAs or MAOIs -Atypical depression: mood reactivity, weight gain, excessive -Minor depressive disorder all activities -Atypical subtype responds best to SSRIs or sleep, heavy sensation, significant social impairment -Bipolar disorder • Insomnia or hypersomnia SNRIs -Catatonic depression: rare form involving disturbances of -Dementia • Change in appetite or weight gain -For refractory depression, confirm dx and motor behavior -Normal grief response • Psychomotor retardation or agitation medication adherence and r/o organic -Postpartum depression: intense, sustained depression -Anxiety disorder • Low energy causes of depression, switch to another experienced within 1 month of giving birth -Domestic violence antidepressant or augment, and consider less • Poor concentration -Seasonal affective disorder: resolves in spring -Hypothyroidism common regimens such as tranylcypromine • Thoughts of worthlessness or guilt -Major depression with psychotic features: delusions or -Parkinsonism or venlafaxine + mirtazapine • Recurrent thoughts about death or hallucinations are present -Stroke -Electroconvulsive therapy is last resort suicide -Drug-induced depression -DSM-IV criteria for MDD: Etiology -Cushing’s syndrome Prognosis • -Multifactorial, involving biologic, psychologic, and social -Borderline personality disorder At least 1 major depressive episode -15% will attempt suicide factors • No h/o mania or hypomania Related Disorders Dysthymia Adjustment disorder with depressed mood -A chronic, milder mood disturbance that has been present for at least 2 years -Occurs when identifiable psychosocial stressor(s) that has occurred within the last 3 months can be attributed to depressed mood Minor depressive disorder -Depressed mood resolves within 6 months after the stressor has ended -Pt meets criteria for 2-4 of MDD criteria -However, if pt meets criteria for MDD this diagnosis will supersede dx of adjustment disorder Pharmacologic Management -Effectiveness of SSRIs, SNRIs, bupropion, TCAs, and MAOIs is generally comparable -May need to use doses higher than what is FDA approved -Begin dose at lowest effective dose and increase incrementally until patient achieves remission -Duration of treatment should be continued until 4-9 months after remission of symptoms for 1st -Switch after 8 weeks of therapy if no response episode, an additional year after that for 2nd episode, and possibly continue treatment indefinitely -If partial response at 8 weeks, can switch or augment therapy with bupropion or buspirone for 3rd episode or more

178

Postpartum Depression -Can occur in women or men Screening Workup -Mood changes will develop in 40-80% of women postpartum -Edinburgh Postnatal Depression Scale -DSM-IV criteria is depression symptoms > 2 weeks with onset and are normal as long as duration is < 2 weeks within 4 weeks of childbirth Signs & Symptoms Risk Factors -Sadness and crying episodes Management -Formula feeding -Fatigue -Attention to infant by other family members or friends -H/o depression -Changes in sleeping and eating habits -Support groups or counseling, home visits -Cigarette smoking -Reduced libido -Psychotherapy -Childcare stress or low social support -Irritability -Healthy diet and sleep patterns -Infant colic -Feelings of hopelessness and low self-esteem -Meds recommended only if support and adequate rest fail to -Low SES -Guilt improve symptoms: sertraline or paroxetine -Unplanned pregnancy -Feeling overwhelmed and inadequate in caring for infant -Inability to be comforted Prognosis -Anhedonia and social withdrawal -Can last several months to a year if untreated -Anxiety and panic attacks -Anger spells Bipolar Disorder -Affects men and women equally Differential Workup -Mean age of onset is 19 -Major depressive disorder -DSM IV-TR criteria for mania are 3 (with elated mood-predominant) or 4 (with -Illness can be further specified as “rapid cycling” (4+ episodes per year, -ADHD irritable mood-predominant) of the following for at least 1 week: separated in time by remission or switching to opposite pole) or “seasonal -Schizophrenia • Inflated self-esteem or grandiosity pattern” -Hyperthyroidism • Decreased need for sleep -Substance abuse • ↑ Talkative or pressure speech Causes -Meds: steroids, thyroxine • Flight of ideas or racing thoughts -Genetic predisposition -CNS disease: tumor, neurosyphilis • Distractibility -Borderline or narcissistic personality • ↑ Goal-directed activity or psychomotor agitation disorder Signs & Symptoms • Excessive involvement in pleasurable activities without regard to -Buying sprees or sexual indiscretions negative consequences -Symptoms in younger children or adolescents will be much more vague Hypomania vs mania? -DSM IV-TR criteria for hypomania are the same as above but last at least 4 and shorter-lived = harder to diagnose in this age group and requires lots -Mania frequently includes psychotic days and are less severe than mania of experience! features and leads to hospitalization

-Most bipolar disorder pts will have at least 1 comorbid psychiatric illness while hypomania does not Prognosis -Neurocognitive deficits in attention, verbal memory, executive function, -Psychosocial functioning in -Rapid cycling subtype associated with poorer long-term course of illness and info processing speed hypomania is either mildly impaired -10-15% death rate by suicide -Suicide attempt or improved while it is markedly -Untreated manic episode usually lasts 3 months and will relapse more -Aggressive behavior impaired in mania frequently as the disease progresses Types of Bipolar Disorder Bipolar I Disorder Bipolar II Disorder Cyclothymic Disorder Bipolar Disorder NOS -1+ manic or mixed episodes -1+ major depressive episode AND 1+ hypomanic -Numerous periods of: -Includes any other pt that -May also have MDD but not required episode • hypomanic episodes for at least 2 years does not meet criteria for -Episodes not better accounted for by -No manic or mixed episodes • depressive sx that don’t meet criteria for bipolar I or II or cyclothymic schizophrenia, delusional disorder, or psychotic -Episodes not better accounted for by schizophrenia, MDD disorder disorder NOS delusional disorder, or psychotic disorder NOS  “bipolar II disorder lite”

179

Management of Bipolar Disorder -Regimen is selected according to phase of the illness pt is Maintenance therapy Acute mania, mixed episodes, or hypomania currently experiencing (acute depression, acute mania or -Goals are to reduce symptoms, delay or prevent new episodes, reduce risk -D/c any antidepressants hypomania, mixed episodes, or maintenance therapy) of suicide, and promote psychosocial functioning -Severe manic or severe mixed episode  (Li or -Pts need to be aware of teratogenicity of drug regimens -Li monotherapy is DOC: rapid absorption, steady state within 3-5 days valproate) + antipsychotic is treatment of choice; switch (ordered from greatest to least teratogenic): valproate > after dose changes, however only 50-60% of pts will respond antimanic if not working; ECT is last resort after 4-6 carbamazepine > Li > lamotrigine > antipsychotics > -2nd line is anticonvulsants or antipsychotics: lamotrigine, risperidone, failed drug therapy trials antidepressants aripiprazole, divalproex, quetiapine, or olanzapine -Hypomania, mild-mod mania, or mixed episode  -Refractory or inadequate response  add a 2nd med risperidone or olanzapine monotherapy Antidepressants -Psychotherapy can also help prevent recurrences and hospitalizations -ECT an option -Efficacy of antidepressants for bipolar disorder is  Most pts will require maintenance therapy for years, and some require it uncertain for life Acute depression -No evidence for use as a maintenance therapy  Li use requires baseline BMP, TSH, EKG, Ca as well as monitoring of -1st line: quetiapine, Li, lamotrigine, or valproate -Certain classes may incite switch to mania BMP and TSH q 6-12 mo; monitoring for signs of toxicity (nausea, tremor, -Dietary supplementation with omega 3 FAs has been -Should never be used as monotherapy, but in conjunction polyuria, thirst, weight gain, diarrhea, cognitive impairment) shown to be effective in improving depressive symptoms with antimanic or 2nd gen antipsychotic

PERSONALITY DISORDERS -Involve deeply ingrained, inflexible patterns of relating to others that are maladaptive and cause -Genetic predisposition significant impairment in social or occupational functioning -These are Axis II diagnoses -Pts lack insight into their problems Cluster A Personality Disorders -Pts are eccentric, peculiar, withdrawn -FH of psychotic disorders Schizoid Personality Disorder Signs & symptoms Workup Management -Lifelong pattern of social withdrawal -DSM-IV criteria: pattern of voluntary social withdrawal and restricted -Psychotherapy is treatment of -Reclusive range of emotion beginning by early adulthood and present in a variety choice -Quiet and unsociable of contexts; with at least 4 of the following present: -Low-dose antipsychotics if -Restricted affect • Neither enjoying nor desiring close relationships transiently psychotic -No desire for close relationships • Generally choosing solitary activities -Antidepressants if comorbid -Prefer to be alone • Little interest in sexual activity with another person major depression is diagnosed • Taking pleasure in few activities Differential • Few close friends and confidants Prognosis -Paranoid schizophrenia: will have fixed delusions • Indifference to praise or criticism -Usually chronic -Schizotypal personality disorder: will have eccentric behavior or magical thinking • Emotional coldness, detachment, or flattened affect Paranoid Personality Disorder Signs & symptoms Workup Management -Pervasive distrust and suspiciousness of others -DSM-IV criteria: must begin by early adulthood and be present in a variety of contexts; with at -Psychotherapy is treatment of choice -Usually interpret motives as malevolent least 4 of the following present: -Anxiolytics -Blame own problems on others • Suspicion without evidence that others are exploiting or deceiving pt -Short course of antipsychotics -Angry and hostile • Preoccupation with doubts of loyalty or trustworthiness of acquaintances • Reluctance to confide in others Prognosis Differential • Interpretation of benign remarks as threatening or demeaning -May eventually become -Paranoid schizophrenia: will have fixed delusions • Persistence of grudges schizophrenic and are psychotic while paranoid personality disorder • Perception of attacks on pt character that are not apparent to others; quick to counterattack -Chronic, lifelong job and marital may only have transient psychosis issues • Recurrence of suspicions regarding fidelity of spouse or lover 180

Schizotypal Personality Disorder Signs & symptoms Workup Management -Eccentric behavior -DSM-IV criteria: a pattern of social deficits marked by eccentric behavior, cognitive or perceptual distortions, and -Psychotherapy is treatment -Peculiar thought patterns discomfort with close relationships, beginning by early adulthood and present in a variety of contexts; 5+ of the of choice following must be present: -Short course of low-dose Differential • Ideas of reference (but not delusions of reference) antipsychotics for transient -Paranoid schizophrenia: will have • Odd beliefs or magical thinking inconsistent with cultural norms (clairvoyance, telepathy, bizarre fantasies, psychosis psychotic features but schizotypal won’t superstitious beliefs) unless under high stress (will be transient) • Unusual perceptual experiences Prognosis -Schizoid personality disorder: won’t • Suspiciousness -Course is chronic have eccentric behavior like schizotypal • Inappropriate or restricted affect -Pts may eventually develop • Odd or eccentric appearance or behavior (cults, strange religious practices) schizophrenia • Few close friends or confidants • Odd thinking or speech • Excessive social anxiety Cluster B Personality Disorders -Pts are emotional, dramatic, or impulsive -FH of mood disorders -Often utilize splitting people/things into good & bad as a coping mechanism Borderline Personality Disorder Signs & symptoms Workup Management -Pattern of instability of interpersonal relationships -DSM-IV criteria: 5+ of the following present in a variety of contexts by early -If comorbid Axis I disorder is present, BPD should -Poor self-image adulthood: be treated first as remission of both disorders often -Unstable affect • Frantic efforts to avoid real or imagined abandonment depends upon successful treatment of BPD -Impulsivity • A pattern of unstable and intense interpersonal relationships characterized -Psychotherapy for at least 20 weeks -Common comorbidities are mood, anxiety, and by alternating between extremes and idealization and devaluation -Consider mood stabilizer for lability, inappropriate substance use disorders • Markedly and persistently unstable self-image or sense of self anger, impulsivity, or aggression -May have suicide attempts • Impulsivity in at least 2 areas that are potentially self-damaging -Consider antipsychotic for perceptual disturbances • Recurrent suicidal behavior, gestures, or threats, or self-mutilating -Consider treating comorbid depression, PTSD, or Differential behavior panic disorder with antidepressants -Mood disorder • Marked reactivity of mood -PTSD • Chronic feelings of emptiness Prognosis -Dissociative identity disorder -Rate of remission is 50% after 4 years and 90% • Inappropriate, intense anger or difficulty controlling anger -Other personality disorders after 10 years • Transient, stress-related paranoid ideation or severe dissociative -Schizophrenia: will have frank psychosis whereas BPD -Remission associated with eventual improvement symptoms may decompensate into transient psychosis during stress in psychosocial functioning

181

Antisocial Personality Disorder -Higher incidence in poor urban areas and in prisoners Workup Management -Genetic component -DSM-IV criteria: pattern of disregard for others and violation of rights of -Psychotherapy is treatment of others since age 15; must be at least 18 years old; history of behavior as choice Signs & symptoms adolescent must be consistent with conduct disorder; additionally 3+ of the -Caution use of pharmacotherapy -Refuse to conform to social norms following are present: for anxiety or depression due to -Lack remorse for actions • Failure to conform to social norms by committing unlawful acts high addiction potential for these -Impulsive and deceitful • Deceitfulness or repeated lying or manipulation of others for patients -Run-ins with the law personal gain -Can appear charming and normal to new acquaintances • Impulsivity or failure to plan ahead Prognosis -Comorbid substance abuse or depression • Irritability and aggressiveness, repeated fights or assaults -Usually chronic -May have somatic complaints • Recklessness and disregard for safety of self or others -May have improvement of • Irresponsibility or failure to sustain work or honor financial symptoms with aging Differential obligations -Drug abuse • Lack of remorse for actions Histrionic Personality Disorder -More common in women Workup Management -DSM-IV criteria: pattern of excessive emotionality and attention -Psychotherapy is Signs & symptoms seeking, present by early adulthood and in a variety of contests, with at treatment of -Attention-seeking behavior least 5 of the following present: choice -Excessive emotionality • Uncomfortable when not the center of attention -Dramatic, flamboyant, and extroverted • Inappropriate seductive or provocative behavior Prognosis -Unable to form long-lasting, meaningful relationships • Uses physical appearance to draw attention to self -Chronic course -Sexually inappropriate and provocative • Has speech that is impressionistic and lacking in detail -May improve • Theatrical and exaggerated expression of emotion with age Differential • Easily influenced by others or situations -Borderline personality disorder: more likely to suffer from depression and suicide attempts, where • Perceives relationships as being more intimate than they histrionic personality disorders are generally more functional really are Narcissistic Personality Disorder Signs & symptoms Workup Management -Sense of superiority -DSM-IV criteria: pattern of grandiosity, need for admiration, and lack of empathy begging -Psychotherapy is treatment -Need for admiration by early adulthood and present in a variety of contexts with 5+ of the following present: of choice -Lack of empathy • Exaggerated sense of self-importance -Antidepressants or lithium -Consider themselves to be “special” • Preoccupied with fantasies of unlimited money, success, brilliance, etc. may be used for comorbid -Will exploit others for own gain • Believes that they are “special” or unique and can only associated with other high- mood disorder -Have an underlying fragile self-esteem status individuals -Become depressed when they don’t get the recognition they feel they • Needs excessive admiration Prognosis deserve • Has a sense of entitlement -Chronic • Takes advantage of others for self-gain -Higher incidence of depression and midlife Differential • Lacks empathy -Antisocial personality disorder: desire material gain or subjugation of crises • Envious of others or believes others are envious of them others while NPD desires status and recognition • Arrogant or haughty

182

Cluster C Personality Disorders Avoidant Personality Disorder Signs & symptoms Workup Management -Intense fear of rejection -DSM-IV criteria: a pattern of social inhibition, hypersensitivity, and -Psychotherapy with -Avoid situations where rejection may occur feelings of inadequacy since early adulthood, with at least 4 of the assertiveness training -Avoidance of social interactions following: -β-blockers for -Seek jobs with little interpersonal contact • Avoids occupation that involves interpersonal contact due to autonomic symptoms -Desire companionship but are extremely shy and easily injured a fear of criticism and rejection of anxiety -Comorbid anxiety and depressive disorders • Unwilling to interact unless certain of being liked -SSRIs for comorbid • Cautious of interpersonal relationships depression Differential • Preoccupied with being criticized or rejected in social -Schizoid personality disorder: have no desire for companionship while APD desires being with situations Prognosis others but are too scared • Inhibited in new social situations because they feel -Usually chronic -Social phobia (social anxiety disorder): not an integral part of the individual’s personality and inadequate -Particularly difficult involves fear of embarrassment in a specific kind of situation whereas APD is an overall fear of • Believes they are socially inept and inferior during adolescence rejection • Reluctant to engage in new activities for fear of -Dependent personality disorder: aggressively seeks relationships whereas APD are slow to get embarrassment involved; both types can be clingy Dependent Personality Disorder -More common in women Workup Management -DSM-IV criteria: a pattern of submissive and clinging behavior due to -Psychotherapy Signs & symptoms excessive need to be taken care of, with at least 5 of the following present: -Treatment of -Poor self-confidence • Difficulty making everyday decisions without reassurance from comorbid anxiety or -Fear of separation others depression -Excessive need to be taken care of • Needs others to assume responsibilities for most areas of their -Allows others to make decisions for them life Prognosis -Feelings of helplessness when left alone • Can’t express disagreement because of fear of loss of approval -Usually chronic -Prone to depression, particularly after loss of person on whom they are dependent • Difficulty initiating projects because of lack of self-confidence -Symptoms may • Goes to excessive lengths to obtain support from others improve with age Differential • Feels helpless when alone -Avoidant personality disorder • Urgently seeks another relationship when one ends -Borderline personality disorder and histrionic personality disorder: inability to form long-lasting • Preoccupied with fears of being left to take care of self relationships while DPD usually have a long-lasting relationship with one individual Obsessive-Compulsive Personality Disorder -More common in men and in oldest children Workup Management -Familial predisposition -DSM-IV criteria: pattern of preoccupation with orderliness, control, and -Psychotherapy perfectionism at the expense of efficiency, present by early adulthood Signs & symptoms and in a variety of contests, with at least 4 of the following present: Prognosis -Pattern of perfectionism, inflexibility, and orderliness • Preoccupation with details, rules, lists, and organization such -Course is -Preoccupation with unimportant details causes inability to complete simple tasks in a timely fashion that the major point of the activity is lost unpredictable -Appear serious, stiff, and formal • Perfectionism that is detrimental to completion of task -Some will develop -Constricted affect • Excessive devotion to work OCD, -Successful professionally but with poor interpersonal skills • Excessive conscientiousness and scrupulousness about morals schizophrenia, or and ethics major depressive Differential • Will not delegate tasks disorder -OCD: have recurrent obsessions and compulsions while OCPD don’t; OCD is ego-dystonic while OCPD is ego- • Unable to discard worthless objects syntonic (aren’t aware of their problem) -Narcissistic personality disorder: achievements are motivated by status whereas OCPD patients are motivated • Miserly by the work itself • Rigid and stubborn 183

