Pain Assessment and Treatment in Children with Significant Impairment of the Central Nervous System Julie Hauer, Amy J
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Differentiating Between Anxiety, Syncope & Anaphylaxis
Differentiating between anxiety, syncope & anaphylaxis Dr. Réka Gustafson Medical Health Officer Vancouver Coastal Health Introduction Anaphylaxis is a rare but much feared side-effect of vaccination. Most vaccine providers will never see a case of true anaphylaxis due to vaccination, but need to be prepared to diagnose and respond to this medical emergency. Since anaphylaxis is so rare, most of us rely on guidelines to assist us in assessment and response. Due to the highly variable presentation, and absence of clinical trials, guidelines are by necessity often vague and very conservative. Guidelines are no substitute for good clinical judgment. Anaphylaxis Guidelines • “Anaphylaxis is a potentially life-threatening IgE mediated allergic reaction” – How many people die or have died from anaphylaxis after immunization? Can we predict who is likely to die from anaphylaxis? • “Anaphylaxis is one of the rarer events reported in the post-marketing surveillance” – How rare? Will I or my colleagues ever see a case? • “Changes develop over several minutes” – What is “several”? 1, 2, 10, 20 minutes? • “Even when there are mild symptoms initially, there is a potential for progression to a severe and even irreversible outcome” – Do I park my clinical judgment at the door? What do I look for in my clinical assessment? • “Fatalities during anaphylaxis usually result from delayed administration of epinephrine and from severe cardiac and respiratory complications. “ – What is delayed? How much time do I have? What is anaphylaxis? •an acute, potentially -
FF #93 THC Cancer AIDS.3Rd Ed
! FAST FACTS AND CONCEPTS #93 CANNABINOIDS IN THE TREATMENT OF SYMPTOMS IN CANCER AND AIDS L Scott Wilner MD and Robert M Arnold MD Introduction The healing properties of cannabis have been asserted for centuries. Popular claims notwithstanding, there are no data to support the use of marijuana in the treatment of asthma, anxiety, depression, epilepsy, glaucoma, alcohol withdrawal, or infection; and limited data to support using cannabinoids as analgesics. Recent scientific studies of cannabinoids for symptom management have focused on nausea/vomiting and appetite stimulation. Terminology Cannabis sativa is the Indian hemp plant. Marijuana is a psychoactive substance derived from the plant. Cannabinoids are the biologically active compounds in the plant. THC is delta )-9 tetrahydrocannabinol, the major cannabinoid. Dronabinol is synthetic THC and the main ingredient in the Schedule 3 drug Marinol. Nabilone is an engineered THC analog that forms the basis of the Schedule 2 drug Cesamet. Pharmacology Cannabinoids act on cannabinoid receptors: the CB1 receptor in the CNS and on the CB2 receptor localized primarily to immune cells. Dronabinol and nabilone are well absorbed orally, but first pass metabolism and protein binding limit bioavailability. Dronabinol has a faster onset of action (~30 minutes), while nabilone has a longer duration of action (typically 8 – 12 hours, but potentially as long as 24 hours in some patients). Alternative delivery systems – including inhalers, suppositories, and transdermal patches – are being evaluated. Anti-emetic Use Dronabinol and nabilone are FDA approved for the treatment of nausea and vomiting associated with cancer chemotherapy in patients who have failed to respond to conventional antiemetics. -
Pharmacologic Interventions for Fatigue in Cancer and Transplantation: a Meta-Analysis
DRUG INTERVENTIONS FOR FATIGUE, Tomlinson et al. REVIEW ARTICLE Pharmacologic interventions for fatigue in cancer and transplantation: a meta-analysis † † ‡ D. Tomlinson RN MN,* P.D. Robinson MD MSc, S. Oberoi MD, D. Cataudella PsyD CPsych, § || # N. Culos-Reed PhD, H. Davis,* N. Duong,* F. Gibson RN PhD, M. Götte PhD, P. Hinds RN PhD,** †† †† † ‡‡ §§ S.L. Nijhof MD PhD, P. van der Torre, S. Cabral, L.L. Dupuis MScPhm PhD,* and L. Sung MD PhD* ABSTRACT Background Our objective was to determine whether, compared with control interventions, pharmacologic interventions reduce the severity of fatigue in patients with cancer or recipients of hematopoietic