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Vignette Pearls

Too busy at the 2008 SGIM meeting to go to all the Clinical Vignette sessions or, worse yet, didn’t get to go to the meeting at all?  Still interested in improving medical knowledge and patient care? Need a quick bulleted fact to bolster your attending rounds or Morning Report discussion but not willing to buy the Clinical Pearls put out by ACP Small Feedings for the Mind? Then visit the SGIM Vignette Pearls 2008.

This year, as a new addition, we collated one teaching point from each of the oral vignettes presented by SGIM members. These highly relevant facts are presented for your review below. 78 pieces of information may be a lot to swallow in one sitting but they make for some good reading over a period of time.

We hope this serves as a novel yet useful source of continuing medical education at a pace self-determined by busy clinicians. And, we hope this may be something that you can use to facilitate good clinical discussion on the wards, in clinic, or during conferences. We welcome your feedback.

Sincerely,
 
Rosanne Granieri, MD
Chair, 2008 SGIM Clinical Vignette Committee
 
Radhika Ramanan, MD, MPH
Co-Chair, 2008 SGIM Clinical Vignette Committee

SGIM Pittsburgh 2008
78 PEARLS FROM CLINICAL VIGNETTES

1. DRESS (Drug Reaction with Eosinophilia and Systemic Symptoms) syndrome has a mortality rate of 10%.
2. AIP is the most common and most severe porphyria.  It results from low levels of porphobilinogen deaminase activity. Remember AIP as a diagnosis in a patient with psychiatric disease and abdominal pain.
3. Less than 10% of all post-streptococcal glomerulonephritis occurs in adults over the age of 40.  
4. HIV associated nephropathy is characterized by progressive renal insufficiency with nephrotic-range proteinura, without significant edema. HAART has been shown to have beneficial effects on prevalence and progression of HIV associated nephropathy.
5. Tenofovir has been associated with proximal RTA and Fanconi syndrome.
6. Calciphylaxis has a mortality rate of 60-80%.
7. Chronic Corynebacterium urealyticum UTI can result in extensive urothelial calcification with post obstructive uropathy in the absence of hydronephrosis. Treatment options include antibiotic therapy, acidifying the urine and surgical removal of the encrustations.
8. Risk factors for venous thromboembolism may differ from risk factors for arterial thromboembolism.
9. Left ventricular noncompaction, a disease in which the normal progression of myocardial morphogenesis is arrested at an early stage, is a rare cause of CHF. The varying extent of noncompaction leads to significant age range at diagnosis. Some patients present as children or young adults, but significant symptoms may be delayed until the sixth decade.
10. Tabosubo’s cardiomyopathy classically occurs in postmenopausal women without atherosclerotic CAD in times of metabolic or psychiatric stress.
11. Anaphylaxis and/or treatment of anaphylaxis with epinephrine are a now reported cause of Tabosubo cardiomyopathy.
12. Ectopic secretion of ACTH and the development of Cushing syndrome due to small cell lung cancer occurs in <5% of patients with small cell cancer. 
13. In a patient with recurrent URIs, common variable immunodeficiency may be present. Low levels of serum IgG are generally seen, although, in most cases, serum IgA and IgM are also reducced. 
14. Kikuchi’s disease is a rare self-limited disease presenting with fever, fatigue and cervical lymphadenopathy. KD has been associated with the development of SLE.
15. Churg Strauss should be considered in patients with refractory asthma. The presence of 4 out of the 6 criteria (asthma, peripheral eosinophilia, neuropathy, radiographic pulmonary opacities, paranasal sinusitis and extravascular eosinophilia on biopsy) has a sensitivity of 85% and specificity of 99.7.
16. C. difficile infections should be considered in patients with reactive arthritis.
17. Henoch-Schonlein Purpura presents with palpable purpura, abdominal pain, arthralgias and nephritis. Approximate 10% HSP cases occur in adults. Renal involvement tends to be more frequent and severe in adults. The GI manifestations tend to be self limited.
18. Tumor-related HCG can trigger thyroid storm.
19. Women are using non-FDA approved supplements containing androgenic steroids.
20. The use of dexamethasone before or during antibiotics for suspected pneumococcal meningitis significantly reduces the incidence of hearing loss.
21. Although HSV encephalitis usually presents acutely, it can present indolently.
22. Posterior reversible encephalopathy syndrome (PRES) is characterized by headache, altered consciousness, visual disturbances and seizes. The most commo
23. DKA can occur in patients with Type II DM. Three proposed mechanisms include insulin insufficiency, elevation of counter-regulatory hormones secondary to stress and increase free fatty acid as substrates for ketoacid formation.  Include pancreatic cancer as a possible etiology of DKA in Type II diabetes.