Personality Disorder NOS -Reserved for personality disorders that don’t fit into cluster A, B, or C Passive-aggressive personality disorder -Stubborn, inefficient procrastinators Other subtypes -Alternation between compliance and defiance -Depressive personality disorder -Passively resist fulfillment of tasks -Sadomasochistic personality disorder -Frequently make excuses for self -Sadistic personality disorder -Lack assertiveness -Attempt to manipulate others to do chores and errands -Frequently complain about own misfortunes -Treated with psychotherapy PSYCHOTIC DISORDERS -Psychosis involves a break from reality and involves delusions, perceptual disturbances, and/or disordered thinking

Differential -Psychosis secondary to general medical condition: CNS disease, endocrinopathies, nutritional deficiency, connective tissue disease, porphyria -Substance-induced psychosis -Delirium -Dementia -Bipolar disorder -Major depression with psychotic features -Schizophrenia, schizophreniform disorder, or schizoaffective disorder -Delusional disorder Psychotic Examples Definition S/S Disturbed Delusions -Fixed, bizarre, unrealistic beliefs not subject to rational argument and not accounted for by accepted cultural or religious beliefs thought -May be delusions of paranoia, grandeur, religion, nihilism, guilt, or somatic delusions content -Includes ideas of reference (a belief or perception that irrelevant, unrelated or innocuous phenomena in the world refer to a person directly or have special personal significance) and thought broadcasting (the belief that one’s thoughts can be heard by others) Paranoid thoughts -General mistrust or suspicion -Beliefs are plausible but false -Elaborate delusional systems Loss of ego boundaries -Perceived loss of boundaries between self and the environment Suicidal or homicidal thoughts Phobias Obsessions -Commonly about contamination, losing control, harm, unwanted sexual thoughts, religious, or perfectionism Compulsions -Washing and cleaning, checking, repeating, mental review of events o prevent harm, counting, cancelling or undoing, collecting, arranging Disturbed Tangentiality -Mental condition in which one tends to digress from the topic under discussion, especially by word association thought processes Loosening of -A disorder of thinking in which associations of ideas become so shortened, fragmented, and disturbed as to lack logical relationship associations Poverty of thought -A global reduction in the quantity of thought and thought perseveration where a person keeps returning to the same limited set of ideas

Thought blocking -When a person's speech is suddenly interrupted by silences that may last a few seconds to a minute or longer Abnormal Poverty of speech -A general lack of additional, unprompted content seen in normal speech speech Verbigeration -An obsessive repetition of meaningless words and phrases Mutism -Unwillingness or refusal to speak Neologisms -Making up words 184

Clang associations -A mode of speech characterized by association of words based upon sound rather than concepts Perceptual Illusions -Stimulus is real but is misinterpreted disturbances Hallucinations -Manufacturing a stimulus that is not really present -May be auditory (common with schizophrenia), visual or tactile (EtOH and opiate withdrawal), olfactory, or gustatory Postpartum Psychosis -Rare Signs & Symptoms Differential Management -Thought to be a manifestation of bipolar I disorder -Hallucinations, delusions, mania, -Postpartum depression -Increased risk of suicide and infanticide during psychosis = and/or depression with psychotic features hospitalization with separation from infant required Causes -Waxing and waning delirium -Schizoaffective disorder -1st line therapy is atypical antipsychotic -Genetic predisposition -Usually within 2 weeks of -Schizophrenia -Adjunct therapy with benzos -Recent d/c of Li or other mood stabilizer childbirth -Treat underlying disorder, may need antidepressants or mood stabilizers Schizophrenia -Rarely presents before 15 or after 45 Signs & Symptoms Differential Management -Associated with lower SES -Positive symptoms: -Major depressive or manic episode with psychotic features -Antipsychotic therapy is 1st line hallucinations, delusions, -Drug or substance abuse or withdrawal for acute and maintenance therapy Causes bizarre behavior, disordered -Dementia -Avoid clozapine and olanzapine -Strong genetic predisposition thought -Delirium unless symptoms are refractory due -Neurotransmitter abnormalities -Negative symptoms: -CNS disease to adverse effects -Seasonal theory: people born in winter and blunted affect, anhedonia, -Nutritional deficiency -Can augment antipsychotic early spring have higher incidence apathy, inattentiveness -Heavy metal poisoning treatment with CBT, social skills -Disheveled appearance -Endocrinopathy training, supported employment, Phases -Intact memory and -Other psychotic disorder: schizoaffective, schizophreniform, delusional and assertive community treatment 1.) Prodromal phase: functional decline that orientation disorder, brief psychotic disorder precedes the first psychotic episode, where pt -Concrete understanding of -Personality disorder Prognosis may become socially withdrawn, irritable, similarities or proverbs -Disease is usually chronic and have physical complaints, or newfound -Lacks insight into disease Workup debilitating interest in religion or the occult -Delusions may be bizarre or -DSM-IV criteria: illness causes significant social or occupational functional -40-50% remain significantly 2.) Psychotic phase: perceptual disturbances, non-bizarre and can be deterioration, duration of illness for at least 6 months, and 2+ of the following impaired after diagnosis delusions, disordered thought grandiose, paranoid, for at least 1 month: -Worse prognostic indicators: early 3.) Residual phase: occurs between episodes nihilistic, or erotomatic • Delusions onset, poor social support, negative of psychosis; marked by flat affect, social -Often has comorbid • Hallucinations symptoms, FH, gradual onset, male withdrawal, and odd thinking or behavior substance abuse • Disorganized speech sex, many relapses, poor premorbid • Grossly disorganized or catatonic behavior functioning • Negative symptoms Paranoid Type Schizophrenia Disorganized Type Catatonic Type Schizophrenia Undifferentiated Residual Type Schizophrenia Type Schizophrenia Schizophrenia -Highest functioning -Poor functioning -Rare -Has features of -Prominent negative -Older age of onset -Early onset -Motor immobility or excessive purposeless motor activity more than one symptoms -Preoccupation with 1+ delusions or -Disorganized speech -Extreme negativism or mutism schizophrenia -Minimal evidence of frequent auditory hallucinations and behavior -Peculiar voluntary movements or posturing type or none of positive symptoms -No predominance of disorganized speech, -Flat or inappropriate -Echolalia or echopraxia the types disorganized or catatonic behavior, or affect inappropriate affect

185

Related Disorders Schizophreniform disorder Brief psychotic disorder -Same criteria as schizophrenia but symptom duration is < 6 months -Rare -Manage with hospitalization, 3-6 mos of antipsychotics, supportive psychotherapy -Same criteria for schizophrenia but duration is 1 day to 1 month

Schizoaffective disorder Delusional disorder -DSM-IV criteria: -Delusions may be erotomanic, grandiose, somatic, persecutory, jealous, or • Meets criteria for MDD or manic episode or mixed episode mixed • Additionally has had delusions or hallucinations for at least 2 weeks in absence of mood disorder symptoms ( -DSM-IV criteria: = period of time where psychotic symptoms don’t coexist with mood symptoms; otherwise this would be • Nonbizarre, fixed delusions for at least 1 month (schizophrenia mood disorder with psychotic features) may be bizarre or nonbizarre) • However, mood symptoms are present for substantial portion of psychotic illness • Does not meet criteria for schizophrenia • Symptoms not attributable to general medical condition or drugs Functioning in life not significantly impaired -Manage with hospitalization, supportive psychotherapy, short-term antipsychotics, maintenance therapy with mood stabilizers or antidepressants

SOMATOFORM DISORDERS -Not all patients with somatization Differential Management of somatoform disorders have a true disorder, but it is more -Factitious disorder = intentionally faking physical or mental in order to assume -Investigate all symptoms likely to be a psychiatric illness if the sick role, without gain of external incentives, frequently present with wound -Don’t try to reason away symptoms as they are not a conscious process there are many organ systems healing problems, excoriations, infection, bleeding, hypoglycemia, and GI -Focus on care rather than cure: provide reassurance and schedule brief, involved in symptoms, if symptoms ailments, often have health care work experience or h/o abuse or neglect regular PCP visits that don’t coincide with symptoms fluctuate, if there is comorbid anxiety -Munchausen syndrome: a factitious disorder with predominantly physical -Insight-directed psychotherapy or depression, if symptoms lead to complaints, often demand specific meds, highly skilled at feigning symptoms -Hypnosis psychological or emotional gain, if necessitating hospitalization -Relaxation therapy symptoms are chronic, or if there is -Munchausen by proxy: intentionally producing symptoms in someone else who -Treat comorbid psychiatric conditions idiosyncratic response to meds is under one’s care in order to assume to sick role by proxy -Minimize polypharmacy and secondary gain -Malingering = intentionally faking or grossly exaggerating symptoms for an obvious incentive such as avoiding work or criminal prosecution, obtaining financial compensation, room and board, or obtaining medications -Depression -Panic disorder -Substance abuse Conversion Disorder -The most common somatoform disorder Differential -Increased incidence in women and low SES -Underlying medical cause (50% will eventually receive)

Signs & symptoms Workup -DSM-IV criteria: -Voluntary motor or sensory deficits that suggest neurologic condition but are medically 1. At least 1 neuro symptom unexplainable 2. Psychological factors are associated with initiation or exacerbation of symptom -Preceded by psychological distress, can have relapses 3. Symptom not intentionally produced -Typically there is onset of a dramatic but physiologically impossible condition such as shifting 4. Can’t be explained by medical condition or substance use paralysis, aphonia, blindness, deafness, feeling a lump in the throat, or pseudoseizures 5. Causes significant distress or impairment in social or occupational functioning -Pt is surprisingly calm and unconcerned when describing dramatic symptoms (“la belle 6. Not accounted for by somatization disorder or other mental disorder indifference”) 7. Not limited to pain or sexual symptoms -Focus is on one symptom rather than multiple in somatization disorder Prognosis -Comorbid schizophrenia, major depression, or anxiety disorders -Symptoms usually resolve within 1 month, 25% will have relapses

186

Somatization Disorder Hypochondriasis Other Somatoform Disorders -More common in females Signs & symptoms Body dysmorphic disorder -Greater prevalence with low SES -Prolonged, exaggerated concern about healthy and possible illness -Preoccupation with an imagined or exaggerated defect in physical appearance -Genetic and familial predisposition -Pt fear disease or are convinced one is present -Spend significant time trying to correct perceived flaws with makeup, derm -Misinterpret normal bodily symptoms as indicative of disease procedures, or plastic surgery Signs & symptoms -Waxing and waning of symptoms, with exacerbation when under -Comorbid depression, anxiety, or psychotic disorder -Multiple vague complaints involving many stress -SSRIs may reduce symptoms organ systems -Comorbid MDD or anxiety disorder -Long history of numerous office visits Somatoform pain disorder Differential -Pain in one or more sites associated with psychological factors that have an Workup -Somatization disorder: focus is on symptoms, whereas important role in the onset, severity, exacerbation, or maintenance of the pain -DSM-IV criteria: hypochondriacs focus on disease -Pain not due to medical disorder 1. At least 2 GI symptoms -Symptom is not intentionally produced or feigned 2. At least 1 sexual symptom Workup -SSRIs may be useful 3. At least 1 neuro symptom -DSM-IV criteria: 4. At least 4 pain symptoms 1. Fear of having a serious medical condition based on Undifferentiated somatoform disorder 5. Onset before age 30 misinterpretation of normal bodily symptoms -“Somatoform light” 6. Can’t be explained by general 2. Fears persist despite appropriate medical evaluation -For patients not fitting existing category criteria medical condition or substance use 3. Fears present for at least 6 months -Diagnostic criteria: 1+ medically unexplained physical complaints persisting for more than 6 months

SUBSTANCE USE DISORDERS Substance Abuse Substance Dependence -DSM-IV criteria: pattern of substance use leading to -AKA “addiction” although this is not considered a scientific term impairment or distress for at least 1 year with 1+ of -DSM-IV criteria: substance use leading to impairment or distress manifested by at least 3 in a 12-month period: the following manifestations: • Tolerance: the need for increased amounts of substance to achieve the desired effect or diminished effect if using the same amount of • Failure to fulfill obligations at work, substance school, or home • Withdrawal: the development of a substance-specific syndrome due to cessation of use that has been heavy and prolonged • Use in dangerous situations • Using substance more than originally intended • Recurrent substance-related legal problems • Persistent desire or unsuccessful efforts to cut down on use • Continued use despite social or • Significant time spent in getting, using, or recovering from substance interpersonal problems due to the • Decreased social, occupational, or recreational activities because of use substance use • Continued use despite subsequent physical or psychological problems Cocaine Abuse & Dependence -Cocaine blocks DA reuptake from Signs & symptoms of intoxication Withdrawal Management of acute intoxication the synaptic cleft  stimulant effect -Euphoria -Cocaine withdrawal symptoms last 1-2 weeks and are -Inpatient -HTN or hypotension predominately psychosocial -Benzos for mild agitation Signs & symptoms of abuse -Tachycardia or bradycardia -Malaise, fatigue, depression, hunger, constricted pupils, vivid -Haloperidol for severe agitation -Needle or track marks -Dilated pupils dreams, psychomotor agitation or retardation -Control HTN and arrhythmias -Nasal septal perforation -Weight loss -Psychomotor agitation or depression Differential Management of dependence -Chills -Amphetamine or PCP intoxication -Cocaine withdrawal can be managed -Diaphoresis -Sedative withdrawal outpatient -Respiratory depression -Tell patient to sleep off the crash -Seizures Workup -Psychotherapy -Arrhythmias -Cocaine can be detected in urine for 2-4 days, up to 14 for -TCAs -Tactile hallucinations heavy users (perpetuated by alcohol use as well) -DA agonists: bromocriptine, amantadine -MI or CVA from vasoconstriction 187

Amphetamine Abuse & Dependence -Classic amphetamines: dextroamphetamine (Dexedrine), Signs & symptoms of intoxication Workup methylphenidate (Ritalin), methamphetamine (crystal meth, speed)  -Similar to cocaine intoxication -UDS will be + for 1-2 days although most are not very sensitive cause release of DA from nerve endings  stimulant effects -Psychosis -Designer amphetamines: MDMA (ecstasy), MDEA (Eve)  release DA Management of acute intoxication and serotonin from nerve endings  stimulant effects as well as Withdrawal -Treat with lorazepam, activated charcoal, NS IVF hallucinogenic -Similar to cocaine Management of dependence Differential -Similar to cocaine -Cocaine or PCP intoxication Sedative or Hypnotic Abuse & Intoxication -Benzodiazepines, barbiturates (phenobarbital), and GHB (date rape drug) Withdrawal Management of acute intoxication -Potentiate effects of GABA -Autonomic hyperactivity: tachycardia, sweating, insomnia, -Activated charcoal anxiety, tremor, n/v, delirium, hallucinations -Flumazenil for benzo overdose Signs & symptoms of intoxication -Seizures -Alkalinize urine with sodium bicarb for barbiturate -Drowsiness overdose to facilitate renal clearance -Slurred speech Differential -Support respiratory status and BP -Incoordination and ataxia -Alcohol intoxication -Mood lability -Delirium -Impaired judgment Management of dependence -Nystagmus Workup -Can’t abruptly DC or risk death! -Respiratory depression -UDS will be + for 1 week -Tapered dose of long-acting benzo -Coma -Tegretol or valproate for seizures control Phencyclidine (PCP) Abuse & Dependence -AKA “angel dust” Signs & symptoms of intoxication Withdrawal Workup -NMDA gluatamate-R antagonist -Recklessness -No withdrawal syndrome is observed -UDS will be + for > 1 week -Dopaminergic neuron activator -Impulsiveness -May have “flashbacks” -Elevated CPK and AST -Similar to the anesthetic ketamine -Impaired judgment -Assaultiveness Differential Management of acute intoxication -Rotatory nystagmus -Acute psychosis -Monitor BP, temp, electrolytes -Ataxia -Schizophrenia -Acidify urine with ammonium chloride and -HTN  ICH, MI, or aortic dissection vitamin C to facilitate clearance -Tachycardia -Benzos to control agitation, muscle spasms, -Muscle rigidity and seizures -High pain tolerance -Haloperidol for severe agitation or psychosis -Seizures or coma with overdose -Hyperthermia -Hyponatremia -DIC -Rhabdomyolysis -Serotonin syndrome

188

Opiate and Opioid Abuse & Dependence -Opiates are naturally occurring Signs & symptoms of intoxication Differential Management of acute chemical compounds include opium, -Drowsiness -Sedative or hypnotic intoxication morphine, and codeine -N/v intoxication -Naloxone -Opioids are synthetic chemicals that -Constipation -Severe EtOH intoxication -May need respiratory support bind to these same receptors (heroin, -Slurred speech hydrocodone, hydromorphone, -Constricted pupils (except for meperidine which dilates!) Workup Management of dependence oxycodone, oxymorphone, -Seizures -Opioid metabolites can be -Withdrawal is NOT life- buprenorphine, fentanyl, methadone, -Respiratory depression detected within 4 days of last threatening tramadol, dextromethorphan, use or longer in chronic -Withdrawal can be treated meperidine (Demerol)) Withdrawal users (false + in rifampin, with clonidine and/or -We all have a small amount of -Begins in 8 hours and lasts up to 3 days: anxiety, insomnia, yawning, stomach cramps, quinolone, or poppy seed buprenorphine for moderate endogenous opiates (endorphins) that lacrimation, rhinorrhea, diaphoresis, vomiting, diarrhea, fever, chills, tremor, tachycardia, ingestion) symptoms or with methadone normally act on these receptors piloerection, HTN, seizures for severe symptoms

Hallucinogen Abuse -Includes psilocybin (shrooms), Signs & symptoms of intoxication Workup Sequelae mescaline (peyote), and lysergic acid -Perceptual changes -Hallucinogens are not detected on -Pts can experience diethylamide (LSD) -Pupillary dilation urine drug screen “flashbacks” and symptom -Dependence and withdrawal do not -Tachycardia recurrence later in life due to occur with hallucinogens -Tremors Management of acute intoxication reabsorption from lipid stores -Incoordination -Guidance and reassurance -Sweating -Pts can be put in a quiet room until -Palpitations symptoms abate, PRN lorazepam -LSD intoxication will have awake pt who is aware symptoms are drug-induced Marijuana (Ab)use -Active component is THC which acts on cannabinoid receptors in the brain Signs & symptoms of Discontinuation effects -No dependence or true withdrawal syndrome intoxication -Lasts 7-14 days or up to several weeks and has symptoms of sleep disturbance, -Euphoria irritability, and physical tension Medicinal uses -Impaired coordination -Depression -Mild tachycardia Workup -Antiemetic in cancer patients -Conjunctival injection -Cannabinoids can be detected in urine for 7-10 days in a casual user, 2-4 weeks -Appetite stimulant in AIDS pts -Dry mouth in a heavy user, and months in a chronic heavy user -Increased appetite Inhalant Abuse -Includes solvents, glue, paint thinners, Signs & symptoms of intoxication Discontinuation effects fuels, and isobutyl nitrates  act as -Impaired judgment -Irritability and hallucinations CNS depressants -Belligerence -N/v -No dependence or withdrawal -Impulsivity -Tachycardia syndrome -Perceptual disturbances -Lethargy Workup -Dizziness -Serum drug screen is + for 4-10 hours -Nystagmus -Slurred speech Management of acute intoxication -Euphoria -ABCs -Stupor or coma -Respiratory depression: can be fatal Management of abuse -Damage to CNS, PNS, liver, kidneys, and muscle with long-term use -Psychotherapy 189

