(MCQS 319-321):A 30 year-old woman relates an 8 month history of recurrent pruritic skin lumps. Endoscopy eveals a duodenal bulb ulcer

Question:319
 
Correct Answer: E
Explanation:
Methemoglobinemia can be the result of an inherited disorder or can be acquired through exposure to chemicals such as nitrates (nitrate contaminated water), aniline dyes and potassium chlorate. The autosomal recessive condition results from an absence of an enzyme that normally converts methemoglobin back to normal hemoglobin thereby leading to an increase in methemoglobin and a decrease in the ability of blood to carry oxygen. Hemoglobin M disease, is an autosomal dominant trait characterized by the production of an abnormal methemoglobin, and is usually milder in its degree of cyanosis. Exposure to environmental chemicals causes an increase in production of methemoglobin in affected people. If the patient is symptomatic, which is often the case in deliberate or accidental overdoses or in toxin ingestion, specific therapy is required. Methylene blue, 1 to 2 mg/kg over 5 minutes, provides an artificial electron acceptor for the reduction of methemoglobin by means of the NADPH-reductase dependent pathway. Response is usually rapid; the dose may be repeated in 1 hour but is frequently unnecessary. Before administering methylene blue, populations with a high incidence of G6PD deficiency (e.g., blacks, Mediterraneans, and Southeast Asians) should be screened. Patients with G6PD deficiency should not receive methylene blue but can receive ascorbic acid
 
Question:320
 
Correct Answer: E
Explanation:
Systemic mastocytosis results in mast cell infiltration of the skin, gastrointestinal mucosa, liver, and spleen. Cutaneous manifestations include small, reddish-brown papules which would be characterized histopathologically as having excess numbers of mast cells. Histamine-mediated hypersecretion of gastric acid accounts for an increased incidence of gastritis and peptic ulcers in patients with systemic mastocytosis. Bone pain, organomegaly, or lymphadenopathy may also be seen. In addition to documentation of mast cells in various organs, biochemical confirmation can be made by urine collection for histamine metabolites or by measuring increased blood levels of histamine or mast cell-derived neutral protease tryptase. Mast cell disease may be indolentor more aggressive characterized by mast cell infiltration of liver and spleen that may lead to mast cell leukemia
 
Question:321
 
Correct Answer: F
Explanation:
Kawasaki's syndrome, also known as mucocutaneous lymph node syndrome is an acute diffuse vasculitis of an unknown etiology. The disease usually affects children and 80% of the patients are younger than 4 years of age. The male to female ratio is 1.6 to 1. Kawasaki's is often seen in Asian populations, especially of Japanese ancestry, but any ethnic group may be affected. There appears to be a seasonal predilection between December and May. The diagnostic criteria for Kawasaki's must include fever of 5 or more days duration and four or more of the following conditions: 1). Bilateral conjunctival injection without exudate 2). Changes in peripheral extremities such as erythema and edema of the hands and feet, or membranous desquamation of the fingertips 3). Polymorphous exanthem 4). Changes in the lips and oral cavity such as erythema, cracked lips, strawberry tongue, diffuse injection of the oral and pharyngeal mucosa 5). acute cervical lymphadenopathy Other clinical signs seen in Kawasaki's syndrome include arthralgias, arthritis, and cardiac involvement. The cardiovascular effects acount for the majority of the morbidity and mortality of this disease. The cardiac abnormalities include aneurysms, myocarditis, arrhythmias, mitral insufficiency, and myocardial infarction. The laboratory findings include mild anemia, thrombocytosis, leukocytosis with a left shift, and an elevated erythrocyte sedimentation rate. The patient may also have a sterile pyuria, proteinuria, cerebrospinal fluid pleocytosis, and mild increases in bilirubin or serum transaminases. The treatment is unknown but high doses of aspirin are recommend for the antiinflammatory and antiplatelet aggregation effects. A dose of 80-100 mg/kg/day has been shown to reduce the duration of the fever. Also, high dose intravenous gamma globulin has been used to prevent cardiac complications. The exact mechanism for the beneficial effect is not well understood. The anterior uveitis is generally self-limited and no treatment is recommended. The prognosis is good with a mortality rate of approximately 0.4%. .


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