WBR0951

Author	[[PageAuthor::Mugilan Poongkunran M.B.B.S [1]]]
Exam Type	ExamType::USMLE Step 3
Main Category	MainCategory::Primary Care Office
Sub Category	SubCategory::Hepatology
Prompt	[[Prompt::A 21 year old male, comes to the office with complaints of severe fatigue and lethargy over the past 1 year. On further review of history, he says he has intermittent mild abdominal pain over the past few months. Of late he is feeling low and does not talk with friends anymore, spending most of his time at home. His past history is otherwise insignificant and his family history is unremarkable. He does not smoke and drink. His vitals are temperature: 36.7 C, blood pressure: 130/70 mmHg, pulse: 72/min and respiration's: 16/min. Abdominal examination reveals mild abdominal pain on the right upper quadrant and mild hepatomegaly. Cardiovascular and respiratory system examinations are normal. Neurological examinations shows mild rigidity in the upper limbs and there is gray green ring around his iris. After confirming with appropriate blood tests, you start the patient on medications. The patient on follow-up in a month says he is experiencing hand tremors on doing work, which he dint have earlier. What is the most appropriate treatment for this patient at this point?]]
Answer A	AnswerA::Labetolol
Answer A Explanation	[[AnswerAExp:: Incorrect  : Labetolol is the drug of choice for essential tremor.]]
Answer B	AnswerB::Zinc
Answer B Explanation	[[AnswerBExp:: Correct  : Zinc should be considered in this patient and has to be given several hours apart from the D-penicillamine agent, atleast twice daily.]]
Answer C	AnswerC::Levo-dopa
Answer C Explanation	[[AnswerCExp:: Incorrect  : Leva-dopa is the drug of choice for parkinsonism, which is also a feature of Wilson’s disease. However treating Wilson’s disease with D-penicillamine provides relief of the neurological symptoms.]]
Answer D	AnswerD::Pyridoxine
Answer D Explanation	[[AnswerDExp:: Incorrect  : Penicillamine inactivates pyridoxine. Thus, small doses of pyridoxine, 25 mg per day, should be given to patients treated with D-penicillamine to prevent pyridoxal phosphate deficiency. However it is not required right now in this patient.]]
Answer E	AnswerE::Vitamin B12
Answer E Explanation	[[AnswerEExp:: Incorrect  : Vitmain B12 is used to treat megaloblastic anemia.]]
Right Answer	RightAnswer::B
Explanation	[[Explanation::Wilson's disease or hepatolenticular degeneration is an autosomal recessive genetic disorder in which copper accumulates in tissues; this manifests as neurological or psychiatric symptoms and liver disease. It is treated with medication that reduces copper absorption or removes the excess copper from the body, but occasionally a liver transplant is required. D-Penicillamine contains a free sulfhydryl group that functions as a copper chelating moiety. Its major effect is to remove copper from less tightly bound sites on proteins, peptides, and membranes, and promote its urinary excretion, although it may also function by other mechanisms such as stimulation of endogenous metallothionein. Penicillamine is associated with multiple side-effect like sensitivity reactions, proteinuria, elastosis perforans serpiginosa, pemphigus, lichen planus, aphthous stomatitis, nausea, vomiting, aplastic anemia and one predominant effect is worsening of the neurologic symptoms with the initiation of treatment. Furthermore, new neurologic symptoms may appear in some patients. Although there are limited data, generally a switch to trientine or to zinc should be considered in these patients. For severely symptomatic patients and for those individuals whose symptoms worsen on D-penicillamine or trientine alone, we consider using combination therapy. Educational Objective: References: ]]
Approved	Approved::Yes
Keyword	WBRKeyword::Wilson's disease
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