Tobacco Dependence Screening Withdrawal symptoms Nonpharmacologic management -Readiness assessment: 5 -Onset of symptoms 2-3 hours -STAR: set quit date 2 weeks out, tell family and friends, anticipate challenges, remove tobacco products from environment A’s (ask about smoking after last tobacco use with peak -Assess dependence level and suggest dosing using Fagerstrom questionnaire status, give clear in 2-3 days, resolution in 1 -Nonpharmacologic methods: cold turkey, unassisted tapering, assisted tapering (QuitKey), formal cessation programs, aversion personalized advice, month after quitting therapy, acupuncture, hypnotherapy, massage therapy assess readiness to quit -Increased symptoms if > 25 -Electronic cigarette: recent safety issues with battery igniting, not FDA approved and barriers, assist with cigs per day, first cig within 30 quit resources, arrange min of waking, discomfort if Prognosis for quit date and f/u forced to refrain from smoking -Tobacco smoke increases risk of certain cancers: lung, laryngeal, oral cavity, esophagus, bladder, kidney, pancreas, uterus, and cervix Pharmacologic Management: Nicotine Replacement Therapy -Good combination therapy: nicotine patch + lozenge or gum, nicotine patch + nicotine inhaler, nicotine patch + bupropion SR -Increases likelihood of successful quit by 2-3x -Low abuse potential -Pt must not use any tobacco products while using -Most formulations are available OTC although minors need a Rx -Cautions: underlying CV disease (as nicotine causes ↑HR and BP), recent MI, serious arrhythmias, serious or worsening angina, pregnancy, lactation Transdermal patch Nicotine gum Lozenge Inhaler Nasal spray -Nicoderm -Nicorette, generics -Commit, Nicorette -Nicotrol -Nicotrol NS -Avoids first pass metabolism -Need to chew n’ tuck -Can’t chew, need to let large -Rx only -Rx only -Can’t cut patches as this causes -Can’t eat or drink for 15 before lozenge dissolve over 30 min -Benefit of hand-to-mouth behavior -Benefit of nicotine bolus that mimics nicotine evaporation and ↓ and after (although new mini lozenge will -Not meant to be inhaled all the way burst from cigs  fast reduction of effectiveness -Helps with acute ravings dissolve faster) into lungs cravings but ↑ abuse potential -Must remove patches before MRI -AEs: n/v, abd pain, hiccups, -Dose based on time to first -Open cartridge only potent for 24 hours -AEs: local nasopharyngeal irritation, to avoid burns mouth irritation, sore jaw, cigarette -AEs: mouth and throat irritation, cough runny nose, sneezing, cough, throat and -Not for relief of acute cravings unpleasant taste -Helps with acute craving relief -Caution with severe reactive airway eye irritation, HA -AEs: skin irritation, insomnia, -Contraindicated with TMJ, poor -AEs: mouth irritation or ulcers, disease -Caution with severe reactive airway nightmares or vivid dreams dentition, dentures abd pain, n/v/d, HA, palps disease Other Pharmacologic Management Bupropion Varenicline (Chantix) Other 2nd line -1-2 daily doses, starting 1 week prior to quit date to allow time for accumulation of norepinephrine -Blocks nicotine from cigs from binding therapies: and DA in the body -Begin 7 days before quit date, with differing doses throughout -Nortriptyline -Try at least 7 weeks before d/c treatment -Clonidine -Best option for patients with CV disease -AEs: insomnia, nausea, abnormal dreams, impaired driving or -AEs: insomnia, dry mouth, suicidality operating machinery, suicide risk (accounts for most cases of -Contraindications: pt with seizure disorder, pt with h/o anorexia or bulimia, pts undergoing abrupt d/c suicide attempt while undergoing smoking cessation), CV risk of ethanol or sedatives

190

Alcohol Abuse & Dependence -The most commonly abused substance in Differential for intoxication Outpatient management the US -Hypoglycemia -Brief intervention for nondependent -Strong genetic risk factors, with -Hypoxia alcohol abusers heritability similar to DM or HTN -Drug overdose -Alcoholics Anonymous -Associated health risks include HTN, a- -Ethylene glycol or methanol poisoning -Disulfiram (Antabuse) -Hepatic encephalopathy fib, cardiomyopathy, esophagitis, -Psychosis -Psychotherapy gastritis, upper GIB, pancreatitis, -Psychomotor seizures -SSRIs hepatitis, cirrhosis -Can consider outpatient management of -Related illnesses include pneumonia, Withdrawal dependence if there are no risk factors TB, and cancers of the breast, liver, -Triggered by abrupt cessation or reduction of intake in dependent individuals throat, and esophagus -Caused by new homeostatic set point of increased inhibitory tone (activates GABA-R) as well as inhibition Inpatient management -Alcohol activates GABA-R and of excitatory tone  alcohol tolerance where individuals retain arousal at concentrations of GABA that -Needed for possible withdrawal if there serotonin-R and inhibits glutamate-R would produce lethargy or coma in a normal adult is h/o seizures, delirium, mental -Onset in 12-24 hours after last drink, with peak intensity at 24-48 hours instability, suicidal or homicidal ideation, Screening -Lasts 4-7 days psychosis, unstable environment, or no -CAGE (2+ is positive): -Most serious form is delirium tremens; 5% of those hospitalized for EtOH withdrawal will develop support or transportation available 1. Wanted to cut back on drinking -Mortality with treated DT is ~5%; untreated 15-20% -Thiamine (given to prevent Wernicke’s 2. Annoyed by criticism of encephalopathy, especially before giving drinking glucose), folate, and B6 supplementation 3. Guilty about drinking -Supplement Mg (alcoholics chronically 4. Needed an eye opener deficient, also an essential cofactor in thiamine metabolism) Signs & symptoms -IV benzodiazepines every 10-15 minutes -Displays at risk drinking habits, defined PRN per CIWA protocol as >14 drinks per week for men or > 7 for -Phenobarbital or propofol for refractory women DTs -Wernicke-Korsakoff syndrome from untreated Wernicke’s encephalopathy: Prognosis impaired recent memory, anterograde -Wernicke-Korsakoff syndrome may be amnesia, confabulation irreversible

191

PERVASIVE DEVELOPMENTAL DISORDERS -Includes autism, Asperger’s syndrome, Rett’s syndrome, and childhood disintegrative disorder -Highly genetic basis with possible environmental factors -Now considered to be a biologic rather than psychologic disorder, more related to mental retardation Autism Differential (many of these can co-exist with autism) -Fragile X syndrome -Pervasive developmental disorder NOS: don’t meet -Rett syndrome (almost exclusively in girls): genetic mutation of MECP2 on X -Angelman syndrome criteria for autism or are still very young for diagnosis chromosome with initial normal development, then onset of loss of hand skills -Turner syndrome -Childhood disintegrative disorder: normal development and social development, decreasing rate of growth of head circumference, -William syndrome for first 2 years of life, then loss of previously acquired problems with gait or trunk movements, severely impaired language and -Asperger’s syndrome: will have normal language skills in language, social skills, bowel or bladder control, psychomotor development, seizures, and cyanotic spells and cognitive development play, or motor skills Screening Management Prognosis -MCHAT administered between 16-30 months -Goals are to maximize functioning, move child towards independence, and -Early detection and treatment can affect course of disease (usually at 18 mo well child check) improve quality of life -Applied behavioral analysis is the best tested method of autism treatment Signs & symptoms -Language therapy: focuses on pictures and visual communication -Markedly impaired eye contact (red flag: lack of -Social skills groups joint attention) -Occupational therapy to aid stimuli sensitivity -Failure to develop peer relationships -Gluten and casein-free diet -Not seeking to share enjoyment or interests (red -Consider meds to target specific symptoms: methylphenidate for inattention flag: doesn’t look up for approval by 2-3 years) or hyperactivity, risperidone for aggression and self-injury, fluoxetine for -Lack of social or emotional reciprocity repetitive behaviors or anxiety or depression, atypical antipsychotic or SSR -Delayed or absent spoken language without for dysregulated mood, melatonin for sleep disturbance attempt to compensate with gestures or mime (red flags: no words by 18 mo, no strings of words by 2 years) -Repetitive language -Inability to initiate and sustain conversation -Lack of spontaneous make-believe play appropriate for developmental level -Repetitive motor mannerisms (rocking, spinning) -Preoccupation with parts of objects -Strong fixations to objects or restricted interests (“little professor”) -Inflexible adherence to rigid routines -May also exhibit sensory seeking or avoidant behavior -Tantrums set off by noise or changes in routine -Comorbid mental retardation or seizure disorder

Workup -Diagnosis is clinical -Send for comprehensive medical evaluation after failed MCHAT or parental concern: basic language and developmental testing via a developmental pediatrician, psychologist, and speech therapist, audiology screen, genetic microarray testing 192

SEXUAL DISORDERS Paraphilia -Sexual disorders characterized by engagement in unusual Management sexual activities or preoccupation with unusual sexual -Insight-oriented psychotherapy, behavior therapy urges or fantasies for at least 6 months that cause -Antiandrogens in men impairment in daily functioning Prognosis Signs & Symptoms -Poor prognosis with early age of onset, comorbid substance abuse, high frequency of behavior, and related arrest causing presentation for -Most common paraphilias are pedophilia, voyeurism, and treatment exhibitionism -Good prognosis with self-referral for treatment, sense of guilt associated with behavior, and history of otherwise normal sexual activity in addition to the paraphilia Sexual Dysfunction Differential Management -Atherosclerosis -True sexual disorder: dual sex therapy, behavior therapy, -DM hypnosis -Pelvic adhesions  dyspareunia -Treat low testosterone -Low estrogen or testosterone or increased progesterone -Erectile disorder: yohimbine, sildenafil, vacuum pumps, -Med AEs: antihypertensives, anticholinergics, antidepressants, antipsychotics constrictive rings, prosthetic surgery -Substance abuse -Dyspareunia: gradual desensitization, muscle relaxation -Depression techniques -True sexual disorder: hypoactive sexual desire disorder, sexual aversion disorder, male erectile disorder, female sexual arousal -Vaginismus: vaginal dilators disorder, orgasm disorder, dyspareunia, vaginismus

Workup -Ask males about morning erections

INTELLECTUAL DISABILITY (MENTAL RETARDATION) -More common in males Causes Differential -85% of cases are mild -Most MR has no identifiable cause -Learning disorder -Genetic disorders: Down’s syndrome, fragile X syndrome Types -Prenatal infections and toxins: toxoplasmosis, syphilis, AIDs, alcohol or Workup -Profound MR: IQ <25 drug exposure, rubella, CMV, HSV -DSM-IV criteria: -Severe MR: IQ 25-40 -Perinatal complications: anoxia, prematurity, birth trauma 1. Significantly subaverage intellectual functioning with IQ ≤ 70 -Moderate MR: IQ 40-50 -Postnatal causes: hypothyroidism, malnutrition, toxin exposure, trauma, 2. Deficits in adaptive skills appropriate for age group -Mild MR: IQ 50-70 encephalitis or meningitis 3. Onset before age 18 Learning Disorders Causes Types Workup -Usually due to deficits in cognitive processing -Reading disorder: occurs in 4% of kids -DSM-IV criteria: (abnormal attention, memory, visual perception, -Mathematics disorder: occurs in 5% of kids 1. Achievement in reading, math, or written expression that is significantly lower etc.) -Disorders of written expression: occurs in 3-10% than expected for chronological age, level of education, and level of intelligence -Genetic factors of kids 2. Affects academic achievement or daily activities and can’t be explained by sensory -Abnormal development deficits, poor teaching, or cultural factors -Perinatal injury Differential -Neurologic or medical condition -Hearing or vision deficit Management -Remediation tailored to child’s specific needs

193

OTHER BEHAVIORAL & EMOTIONAL DISORDERS Child and Elder Abuse Differential Signs & Symptoms Workup Management -Child: coagulopathy, -Inconsistent history for mechanism of injury and injuries observed -“Skeletal survey” radiographs -Mandatory reporting of suspected salicylate ingestion, -Vague history or no history offered mandatory in children under 2 in whom child abuse and neglect to law vasculitis, Mongolian -Story changing abuse is suspected enforcement and DSS spots, complimentary or -Injury attributed to actions of siblings -Noncontrast CT for suspected inflicted -Parents must be made aware of alternative medicine -History inconsistent with child’s developmental stage or elder’s cognitive status head trauma claim and rationale and what will treatments, hereditary -Implausible history -Consult social worker and abuse happen next sensory autonomic -Evidence of poor caretaking specialist if available -Community-dwelling older adults neuropathies -Child who is too compliant with painful or disturbing examination of injuries -Photographs of injuries (may require covered in most states by Adult -Child who is pseudomature or withdrawn, passive, depressed, violent or hyperactive parental permission in some states) Protective Services -Sudden onset AMS not attributed to medical illness or poisoning -Older adults: pressure ulcers, dehydration, malnutrition, failure to get needed meds Sexual Assault Management -Evaluation and physical examination of victim by trained personnel -Evidence collection kit -Empiric STD treatment -Acute crisis counseling -Follow-up STD and pregnancy testing

Intimate Partner Violence (Domestic Violence) -Refers to actual or threatened psychological, Signs & Symptoms Management physical, or sexual harm by a current or former -Inconsistent explanation of injuries -Provider expression of empathy and continued ability to support and assist partner or spouse -Delay in seeking treatment or missed appointments patient -May begin or escalate during pregnancy -Frequent ED visits -Consult domestic violence advocate to explore resources -Late prenatal care -Caution with providing written materials that may be seen by perpetrator -Inappropriate affect -Don’t confront perpetrator -Overly attentive partners -Restraining orders have inconsistent effectiveness -Reluctance to be examined -Somatization Adjustment Disorder -An excessive, prolonged reaction to a stressful event or situation or combination Differential of situations serious enough to impair social and occupational functioning -PTSD: occurs in face of life-threatening event while adjustment disorder is a result of non life-threatening event -More common in females and adolescents -Further coded based on predominance of depressed mood, anxiety, disturbance of Workup conduct (aggression), or a combination of these -DSM-IV criteria: 1. Development of emotional or behavioral symptoms within 3 mos after a stressful event that produce Causes either severe distress in excess of what would be expected or significant impairment in daily -Relationship problems, financial difficulties, family conflict, school or work functioning changes, major life changes, health problems, divorce, death, moving, sexuality 2. Symptoms are not those of bereavement. issues 3. Symptoms resolve within 6 months after stressor has terminated.

Signs & symptoms Management -Common comorbidities: depression, anxiety, disturbance of conduct, eating -1st line is psychotherapy disorder -Treat associated symptoms: insomnia, anxiety, depression

194

Grief Normal grief Abnormal grief -Feelings of guilt and sadness -Feelings of severe guilt and worthlessness -Mild sleep disturbance and weight loss -Significant sleep disturbance and weight loss -Illusions of the deceased (visual or auditory) -Hallucinations or delusions -Attempts to resume daily activities and work -No attempt to resume activities -Symptoms resolve within a year -Suicidal ideation -Symptoms persist > 1 year Conduct Disorder Causes Workup Management -Genetic and psychosocial factors -DSM-IV criteria: a pattern of behavior that involves violation of the basic -Consistent reinforcement of firm rules rights of others or of social norms and rules, with at least 3 acts within the -Psychotherapy focused on behavior modification and problem-solving skills Signs & symptoms following categories in the past year: -Antipsychotics or Li for aggression -Comorbid ADD or learning disorders A. Aggression towards people and animals -SSRIs for impulsivity, irritability, and mood lability B. Destruction of property C. Deceitfulness Prognosis D. Serious violations of others -40% risk of developing antisocial personality disorder in adulthood Oppositional Defiant Disorder -Prevalence is ~20% in kids over 6 Workup Management -DSM-IV criteria: at least 6 months of negativistic, hostile, and defiant behavior during which at -Psychotherapy with behavior modification Signs & Symptoms least 4+ of the following are present: and problem-solving skills -Comorbid substance abuse, mood disorders, • Frequent loss of temper -Parenting skills training and ADD • Arguments with adults • Defying adults’ rules Prognosis Differential • Deliberately annoying people -Remits in 25% of children -Conduct disorder: involves violation of basic • Easily annoyed -May progress to conduct disorder rights of others while ODD does not • Anger and resentment • Spitefulness • Blaming others for mistakes or misbehaviors Suicide -In adolescents there are 50-100 attempts for Risk factors for ideation Workup Management every suicide -Psych disorder -Evaluate intent and lethality of plan -Ensure immediate safety -Hopelessness -Address precipitating events and life -Prior suicide attempt or threat circumstances -High impulsivity -Counseling -Substance abuse -Involvement of support systems

195

ADHD Etiology Differential Workup Diagnostic criteria -Genetic factors -Learning disability -Schools are federally mandated to perform appropriate evaluations at -Symptoms must be present and impair -Prenatal trauma or toxin exposure -Language or no cost to the family if a child is suspected of having a disability that function in more than one setting (school, -Neurochemical and neurophysiologic factors communication impairs functioning, but the waiting period can be months home, work) -Psychosocial factors disorder -Primary care toolkit available online via the NICHQ -Symptoms must persist for at least 6 -Autism spectrum -Psychometric testing is not necessary for routine evaluation for months Subtypes disorder ADHD and does not distinguish children with ADHD from those -Symptoms must present before the age of -Predominantly inattentive type -Anxiety disorder without ADHD but can be valuable in excluding other disorders and 7 -Predominantly hyperactive-impulsive type -Mood disorder pinpointing specific ADHD problem areas -Symptoms must be excessive for the -Combined type -Oppositional defiant -Specialist evaluation indicated for suspected intellectual disability, developmental level of the child (ie beyond disorder developmental disorder, learning disability, hearing or vision normal hyperactivity for a child’s age) Signs & symptoms -Conduct disorder impairment, history of abuse, severe aggression, seizure disorder, -Peak severity at 7-8 years -OCD continued dysfunction despite treatment Management -Hyperactivity: excessive fidgetiness, talking, -PTSD -Additional evaluations in speech and language, occupational therapy, -Methylphenidate (Ritalin) is first-line difficulty remaining seated, difficulty playing quietly, -Adjustment disorder and mental health as needed therapy frequent restlessness -Stressful home -DSM-IV criteria -Other CNS stimulants: -Impulsivity: difficulty waiting turns, blurting out environment 1. At least 6 symptoms involving inattentiveness, dextroamphetamine, pemoline answers, disruptive classroom behavior, intruding or -Inappropriate hyperactivity, or both that have persisted for at least 6 -Individual psychotherapy interrupting other’s activities, peer rejection, educational setting months -Parental counseling unintentional injury -Hearing or vision a. Inattention: problems listening, concentrating, -Group therapy to improve social skills -Inattention: forgetfulness, easily distracted, losing or impairment paying attention to details, or organizing tasks; -Reevaluation whenever symptoms change misplacing things, disorganization, academic easily distracted, often forgetful or worsen underachievement, poor follow-through with b. Hyperactivity-impulsivity: blurting out, -Treat comorbid anxiety, depression, and assignments or tasks, poor concentration, poor interrupting, fidgeting, leaving seat, talking learning disorders attention to details excessively -Teacher-reported symptoms should have a duration 2. Onset before age 7 Prognosis of at least 4-6 months! 3. Behavior inconsistent with age and development -Most cases remit in adolescence -Comorbid mood disorders, personality disorders, -20% will have symptoms into adulthood conduct disorder, or ODD