stem-cell transplantation (hsct). Methods For a systematic review, we searched medline, embase, the Cochrane Central Register of Controlled Trials, cinahl, and Psychinfo for randomized trials of systemic pharmacologic interventions for the management of fatigue in patients with cancer or recipients of hsct. Two authors independently identified studies and abstracted data. Methodologic quality was assessed using the Cochrane Risk of Bias tool. The primary outcome was fatigue severity measured using various fatigue scales. Data were synthesized using random-effects models. Results In the 117 included trials (19,819 patients), the pharmacologic agents used were erythropoietins (n = 31), stimulants (n = 19), l-carnitine (n = 6), corticosteroids (n = 5), antidepressants (n = 5), appetite stimulants (n = 3), and other agents (n = 48). Fatigue was significantly reduced with erythropoietin [standardized mean difference (smd): –0.52; 95% confidence interval (ci): –0.89 to –0.14] and with methylphenidate (smd: –0.36; 95% ci: –0.56 to –0.15); modafinil (or armodafinil) and corticosteroids were not effective. -
Review Cutaneous Patterns Are Often the Only Clue to a a R T I C L E Complex Underlying Vascular Pathology
pp11 - 46 ABstract Review Cutaneous patterns are often the only clue to a A R T I C L E complex underlying vascular pathology. Reticulate pattern is probably one of the most important DERMATOLOGICAL dermatological signs of venous or arterial pathology involving the cutaneous microvasculature and its MANIFESTATIONS OF VENOUS presence may be the only sign of an important underlying pathology. Vascular malformations such DISEASE. PART II: Reticulate as cutis marmorata congenita telangiectasia, benign forms of livedo reticularis, and sinister conditions eruptions such as Sneddon’s syndrome can all present with a reticulate eruption. The literature dealing with this KUROSH PARSI MBBS, MSc (Med), FACP, FACD subject is confusing and full of inaccuracies. Terms Departments of Dermatology, St. Vincent’s Hospital & such as livedo reticularis, livedo racemosa, cutis Sydney Children’s Hospital, Sydney, Australia marmorata and retiform purpura have all been used to describe the same or entirely different conditions. To our knowledge, there are no published systematic reviews of reticulate eruptions in the medical Introduction literature. he reticulate pattern is probably one of the most This article is the second in a series of papers important dermatological signs that signifies the describing the dermatological manifestations of involvement of the underlying vascular networks venous disease. Given the wide scope of phlebology T and its overlap with many other specialties, this review and the cutaneous vasculature. It is seen in benign forms was divided into multiple instalments. We dedicated of livedo reticularis and in more sinister conditions such this instalment to demystifying the reticulate as Sneddon’s syndrome. There is considerable confusion pattern. -
Megestrol Acetate: Drug Information
Official reprint from UpToDate® www.uptodate.com ©2017 UpToDate® Megestrol acetate: Drug information Copyright 1978-2017 Lexicomp, Inc. All rights reserved. (For additional information see "Megestrol acetate: Patient drug information" and see "Megestrol acetate: Pediatric drug information") For abbreviations and symbols that may be used in Lexicomp (show table) Brand Names: US Megace ES; Megace Oral Brand Names: Canada Megace OS; Megestrol Pharmacologic Category Antineoplastic Agent, Hormone; Appetite Stimulant; Progestin Dosing: Adult Note: Megace ES suspension is not equivalent to other formulations on a mg-per-mg basis. Anorexia or cachexia associated with AIDS: Oral: Suspension: U.S. labeling: Initial: 625 mg daily (of the 125 mg/mL suspension) or 800 mg daily (of the 40 mg/mL suspension); daily doses of 400 mg to 800 mg have been found to be effective Canadian labeling: Usual dose: 400 to 800 mg once daily for at least 2 months Breast cancer, advanced: Oral: Tablet: U.S. labeling: 160 mg per day in divided doses of 40 mg 4 times daily for at least 2 months Canadian labeling: 160 mg or 125 mg/m2 daily (40 mg 4 times daily or 160 mg once daily) for at least 2 months Endometrial cancer, advanced: Oral: Tablet: U.S. labeling: 40 to 320 mg daily in divided doses