USMLE Step 1 Question of the Day
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A 14 year old male patient presents with altered mental status, and RUQ pain. On physical exam, brown corneal deposits are noted on slit-lamp exam. Hepatomegaly is noted with a span 12cm, and his asterixis is noted. Laboratory values are notable for elevated transaminases. What is most appropriate medical therapy?
Correct Answer: A. D-penicillamine
This patient is suffering from Wilson’s Disease. The signs of Wilson’s are Kayser-Fleischer rings (brown rings around iris), and liver disease which can progress to cirrhosis. Wilson’s disease is caused by the accumulation of copper which is treated with dpenicillamine (option A).
Deferoxamine is used for the treatment of increased iron usually used in those with multiple blood transfusions. Therapeutic phlebotomy (option E) is used in hematochromatosis which is also caused by the build up of iron.
Dimercaprol (option C) is used to treat heavy metal posioning, and is only the last line of defence in Wilson’s disease.
N-acetylcysteine is used for acetaminophen overdoses.
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Category: PharmacologyA 14 year old male patient presents with altered mental status, and RUQ pain. On physical exam, brown corneal deposits are noted on slit-lamp exam. Hepatomegaly is noted with a span 12cm, and his asterixis is noted. Laboratory values are notable for elevated transaminases. What is most appropriate medical therapy?
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