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Clinical Classification of Caroli's Disease: an Analysis of 30 Patients
View metadata, citation and similar papers at core.ac.uk brought to you by CORE provided by Elsevier - Publisher Connector DOI:10.1111/hpb.12330 HPB ORIGINAL ARTICLE Clinical classification of Caroli's disease: an analysis of 30 patients Zhong-Xia Wang1,2*, Yong-Gang Li2*, Rui-Lin Wang2, Yong-Wu Li3, Zhi-Yan Li3, Li-Fu Wang2, Hui-Ying Yang2, Yun Zhu2, Yao Wang2, Yun-Feng Bai2, Ting-Ting He2, Xiao-Feng Zhang2 & Xiao-He Xiao1,2 1Department of Graduate School, 301 Hospital, 2Integrative Medical Centre, and 3Imaging Centre, 302 Hospital, Beijing, China Abstract Background: Caroli's disease (CD) is a rare congenital disorder. The early diagnosis of the disease and differentiation of types I and II are of extreme importance to patient survival. This study was designed to review and discuss observations in 30 patients with CD and to clarify the clinical characteristics of the disease. Methods: The demographic and clinical features, laboratory indicators, imaging findings and pathology results for 30 patients with CD were reviewed retrospectively. Results: Caroli's disease can occur at any age. The average age of onset in the study cohort was 24 years. Patients who presented with symptoms before the age of 40 years were more likely to develop type II CD. Approximately one-third of patients presented without positive signs at original diagnosis and most of these patients were found to have type I CD on pathology. Anaemia, leucopoenia and thrombocytopoenia were more frequent in patients with type II than type I CD. Magnetic resonance cholangiopancreatography (MRCP) and computed tomography (CT) examinations were most useful in diagnosing CD. -
Biliary Tract
2016-06-16 The role of cytology in management of diseases of hepatobiliary ducts • Diagnosis in patients with radiologically/clinically detected lesions • Screening of dysplasia/CIS/cancer in risk groups biliary tract cytology • Preoperative evaluation of the candidates for liver transplantation (Patients with cytological low-grade and high-grade Mehmet Akif Demir, MD dysplasia/adenocarcinoma are currently referred for liver transplantation Sahlgrenska University Hospital in some institutions). Gothenburg Sweden Sarajevo 18th June 2016 • Diagnosis of the benign lesions and infestations False positive findings • majority of false positive cases have a Low sensitivity but high specificity! background of primary sclerosing cholangitis. – lymphoplasmacytic sclerosing pancreatitis and cholangitis, – primary sclerosing cholangitis, – granulomatous disease, – non-specific fibrosis/inflammation – stone disease. False negative findings • Repeat brushing increases the diagnostic yield and should be performed when sampling • Poor sampling biliary strictures with a cytology brush at ERCP. • Lack of diagnostic criteria for dysplasia-carcinoma in situ • Difficulties in recognition of special tumour types – well-differentiated cholangiocarcinoma with tubular architecture • Predictors of positive yield include – gastric foveolar type cholangiocarcinoma with mucin-producing – tumour cells. older age, •Underestimating the significance of the smear background – mass size >1 cm, and – stricture length of >1 cm. •The causes of false negative cytology –sampling -
Journal of Medical Genetics April 1992 Vol 29 No4 Contents Original Articles
Journal of Medical Genetics April 1992 Vol 29 No4 Contents Original articles Beckwith-Wiedemann syndrome: a demonstration of the mechanisms responsible for the excess J Med Genet: first published as on 1 April 1992. Downloaded from of transmitting females C Moutou, C Junien, / Henry, C Bonai-Pellig 217 Evidence for paternal imprinting in familial Beckwith-Wiedemann syndrome D Viljoen, R Ramesar 221 Sex reversal in a child with a 46,X,Yp+ karyotype: support for the existence of a gene(s), located in distal Xp, involved in testis formation T Ogata, J R Hawkins, A Taylor, N Matsuo, J-1 Hata, P N Goodfellow 226 Highly polymorphic Xbol RFLPs of the human 21 -hydroxylase genes among Chinese L Chen, X Pan, Y Shen, Z Chen, Y