196

DERMATOLOGIC SYSTEM ECZEMATOUS ERUPTIONS Dermatitis -Substances are either irritants ( = not immunologically mediated) or allergens (type IV hypersensitivity) Management -May require sunlight acting on substance to cause the dermatitis -Trigger avoidance -Inflammation may be acute, subacute, or chronic -Topical or systemic steroids -Distinguish irritant from allergic dermatitis by provocation testing: apply substance to AC fossa twice daily for a -Emollients or other barriers week; contact urticaria 15-30 min after application suggest allergic etiology -Oral antihistamines -Patch testing is only indicated when dermatitis is chronic, recurrent, or deters work or life activities (this tests for type IV hypersensitivities rather than type I, which is what skin scratch tests check) -Does NOT include latex hypersensitivity as this is a type I reaction Allergic Contact Dermatitis Irritant Contact Dermatitis Atopic Dermatitis (Eczema) -Causes a killer T-cell response -Accounts for most cases of dermatitis -Inflammatory, acute or Signs & symptoms -Common allergens: metallic salts, plants -Common irritants: water, soaps, detergents, wet work, solvents, chronically relapsing, not -May have concomitant food allergy, asthma, or (poison ivy), fragrances, nickel, greases, acids, alkalis, fiberglass, dusts, humidity, chrome, lip contagious allergic rhinitis preservatives, formaldehyde, propylene licking or other trauma -Infantile phase: affects cheeks, forehead, scalp, and glycol, oxybenzone, bacitracin, neomycin, Etiology extensor surfaces of limbs; lesions are vesicular, bleached rubber, chrome, sorbic acid Signs & symptoms -Genetic predisposition edematous, weepy, and crusty -Acute with bullae, erythema, and sharp borders -Defects in skin barrier -Childhood phase: affected areas are less vesicular, Signs & symptoms -Chronic with poorly-demarcated erythema, scales, and pruritus function more papules and plaques that become lichenified -Acute with macules, papules, vesicles, and -Fissured, thickened, dry skin -Immune dysregulation -Post-pubertal phase: skin becomes thickened, dry, bullae -Usually palmar and lichenified, may affect dorsal surfaces as well as -Chronic with lichenification, scaling, flexural skin, dyshidrotic changes maybe present on fissures Workup the palms and soles -Uncommon on scalp, palms, soles, or other -Negative patch test thick-skinned areas that allergens can’t get -Healing proceeds without plateau on removal of the offending Workup through agent -Patch test to look for pustular reactions Dyshidrosis (Acute Palmoplantar Eczema or Pompholyx) -Recurrent, pruritic vesicular eruption affecting Differential Management the palms, soles, or both -Allergic contact dermatitis -Avoidance of irritants or -Bullous tinea: usually on the feet, unilateral, + KOH prep exacerbating factors Signs & symptoms -Irritant contact dermatitis -Super high potency or high -Acute eruption of intensely pruritic vesicles or -Atopic dermatitis potency topical steroids bullae that persist for weeks, desiccate, and -Herpetic infection: painful BID for 2 weeks resolve with desquamation -Autoimmune bullous disease -Oral steroids for severe -Episodes recur at intervals of 3-4 weeks for cases (prednisone with taper months or years Workup for 3 weeks) -Diagnosis is clinical -Refractory cases: -Can consider patch testing to check for component of allergic phototherapy, topical contact dermatitis tacrolimus

197

Lichen Simplex Chronicus (Neurodermatitis) -Thick, leathery, brownish Signs & symptoms Workup skin due to chronic itching -May begin with something that rubs or irritates the skin, or an insect bite -KOH scraping to exclude fungal cause and scratching -Cycle of scratching, itching, and thickening skin -Commonly found on the scalp, nape of neck, extensor forearms and elbows, Management vulva, scrotum, upper medial thighs, knees, lower legs, or ankles -Steroid cream -Pruritus worse when sitting still or quiet and may be nonexistent when patients -Antihistamines: Benadryl, are active hydroxyzine -Affected area may spread rapidly throughout rest of body -Anxiolytics: doxepin, clonazepam -May have associated atopic dermatitis or psoriasis -Comorbid anxiety or depression

PAPULOSQUAMOUS AND DESQUAMATION DISEASES Pityriasis Rosea -A common, benign self-limited dermatosis Differential -May have viral origins: HSV-7 or 8 -Secondary syphilis

Signs & symptoms Management -Typically asymptomatic other than skin presentation -Oral antihistamines and topical steroids -May have recent h/o infection with fatigue, HA, sore throat, lymphadenitis, -UVB phototherapy fever -Initially just one herald lesion that is raised with fine scale (“collarette scale”) -7-14 days later there are diffuse eruptions on trunk dermatomes that are salmon in white pts and hyperpigmented in black patients -Lesions may be pruritic at night or with heat

198

Classic Drug Eruptions Type Information Type Information

Drug-Induced -The most common drug eruption Urticaria & Mechanism can be type I hypersensitivity, or Exanthem -Thought to be a delayed type IV Angioedema via a non-IgE mechanism  = can be hypersensitivity reaction immediate, accelerated (hours later), or -Common offenders are antibiotics, delayed (days later) especially sulfa -Urticaria is mediated by mast cells in the -Described as morbilliform, macular, or superficial dermis papular eruptions -Angioedema is swelling of the deeper dermis -Eruptions begin in dependent areas (= and subcutaneous tissues that can be mediated lower than the heart) and then generalize by mast cells (although not always) and can -Onset is usually within 2-3 weeks of coexist with urticaria beginning new drug or within days if -Common offenders causing type I there was prior exposure hypersensitivity are antibiotics, especially -May have mucous membrane erythema, penicillins, cephalosporins, and sulfonamides pruritus, low grade fever -Common offenders causing a non-IgE type -Can progress to more serious reactions hypersensitivity are morphine and codeine such as S-J, toxic epidermal necrolysis, (“red man syndrome”) hypersensitivity syndrome, or serum sickness -Described as intensely pruritic , circumscribed, -D/c of offending drug usually results in resolution of rash in 7-14 days raised, and erythematous eruption with central -Drugs causing a morbilliform eruption should only be continued when pallor there is no alternative therapy available -Lesions may coalesce -Typically disappear over a few hours, although urticaria can manifest in a chronic form (present > 6 weeks) which is usually due to autoimmunity or chronic disease -Can be treated with H1 or H2 blockers, doxepin, glucocorticoids if acute, epinephrine, or around-the-clock antihistamines if chronic Anaphylaxis -A type I hypersensitivity Hypersensitivity -AKA drug-induced vasculitis, leukocytoclastic vasculitis, serum sickness, that affects multiple organs, Vasculitis serum sickness-like reaction, or allergic vasculitis including pruritus, urticaria, -Symptoms usually begin 7-10 days after exposure angioedema, laryngeal -ACR criteria: age > 16, use of a possible offending drug in temporal relation edema, wheezing, nausea, to the symptoms, palpable purpura, maculopapular rash, skin biopsy showing vomiting, tachycardia, neutrophils around an arteriole or venule sense of impending doom, -Other S/S include fever, urticaria, arthralgias, lymphadenopathy, low serum and occasionally shock complement levels, and elevated ESR -Common offenders include penicillins, cephalosporins, sulfonamides (including loop and thiazide diuretics), phenytoin, and allopurinol -Resolves after days to weeks, NSAIDS or steroids may be needed for more severe cases

199

Type Information Type Information Exfoliative -Begins with generalized eczema or Stevens- -Severe mucocutaneous eruptions Dermatitis morbilliform erythema that progresses Johnsons characterized by epidermal (Erythroderma) into chronic erythema and scale Syndrome & necrosis and sloughing of involving > 50% of the body surface Toxic Epidermal mucous membranes and skin area Necrolysis -Whether it is S-J or TEN -Can be caused by drugs as well as depends on % of body surface atopic dermatitis, malignancy, or involved, if > 10% it is S-J, if > psoriasis 30% it is TEN -Common offenders include penicillins, barbiturates, gold salts, arsenic, and mercury Erythema -A different condition than S-J Photosensitivity -Phototoxic variety: result of direct tissue Multiforme -An acute eruption with or cellular damage following UV distinctive target skin lesions irradiation of a phototoxic agent that has that tend to affect the distal been ingested or applied to the skin extremities including the palms (tetracyclines, thiazides, sulfonamides, and soles fluoroquinolones, NSAIDs, -Often caused by infections phenothiazines, griseofulvin, voriconazole, such as HSV or Mycoplasma retinoids, St. John’s wort) pneumoniae, but it has had -Photoallergenic variety: a delayed-type some reports with medication hypersensitivity reaction to an allergen whose antigenicity has changed after use UV exposure (sunscreens, antimicrobials, NSAIDs, fragrances, griseofulvin, quinolones, sulfonamides, ketoprofen, piroxicam)

Fixed Drug -A distinctive reaction characterized acutely by erythematous and Symmetrical -AKA baboon syndrome Eruption edematous plaques with a grayish center or bullae, dark Drug-Related -Rare postinflammatory pigmentation Intertriginous -Occurs hours to days after -Usually occurs on the lips, tongue, genitalia, face, and acral areas and Flexural administration of offending drug -Common Exanthem -Sharply demarcated V-shaped erythema offenders are in the gluteal, perianal, inguinal, or laxatives, perigenital areas, often with involvement tetracyclines, of at least one other flexural or barbiturates, intertriginous fold sulfonamides, -Common offenders are amoxicillin, NSAIDs, and ceftriaxone, penicillin, clindamycin, erythromycin, iodinate contrast, salicylates pseudoephedrine, valacyclovir -Treat with topical or systemic steroids

200

Hypersensitivity Syndrome -AKA drug reaction with eosinophilia and systemic symptoms (DRESS) or drug-induced hypersensitivity syndrome (DIHS) -Infection with HHV-6 may play a role -Reaction typically occurs 2-6 weeks after drug is first used -Usually begins with morbilliform or erythrodermic eruption with possible erythematous follicular papules, pustules, bullae, or purpura, as well as fever, hepatitis, arthralgias, lymphadenopathy, hematologic abnormalities, pneumonitis, renal failure, myocarditis, thyroiditis, and neurologic symptoms -Severity is dependent on length of time drug is continued -Can be life-threatening no some cases -Common offenders include phenytoin, carbamazepine, phenobarbital, sulfonamides, lamotrigine, valproate, allopurinol, minocycline, antidepressants, NSAIDs, ACEIs, beta blockers

Lichen Planus -Chronic mucocutaneous Signs & symptoms Management Prognosis disease of uncertain etiology -Can affect skin, nails, mucous membranes -Topical steroids -Disease may remit in 1-2 -May involve CD8 activation -Lichen planus of the scalp (lichen planopilaris) can cause -May need intralesional steroid injections for scalp or years or may be chronic against keratinocytes alopecia hypertrophic forms -Oral lichen planus is risk -May be related to hepatitis C -Cutaneous presentation: pruritic violaceous papules or -Systemic steroids for systemic disease factor for SCC -Can be drug-induced: ACEI, plaques with overlying white or lacey pattern -Phototherapy thiazide, antimalarial, β-blocker -May also have hypertrophic or vesicobullous lesions -Refractory disease: tetracycline, hydroxychloroquine, or -Mostly affects middle-aged -Oral presentation: papular, atrophic, or erosive lesions mycophenolate mofetil adults -Vulvar presentation: vaginal discharge, pruritus, burning, -Vulvar lichen planus: DOC is hydrocortisone suppositories with dyspareunia, narrowing of introitus 2% clotrimazole for candidiasis prophylaxis

201

Psoriasis -A hyperproliferation of the Signs & symptoms Differential Management epidermis with altered -Red scaling papules that coalesce into round-oval plaques with a -Atopic dermatitis (eczema) -Initial treatment with topical differentiation  inflammation of silvery white adherent scale -Contact dermatitis corticosteroids and emollients for mild the epidermis and dermis with -Pustules may border lesions -Nummular eczema to moderate plaque psoriasis accumulation of T-cells and -Lesions are most commonly on -Tinea -Alternatives: tar, topical retinoids, cytokines the scalp, elbows, legs, knees, -Candidiasis topical vitamin D, topical tacrolimus or -Can be flared by strep infections, arms, trunk, lower body, palms, -Intertrigo pimecrolimus injury, trauma, drugs, low humidity, and soles, and occur at sites of -Seborrheic dermatitis -Localized phototherapy emotional stress, and overtreatment trauma -Pityriasis rosea -Systemic therapy for refractory cases: -Variable pruritus -Secondary syphilis methotrexate, cyclosporine, biologics -Extracutaneous manifestations: -Onychomycosis -Treating coexisting depression can onycholysis, geographic tongue, -Cutaneous features of reactive arthritis also help the psoriasis destructive polyarthritis, -Cutaneous T-cell lymphoma ankylosing spondylitis, DIP arthritis, CV disease, depression, -Lichen simplex chronocus lymphoma

Chronic Plaque Psoriasis Erythrodermic Psoriasis Pustular Psoriasis Guttate Psoriasis Intertriginous (Inverse) Psoriasis -Sharply defined erythematous scaling -Generalized erythema with scaling and exfoliation -Individual or coalescing -Multiple small papules of short -Presentation involves the plaques in symmetric distribution -Accounts for 10% of cases non-infectious pustules 1- duration inguinal, perineal, genital, -The most common type of psoriasis -Patients are very sick, with hypo or hyperthermia, 10 mm that are -Associated with recent strep intergluteal, axillary, and -Lasts months to years protein loss, dehydration, renal and cardiac failure generalized or localized infection inframammary regions -May have nail involvement -May need a punch biopsy to differentiate from contact -Can also see typical plaque dermatitis lesions on the knees and elbows

202

VESICULAR BULLAE Bullous Pemphigoid -Uncommon autoimmune subepithelial Differential Management blistering disease -Broad! -High potency topical steroid -On the same spectrum as linear IgA disease, -Aphthous stomatitis -Systemic steroid pemphigus, bullous lupus erythematosus, -Contact dermatitis -May need long-term therapy with dermatitis herpetiformis, and epidermolysis -Contact dermatitis or eczema mycophenolate mofetil, azathioprine, or bullosa acquisita -Dermatitis herpetiformis methotrexate as this disease often persists for -May be a result of genetic factors as well as -Drug reaction years environmental exposures -Lichen planus -Most common in older adults -Stevens-Johnson or TEN Prognosis -Viral infection -Remission may occur after months or years Signs & symptoms -Many more -May be fatal secondary to infection or use of -Prodromal phase with pruritic inflammatory immunosuppressive agents plaques resembling eczema or urticaria Workup -Followed by development of multiple tense 1- -Lesion biopsy needed 3 cm bullae with erosions and crusts -Heal without scarring

ACNEIFORM LESIONS Acne Vulgaris Etiology Differential Management -Multifactorial, involving hormones, -Hydradenitis suppurativa (acne 1.) Behavioral modification: no picking, mild cleanser BID, oil-free non-comedogenic products keratin, sebum, and bacteria inversa): usually occurs in the axillae, 2.) Topical comedolytics (allow 4-6 weeks to work): retinoid (pregnancy D), azelaic acid (better for pregnancy), -Proliferation of P. acnes in this inguinal folds, and perianal area; glycolic acid, salicylic acid environment  foreign body hallmark is double comedones 3.) Topical antibacterials: benzoyl peroxide (DOC, no bacterial resistance), clindamycin, erythromycin (lots of reaction -Steroid acne resistance)), sulfur-containing preparations, metronidazole, dapsone (for inflammatory acne) -Meds: Li, tetracyclines (paradox), 4.) Oral therapies: antibiotics (minocycline, doxycycline, tetracycline, erythromycin; need 2-4 weeks to work), 5 mo Types phenytoin, OCPs, isoniazid course of isotretinoin (regulated by FDA iPledge due to pregnancy X), OCPS (for adult acne, hirsutism, PCOS, -Comedonal -Infectious folliculitis premenstrual flares), spironolactone (for poor OCP candidates, pregnancy X) -Inflammatory -Cutting oils and other occlusives -Cystic: characterized by cysts, -Rosacea Prognosis fissures, abscess formation, deep -Perioral dermatitis -Usually ends by age 25 scarring

203

Rosacea Etiology not well understood: spicy food, alcohol, exercise, Treatment sun? -Topical metronidazole, sulfacetamide + sulfur, or azelaic acid -Time-released doxycycline Signs & symptoms -Laser therapy -Resembles acne but also has flushing, telangiectasia, lingering erythema on forehead, chin, ± eyes -No comedones -Late manifestation is rhinophyma (big bulb-shaped nose)

VERRUCOUS LESIONS Actinic Keratosis -Common cutaneous Signs & symptoms Workup lesions as a result of -Scaly, erythematous macules or papules -Biopsy uncertain lesions, esp if > 1 cm, indurated, ulcerated, rapidly proliferation of atypical on sites of chronic skin exposure growing, or unresponsive to therapy epidermal keratinocytes -May be pigmented, nonerythematous, or free of scale Management Risk factors -Common sites are scalp, face, lateral -Liquid nitrogen cryotherapy for isolated lesions -Chronic sun exposure neck, dorsal forearms, dorsal hands -Surgical curettage or shave removal -Fair skin -Actinic cheilitis is a variant involving the -Multiple lesions in a given area: 5-FU, imiquimod, or photodynamic -Advancing age lip therapy -Male sex -Avoid sun exposure and monitor lesion recurrence or malignancy Differential transformation -Lentigo maligna -SCC Prognosis -May progress to SCC Seborrheic Keratosis -Common epidermal tumor from benign proliferation of immature Signs & symptoms Differential Management keratinocytes -May be 1 isolated lesion or hundreds -Nevus: will not have stuck on or warty -Liquid nitrogen -Stuck-on, warty, well-circumscribed, often scaly appearance, no scale therapy ± curettage Sign of Leser-Trelat = sudden appearance of multiple seborrheic hyperpigmented lesions -Melanoma: look for blurred borders, -Shave excision with keratoses in association with skin tags and acanthosis nigricans; a sign -Most commonly on asymmetry, or h/o change 1% lidocaine of GI or lung cancer the trunk, face, and -Pigmented BCC: h/o change, waxy -Electric removal upper extremities appearance, dilated blood vessels, Dermatosis papulosa nigra = ulceration variant commonly seen on the face of black patients, may be pedunculated

Risk factors -FH -Inflammatory skin disease

204

INSECT & PARASITIC INFESTATIONS Lice Signs & symptoms Management -Scalp or neck pruritus -Resistance has been reported and varies geographically -May be asymptomatic -First-line treatments are pyrethroids, malathion, benzyl alcohol, or spinosad -Cervical and nuchal lymphadenopathy -Mechanical wet combing is an alternative therapy for kids too young for medical therapy ( < 2 months) -Secondary infection -Lindane is restricted due to neurologic effects -Treat bedmates prophylactically and examine all housemates and close contacts for nits Workup -Wash all clothing and bedding used in last 2 days in hot water -Diagnosis is with visualization of the nits on hair -Store non-washable items in a plastic bag for 2 weeks shaft as well as crawling nymphs and adults -2nd treatment with insecticide 7-10 days after first treatment to kill any surviving nits -Kids may return to school after first application of insecticide

Scabies -Caused by mites Workup -Spread by skin to skin or sex, with incubation period of 21 days -Skin scraping under oil immersion for -Can live for 48 hours on clothing, bedding, and furniture mites, feces, and eggs

Signs & symptoms Treatment -Intensely pruritic papules and pustular rash that is worse at -Permethrin cream, usually only single bedtime application needed -Predilection for finger webs, wrist flexors, elbows, axillae, -Treat all family members penis, external genitalia, feet, ankles -Wash all bedding and clothing in hot -Babies < 1 year can get scabies from the neck up water -“Norwegian” scabies make severe crusting and have heavy infestation

205

Spider Bites -Most lesions attributed to spider bites are caused by something else Differential Signs & symptoms -Infection -Solitary papule, pustule, or wheal -Other bite or sting: triatomid, ant, flea, bedbug, blister -Systemic symptoms of evenomation with certain spiders beetle, tick, mite, mosquito, biting fly -Recluse bite: malaise, n/v, fever, myalgias, progression of lesion to -Scorpion sting necrosis -Common dermatosis: poison ivy, poison oak

Management -Widow bite: depends on severity of evenomation; pain control, wound cleansing, tetanus prophylaxis, antiemetics, benzos, widow antivenom -Recluse bite: local wound care, pain meds, tetanus prophylaxis, antibiotics for cellulitis, dapsone for necrosis

-Widow spider: muscle pain or spasm, local paresthesia, HA, n/v, no necrosis

206

NEOPLASMS Basal Cell Carcinoma -Most common skin cancer, and most common human cancer -Slow growing, locally destructive -No mets -Risk factors: sun, sunburns < age 14, arsenic ingestion, radiation -More common in males -Usually after age 40 -Several subtypes with different treatments Type of BCC Info Investigation & Picture Type of BCC Info Investigation & Picture Treatment Treatment Nodular BCC Most common BCC. ED&C, Pigmented Resembles melanoma. ED&C, excision, Pearly white or pink excision, Mohs. BCC Mohs. dome shaped papule with overlying telangiectasias  ulceration, raised borders, bleeding, scaling.