for at least 2 months Canadian labeling: 80 to 320 mg or 62.5 to 250 mg/m2 daily in divided doses (40 to 80 mg 1 to 4 times daily or 160 to 320mg daily) for at least 2 months Cancer-related cachexia: Canadian labeling: Oral: Tablet: 400 to 800 mg once daily for at least 2 months Cancer-related cachexia (off-label use/dosing in U.S.): Oral: Doses ranging from 160 to 800 mg per day were effective in achieving weight gain, higher doses (>160 mg) were associated with more weight gain (Beller, 1997; Loprinzi, 1990; Loprinzi, 1993; Vadell, 1998); based on a meta-analysis, an optimal dose has not been determined (Ruiz Garcia, 2013) Dosing: Geriatric Use with caution; refer to adult dosing. -
Skin Manifestations of Illicit Drug Use Manifestações Cutâneas
RevABDV81N4.qxd 12.08.06 13:10 Page 307 307 Educação Médica Continuada Manifestações cutâneas decorrentes do uso de drogas ilícitas Skin manifestations of illicit drug use Bernardo Gontijo 1 Flávia Vasques Bittencourt 2 Lívia Flávia Sebe Lourenço 3 Resumo: O uso e abuso de drogas ilícitas é um problema significativo e de abrangência mun- dial. A Organização das Nações Unidas estima que 5% da população mundial entre os 15 e 64 anos fazem uso de drogas pelo menos uma vez por ano (prevalência anual), sendo que meta- de destes usam regularmente, isto é, pelo menos uma vez por mês. Muitos dos eventos adver- sos das drogas ilícitas surgem na pele, o que torna fundamental que o dermatologista esteja familiarizado com essas alterações. Palavras-chave: Drogas ilícitas; Drogas ilícitas/história; Drogas ilícitas/efeitos adversos; Pele; Revisão Abstract: Illicit drug use and abuse is a major problem all over the world. The United Nations estimates that 5% of world population (aged 15-64 years) use illicit drugs at least once a year (annual prevalence) and half of them use drugs regularly, that is, at least once a month. Many adverse events of illicit drugs arise on the skin and therefore dermatologists should be aware of these changes. Keywords: Street drugs; Street drugs/history; Street drugs/adverse effects; Skin; Review HISTÓRICO Há mais de cinco mil anos na Mesopotâmia, minorar o sofrimento dos condenados. região onde hoje se situa o Iraque, os poderes cal- Provavelmente seja o ópio a droga nephente à qual mantes, soníferos e anestésicos do ópio (do latim Homero se referia como “o mais poderoso destrui- opium, através do grego opion, ‘seiva, suco’) já eram dor de mágoas”.1,2 conhecidos pelos sumérios. -
Quick Tips on Recognizing Cutaneous Manifestations of Systemic Disease
FEATURE ARTICLE 2.0 Quick Tips on Recognizing Contact Hours Cutaneous Manifestations of Systemic Disease Jennifer Cittadino ABSTRACT: Although many skin manifestations are isolated This article will concentrate on nonmalignant cutaneous to a dermatological disorder, some skin manifestations can manifestations of systemic disease. The body systems will berelatedtoadeeper,moresystemicissue.Theskinis be reviewed, and their associated skin manifestations will be related to other body systems and functions. The impor- discussed. Some of the skin manifestations can be seen in tance of being able to recognize these dermatological multiple systemic disorders, such as erythema nodosum, manifestations and associate them with potential systemic which can be seen in pulmonary disorders, collagen and disease can be a critical diagnostic tool for the practitioner. vascular disorders, and gastrointestinal disorders. Pictures Sometimes, these skin manifestations can be the first sign will be provided when possible. of an internal disease. Although challenging, recognition, prompt diagnosis, and treatment can alter the course of a CARDIOVASCULAR AND PULMONARY SYSTEM life-threatening illness. There are several general cutaneous signs of cardiovascular Key words: Cutaneous, Dermatology, Systemic, Skin disease that may commonly be seen. These findings may include edema, cyanosis, clubbing of the nails, and corneal he skin is the largest organ in the body and one arcus. The corneal arcus appears as a gray-white ring around of the most visible body systems. The skin has the cornea and correlates with age and cholesterol levels. a reciprocal relationship between what is visu- A diagonal earlobe crease is also reported to be a marker for alized on the surface and what is occurring coronary artery disease (Uliasz & Lebwohl, 2008). -