Zhang, R Chen 231 Screening of microdeletions of chromosome 20 in patients with Alagille syndrome C Desmaze, J F Deleuze, A M Dutrillaux, G Thomas, M Hadchouel, A Aurias 233 Confirmation of genetic linkage between atopic IgE responses and chromosome 1 1 ql 3 R P Young, P A Sharp, J R Lynch, J A Faux, G M Lathrop, W 0 C M Cookson, J M Hopkini 236 Age at onset and life table risks in genetic counselling for Huntington's disease P S Harper, R G Newcombe 239 Genetic and clinical studies in autosomal dominant polycystic kidney disease type 1 (ADPKD1) E Coto, S Aguado, J Alvarez, M J Menendez-DIas, C Lopez-Larrea 243 Short communication Evidence for linkage disequilibrium between D16S94 and the adult onset polycystic kidney disease (PKD1) gene S E Pound, A D Carothers, P M Pignatelli, A M Macnicol, M L Watson, A F Wright 247 Technical note A strategy for the rapid isolation of new PCR based DNA polymorphisms P R Hoban, M F Santibanez-Koref, J Heighway 249 http://jmg.bmj.com/ Case reports Campomelic dysplasia associated with a de novo 2q;1 7q reciprocal translocation I D Young, J M Zuccollo, E L Maltby, N J Broderick 251 A complex chromosome rearrangement with 10 breakpoints: tentative assignment of the locus for Williams syndrome to 4q33-q35.1 R Tupler, P Maraschio, A Gerardo, R Mainieri G Lanzi L Tiepolo 253 on September 26, 2021 by guest. -
Pediatrics-EOR-Outline.Pdf
DERMATOLOGY – 15% Acne Vulgaris Inflammatory skin condition assoc. with papules & pustules involving pilosebaceous units Pathophysiology: • 4 main factors – follicular hyperkeratinization with plugging of sebaceous ducts, increased sebum production, Propionibacterium acnes overgrowth within follicles, & inflammatory response • Hormonal activation of pilosebaceous glands which may cause cyclic flares that coincide with menstruation Clinical Manifestations: • In areas with increased sebaceous glands (face, back, chest, upper arms) • Stage I: Comedones: small, inflammatory bumps from clogged pores - Open comedones (blackheads): incomplete blockage - Closed comedones (whiteheads): complete blockage • Stage II: Inflammatory: papules or pustules surrounded by inflammation • Stage III: Nodular or cystic acne: heals with scarring Differential Diagnosis: • Differentiate from rosacea which has no comedones** • Perioral dermatitis based on perioral and periorbital location • CS-induced acne lacks comedones and pustules are in same stage of development Diagnosis: • Mild: comedones, small amounts of papules &/or pustules • Moderate: comedones, larger amounts of papules &/or pustules • Severe: nodular (>5mm) or cystic Management: • Mild: topical – azelaic acid, salicylic acid, benzoyl peroxide, retinoids, Tretinoin topical (Retin A) or topical antibiotics [Clindamycin or Erythromycin with Benzoyl peroxide] • Moderate: above + oral antibiotics [Minocycline 50mg PO qd or Doxycycline 100 mg PO qd], spironolactone • Severe (refractory nodular acne): oral -
Value of 48- Or 72-Hr Urine Collections in Performing the Schilling Test
VALUE OF 48- OR 72-HR URINE COLLECTIONS IN PERFORMING THE SCHILLING TEST Edward B. Silberstein Radioisotope Laboratory, Cincinnati General Hospital, Cincinnati, Ohio In 21 % of 71 consecutive, normal Schilling serum vitamin B,2 levels less than 50 pg/mI, and tests evaluated in the Radioisotope Laboratory patients with abnormal vitamin B12 absorption as of the Cincinnati General Hospital, normal 57Co measured by a whole-body counter (6—10). cyanocobalamin excretion (greater than 8% of The test was performed, as previously described a test dose of 0.5 @g)was not achieved until 48— (5), with 0.5 @gof 57Co-cyanocobalamin, 1 @@Ci/ 72 hr. it is recommended that the vitamin B,2 @Lg;however, instead of a single day's collection, adsorption test, as described by Schilling, be serial 24-hr urines were collected for 48—72 hr with altered to routinely include at least a 48-hr additional “flushing―doses of 1 mg of cyanoco urine collection. balamin given intramuscularly at the beginning of the second and third days of the test. Each individual in this study produced at least 500 ml of urine per The Schilling test remains an important diagnos 24 hr with creatinine content exceeding 15 mg/kg tic procedure in the study of patients with megalo body weight if volume was under 500 ml to prove blastic anemia and/or peripheral neuropathy. In the that a full day's collection was made (1 1) . The 72-hr original description of the vitamin B,2 absorption collection was made if there was azotemia (BUN test by Schilling ( 1) , a 24-hr urine collection was exceeding 25 mg% ) or in any patient older than obtained after the oral administration of radioactive 65 years. -