Superficial Least aggressive BCC. ED&C, Morpheaform Least common variant. Mohs needed. BCC Erythematous scaly excision. BCC White to yellow patch with plaques or papules +/- poorly-defined borders. rolled borders. Can look like psoriasis, eczema, others.

Kaposi Sarcoma -Neoplasm characterized by Signs & symptoms Management abnormal angiogenesis -Purplish, reddish blue, or -Observation for limited -Requires prior infection dark brown or black asymptomatic lesions that do with HHV-8 lesions on the lower not impair function -Most common in elderly extremities, often with -Compression stockings for LE men of Mediterranean and lymphedema edema European descent -Slow growing and -Local treatment: radiation localized, but can become therapy, excision, cryotherapy, disseminated laser ablation -Visceral involvement -Chemo: PLD with HIV

Workup -Biopsy required

207

Melanoma -Flat, raised, nodular, or ulcerated Workup -Variable color -Lymph node palpation -Consider in any new mole or a mole changing shape, size, or color Punch or incisional biopsy Type of Melanoma Info Picture Type of Melanoma Info Picture Lentigo maligna (melanoma Melanoma restricted to Acral lentiginous Primarily on hands, feet, in situ) epidermis. melanoma nails. Most common type of melanoma in blacks and Asians. Common in males.

Superficial spreading Most common type of Amelanotic melanoma Innocent-appearing pink to melanoma melanoma. Asymmetric, flat red colored papules that lesions > 6 mm. Vary in enlarge to plaques and color. Lateral spread. nodules. Scary.

Nodular melanoma Rapid growth vertically from and through skin. Most common on extremities.

Squamous Cell Carcinoma -AKA Bowen’s disease if SCC in situ Presentation -Potentially invasive malignancy of keratinocytes in the skin or -Flesh, pink, yellow, or red indurated papules plaques, or mucous membranes nodules with scale -Most caused by UV radiation but other risks are chemicals, -Can have ulcerations and erosions tobacco, infection, burns, HPV -Erythroplasia of Queyrat is SCC of the penis Workup & Management -Palpate regional lymph nodes for mets. -ED&C, excision, Mohs. -Bowen’s: 5-FU, cryo, ED&C, excision, Mohs.

208

HAIR & NAILS Alopecia Signs & symptoms Differential Workup -Alopecia areata: smooth, circular, discrete areas of -Scarring (cicatricial) alopecia: chemical or physical trauma, lichen planopilaris, -May need microscopic examination of hair or scalp complete hair loss developing over a few weeks, bacterial or fungal infection, shingles, discoid lupus, scleroderma, ionizing biopsy may have nail pitting radiation -Male pattern baldness: slow, progressive transition -Nonscarring: male pattern baldness, telogen effluvium (stress-related), alopecia Management of terminal hairs on the frontal scalp to shorter, areata (immune-related, exclamation point hairs), trichotillomania, drug-induced, -Alopecia areata  topical steroids thinner, more vellus hairs SLE, secondary syphilis, hyperthyroid, hypothyroid, iron-deficiency anemia, -Male pattern baldness  minoxidil or finasteride, hair pituitary deficiency transplant, antiandrogen in women (spironolactone) Paronychia -Infection of nail base Signs & Symptoms Management -Pain -I&D for abscess Agents -Erythema -Raise nail to express pus -Acute: Staph -Abscess formation -Warm moist compresses for 24 hours followed by dry -Chronic: Candida -Swelling dressings for 3-4 days

VIRAL DISEASES Childhood Exanthems Disease Information Signs & symptoms Picture Treatment Measles (Rubeola -Agent is measles -Cough -Self-resolution in 7-10 or First Disease) virus -Coryza days -Prevent with -Conjunctivitis -Supportive care MMR vaccination -Koplik’s spots -Complication: subacute (indicated to -Maculopapular rash starting at hairline and sclerosing panencephalitis, prevent death) spreading down to confluence a rare fatal infection years after initial infection Scarlet Fever -Agent is GAS -Pharyngitis -Penicillin VK or (Second Disease) -Strawberry tongue amoxicillin administered -Sandpapery rash that is worse in the groin and to prevent sequelae of axilla with desquamation of palms and soles rheumatic fever

Rubella -Agent is rubella -Mild fever -Resolves in 3 days (German Measles, virus -Conjunctivitis -Sequelae of arthralgias 3 Day Measles, -Prevent with -Arthralgias Third Disease) vaccination -Postauricular and occipital adenopathy (indicated to -Maculopapular rash on face that spreads prevent congenital rubella syndrome)

209

Disease Information Signs & symptoms Picture Treatment Erythema -Agent is human -Mild flulike illness -Treatment is supportive Infectiosum parvovirus B19 -Rash at days 10-17: initially appears as flushed with NSAIDs for (Fifth Disease) cheeks, then encompasses whole body as a arthralgias and fever maculopapular rash, becoming lacy in the arms and legs -Low grade fever -Migratory arthritis in older patients that can last 6-8 weeks -“Papular purpuric glove & sock syndrome” in older adolescents, lasts 1-2 weeks

Roseola -Agent is HHV-6 -High fevers to 104 for 3-7 days with no rash -Antipyretics and (Sixth Disease) or HHV-7 -Rash after fever goes away hydration -Affects young children, 6 mo to 3 years

Varicella -Caused by HHV-3 -Intensely pruritic lesions on the trunk first, -Symptomatic only (VZV) then face, head, extremities, possibly mucous -Consider acyclovir in -Child will be membranes teens contagious for 1 -Lesions come in crops over 3-4 days and crust week over in 3-5 days

Hand-Foot-Mouth -Agent: Coxsackie -Vesicles on tongue, oral mucosa, hands, -Symptomatic Disease A16 virus possibly feet -Sequelae of myocarditis, (Herpangina) -Highly contagious -May have generalized scarlatiniform rash substernal chest pain, -Low-grade fever dyspnea -Overall child feels well

210

Disease Information Signs & symptoms Picture Treatment Gianotti-Crosti -Usually caused by -Symmetric red-purple papules and -Self-limiting over 3-4 Syndrome EBV, Hep B, or papulovesicles on the face, buttocks, and weeks (Papular HHV-4 extremities Acrodermatitis of -Affects 6-14 -Lymphadenopathy Childhood) month olds -Low-grade fever

Enterovirus -Varied rash; may be maculopapular, vesicular, petechial, or urticarial Exanthems -May involve other organ systems Molluscum Contagiosum -Agent is a poxvirus Management -Spread by autoinoculation -Self-limiting after about 1 year -Cryotherapy Signs & symptoms -Flesh-colored dome-shaped lesions with umbilicated central core

Verrucae -Agent is HPV, which infects the skin Subtypes Management keratinocytes to cause warts -Verruca vulgaris: the common wart; verrucous surface, thrombosed capillaries, loss of -Wait for regression via cell-mediated -Occur in areas of skin trauma dermatoglyphics, may have fingerlike projections immunity -100 serotypes = many infections possible over -Verruca plana: the flat wart; flat-topped pink to brown papules, usually in linear formation, with -Destruction: cryotherapy, laser, cautery, a lifetime predilection for the face, dorsal hands, wrists, and knees, commonly spread by shaving duct tape occlusion, excision, beetle juice, -Don’t have “roots”, rather they are confined -Verruca plantaris: the plantar wart; verrucous surface, thrombosed capillaries, often coalesce into a podophyllin gel, retinoids, salicylic acid, 5- to the epidermis “mosaic”, with predilection for pressure points of the feet fluorouracil -Cause necrosis of capillaries -Immunomodulating agents -Have oncogenic potential Differential -Callus: won’t interrupt skin lines

211

Condyloma Acuminatum -Anogenital warts associated with HPV Differential subtypes 6 and 11 -Condyloma lata of 2° syphilis -The most common STD in the US -Micropapillomatosis of the vulva -SCC Transmission -Vulvar intraepithelial neoplasia -Sex, including digital/anal, oral/anal, and -Molluscum contagiosum digital/vaginal contact -Skin tag -Possibly fomites Management Prevention -Chemical or physical destruction -Gardasil vaccine -Immunologic therapy -Surgical excision Signs & symptoms -For recurrence  surgical excision or fulguration therapy followed by -Many are asymptomatic adjuvant treatment -Smooth papules or verrucous lesions -Pruritus -Bleeding -Burning -Tenderness -Vaginal discharge

BACTERIAL INFECTIONS Impetigo Etiologies Signs & symptoms Management -Usually Staph aureus -Oozing lesions -Topical mupirocin for small number of non-bullous lesions -Also Strep -May be pruritic -Oral therapy for anything else: dicloxacillin, cephalexin, or -If deep and extending into the dermis with ulceration and a clindamycin tender yellow-gray crust, it is called ecthyma (agents are Strep, -Suspect MRSA  clindamycin or linezolid Pseudomonas, Staph) Sequelae -Risk factors: trauma, underlying eczema or HSV, poor hygiene, -May be followed by poststreptococcal glomerulonephritis or previous antibiotics, warm temps, high humidity rheumatic fever -Lesions are spread by auto-inoculation

212

Cellulitis -Risk factors: skin trauma, inflammation (eczema or radiation), Signs & symptoms Management pre-existing impetigo or tinea pedis, edema -Skin erythema, edema, and warmth -Can usually be treated with oral antibiotics -Indolent onset with development of symptoms over days -Careful, erysipelas must be covered with IV therapy for initial Agents -May have purulent drainage or exudate management -Most commonly GAS or GBS -IV AB for systemic toxicity or rapid progression -Staph aureus Differential -Empiric therapy for nonpurulent cellulitis to cover Strep and -Gram negs -Necrotizing fasciitis MSSA  oral dicloxacillin (or cephalexin or clindamycin), IV -H. flu -Gas gangrene cefazolin (or oxacillin, nafcillin, or clindamycin) -Clostridium -TSS -If risk factors for MRSA (recent hosp, LTC resident, recent -Pseudomonas -Bursitis AB, HIV, MSM, IVDU, HD, incarceration, military, DM) with -Osteomyelitis nonpurulent cellulitis oral clindamycin (or amoxicillin + Differential -Herpes zoster Septa or doxy, or linezolid) -Erysipelas: more acute onset that involves the upper dermis and -Erythema migrans -Purulent cellulitis ( = risk for MRSA)  clindamycin, Septra, superficial lymphatics while cellulitis involves the deeper doxy, or linezolid dermis and subcutaneous fat; lesions will be raised above Workup -May need suppressive therapy for recurrent cellulitis surrounding skin with clear demarcation between involved and -Diagnosis is clinical uninvolved tissue -Cultures if there is systemic toxicity, extensive skin -If it involves the ear, it’s erysipelas! (no deep tissue here) involvement, underlying comorbidities, unusual exposures like animal bites or water-associated injury, or recurrent or persistent cellulitis

213

FUNGAL INFECTIONS Dermatophytoses -Caused by Microsporum, Trichophyton, or Epidermophyton spp -Risk factors: atopy, immunosuppression, existing skin condition, DM, sweating, humidity -May be transmitted person-to-person, soil-to-person, or zoonotically -AKA “tinea” or “ringworm” Differential -Atopic dermatitis -Trichotillomania -Pityriasis versicolor -Discoid lupus erythematosus -Erythrasma -Dyshidrotic dermatitis -Traction alopecia -Pseudofolliculitis barbae -Contact dermatitis -Friction blister -Lichen simplex chronicus -Alopecia areata -Seborrheic dermatitis -Candidal intertrigo -Onychogryphosis -Psoriasis -Erythema chronicum migrans -Acne rosacea Disease Information Management Information Disease Tinea barbae: -Oral antifungal: griseofulvin, -Located on the non- Tinea beard terbinafine, or itraconazole bearded face faciale -Air exposure -Children often acquire -Topical antifungal: terbinafine, from cats or dogs naftifine, butenafine -May be brought on by ± Soaks with aluminum acetate sunlight exposure -2nd line: topical azole

Majocchi -Deep cutaneous -Erythema, scaling, vesicles Tina pedis (trichophytic) infection -Maceration of web spaces granuloma -May have toenail involvement or bacterial 2° infection -“Moccasin” pattern with involvement of heels, soles, and lateral feet -May be inflammatory with bullae Tinea Similar to tinea pedis -Erythematous, well- Tina manuum: hand but on the hand demarcated scaling plaques cruris: -May be more -Must differentiate from “jock itch”; aggressive erythrasma or candidiasis groin and (will fluoresce differently thighs under Wood’s lamp)

Tinea -Sharp-bordered corporis: erythematous plaques of infection of varying sizes neck, trunk, or -May have pustules or extremities vesicles within the border -Lesions will enlarge peripherally and may have an area of central clearing

214

Disease Information Management Disease Information Management Tinea capitis: -Black dot hair loss -Oral antifungal: Tinea unguium -3 forms: distal subungual, proximal subungual, -1st line is oral head -Scalp erythema and scaling griseofulvin, terbinafine, (onychomycosis) and white superficial terbinafine -May see kerions or itraconazole -Most pts will also have tinea pedis -2nd line is oral azole or ± Antifungal shampoo: -Infection may be yeast or nail dystrophy = must ciclopirox topical lacquer selenium sulfide, to KOH scrape to be sure before starting therapy -3rd line is repeat therapy ketoconazole as terbinafine won’t cover candidiasis or nail removal

Tinea -Hypo or hyperpigmented macular lesions -Topical selenium versicolor: -Especially on trunk sulfide, pyrithione zinc, caused by -Fine rim of scale propylene glycol, Malasezzia -KOH prep for spaghetti and meatballs ciclopirox, azole, or furfur = not terbinafine really a tinea ± UV light therapy -Systemic ketoconazole if recurrent or refractory

OTHER DERMATOLOGY TOPICS Acanthosis Nigricans -19% of primary care patients will have this, with rates up to 47% in Native Signs and symptoms Management Americans -Velvety, hyperpigmented plaques that -Treat underlying disorder most frequently occur on the neck and -Weight loss, metformin or rosiglitazone Etiologies axillae but can appear on other skin sites -D/c offending meds -Acquired or inherited or mucosal surfaces -Topical retinoids, vitamin D analogs, and keratolytics -Most commonly a result of obesity or endocrine or metabolic disorders, but -Rapid onset if associated with can also be associated with genetic syndromes, familial acanthosis nigricans, malignancy malignancy, or drug reactions Lipomas -Collections of mature fat cells Signs & symptoms Management Prognosis encased in thin fibrous capsules -Small golf ball under skin -Treatment only indicated for pain, cosmesis, or concern for -Recurrence of excised lipoma is -The most common benign soft -Soft, asymptomatic, mobile malignancy uncommon tissue neoplasm -Do not enlarge quickly -Surgical excision -Multiple sites with familial multiple lipomatosis

215

Burns First Degree Burns Third Degree Burns Calculation of Burn Area Management -Superficial burns only involving the epidermis -Full thickness burn, entire -“Rule of 9s” for adults -Remove clothes -Ex. sun burns, “flash” burns epidermis and dermis is gone, -Irrigate with room temp saline (not cold, will cause -No blisters or edema, skin is pink or red, dry extends to subcutaneous fat vasoconstriction) -Will heal on their own in 3-6 days -A result of prolonged exposure -Cover wound to prevent heat loss -Are not included in burn calculations to any heat source -Use LR for IVF using Parkland formula to estimate -Extensive edema, dry, leathery, needed amount Second Degree Burns charred skin, no sensation or -Keep room warm -Partial thickness burn that involves the dermis circulation -IV morphine -Superficial partial = small amount of dermis involved; -Will not heal spontaneously, -NG decompression caused by flame, scalding, or chemicals; moist, pink/red, requires skin grafting -Tetanus booster edema, blistering, extremely sensitive to touch; heals in 10- -Assess for smoke injury: facial burn, singed nose or 21 days Fourth Degree facial hair, carbonaceous deposits in oropharynx -Deep partial = significant amount of dermis involved, -Penetration to the bone -Surgical management may be needed more than 50%; caused by grease, flame, or chemicals; -Usually requires amputation -Biologic dressings for several weeks with daily cleansing fewer capillaries left = appears white, dry, moderate and debridement, topical antibiotics edema, decreased sensation & circulation  minimal pain; -Vaseline cause during exudative phase may scar; may convert to full thickness burn; healing takes -Indications for referral to burn center: > 10% body > 21 days surface are with 2nd degree burns, any 3rd degree burns,  Tell these apart by degree of pain and pressure sensation electric or chemical burn, inhalation injuries, burns accompanied by trauma