24. Myxedema coma is an uncommon presentation of hypothyroid disease but is more common in the elderly and in demented patients.  Thyroid function testing should be considered in the workup of dementia or delirium.
25. An otogenic focus can be found in one third of patients with pneumococcal meningitis. Dexamethasone decreases mortality in patients with pneumococcal meningitis.
26. Acute mania is a very rare presentation of neurosyphilis.
27. Hypertensive encephalopathy is caused by breakthrough hyperperfusion from severe and sudden rises in blood pressure, generally greater than 180/120.
28. Brucellosis is caused by consumption of unpasteurized goat, sheep and camel milk. Travel and global migration require its consideration in a fever work-up in non-endemic countries.
29. Aeromonas infection and cellulitis often develop within 8-48 hours of exposure to the organism. Aeromonas lives in freshwater and marine environments in the Northern Hemisphere and is more common in warmer months. Skin lesions include hemorrhagic bullae, subcutaneous bleeding and purpura.
30. Candida endophthalmitis can occur as a complication of candidemia through hematogenous seeding of the retina. Risk factors in immunocompetent patients include central lines, prior abdominal surgery, TPN and steroid therapy. Prompt identification and treatment is necessary to preserve visual acuity.
31. Pneumomediastinum is a rare complication of Pneumocysitis jiroveci pneumonia.
32. 10-20% of patients with acute mononucleosis will have a negative monospot in the first 2 weeks of infection.
33. Consider thiamine deficiency in patients with heart failure, especially those on furosemide. Some experts suggest that long-term furosemide can lead to thiamin deficiency due to increased urinary thiamine excretion.
34. The diagnosis of acute MI is challenging in the patient with LBBB. Sgarbossa’s EKG criteria (ST elevation of 1 mm concordant with the QRS) is specific (>90%) but not sensitive for diagnosis of acute myocardial infarction in LBBB. 
35. Hyperlipidemia in HIV patients is mainly due to the antiretroviral agents used in treatment. Protease inhibitors have the largest impact on triglycerides and total cholesterol.  The NNRTI class tends to be the most lipid neutral.
36. In order to consider the diagnosis of Tabosubo’s cardiomyopathy, you must rule out atherosclerotic disease.
37. A fixed split S2 is a sign for ASD.
38. A vascular examination should be included in all patients who present with lower extremity weakness. Leriche’s syndrome refers to buttock, hip and thigh claudication in the setting of aortoiliac occlusive disease.
39. Aortic dissection should be in the differential diagnosis of patients who present with neurological and vascular symptoms that are unexplained.
40. Thyrotoxic hypokalemic periodic paralysis most commonly affects males of Asian descent. The hypokalemia is due to intracellular shifts of potassium. Treating underlying hyperthyroidism is the key to treating the hypokalemia..
41. In patients who present with both neurological symptoms (headache, confusion, cerebellar ataxia, stupor) and pulmonary symptoms, Legionella pneumonia should be high on the differential.
42. In high risk patients with signs and symptoms of peripheral neuropathy, tabes dorsalis should be considered. Symptoms may appear even decades after initial infection...
43. Coccidioides infection is endemic to the Central Valley of Southern California and Mexico. Many infections are sub-clinical and those presenting with symptoms develop self-limited pulmonary syndromes.
44. Thyroid abscesses in adults are usually due to direct trauma from FNA or foreign body.  Thyroid abscesses occurs mostly in adolescents in the setting of embryological anomalies of the neck, the most common being pyriform sinus fistula.
45. Orbital apex syndrome (OAS) is manifested by multiple cranial nerve palsies with optic nerve involvement. The most common infectious etiology is bacterial infection from paranasal sinusitis or contiguous spread from facial cellulitis. Fungal infection should be suspected in immunocompromised patients.
46. Salmonella UTI in healthy patients without enteritis occurs.
47. Symptoms of anaphylaxis in a patient who has been previously infected with Plasmodium should prompt consideration of a recurrence.
48. Elevated IgG4 is part of the criteria for the diagnosis of autoimmune pancreatitis.
49. Giant cell arteritis may present with a normal sedimentation rate and CRP in 5-22% of patients.
50. Delayed diagnosis of late onset SLE is likely due to its atypical presentation.
51. Elderly patients have atypical presentation of certain diseases. Thyrotoxicosis may present in the elderly with depression (apathetic thyrotoxicosis).
52. Adenosine deaminase level of >45 has a sensitivity of 100% and a specificity of 97% in the diagnosis of in TB peritonitis
53. Recurrent hemoptysis is the most widespread presenting symptom of an ABF.