39 Voiding Dysfunction in Patients with Dysautonomia
39 Shridharani A1, Guralnick M1, Barboi A1, Jaradeh S1, Prieto T1, Yellick M1, O'Connor R C1 1. Medical College of Wisconsin VOIDING DYSFUNCTION IN PATIENTS WITH DYSAUTONOMIA Hypothesis / aims of study Dysautonomia, or autonomic dysfunction, is a primary neurologic condition resulting from failure of the sympathetic or parasympathetic nervous systems. The disorder has a myriad of clinical presentations including dysregulation of body temperature, orthostatic intolerance, gastrointestinal motility disorders and chronic pain syndromes. Urologically, while sexual dysfunction has been recognized as part of the autonomic dysfunction spectrum, voiding symptoms have been inadequately characterized. We present the chief urologic complaints, results of urodynamic studies and treatments of patients with a known history of dysautonomia referred to our neuro-urology clinic. Study design, materials and methods Retrospective chart review was performed on all patients seen between 2003 and 2008 in the neuro-urology clinic for voiding dysfunction with the concomitant diagnosis of dysautonomia. Patients with other neurologic diagnoses, such a multiple sclerosis or a history of spinal surgery, were excluded from the analysis. All patients underwent focused history and physical examination as well as video urodynamic studies. Upper tract imaging by renal ultrasound or computerized tomography of the abdomen/pelvis was performed on select patients. Treatment modalities that subjectively and objectively improved the patient’s symptoms were recorded. Objective improvements were measured via post void residual bladder volume, uroflowmetry and/or urodynamic studies. Results Of 443 patients with the diagnosis of dysautonomia, 37 (8%) were referred for evaluation of voiding dysfunction. Mean age was 47 years (range 12 - 80) and 31/37 (84%) patients were female. -
Neuromuscular Weakness in The
Neuromuscular Disorders in the Intensive Care Unit -when and how M. S. Damian, Addenbrookes Hospital, Cambridge Background to this talk? • Neuromuscular admissions to the ICU are increasing • The incidence of individual conditions is not clear • The true outcomes of these patients are unclear • Mortality rates in patients treated with Myasthenia and Guillain Barre Syndrome have not significantly improved in the last 20 years, neither have treatments [Damian MS, Howard R, Int Care Med 2013] • Bad medicine is expensive (Example: An MG case with recurrent crises. 1 year pre-Rituximab: £39,810 costs incl. 14d ICU stay 1 year on Rituximab: ca. £8,000 total costs, no ICU stay M. S. Damian, Addenbrookes Hospital, Cambridge Which neuromuscular symptoms may require treatment in the ICU? • Severe respiratory weakness • Bulbar weakness and aspiration • Cardiomyopathy and heart failure • Arrhythmia • Dysautonomia • Acute rhabdomyolysis and renal failure M. S. Damian, Addenbrookes Hospital, Cambridge 3 main groups of patients with neuromuscular disease may require treatment in the ICU I. Patients with severe new onset of neuromuscular disease II. Patients with pre-existing chronic neuromuscular conditions who develop acute complications III. Patients whose neuromuscular disorder arises in the ICU M. S. Damian, Addenbrookes Hospital, Cambridge Severe new onset neuromuscular disease in the ICU • Guillain Barre Syndrome • Severe acute neuropathy • Acute flaccid paralysis syndrome (WNV, enterovirus 71- mostly with encephalitis) • Myasthenic crisis and -
EDS, Autonomic Dysfunction and MCAS Info
Miguel Trevino, MD Internal Medicine Ehlers-Danlos Syndromes Are a Clinical Subtype of Characterized by: Connective Tissue Disorders Joint Hypermobility (joints They can be inherited and are that stretch further than varied in: normal) • How they affect the body Skin Hyper Extensibility (skin • In their genetic causes that can be stretched further than normal) Tissue Fragility, Easy Bruising Generalized Joint Hypermobility A B C • Five or more of the • Positive family history • Pain in two or more following: • In first-degree extremities The 2017 International • Soft velvety skin relatives diagnosed • For three + month with these criteria Diagnostic Criteria for • Skin hyper extensibility • Recurrent