THE ACUTE ABDOMEN Definition Abdominal Pain of Short Duration
THE ACUTE ABDOMEN Definition Abdominal pain of short duration that is usually associated with muscular rigidity, distension and vomiting, and which requires a decision whether an emergent operation is required. Problems and management options History and physical examination are central in the evaluation of the acute abdomen. However, in an ICU patient, these are often limited by sedation, paralysis and mechanical ventilation, and obscured by a protracted, complicated inhospital course. Often an acute abdomen is inferred from unexplained sepsis, hypovolaemia and abdominal distension. The need for prompt diagnosis and early treatment by no means equates with operative management. While it is a truism that correct diagnosis is the essential preliminary to correct treatment, this is probably more so in nonoperative management. On occasions, the need for operation is more obvious than the diagnosis and no delay should be incurred in an attempt to confirm the diagnosis before surgery. Frequently fluid resuscitation and antibiotics are required concurrently with the evaluation process. The approach is to evaluate the ICU patient in the context of the underlying disorder and decide on one of the following options: ∙ Immediate operation (surgery now)– the ‘bleeder’ e.g. ruptured ectopic pregnancy, ruptured abdominal aortic aneurysm (AAA) in the salvageable patient ∙ Emergent operation (surgery tonight)– the ‘septic’ e.g. generalized peritonitis from perforated viscus ∙ Early operation (surgery tomorrow)– the ‘obstructed’, e.g. obstructed colonic cancer ∙ Radiologically guided drainage – e.g. localized abscesses, acalculous cholecystitis, pyonephrosis ∙ Active observation and frequent reevaluation – e.g localized peritoneal signs other than in the RLQ, selected cases of endoscopic perforation . -
Gastroenterostomy and Vagotomy for Chronic Duodenal Ulcer
Gut, 1969, 10, 366-374 Gut: first published as 10.1136/gut.10.5.366 on 1 May 1969. Downloaded from Gastroenterostomy and vagotomy for chronic duodenal ulcer A. W. DELLIPIANI, I. B. MACLEOD1, J. W. W. THOMSON, AND A. A. SHIVAS From the Departments of Therapeutics, Clinical Surgery, and Pathology, The University ofEdinburgh The number of operative procedures currently in Kingdom answered a postal questionnaire. Eight had vogue in the management of chronic duodenal ulcer died since operation, and three could not be traced. The indicates that none has yet achieved definitive status. patients were questioned particularly with regard to Until recent years, partial gastrectomy was the eating capacity, dumping symptoms, vomiting, ulcer-type dyspepsia, diarrhoea or other change in bowel habit, and favoured operation, but an increasing awareness of a clinical assessment was made based on a modified its significant operative mortality and its metabolic Visick scale. The mean time since operation was 6-9 consequences, along with Dragstedt and Owen's years. demonstration of the effectiveness of vagotomy in Thirty-five patients from this group were admitted to reducing acid secretion (1943), has resulted in the hospital for a full investigation of gastrointestinal and widespread use of vagotomy and gastric drainage. related function two to seven years following their The success of duodenal ulcer surgery cannot be operation. Most were volunteers, but some were selected judged only on low stomal (or recurrent) ulceration because of definite complaints. There were more females rates; the other sequelae of gastric operations must than males (21 females and 14 males). The following be considered. -
Hereditary Pancreatitis