54. Remember acquired factor deficiency as a cause of bleeding in older patients.  Treatment goals of acquired factor VIII deficiency are initial treatment of bleeding and removal or eradication of the inhibitor with immunosuppression.
55. Patients with sickle cell disease are especially prone to hyperhemolysis syndrome, due to accumulation of antibodies to “i” antigens after sensitizing transfusions.
56. Acquired hemophilia A. is rare. It usually presents in elderly patients and it is associated with malignancy, autoimmune disease and allergic drug reactions... The diagnosis is suspected in a patient with prolonged PTT associated with reduced Factor VIII level. Confirmation is via demonstration of inhibitory antibody to Factor VIII.
57. Discordance between a strongly positive urine dip for blood and urine microscopy should prompt evaluation for hemoglobinuria or myoglobinuria.
58. A patient with a history of radiation and Hodgkin’s disease is at high risk for GI tumor.
59. Erythroid leukemia can be mistaken for severe pernicious anemia with pancytopenia in the presence of elevated MCV. Bone marrow biopsy with flow cytometry and cytogenetic analysis are essential in confirming the diagnosis of erythroid leukemia and differentiating it from nutritional deficiency syndromes.
60. A nonresolving pleural effusion should be further investigated for chylothorax.
61. Bronchoalveolar carcinoma may present with signs and symptoms identical to pneumonia or inflammatory lung disease. If there is stretching, squeezing or widening of the branching angle of the air-filled bronchus within consolidation, it is likely to be BAC rather than pneumonia.
62. Light chain deposition disorder (LCCD) is an uncommon but important monoclonal gammopathy to consider in a patient with renal disease and lymphoplasmacytic disorders. Diseases that produce monoclonal light chains include multiple myeloma, macroglobulinemia, lymphoma and CLL.
63. DRESS (Drug Reaction with Eosinophilia and Systemic Symptoms) syndrome, also known as eosinophilic hypersensitivity reaction, is a type of severe hypersensitivity reaction defined by widespread papulopustular skin lesions.
64. Pericarditis and constrictive cardiomyopathies are rare complications of methotrexate therapy.
65. Anaphylaxis to cephalosporins can occur even in the absence of penicillin allergy.
66. Ondansetron can be associated with heart block.
67. Coumadin induced skin necrosis will occur within days of initiating therapy. Warfarin induced hypersentivity vasculitis occurs after weeks of therapy.
68. Approximately 2% of the cases of multiple myeloma occur in patients younger than 40 years.
69. The red flags of back pain include immunosuppressive state, history of cancer, IV drug use, bladder/bowel incontinence, age greater than 70, unexplained fever, unexplained weight loss and duration of pain greater than 6 weeks... Presence of these warning signs merit further evaluation in the patient with acute back pain.
70. Mycotic aneurysm is a rare cause of low back pain. The aneurysm is formed by exposure of a preexisting aneurysm to bacteremia, most commonly Staph aureus (2/3 of which are methicillin resistant). Surgical repair is always required.
71. The two most common causes of mid-esophageal diverticula are congenital lesions and traction diverticula secondary to mediastinal inflammation. Most are asymptomatic but trapped food or pills can cause chest pain.
72. Eosinophilic esophagitis (EE) is a newly described entity with symptomatology similar to gastroesophageal reflux disease (GERD). Early recognition and differentiation from GERD is crucial to prevent significant morbidity associated with EE. Treatment options include corticosteroids, leukotriene receptor antagonists and biological molecules.
73. Endometriosis is an uncommon cause of small bowel obstruction. The most common location of extrapelvic endometriosis is the gastrointestinal tract. The most common sites in the large bowel are rectum and sigmoid colon; the most common sites in the small bowel are terminal ileum and appendix. 
74. Splenic rupture is a documented but rare complication of routine colonoscopy.  Risk factors for splenic rupture are previous abdominal surgery, inflammatory bowel disease, difficult colonoscopic and therapeutic procedures and being in the supine position during colonoscopy.
75. An elevated Ca19-9 is not always due to malignancy. However, at levels >1000 U/ml, the specificity for pancreatic cancer is greater than 99%.  Conditions other than malignancy known to cause elevations of this assay include cirrhosis and ascending cholangitis.
76. After acquisition of hepatitis C, partial control of the virus before infection becomes chronic can result in transiently undetectable HCV RNA levels. Thus patients should be retested after a few weeks.
77. Lofgren’s syndrome is characterized by bilateral hilar lymphadenopathy, erythema nodosum and migratory polyarthralgias.
78. Severe eosinophila (total eosinophil count >1500) is more likely associated with neoplasm, eosinophilic gastroenteritis, hyper IgE syndrome and parasites.