Hidradenitis Suppurativa -Chronic inflammatory skin disorder characterized by pustules, Signs & Symptoms Management inflammatory nodules, and sinus tract development, usually in -Affected areas may be in the axillae, genitofemoral -Hot packs for mild cases intertriginous areas region, gluteal folds, or perianal areas -Topical or systemic antibiotics: clindamycin or doxycycline -Small, painful subcutaneous nodules can be palpated -Retinoids Prevention -Pruritus -Accutane -Avoiding skin trauma -Erythema -Surgical removal, I&D, or skin grafting for severe cases -Careful skin hygiene -Burning pain -I&D will not alter course of disease and should be reserved for pt -Smoking cessation -Local hyperhidrosis comfort in times of tight skin abscess formation -Reducing carb intake -Sinus tract formation -Weight reduction -Hyperpigmentation, scars, and pitting of the skin Melasma -Disorder of hyperpigmentation affecting sun-exposed areas of skin Signs & Symptoms -Usually appears on the face Causes -More pronounced in those with darker complexions -Pregnancy (occurs in up to 75% of pregnant women) -OCPs -Genetics Workup -Sun exposure -Diagnosis is clinical -Cosmetics -Thyroid dysfunction Management -Antiepileptics -Broad spectrum sun protection -Hydroquinone 4% cream

216

Epidermal Inclusion Cysts (Epidermoid Cysts) -Discrete nodules with keratin-producing cell Signs & symptoms Differential Management wall -Discrete, freely movable cyst or nodule, often -Pilar cyst: derived -Be cautious of cysts that have been present on scalp since birth as -Erroneously referred to as sebaceous cysts but with central punctum from hair root sheath these may communicate with CNS there is no sebaceous component -Can have spontaneous inflammation and -Kenalog injection for inflamed cysts rupture -I&D of infected cyst Gardner’s syndrome = rare condition of -May grow larger -Surgical excision of non-inflamed cyst multiple epidermal inclusion cysts associated with colon cancer Prognosis -May resolve spontaneously without therapy Pilonidal Disease -An acquired condition likely related to mechanical forces on Signs & Symptoms Management the skin overlying the natal pilonidal cleft  cavity formation -May have asymptomatic cyst -Acute abscess needs I&D with debridement of all visible hair containing hair, debris, and granulation tissue -Tenderness, erythema, and abscess formation just -Refer for surgical excision of recurrent pilonidal disease above the gluteal cleft -Primary closure associated with faster healing but delayed closure associated with lower likelihood of recurrence -Antibiotics only for cellulitis Decubitus Ulcers (Pressure Ulcers) -Infections of these are Staging Differential Management typically -Stage 1 = intact skin but with non-blanchable redness for > 1 -Diabetic neuropathy ulcer -Comprehensive analysis of patient’s general medical condition and polymicrobial, hour after relief of pressure -Arterial or venous evaluation of the wound including staph and -Stage II = blister or other break in the dermis ± infection insufficiency ulcer -Daily monitoring using healing and staging scales strep, enterococci, -Stage III = full thickness tissue loss, may see subcutaneous -Adequate pain control Enterobacter, Proteus, fat, destruction extends into muscle ±infection, may have Workup -Nutritional supplementation if needed and anaerobes undermining or tunneling -MRI is best for evaluation -Repositioning every 2 hours -Stage IV = full thickness skin loss with involvement of bone, of osteomyelitis -Dressing choice depends on wound characteristics: wet to dry, Risk factors tendon, or joint ± infection, often with undermining or -Wound cultures: gold semiocclusive, occlusive, sterile maggots, etc. -Shearing forces, tunneling standard sample is of a deep -Stage 1 ulcers need transparent film dressing friction, moisture, -Unstageable = full thickness tissue loss in which ulcer base is tissue specimen from a -Stage 2 ulcers need a moist wound dressing immobility, covered by slough or eschar surgically cleaned and -Stage 3 and 4 ulcers need infection treatment, debridement, and other incontinence, poor -Suspected deep tissue injury = purple or maroon localized ara debrided ulcer; superficial special dressings nutrition, decreased of discolored intact skin or blood-filled blister due to damage cultures tend to represent -For superficial infections, topical antibiotics such as sulfadiazine are used skin perfusion of underlying tissue from pressure or shear colonization rather than true to reduce bacterial counts wound infection -For deeper infections or complicated infections, systemic therapy is needed

Prognosis -Complications: bacteremia, sepsis, death Vitiligo -Acquired skin depigmentation as a result of autoimmunity against Differential Management melanocytes -Postinflammatory hypopigmentation -Repigmentation therapies -Chemically-induced depigmentation -Topical steroids Signs & symptoms -Tinea versicolor -Topical calcineurin inhibitors -Usually is generalized (vitiligo vulgaris): widespread macules and patches -Pityriasis alba -UV light that are often symmetric -Morphea -Comorbid autoimmune disease: autoimmune thyroiditis, pernicious anemia, -Lichen sclerosus SLE, Addison disease -Leprosy

217

Leg Ulcers Risk factors -Poor circulation, venous insufficiency, disorders of clotting, diabetes, sickle cell, neuropathy, renal failure, HTN, lymphedema, inflammatory skin diseases, smoking, genetics, malignancy, meds Diabetic (Neurotrophic) Ulcers Venous Insufficiency Ulcers Arterial (Ischemic) Ulcers Screening -Classified by CEAP system, which helps distinguish initial disease severity as well Signs and symptoms -Recommended annually with as changes over time -PAD symptoms: pain and claudication with visual examination and -Varicose veins in the absence of skin changes are NOT chronic venous walking that is relieved by rest (however may monofilament test (checks most insufficiency! have more pain with leg elevation in severe common sites of ulceration) disease) Risk factors -Ulcers are usually on the feet at points of -Advancing age, FH, increased BMI, friction and appear punched-out smoking, h/o LE trauma, prior DVT, -Feet will turn red when dangled and pale white pregnancy or yellow when elevated

Signs & symptoms -C/o tired, heavy legs, leg pain, or leg swelling -Telangiectasias, reticular veins, and varicose veins -Edema, inflammation, pruritic dermatitis -Ulcers with irregularly shaped borders along the medial ankle or saphenous veins Signs & symptoms that are tender, shallow, exudative, and have a base of granulation tissue -Ulcers with punched-out borders with calloused -Skin discoloration or redness, may appear shiny or tight surrounding skin -20% of symptomatic patients will have no visible clinical signs -Underlying neuropathy

Management Workup -Leg elevation, exercises, and graduated compression stockings -Ankle-brachial index with symptoms of PAD -SCDs for patients refractory to stockings -Horse chestnut seed extract for patients who can’t tolerate or are noncompliant with Management compression therapy -Comprehensive assessment of ulcer and patient’s overall -Skin moisturizers medical condition -Wound debridement PRN -Classification of wound at each follow-up -Barrier creams to protect adjacent skin -Debridement, local wound care, pressure relief, infection -Selection of proper wound dressing control, and proper dressing selection -NOT effective: topical antibiotics, debriding enzymes, growth factors, or honey -Negative pressure wound therapy following debridement -Compression bandages for severe edema, weeping, eczema, or ulceration after infection, necrosis, or amputation -Aspirin therapy accelerates healing -Revascularization for critical wound ischemia -Referral to subspecialty for slowly healing ulcers, persistent dermatitis, or recurrent cellulitis Human & Animal Bites -Risk of infection in cat bites > human bites > dog bites Management -Primary closure of wounds that are cosmetically important, clinically uninfected, < 12 hours old Workup (or < 24 on the face), and not located on hand or foot -Check involved animals for rabies -Leave wounds open that are crush injuries, puncture wounds, bites of the hand or feet, wounds > -CBC, blood and wound cultures if febrile or wound appears infected 12 hours old (or > 24 on the face), cat or human bites, or bite wound in immunocompromised host -Head CT for deep dog bite to the scalp -Tetanus or rabies vaccination if indicated -Primary care f/u in 48-72 hours -Empiric antibiotics for animal and human bites: Augmentin drug of choice -Deep or severe wound infections from animal or human bites require IV antibiotics: Zosyn 218

HEMATOLOGY ANEMIAS Aplastic Anemia -A result of bone marrow failure due to injury or suppression Signs & symptoms Management -Abrupt onset of fatigue, weakness, dyspnea, excess bleeding & -Based on severity of disease Etiologies bruising, petechiae, purpura, pallor, and infections -None if mild -Idiopathic -BMT or immunosuppression if severe -Drugs: phenytoin, sulfas, chemo, radiation, chemicals Workup -Viruses -Labs show pancytopenia, severe normocytic anemia, ↓ retics, -Pregnancy reduced cells in BM with fat replacement -Hereditary (Fanconi’s anemia) Microcytic Anemia = MCV < 80 Iron Deficiency Anemia Chronic Inflammation Anemia (Anemia of Chronic Sideroblastic Anemia Disease) Etiology Management Etiology Etiology -In an adult, this is due to blood loss, likely GI, -Treat blood loss -Usually from reduced erythropoietin stimulation of -Inherited, acquired, or idiopathic heme until proven otherwise -Oral iron with stool softeners, continue 3-6 bone marrow synthesis from alcohol, lead, months post Hb recovery myelodysplasia, leukemia, TB, or Signs & symptoms -Consider parenteral therapy by heme if pt Workup drugs -Fatigue does not tolerate oral therapy or it is not -A disease of exclusion -Dyspnea on exertion rapid enough Workup -Tachycardia -Recheck CBC in 3-4 weeks and ferritin in 8 Management -BM biopsy showing ringed -Cheilosis weeks -Treat only it pt is symptomatic with folate, iron, EPO sideroblasts -Spoon-shaped nails -Pica Management based on cause -Dysphagia due to webbing of the esophagus Macrocytic Anemia = MCV > 100 Vitamin B 12 Deficiency Folate Deficiency Etiology Signs & symptoms Etiology Signs & symptoms -Inadequate diet: vegetarians -Abnormal sensation and peripheral -Inadequate diet: alcoholics -Glossitis -Malabsorption neuropathy in stocking-glove pattern -Dialysis -Diarrhea -Drugs -Glossitis -Malabsorption -Malnourishment -Pallor -Impaired metabolism -Cheilosis -Anorexia -No neuropathies -Diarrhea Hemolytic Anemia Etiologies Signs & symptoms Workup -Hereditary spherocytosis: intrinsic inherited defective spectrins in RBC -Rapid onset of pallor and -Increased hemolysis markers: LDH membrane  weak, deformed spherical RBCs prone to rupture in blood vessels anemia -Reduced or absent serum haptoglobin (since it binds free Hb, which is or spleen -Jaundice with ↑ unconjugated abundant during hemolysis) -Hereditary elliptocytosis bili -May have + Coomb’s test (DAT) -G6PD deficiency: results in oxidation-prone RBCs  intracellular ppt of -Bilirubin gallstones -Increased retic % or retic # as BM responds to anemia oxidized Hb into Heinz bodies  bite cells and hemolysis after journey through -Splenomegaly -Abnormalities on peripheral smear the spleen -Sickle cell disorders Management -Acquired disorders: TTP, HUS, giant hemangioma, artificial heart valves, sepsis, -Treat underlying cause DIC, autoimmune hemolytic anemia, hemolytic disease of the newborn

219

Sickle Cell Disease -Triggers for crisis can be dehydration, Signs & Symptoms Workup Management of crisis infection, and acidosis as they increase -Anemia  pallor and fatigue -Peripheral smear showing sickled cells and -Pain control for opioid naïve: NSAIDS or APAP + codeine cell sickling -Hemolysis  jaundice and gallstones Howell-Jowell bodies reflecting splenic -Next step: oral morphine, oxycodone, or hydromorphone -Usually pts are functionally asplenic -Dactylitis dysfunction -Severe pain: IV fentanyl, morphine, or hydromorphone as their spleen becomes infarcted by a -Leg ulcers -Reticulocytosis from chronic hemolysis -Breakthrough pain: start PCA young age -Priapism -IVF -Disease can be AS (trait), SS, SC, or S -Pulmonary, cerebral, and splenic Outpatient Management -Bronchodilators for wheezing + other Hb variant emboli  functional asplenia, stroke, -Vaccinations: Pneumovax, flu, Hib, Menactra, -Incentive spirometry to prevent atelectasis PE hep B -Supplemental O2 -Retinal artery obstruction  blindness -Prophylactic penicillin until age 5 -May need transfusion for low Hb -Crisis: skeletal pain, fever, anemia, -Folate, MVI, hydroxyurea -DVT prophylaxis (hypercoagulable state) jaundice -Funduscopic exam for proliferative -Empiric ceftriaxone for fevers -Acute chest syndrome retinopathy risk -Splenic sequestration crisis -Screening echo and BNP every 2 years after Complications -Aplastic crisis: worsening of anemia age 21 -Acute chest syndrome: #1 cause of death in sickle cell, from parvovirus B19 infection -BMP and UA twice a year defined as a new radiodensity + fever/resp symptoms, caused -Pulmonary HTN -Mental health assessments by hypoventilation/pulmonary infarct/pulmonary -CKD (may progress to ESRD) -Birth control discussion (risk of crisis ↑ during edema/TRALI, treat with abx to cover CAP (because it can’t -Osteomyelitis pregnancy, hydroxyurea is pregnancy cat D) be distinguished from pneumonia), IVF, bronchodilators, incentive spirometry, supplemental O2, transfusion for Hb < Crisis Workup 10 -↑bili and LDH from hemolysis -Chronic lung disease and pulmonary HTN -Low haptoglobin (binds up the lysed -Stroke Hb) -Bone/joint: osteomyelitis, septic arthritis, osteonecrosis, -Cultures for fever vertebral body collapse, stunted growth, osteopenia -Chronic kidney disease from renal infarcts -Priapism G6PD Deficiency -X-linked disorder of the enzyme that Signs & symptoms Workup Prognosis catalyzes reactions to prevent oxidative -Most affected patients are asymptomatic -Several screening tests available -May incur protection against injury RBCs -Neonatal jaundice malaria -Increased susceptibility to infection Management -Severe episodes of hemolysis Screening -Episodic anemia following exposure to infection, drugs, or chemicals  -Avoid fava beans can be fatal -Not performed routinely in newborns HA, nausea, back pain, chills, fever, hemoglobinuria, jaundice -Avoid certain meds: dapsone, -Some may have chronic hemolysis nitrofurantoin Thalassemia Alpha Thalassemia Beta Thalassemia -Decrease or absence of alpha globulin Management -Decrease or absence of beta Signs & symptoms -At risk: SE Asian, Mediterranean, African -For full disease, globulin -Major disease (Cooley’s)  jaundice, hepatosplenomegaly, bony splenectomy, folate, avoid -Body may compensate by abnormalities, chipmunk face, growth retardation Signs & symptoms Fe increasing % of HbA2 and HbF -Intermediate disease  moderate chronic anemia, Fe overload -HBH disease  splenomegaly, pallor -At risk: Italian, Greek, Asian, -Minor disease  rare anemia -A thal trait  normal clinic presentation under African non-stressful conditions Management -Only severe disease is treated with transfusions, folate, Fe chelation therapy, splenectomy

220

COAGULATION DISORDERS Thrombocytopenias *Remember to distinguish from pseudothrombocytopenia in patients that are “EDTA clumpers” or in patients on aspirin, clopidogrel, prasugrel, NSAIDs, or other antiplatelets Acute Immune Thrombocytopenic Heparin-Induced Thrombotic Hemolytic Uremic Disseminated Other Causes Thrombocytopenia Purpura Thrombocytopenias Thrombocytopenic Syndrome Intravascular Purpura Coagulation -Sudden ↓ in -Due to anti-platelet Abs -HIT type I is non-immune, -Caused by Ab to -Classically caused by E. -A pathological activation -Liver disease platelets from -Can be from viral infection in transient, and improves upon ADAMSTS13  coli damage to endothelia of coagulation that is low destruction, less kids that is self-limiting d/c of heparin extensive and renal arterioles and always associated with an thrombopoietin production, or -In adults tends to be chronic with -HIT type II is due to IgG microthromboses inactivation of underlying disease such as (tx with FPP) sequestration no prior infection, but can also against PF4 formation of throughout body ADAMSTS13  plt sepsis, burns, cancer, head -Vit K -S/s: dried blood in occur after valve replacement, immune complexes that -Assoc with certain activation  trauma, snake bite, or deficiency due to nose, wet purpura in cardiac cath, drugs activate platelets to form meds thrombocytopenia, uremia vasculitis abx killing vit K the mouth, -S/s: neurologic symptoms, fever, microthromboses in small -S/s: AMS, fevers, -Very similar to TTP, -W/u: ↓ plt, schistocytes, + synthesizing gut splenomegaly, ± renal failure vessels throughout body chest pain, dyspnea, distinguish it by renal D-dimer, ↓ fibrinogen (all bacteria, petechiae -W/u: normal PT/PTT, normal -S/s: MAHA, limb necrosis, not urinating, similar dysfunction vs neuro used up), prolonged malnutrition, or -Plt will be < 150k cell lines, normal marrow, normal pulseless extremities to acute impairments PTT/PT (factors all used biliary tract spleen = dx of exclusion -W/u: plt ~50k, normal thrombocytopenia or -Tx: supportive, volume up) disease (tx with -Tx: steroids, immune modulation PT/PTT, PF4 Ab, schistocytes ITP repletion, pressors, plasma -Tx: treat underlying vit K supp + with IV Ig or splenectomy, on peripheral smear -W/u: plt ~20k, normal exchange; only give pRBCs disease, replace blood FFP) plasmapheresis, EPO -Tx: stop heparin, use direct PT/PTT, schistocytes with severe anemia; NO products PRN, may need -90% fatal without treatment thrombin inhibitors, and begin -Tx: plasmapheresis to abx as it will worsen HUS, LMWH to inhibit further warfarin after plt normalize remove Ab, steroids NO plt as this will worsen clotting MAHA Thrombopathies -Characterized by prolonged bleeding time despite normal platelet counts Von Willebrand Disease Glanzmann’s Bernard-Soulier Syndrome Hemophilia Other Thrombopathies Thrombasthenia -The most common hereditary coagulation abnormality -Rare genetic disease -Rare genetic disease that causes a -Hemophilia A is factor 8 -Decreased factor 8 due to -A result of a functional or quantitative deficiency of vWF where platelets lack deficiency of the receptor for vWF deficiency autoimmunity (differentiate -A spectrum of disease glycoprotein IIb/IIIa = no -Giant platelets dominate = -Hemophilia B is factor 9 from hemophilia by doing a -W/u: electrophoresis shows decreased vWF multimers fibrinogen or vWF functional thrombocytopenia deficiency mixing study, it won’t correct) (the opposite of TTP), hyperagglutination with ristocetin, bridging can occur -W/u: giant platelets on peripheral -S/s: hemarthroses, muscle bleeds, -Acquired thrombopathy from prolonged closure time for collagen and ADP -W/u: platelet agg studies smear, platelet agg studies are the other abnormal bleeding drugs, infection, renal disease, -Tx: only treat severe cases with vWF & factor 8 are the opposite of Von opposite of Von Willebrand -W/u: prolonged PTT, decreased hepatic disease, AIDS, concentrates, or desmopressin for quick release of vWF Willebrand disease disease factor 8 or 9 NSAIDs from endothelial stores -Tx: factor concentrates Hypercoagulable States Hereditary Acquired Factor V Leiden Protein C or protein S deficiency Virchow’s triad Others -Hereditary resistance to factor 5 inactivation by protein -Deficiency of natural vitamin K- -Vascular damage, hypercoagulability, vascular -Hormones: pregnancy, OCPs -PTT may be shortened and does not correct dependent anticoagulants stasis = after surgical procedures -Malignancy -Definitive PCR test -Smoking Antithrombin III deficiency Anti-PL syndrome -Immobilization Prothrombin G20120A -Reduced inhibition of the conversion -Recurrent spontaneous abortions -Surgery -Inherited mutation that causes increased prothrombin levels of fibrinogen to fibrin -Livedo reticularis -Definitive DNA test -Fatal if homozygous -Treat indefinitely with warfarin