Fact Sheet - Hereditary Pancreatitis Hereditary Pancreatitis (HP) is a rare genetic condition characterized by recurrent episodes of pancreatic attacks, which can progress to chronic pancreatitis. Symptoms include abdominal pain, nausea, and vomiting. Onset of attacks typically occurs between within the first two decades of life, but can begin at any age. In the United States, it is estimated that at least 1,000 individuals are affected with hereditary pancreatitis. HP has also been linked to an increased lifetime risk of pancreatic cancer. Pancreatic cancer is the 4th leading cause of cancer deaths among Americans. Individuals with hereditary pancreatitis appear to have a 40% lifetime risk of developing pancreatic cancer. This increased risk is heavily dependent upon the duration of chronic pancreatitis and environmental exposures to alcohol and smoking. One recent study suggested that individuals with chronic pancreatitis for more than 25 years had a higher rate of pancreatic cancer when compared to individuals in the general population. This increased rate appears to be due to the prolonged chronic pancreatitis rather than having a gene mutation (all cationic trypsinogen mutations). It is important to note that these risk values may be higher than expected because these studies on pancreatic cancer use a highly selective population rather than a randomly selected population. At this time, there is no cure for HP. Treating the symptoms associated with HP is the choice method of medical management. Patients may be prescribed pancreatic enzyme supplements to treat maldigestion, insulin to treat diabetes, analgesics and narcotics to control pain, and lifestyle changes to reduce the risk of pancreatic cancer (for example, NO SMOKING!). -
Idiopathic and Hereditary Pancreatitis Testing
Idiopathic and Hereditary Pancreatitis Testing Pancreatitis is a relatively common disorder with multiple etiologies that causes inammation in the pancreas. Acute pancreatitis (AP) is a result of sudden inammation, and patients may present with increased pancreatic enzyme concentrations. Chronic Tests to Consider pancreatitis (CP) is a syndrome of progressive inammation that may lead to permanent damage to pancreatic structure and function. Genetic testing can be utilized to determine Pancreatitis, Panel (CFTR, CTRC, PRSS1, a genetic cause of idiopathic or hereditary AP or CP and/or to assess risk of disease in SPINK1) Sequencing (Temporary Referral as of 12/7/20) 2010876 family members. Method: Polymerase Chain Reaction/Sequencing Preferred test for individuals with history of Disease Overview idiopathic pancreatitis Pancreatitis (CTRC) Sequencing 2010703 Incidence/Prevalence Method: Polymerase Chain Reaction/Sequencing Chronic pancreatitis For adults with idiopathic pancreatitis if other components of panel (CFTR , PRSS1 , SPINK1 ) Incidence: ~4-12/100,000 per year 1 have been sequenced without providing a Prevalence: ~37-42/100,000 1 complete explanation for the pancreatitis Idiopathic chronic pancreatitis is more common than previously thought 2 Pancreatitis (PRSS1) Sequencing and Deletion/Duplication (Temporary Referral Symptoms/Presentation Etiologies as of 01/14/21) 3001768 Method: Polymerase Chain Reaction/Sequencing Acute Sudden onset of pain in Common and Multiplex Ligation Dependent Probe Amplic- pancreatitis the upper abdomen, -
Managing Envenomations
ResidentOfficial Publication of the Emergency Medicine Residents’ Association June/July 2021 VOL 48 / ISSUE 3 Managing Envenomations How to Sustain a Career: Peer Support Guide to ABEM Certi ication We Help Healers SCP Reach New Heights Health Meet Your Medical Career Dream Team SCP Health Step Right Up, Residents! As you’re transitioning from residency to begin your career, our team is here to create a tailored environment for you that fosters growth and delivers rewarding daily work experiences. Explore clinical careers at scp-health.com/explore TOGETHER, WE HEAL Welcome to a New Academic Year! uly marks a turning point each year, as new interns arrive in programs throughout Jthe country, newly graduated residents launch the next phase of their