joint hEDS have: dislocations • Striae (stretch marks ) • Atraumatic joint • Piezogenic heel papules Three Criteria (A,B,C) instability • Hernias • Atrophic scarring • Prolapse of pelvic floor ALL of which MUST be • Rectum or uterus present: • Dental crowding and high palate • Arachnodactyly (long, slender fingers) • Arm-span-to-height ratio >1.05 • Mitral valve prolapse or aortic root dilatation Evaluation of HSD There are NO Diagnostic Laboratory Tests for HSD The Beighton Score is a Screening Technique for hypermobility Used to Evaluate/Assess the Range Of Movement in some joints Joint Hypermobility or Laxity is the Hallmark of most types of EDS Beighton Hypermobility Scale is widely used The following maneuvers are performed: Flexion of waist with Passive dorsiflexion palms on the floor of the fifth finger (and with the knees >90 degrees -
The Latest in Research in Familial Dysautonomia
2018 – 2019 YEAR IN REVIEW THE LATEST IN RESEARCH IN FAMILIAL DYSAUTONOMIA _______ A MESSAGE FROM OUR DIRECTOR______ ver the last 12 months, the Center’s research efforts have continued us on the path of finding better treatments. There has never been a more O exciting time when it comes to developing new therapies for neurological diseases. In other rare diseases, it has been possible to edit genes, fix protein production, and even cure illnesses with a single infusion. These new treatments have been accomplished thanks to basic scientists and clinicians working together. Over the last 11-years, I have watched the Center grow into a powerhouse of clinical care as well as research built on training, learning, and collaboration. The team at the Center has built a research framework on an international scale, which means no patient will be left behind when it comes to developing treatments. We now follow patients in the United States, Israel, Canada, England, Belgium, Germany, Argentina, Brazil, Australia and Mexico. The natural history study where we collect all clinical and laboratory data is helping us design the trials to get new treatments in to the clinic as required by the US Food and Drug Administration (FDA). In December 2018, I visited Israel to attend the family caregiver conference and made certain that all Israeli patients participate in the natural history study, a critical step to enroll the necessary number of patients. Because FD is a rare disease, we need patients from all corners of the globe to participate. Geographical constraints should not limit be a limit to the progress we can make for FD. -
(MPN) Symptoms
RECOGNIZING SYMPTOMS RECOGNIZING YOUR MPN SYMPTOMS The MPN LANDMARK SURVEY* is a large-scale analysis of patients with myeloproliferative neoplasms (MPNs) and Healthcare Professionals (HCPs) who treat these rare, chronic blood cancers (813 patients; 457 hematologists/oncologists). The results from LANDMARK provide new information that validates previous findings about the effect of MPN symptoms and their impact on the lives of patients, even those with low-risk MPNs. Fatigue is a symptom of particular note. Across diseases it was reported as the most common and severe symptom, and the one that patients most wanted to resolve. Symptoms such as fatigue can be vague and challenging to quantify. It is difficult to measure tiredness and its impact on daily life. In addition to physical symptoms, patients in the survey reported emotional difficulties—feeling irritable, angry or depressed. Many patients surveyed suffered with their MPN symptoms for a year or more before diagnosis. LANDMARK helps to confirm that MPN symptoms have a significant, negative impact on daily activities and quality of life, often resulting in limited or canceled family and/or social activities. For some, it means having to miss days from work, reduce hours, or even leave their job. These issues create stress, anxiety, and financial hardship for MPN patients. This information is intended to help patients validate the impact of MPN symptoms and know that they are not alone. SYMPTOM RECOGNITION Patients in the survey often did not recognize the connection between certain symptoms they were experiencing and their MPN. For example, patients with MPNs often have difficulty sleeping; however, some patients who reported this symptom did not realize it was related to their MPN.