221

Disseminated Intravascular Coagulation -Can be acute or chronic Signs & Symptoms Workup Management -Bleeding diathesis: petechiae, ecchymosis, oozing -Peripheral smear shows -Treat underlying disease Etiologies from wounds and IVs microangiopathic hemolytic -Hemodynamic support -Usually occurs when shock causes widespread activation of the clotting -Thromboembolism anemia -Most coagulopathies are short- cascade -Renal dysfunction: AKI -Low platelets and clotting lived but some pts with severe -Sepsis -Hepatic dysfunction: jaundice factors (may be near normal in bleeding may need platelets or -Trauma and tissue destruction -Respiratory dysfunction: hemoptysis, dyspnea chronic DIC) FFP transfusions -Malignancy -Shock -Obstetric complications: placental abruption, HELLP syndrome, -CNS involvement: coma, delirium, TIAs hemorrhage, septic abortion

MALIGNANCIES Acute Lymphoblastic Leukemia -Cancer of the lymphoid progenitor, affecting B or T cell lineage Differential Workup Management -Incidence peaks @ 2-5 years and drops @ 8-10 years = more common in -Infection -CBC with differential showing 1-2 -3-4 agent induction therapy children -Aplastic anemia cytopenias -Radiation if CNS disease is present -Juvenile RA -Confirmatory blood smear or bone -Continuous therapy for 2-3 years Signs & symptoms -Other malignancy marrow aspirate showing blasts -Illness over days to weeks -LP to eval for CNS involvement Prognosis -Fever, pallor, petechiae, ecchymoses, lethargy, malaise, anorexia, bone or joint -Overall cure rate 20-40% for adults pain, meningitis, weight loss, lymphadenopathy, splenomegaly, dyspnea Acute Myelogenous Leukemia -Cancer of the myeloid progenitor Signs & symptoms Workup Management (gives rise to all WBCs other than -From cell deficiencies: pallor, fatigue, dyspnea, thrombocytopenia, -Differentiate from ALL by peripheral smear showing -Aggressive chemo B/T and NK cells), where cells do petechiae, hematomas, bleeding, neutropenia with sepsis, cellulitis, Auer rods not mature and do not die and take pneumonia Prognosis up the bone marrow space of other -From hyperleukocytosis: obstruction of capillaries and small arteries -Overall survival of needed cells with high 30% -Most common in first 2 years at -From CNS involvement: HA, AMS, CN issues life, peaks again in adolescence -Leukemia cutis lesions numbers of blasts -DIC -Tumor lysis syndrome Chronic Lymphocytic Leukemia -Clonal proliferation and accumulation of mature- Workup Management Prognosis appear B cells -CBC showing lymphocytosis with WBCS > 20k with concomitant -Observation -Typically slow-growing, but has -The most commonly occurring leukemia anemia and peripheral smear showing mature small lymphocytes -Chemo with tumor lysis potential for Richter’s -Mostly occurs in those > 50, and more common in and cobblestone-appearing smudge cells prophylaxis transformation to aggressive males -Coexpression of CD19 and CD5 -BMT disease -RAI system for staging -High IgG -Radiation for -Worse prognosis with deletion of lymphadenopathy chromosome 17 Signs & symptoms -Average 5 year survival rate of -Fatigue, night sweats, weight loss, persistent 50% infections, lymphadenopathy, hepatomegaly, splenomegaly

222

Chronic Myelogenous Leukemia -Excess proliferation of the myeloblast or its progeny with no Signs & symptoms Workup Management negative feedback -Fever, bone pain, LUQ pain with -Detection of Philadelphia chromosome -Chemo -Usually occurs in young to middle age adults splenomegaly, weakness, night seats, via FISH or RT-PCR -BMT bleeding & bruising, petechiae -CBC showing leukocytosis and Categories thrombocytopenia Prognosis 1.) Chronic: < 15% blast component of bone marrow or blood -Average survival is 6 years with 2.) Accelerated: peripheral blood > 15% blasts or > 30% blasts + treatment promyelocytes, or > 20% basophils 3.) Acute: when blasts comprise > 30% of BM; final phase of CML

Multiple Myeloma -Malignancy of plasma cells where replacement of bone Signs & symptoms Workup Management marrow leads to failure -Forms lytic lesions on bone  -Bone marrow biopsy shows > 5% plasma cells -Chemotherapy -Etiology is unknown, but there is increased incidence with bone pain, pathologic fx, and -Lytic lesions on metastatic bone survey x-ray series -Local radiation for pain control h/o pesticides, paper production, lather tanning, nuclear hypercalcemia -Spikes in M protein in protein electrophoresis (differentiate -Autologous BMT radiation exposure, and abnormalities of chromosome 13 -Renal failure from excretion of from MGUS, where M protein level will still be WNL) -Bisphosphonates for -Multi-hit hypothesis that development of MM requires 2 proteins -IgG and IgA spikes on electrophoresis hypercalcemia oncologic events: MGUS (a common, age-related medical -Fatigue -Peripheral smear showing rouleaux formations (poker condition characterized by accumulation of monoclonal -Recurrent infections chips) Prognosis plasma cells in the BM  moderate IgG spike on -Spinal cord compression -Urine has Bence-Jones proteins (produced by malignant -Average survival with chemo electrophoresis) + 2nd hit causing transition of MGUS to -Hyperviscosity syndrome from plasma cells) is 3 years, 7 years for BMT severe MM high circulating Ig of all kinds -Hypercalcemia -Anemia

Lymphoma Hodgkin Lymphoma Non-Hodgkin Lymphoma -A group of cancers characterized by orderly spread Workup -A diverse group of blood cancers that include any Workup of disease from one lymph node to another and by the -Peripheral smear showing Reed-Sternberg kind of lymphoma except Hodgkin (includes CLL, -CBC and smear are usually normal development of systemic symptoms with advanced cells Waldenstrom’s, and multiple myeloma) -Lymph node biopsy disease -Associated with congenital or acquired -CT scans of chest, abdomen, and -Extranodal presentation in the lung, liver, or BM in -CT scans of chest, abdomen, and pelvis immunodeficiency pelvis some cases -PET scan -Single or multiple areas of involvement -Peaks in adolescence and young adulthood, and in -BM biopsy -Low, intermediate, and high grades Management ages 50+ -Lymph node biopsy -Incidence increases with age -Systemic chemo -Association with EBV Management Signs & symptoms Prognosis Signs & symptoms -Chemo -Lymphadenopathy  hydronephrosis, bowel -70-90% survival rate -Painless, firm lymphadenopathy (often -Low dose radiation obstruction, jaundice, wasting, SVC syndrome supraclavicular and cervical areas), mediastinal mass -Abdominal pain causing cough or SOB, fever, weight loss Prognosis -Fever, weight loss, night sweats -Overall survival 90% but there are 3 -Edema separate risk groups

223

INFECTIOUS DISEASES Fungal Disease Cryptococcosis -Cryptococcus neoformans Signs & symptoms Workup -Invasive fungal infection that is becoming increasingly -Pulmonary infections  solitary, non-calcified nodules -Must culture organism from CSF for definitive dx of prevalent in the immunocompromised population (AIDS, -Meningoencephalitis: seen in HIV, sx occur over 1-2 weeks, meningitis but can presumptively ID with CSF Ag testing prolonged steroids, organ transplant, malignancy, sarcoidosis) -Associated with soil frequented by birds and with rotting Treatment vegetation -Amphotericin B and flucytosine for meningitis -Worldwide distribution Pulmonary Histoplasmosis -Histoplasma capsulatum Signs & symptoms Differential Workup -The most prevalent endemic mycosis in the -Symptoms begin weeks to months following exposure -Sarcoidosis -CXR looks just like sarcoid US -Pneumonia with mediastinal or hilar lymphadenopathy -TB -Histo serologies -Associated with bird and bat droppings, -Mediastinal or hilar masses -Malignancy -Special stains on biopsy chicken coops, farm buildings, abandoning -Pulmonary nodule specimens buildings, caves, wood lots -Cavitary lung disease -EIA: urine, blood, or BAL -Most infections will be asymptomatic or self- -Pericarditis with mediastinal lymphadenopathy limiting -Arthritis or arthralgia + erythema nodosum Management -Dysphagia from esophageal narrowing -Itraconazole for moderate -SVC syndrome infection, amphotericin B for -With dissemination: fever, fatigue, weight loss, GI, CNS, adrenal manifestations severe

Candidiasis -Candidiasis of the esophagus, bronchial tree, or bladder are a result of severe immunosuppression  think HIV Oral Candidiasis (Thrush) Vaginal Candidiasis Cutaneous/Intertriginous -Risk factors: inhaled steroid use, AIDS, antibiotic use, Workup See section on -Commonly affected sites are under the radiation therapy -KOH prep of mouth scrapings vaginitis breasts, abdominal folds, axillae, groin, web spaces, diaper areas Presentation Management -Pseudomembranous candidiasis: most common; white plaques -Nystatin swish or cream Presentation with underlying red mucosa -Disinfect dentures -Beefy red plaques with satellite -Erythematous candidiasis: no white component; agent of -Oral fluconazole if severe lesions denture stomatitis -Infants: oral nystatin swabs for 7-14 days, boiling of bottle -Hyperplastic candidiasis: thick white patches that can’t be nipples and pacifiers Management scraped off -Nystatin powder -Angular cheilitis: corners of mouth -Glossitis with broad spectrum antibiotic use -Cottony feeling in mouth -Loss of taste -Pain with eating and swallowing -May be asymptomatic

224

Pneumocystis Pneumonia -Pneumocystic jiroveci Signs & symptoms Workup Management -Occurs in immunosuppressed individuals: HIV, stem cell or organ -Fever -CXR typically shows diffuse, bilateral -Begin empiric therapy if transplant, cancer pts, chronic steroid therapy, chemotherapy -Dry cough interstitial infiltrates suspecting with Bactrim, -Fulminant respiratory failure -Sputum induction or BAL with Gram atovaquone, or IV pentamidine Prevention stain -Adjunct steroids with PaO2 < 70 -PCP prophylaxis for HIV+ individuals with h/o PCP, CD4 < 200, or h/o or hypoxemia candidiasis with Bactrim DS daily

Bacterial Disease Acute Rheumatic Fever -Sequelae of GAS pharyngitis 2-4 Signs & Symptoms Management weeks after infection (does not -Onset of symptoms 1-5 weeks from start of infection -Treat whether or not pharyngitis is present with penicillin, occur with impetigo)  -Major Jones criteria: migratory arthritis, carditis, cephalexin, or azithromycin, and treat all household inflammatory lesions of heart, valvulitis, CNS involvement (chorea), erythema contacts with + throat cultures joints, and subcutaneous tissue marginatum, subcutaneous nodules -Aspirin is DOC for anti-inflammatory, even in kids -Peak incidence in 5-15 -Minor Jones criteria: arthralgia, fever, elevate ESR or -HF management if present CRP, prolonged PR interval -Continuous prophylaxis against GAS (due to increased Screening severity with subsequent infection) with IM penicillin G -Echo for children and young Workup after end of initial treatment until 18-25 years of age, adults in countries where rheumatic -2 major or 1 major + 2 minor criteria indicate a high indefinitely if valvular disease is present, and for one year if fever is endemic probability of acute rheumatic fever reactive arthritis is present Botulism -Rare but life-threatening neuroparalytic syndrome Infant botulism Signs & symptoms Differential resulting from a neurotoxin released by the -Accounts for most US cases of botulism -Acute onset of bilateral -Myasthenia gravis bacterium Clostridium botulinum -S/s: constipation, weakness, feeding difficulties, hypotonia, cranial neuropathies -Tick paralysis -Clostridia are ubiquitous in the environment, drooling, irritability, weak cry -Symmetric descending -Guillain-Barre syndrome including soil, seafood, and on the surfaces of fruits weakness -Polio and vegetables Wound botulism -Absence of fever -Stroke -110 cases per year occur in the US -Can occur with all kinds of wounds, not just puncture wounds -Patient remains responsive -Heavy metal intoxication -S/s: no prodromal GI symptoms -Normal or slow HR and Foodborne botulism normal BP Management -Ingested preformed botulinum toxin Adult enteric infectious botulism -No sensory deficits except -Botulinum antitoxin -Incubation of hours to 1 week -Enteric colonization without source of preformed toxin ingestion for blurred vision -Additionally, penicillin G for wound S/s: n/v/d, abdominal pain, dry mouth, followed by S/s are same as foodborne botulism -Nonspecific GI symptoms botulism CN involvement and weakness Inhalational botulism Chlamydia -Most commonly reported STI in US Signs & symptoms Workup Prognosis -Frequent coinfection with gonorrhea -May be asymptomatic -Cervical swab with PCR is best -Need retesting 3 months -Vaginal discharge -Urine test for men after treatment Screening -Dysuria -Complications: PID, -Every year for women < 26 -Cervical friability or ectropion Treatment epididymitis, urethritis, -When there is a new sex partner in last 60 -Pelvic or lower abdominal pain -1st line is azithromycin or doxycycline sterility days -Ectopic pregnancy -2nd line is erythromycin or levofloxacin -With > 2 new sex partners in a year -Perihepatitis -Sexual abstinence for 7 days from initiation of therapy -Lymphogranuloma venereum with L serotypes -Treat for gonorrhea as well

225

Cholera -Vibrio cholerae Signs & symptoms Workup -US cases are only acquired overseas or via consumption of -Severe, watery diarrhea -Stool Gram stain for curved Gram neg rods contaminated seafood -Vomiting -PCR for toxinogenic strains

Prevention Management -Dukoral vaccine available -Begin treatment before definitive diagnosis! -Rehydration -Antibiotics: doxycycline, FQ if resistant Diphtheria -Agent is Corynebacterium diphtheriae Signs & symptoms Workup -Transmission is direct or droplet -May be asymptomatic -Throat and membrane cultures -Humans are the only reservoir and -Sore throat immunization does not prevent carriage -Low grade fever Treatment -Some strains produce respiratory toxin  -Malaise -Erythromycin or penicillin heart and nervous system damage (why we -Cervical lymphadenopathy -Airway management vaccinate) -Diphtheria membrane -Prophylax close contacts -Cutaneous diphtheria

Gonorrhea Screening Signs & symptoms Workup Treatment -Every year for women < 26 -Vaginal discharge -Cervical swab with PCR is best -1st line is ceftriaxone injection -When there is a new sex -Abdominal pain -Urine test for men -2nd line is cephalosporin partner in last 60 days -Cervicitis -May need to culture rectum -If pharyngitis is present add azithromycin or doxycycline -With > 2 new sex partners -Most men will be symptomatic with purulent -Treat for chlamydia as well in a year discharge, dysuria, urethritis -Pharyngitis Prognosis -Complications: PID, tubo-ovarian abscess, perihepatitis, vertical transmission

Tetanus -Caused by the soil anaerobe Clostridium tetani Workup -Diagnosis is clinical Signs & Symptoms -Muscle spasms Management -Inadequate vaccination history -Neutralize unbound toxin using tetanus Ig -Metronidazole to eradicate remaining Clostridia -Benzos to control spasms -Labetalol for autonomic hyperactivity Methicillin Resistant Staph Aureus (MRSA) -Healthcare-associated MRSA (HA-MRSA) is associated with severe, invasive disease in Management hospitalized patients -Invasive infections  vancomycin or daptomycin -Community-acquired MRSA (CA-MRSA) can occur in skin and soft tissue infection in -Osteomyelitis  vancomycin or daptomycin young healthy adults with no recent healthcare exposure -Outpatient management of MRSA skin and soft tissue infection  clindamycin, Septra, or -Transmission occurs via contact with a colonized individual or contaminated fomite doxycycline -Inpatient treatment of severe MRSA skin and soft tissue infection  vancomycin, linezolid, or daptomycin 226

Pertussis Prevention Signs & symptoms Workup -Dtap vaccine series for kids -Initial: cold-like; rhinorrhea, lacrimation, dry cough with episodes of severe -Bordetella culture or PCR from nasopharyngeal swab -Tdap vaccination for adults to protect kids cough, low-grade fever; post-tussive emesis -Paroxysmal stage: coughing becomes more severe and may persist up to 10 weeks Management at this stage; paroxysmal whooping may be heard -Macrolides are DOC -Convalescent stage: coughing diminishes as patient recovers and disappears over -Septra is an alternative 2-3 weeks but may recur with subsequent URIs Prognosis -May be infectious for several weeks if untreated

MYCOBACTERIAL DISEASE Tuberculosis Signs & symptoms Workup Active TB drug regimens Monitoring -Latent or primary infection: Asymptomatic -If high suspicion, most clinics don’t workup but put a -Initial for 2 months: isoniazid, rifampin, -Sputum smears and cultures -Active infection: cough, fever, weight loss, mask on and send to ER pyrazinamide, ethambutol throughout treatment night seats, hemoptysis, fatigue, decreased -CXR: active infection (infiltrates in mid or lower fields, -Continuation for 4-7 months: isoniazid and rifampin -Vision checks and color vision appetite, chest pain hilar adenopathy, cavitation, emyema) or previous testing with ethambutol (pulmonary nodules, apical fibrosis, Ghon lesion) Latent TB drug regimens -CMP, CBC, and bili -TB skin test, AFB smear -9 months of isoniazid or 4 months of rifampin Atypical Mycobacterial Disease -Mycobacterium tuberculosis Signs & symptoms Workup -Mycobacterium leprae -MAC: pulmonary disease with cough, fatigue, malaise, -Sputum or BAL culture -Non-tuberculous mycobacteria: MAC, Mycobacterium weakness, dyspnea, chest discomfort, occasional hemoptysis kansasii, Mycobacterium abscessus -M. kansasii presents as lung disease that is very similar to TB Management -Superficial lymphadenitis -3 drug regimen for 12 months+ -Disseminated disease in the immunocompromised -Skin and soft tissue infection from direct inoculation

PARASITIC DISEASE Malaria -At greatest risk for severe Signs & symptoms Differential Workup malaria are young children -Plasmodium falciparum incubation is 12-14 days, P. vivax and P. ovale can cause illness -Viral infection -Light microscopy of peripheral smear is test of and pregnant women weeks or months after initial infection -Meningitis choice but can’t detect low parasitemia -Older children and adults -Fever, chills, malaise, fatigue -Pneumonia -Antigen or antibody tests typically develop partial -Tachycardia and tachypnea -Bacteremia immunity after repeated -Diaphoresis -Leptospirosis Management infection and are at low risk -HA -Typhus -Uncomplicated malaria: chloroquine or for severe disease -Cough -Enteric fever quinine in drug-resistant areas -Anorexia Prevention -N/v/d -Mosquito bite prevention -Abdominal pain -Chemoprophylaxis for -Diarrhea travelers to endemic areas -Arthralgias -Myalgias -Severe malaria (hyperparasitemia)  cerebral malaria, hypoglycemia, acidosis, renal impairment, noncardiogenic pulmonary edema, anemia, liver dysfunction 227