careers, and medical students take the next steps in their journey to residency. DO YOU KNOW HOW EMRA CAN HELP? Resources for Interns Resources for PGY2+ Resources for Students All EM programs will receive EMRA residents members receive the EMRA shows up for medical students Intern Kits this summer with nearly 10-pound EMRA Resident Kit upon interested in this specialty. From resources that offer immediate first joining EMRA. It is packed with clinical new advising content every month to backup for those first nerve-wracking resources for every rotation — some you’ll opportunities for leadership and growth, shifts. The high-yield EMRA Intern need only rarely (but prove to be clutch), and EMRA student membership is high-yield. Kit is sent for free to all EM interns some you’ll use every single shift (EMRA Plus, our online resources are unparalleled: (membership not required) and Antibiotic Guide, anyone?). -
Historical Note
East African Orthopaedic Journal HISTORICAL NOTE AMBROISE PARE 1510-1590 Pare was born in 1510 in Bourge-Hersent in North- were failing to emerge from the gums due to lack of West France. He initially worked with his brother who a pathway, and this failure was a cause of death. This was a surgeon cum barber. He practiced at Hotel Dieu, belief and practice persisted for centuries, with some Frances oldest Hospital. He was an anatomist and exceptions, until towards the end of the nineteenth invented several surgical instruments. He became a century lancing became increasingly controversial and war injury doctor at Piedmont where he used boiled oil was then abandoned (2). for treating gunshot wounds. One day he improvised In 1567, Ambroise Pare described an experiment using oil of roses, egg white and turpentine with very to test the properties of bezor stones. At the time, the good results. Henceforth he stopped the cauterization stones were commonly believed to be able to cure and hot oil method. the effects of any poison, but Pare believed this to be Pare was a keen observer and did not allow the impossible. It happened that a cook at Pare court was beliefs of the day to supersede the evidence at hand. In caught stealing fine silver cutlery, and was condemned his autobiographical book, Journeys in Diverse Places, to be hanged. The cook agreed to be poisoned, on the Pare inadvatienty practiced the scientific method when conditions that he would be given a bezoar straight he returned the following morning to a battlefield. -
Chromosome 20
Chromosome 20 ©Chromosome Disorder Outreach Inc. (CDO) Technical genetic content provided by Dr. Iosif Lurie, M.D. Ph.D Medical Geneticist and CDO Medical Consultant/Advisor. Ideogram courtesy of the University of Washington Department of Pathology: ©1994 David Adler.hum_20.gif Introduction Chromosome 20 contains about 2% of the whole genetic material. Its genetic length is ~63 Mb. The long arm (~36 Mb) is a little bit larger than the short arm (~27 Mb). Chromosome 20 contains ~700–800 genes. Less than 10% of these genes are known to be related to human diseases. Deletions or duplications of these genes, which may be found in patients with chromosomal abnormalities, cause mostly functional defects, including a delay of psycho–motor development and seizures. Only a few genes may lead (when deleted) to structural defects of the heart, liver, extremities and other organs. Deletions of Chromosome 20 There is a relatively small number of known conditions caused by deletions and duplications of various segments of chromosome 20. Almost all of these deletions and duplications became recognized after usage of molecular cytogenetics. Only a handful of reports on patients with these abnormalities were available only 10 years ago. Because these methods open wide an opportunity to examine abnormalities of this previously not–well studied chromosome, there are no doubts that some new syndromes caused by deletions (or duplications) of chromosome 20 will be delineated in the near future. Currently, the most frequent forms of chromosome 20 deletions are deletions 20p12, involving the JAG1 gene and Alagille syndrome, and deletions 20q13.13q13.2, involving the SALL4 gene.