Helminth Infestations Helminth Information Treatment Helminth Information Treatment Cestodes -Flat hermaphroditic worms that can live in the human GI tract -Praziquantel Hookworms -Ancylostoma -Albendazole (Tapeworms) -Taenia saginata = beef tapeworm  mostly asymptomatic, duodenale or may have nausea, anorexia, epigastric pain, or peripheral -Necator mebendazole eosinophilia americanus -Diphyllobothrium lata = fish -Penetrate skin tapeworm  megaloblastic anemia directly from B12 deficiency -Spread by feces -Hymenolepis nana = -S/s: acute GI sx, rodent/arthropod tapeworm, chronic nutritional associated with poor sanitation  deficiency, anemia asymptomatic infection or abdominal -Dx: stool exam for pain, diarrhea, anorexia, pruritus ani eggs -Dx: eggs or proglottids in stool Nematodes -Ascaris lumbricoides = tropical worm, associated with poor -Albendazole Trematodes -Schistosomiasis: fresh water transmission  localized -Praziquantel (Roundworms) sanitation, migrate from small intestine to lungs then back to or dermatitis, fever, intestinal, hepatic, urinary, neurologic, or intestine  asymptomatic mebendazole pulmonary disease infection or transient -Prophylactic -Clonorchis sinensis = liver fluke, ingestion of infected pulmonary symptoms, albendazole q fish  fever, anorexia, abdominal pain, myalgia, bowel obstruction, biliary 3-4 months in arthralgia, malaise, urticaria, bile duct obstruction, weight colic, acalculous endemic areas loss, diarrhea, pancreatitis, recurrent cholangitis, liver cholecystitis, ascending abscess, cholangiocarcinoma cholangitis, obstructive jaundice, pancreatitis -Dx: stool microscopy

-Strongyloides stercoralis  direct penetration of skin  -Ivermectin waxing/waning GI symptoms, cutaneous or pulmonary symptoms, unexplained eosinophilia -Dx: at least 2 stool specimens for larvae -Occurs in the southern US

Pinworms -Parasite is Enterobius vermicularis, a roundworm Signs & symptoms Workup Management -The most common parasitic intestinal infection -Severe rectal itching -Diagnosis is usually clinical -Albendazole, with repetition in 2 weeks -Transmission is fecal-oral or by inhalation -UTI -Tape test with microscopy -Vaginitis

228

Toxoplasmosis -Agent is parasite Toxoplasma gondii Signs & Symptoms Differential Management -Transmission is through ingestion of -Infections are generally asymptomatic -Lymphoma -Usually not required in adults contaminated meat or produce, vertical, via -Fevers, chills, sweats -Primary HIV -Congenital toxoplasmosis  treat with blood transfusion or organ transplantation, or -Cervical lymphadenopathy -Mono pyrimethamine + sulfadiazine for 1 year by handling contaminated animal feces (cats) -Congenital toxoplasmosis: chorioretinitis, intracranial calcifications, seizures, jaundice, Workup Prognosis HSM, lymphadenopathy, anemia, -Toxo IgG antibodies will be present in pts -Infection will persist in latency for lifetime of thrombocytopenia, abnormal CSF, hearing previously exposed/immunized, while IgM infected host but can reactive in times of loss, intellectual disability, motor indicates active infection immunosuppression abnormalities, hydrocephalus -Treated infants remain at risk for long-term sequelae Amebiasis -Entamoeba histolytica Workup -Serology or Ag testing along with parasitic stool exam Signs and symptoms -Intestinal amebiasis has a subacute onset of 1-3 weeks with mild diarrhea or dysentery, Management abdominal pain, bloody stools, can have fulminant colitis with bowel necrosis  perf and -Treat with metronidazole, then paromomycin to kill the cysts peritonitis or toxic megacolon -Extraintestinal manifestations present as liver abscesses or pulmonary, cardiac, or brain involvement

SPIROCHETAL DISEASE Lyme Disease -Borrelia burgdorferi Signs & symptoms Workup Management with a tick vector -Early localized disease: erythema migrans ~1 mo after -Dx can be clinical if -Treat with doxycycline, amoxicillin, or cefuroxime, for 10-21 days for -Transmitted by Ixodes exposure, nonspecific flulike viral syndrome erythema migrans is present erythema migrans, 14-21 days for facial nerve palsy, 28 days for meningitis spp deer tick -Disseminated disease: acute neurologic or cardiac or in endemic areas or arthritis -Transmission usually involvement weeks to months after tick bite, secondary -Serology for antibodies -Ceftriaxone for CNS manifestations does not occur until 72 skin lesions, malaise, fatigue, fever, HA, neck pain, (warning, pts may be -IV antibiotics needed for patients with cardiac symptoms or late neurologic hours after attachment myopericarditis, facial palsy antibody negative for first disease -Late disease: months to years after disease; arthritis, several weeks of disease) -No evidence for extended-course antibiotics for presumed chronic Lyme subtle encephalopathy or polyneuropathy, acrodermatitis -Confirmatory Western blot -Can give single dose doxycycline for prophylaxis if attached tick is chronicum atrophicans identified, estimated to be present > 36 hours, local tick Borrelia infection -HA, fatigue, arthralgias may persist for months after rate > 20% treatment but don’t represent active disease Rocky Mountain Spotted Fever -Most cases in NC, SC, Tennessee, Oklahoma, Signs & symptoms Workup and Arkansas -Chills, fever, headache, nausea, vomiting, myalgias, restlessness, -PCR of skin lesions -Agent in US is Rickettsia rickettsii insomnia, and irritability -Serology will not be positive until 2nd week of disease -Transmitted by Dermacentor variabilis (dog -Rash begins as macules then progresses to maculopapules and petechiae, tick) beginning on wrists and ankles, spreading to arms, legs, and trunk, also Treatment soles and palms -Doxycycline -Facial flushing, conjunctival injection, and hard palate lesions may occur -Splenomegaly, hepatomegaly, jaundice, and myocarditis may occur Prognosis -Possible delirium -High mortality rate if untreated -Pneumonitis with respiratory failure

229

Syphilis -Treponema pallidum Signs & symptoms Workup Management -Most cases are MSM -Primary/acute infection lasts 5-6 weeks: contagious chancre, painless rubbery regional -Remember that negative tests do not -Mandatory reporting -Can be transmitted lymphadenopathy, followed by generalized lymphadenopathy exclude a diagnosis of syphilis within 24 hours vertically from mother -Secondary infection 6 weeks-6 months after exposure (not all pts will develop this): fever, malaise, -Darkfield microscopy of chancre -Penicillin G to fetus HA, arthralgias, bilateral papulosquamous rash on the palms and soles, alopecia, denuded tongue, sample -Recheck serologies at 6 condyloma lata -LP for neurosyphilis and 12 months after Screening -Tertiary infection occurs in disease > 4 years’ duration: end organ manifestations, CV symptoms, -Direct fluorescent antibody testing treatment to look for -Recommended for gummas, neurosyphilis -Serology: RPR (has a 3-6 week fourfold reduction in titer pregnant women at the -Latent infection has no clinical manifestations but serology will be reactive latency period) first prenatal visit, with -Congenital syphilis of infant: stillbirth, prematurity, low birth weight, hydrops fetalis, large or pale -HIV test recommended as syphilis repeat at 28 weeks placenta, inflamed umbilical cord, fever, HSM, lymphadenopathy, failure to thrive, edema, syphilitic facilitates this infection rhinitis, maculopapular rash, condyloma lata, jaundice, anemia, thrombocytopenia, leukopenia or leukocytosis, pneumonia

VIRAL DISEASE Cytomegalovirus -Transmission may be sexual, close contact, or blood and tissue Signs & symptoms Workup exposure -Generally asymptomatic or nonspecific in immunocompetent -CBC shows lymphocytosis -HIV patients and transplant patients are at increased risk of host -PCR test reactivation disease -Can have CMV mononucleosis with fever (distinguish from -Serologies EBV by absence of lymphadenopathy and pharyngitis) -Viral culture -Rare associations with colitis, encephalitis, myocarditis -Can have reactivation in critically ill patients Management -Antivirals only for immunocompromised with severe manifestations Epstein-Barr Virus -Cause of infectious Signs & symptoms Workup Management mononucleosis -Majority of primary infections are asymptomatic -CBC -Supportive -Persists as an -Malaise and anorexia -Rapid monospot test -Pain control asymptomatic latent -N/v -Peripheral smear will show lymphocytosis with -Steroids with emergent ENT referral if impending airway infection for life in -HA atypical lymphocytes obstruction most adults -Low-grade fever -No contact sports for 3 weeks -Pharyngitis and palatal petechiae -Cervical lymphadenopathy Prognosis -Splenomegaly -Associated with development of lymphoma and -Young children/infants: OM, diarrhea, URI nasopharyngeal carcinoma -Morbiliform rash if ampicillin is used -Risk of splenic rupture Human Immunodeficiency Virus (HIV) -Progresses from primary infection with seroconversion  Signs & symptoms Workup Management clinical latency  early symptomatic disease  AIDs -Only lymphadenopathy during asymptomatic disease -Serologies are + 3-7 weeks after -Large debate about -Transmission is mostly heterosexual in developing countries -May have mononucleosis-like syndrome during primary infection aggressive treatment of while both MSM and heterosexual in the US infection -Drug resistance testing primary infection vs waiting -Patients are most infectious during primary infection -Febrile illness -Definition of AIDS is when CD4 until disease is symptomatic -Aseptic meningitis count drops to < 200

230

Herpes Simplex -Over 85% of adults will be + for HSV-1 and 20% will be + Differential Complications Management for HSV-2 by serology -Chancroid -Eczema herpeticum: severe infection in -Acyclovir -Precipitating factors: sunlight, dental surgery, cosmetic -Syphilis the immunocompromised -Valacyclovir surgery, wind, trauma, fever, stress -Pyoderma -Herpetic whitlow: fingernail or hand -Famciclovir -Transmission can be through asymptomatic shedding -Trauma infection -Topical corticosteroid for -First outbreak will be the worst and can last up to 21 days -Herpes gladiatorum: infection anywhere orolabial herpes Workup not covered by underwear -7-10 days for first outbreak and Signs & symptoms -Viral culture is gold standard -Pyoderma 3-5 days for subsequent outbreaks -Prodrome of burning or neuralgia -Serology is questionable, as not all + cultures will -Proctitis, esophagitis -Suppressive therapy if needed -Swollen regional lymph nodes have + serology and vice versa, and many are -Keratitis -Pain with urination asymptomatically + -Encephalitis Human Papilloma Virus -Small DNA viruses that are sexually or contact transmitted Prevention Workup -Sexually transmitted strains are associated with squamous neoplasia of the -HPV vaccines cover most of the sexually transmitted subtypes -Pap cytology anogenital region and oropharynx -Colposcopy Signs & Symptoms Risk Factors -Condyloma acuminata: caused by HPV type 6 and 11, can also Management -Multiple sex partners be 16 or 18 -Most sexually transmitted HPV infections will -Young age at first sexual activity -Cervical dysplasia and oropharyngeal lesions: usually HPV 16 self-resolve -H/o STDs and 18 -Follow resolution of infection with sequential -Multiparity -Common cutaneous warts: HPV types 1, 2, 4 Paps -Immunosuppresion -Anal carcinoma in MSM -Uncircumcised male partner Influenza Influenza vs. common cold Prevention Treatment -Flu = abrupt onset (sx worsen over 3-6 hours) with -Inactive vaccine starting at 6 months (first-time vaccination in -Antipyretic/analgesic fever > 101.5, severe myalgias, headache, malaise, kids under 9 requires 2 doses) -Albuterol neb painful dry cough, sore throat, rhinitis, secondary -Live vaccine if 2-49 and healthy (warning: viral shedding) -Ipratropium inhaler (Atrovent) for secretions Staph aureus pneumonia may follow -Consider steroids -Cold = slow, insidious onset, usually no headache Workup -Consider antivirals (oseltamivir, zanamivir) for influenza A or B only with or chills, sore throat, stuffy nose, sneezing, mild -Nasopharyngeal swab (may be done just for epidemiologic hospitalization, severe or progressive disease, age under 2 or over 65, and aches purposes) for outbreak control in institutions or health care workers, AND MUST BE within 48 hours of start of symptoms to help at all Mumps -Agent is paramyxovirus Signs & symptoms Management -Prevent with MMR vaccine (indicated to prevent severe pain) -Parotitis -Scrotal support and ice packs -Stenson’s duct inflammation with yellow discharge -NSAIDs -Orchitis 7-10 days later, with abrupt fever, testicular swelling and tenderness Prognosis -Possible CNS involvement -Rare chance of sterility with orchitis Varicella Signs & symptoms Management Prognosis -Intensely pruritic lesions that come in crops over 3-4 days  see lesions in -Oral acyclovir for adults with uncomplicated varicella -Can be fatal in adolescents and all different stages -IV acyclovir for immunosuppressed host or with disseminated disease such as adults -Acute neuritis with rash most commonly presenting in the thoracic and pneumonia or encephalitis -Will be contagious for 1 week lumbar dermatomes -Acetaminophen for fever -Disseminated lesions in immunocompromised host -Antihistamines for pruritus 231

Varicella Zoster (Herpes Zoster or Shingles) -Primary infection is varicella, secondary is zoster Treatment Complications -High dose acyclovir within 72 hours of onset -Post-herpetic neuralgia Prevention -Prednisone if over age 50 -Ophthalmic complications -Zostavax vaccine indicated after age 60 -Analgesics -Hemiplegia

SURGICAL INFECTIOUS DISEASE Postoperative Fever Etiologies Prevention Workup Management -Not always infectious! -Avoid atelectasis: early ambulation, incentive -Fever in patient < 2 days out from surgery -Change out infected or thrombosed lines -Wind: atelectasis, pneumonia spirometry who is otherwise doing well is usually self- -Wound infection: open infected area, start -Wound infection: usually occur several days -Avoid pneumonia: use humidified O2 limiting and does not require workup antibiotics to 1 week after operation -DVT prophylaxis -CBC -D/c unnecessary meds, NGT, catheters -Water: UTI -Judicious use of Foley catheters with d/c -CXR: may lag behind PE findings -Treat fever with acetaminophen -Walking: DVT or thrombophlebitis ASAP -Consider LE US for DVT -Broad spectrum antibiotics only for -Wonder drugs: medication-induced fever -Clear instructions for home care of wound -Blood cultures? hemodynamically unstable pts while source is (heparin or abx) sites being identified -Women: postpartum fever, endometritis -Blood transfusion Surgical Site Infection -SSI is defined as infection related to Risk Factors Prevention Components of Optimal Wound the operative procedure occurring at -Obesity -Preop showering with antimicrobial soaps Healing or near the surgical incision within 30 -Smoking -Preop prepping of operative site with antiseptics (chlorhexidine superior to iodine) -Well-vascularized wound bed days of an operative procedure or -DM -Washing and gloving of surgeon’s hands (alcohol rubs may be as effective as -Wound free of devitalized tissue within 1 year of an implant -Systemic corticosteroids traditional soap scrubbing) -Wound clear of infection -Occur in 2-5% of patients -Immunosuppression -Use of sterile drapes -Moist wound undergoing surgery -Malnutrition -Use of gowns and masks by OR personnel -Nonteaching hospitals have lower -Preoperative nasal carriage -Good surgical technique: gentle traction, effective hemostasis, removal of devitalized Management of SSI rates than teaching hospitals with Staph aureus tissue, obliteration of dead space, irrigation with saline, use of fine nonabsorbable -Opening, exploration, draining, -Most common source is direct -Presence of remote focus of monofilament suture, judicious use of closed suction drains, wound closure without irrigation of wound inoculation of pt’s endogenous flora infection tension -Sharp surgical debridement of at the time of surgery -Long duration of -Antibiotic prophylaxis: should be administered within 60 min of first incision; may devitalized tissue -Incidence of resistant pathogens preoperative hospitalization need to be repeated more than once depending on length of surgery -Wound can be closed or allowed cultured from SSIs is increasing: -Preoperative severity of pt -Hair removal: may increase risk of surgical site infection, must use clippers or to heal by secondary intention MRSA, MRSE, VRE illness depilatories if removing hair vs razor once granulation tissue is apparent -Incidence of fungi cultured from  Can predict pt risk of SSI -Tight glucose control in diabetic pts SSIs is increasing: Candida albicans with National Healthcare -Perioperative warming (bear huggers) to prevent hypothermia, warmed IVF, hats and Safety Network risk index booties -Minimally invasive and laparoscopic procedures associated with ↓ risk of SSI

232

ADDITIONAL EMERGENCY MEDICINE TOPICS Near Drowning Signs & Symptoms Management Prognosis -Pulse detection may be difficult due to hypothermia -Ventilation is most important -Neuroprotective effects of hypothermia can result in complete -If pt does not respond to 2 rescue breaths, CPR should begin recovery of patients despite cardiac arrest and prolonged resuscitation -Intubation if needed of several hours -Rewarming techniques Carbon Monoxide Poisoning Causes Signs & Symptoms Workup -Poorly functioning heating systems -Headache -ABG cooximetry (pO2 will look normal) -Improperly vented kerosene heaters, charcoal grills, camping -Malaise stoves, gasoline powered generators, or running cars -Nausea and dizziness Management -CO outcompetes with O2 binding Hb -AMS, seizures, or coma -100% O2 via nonrebreather -Cardiac ischemia -?Hyperbaric therapy for severe intoxication Electrical Injury Causes Signs & Symptoms Management -Lightning -Manifestations range from mild superficial burns to severe multiorgan dysfunction and death -Trauma evaluation -AC current: more dangerous -Cardiac: arrhythmias -Telemetry -DC current: travels in one direction so individual is -Skin: superficial to full-thickness burns -Monitoring for compartment syndrome, thrown off of electrical source -Msk: periosteal burns, destruction of bone matrix, osteonecrosis, rhabdo, compartment syndrome rhabdo, and AKI -Renal: AKI from rhabdo -Neuro: coma, cognitive deficits, autonomic dysfunction -Vascular: small vein coagulation, major artery thrombosis -TM rupture -Entry and exit sites for DC current exposure Anaphylaxis -Typically an IgE-mediated Signs & Symptoms Workup Management reaction -Cardiopulmonary arrest: occurs in 5 -Transiently elevated plasma histamine or total -Epinephrine -Common triggers are minutes with iatrogenic anaphylaxis, tryptase -Immediate intubation if signs of impending airway obstruction from foods, insect stings, and 15 minutes in stinging insect angioedema medications anaphylaxis, and 30 minutes in food- -Oxygen via face mask induced anaphylaxis -Rapid NS infusion of 1-2 L -Generalized urticaria -Also consider albuterol, diphenhydramine, ranitidine, and/or -Angioedema methylprednisolone -Flushing -Refractory symptoms  epinephrine infusion, other pressors, -Pruritus glucagon -Referral to allergist/immunologist to confirm diagnosis and triggers

233

234