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Correspondence
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Volume 353:2410-2411 December 1, 2005 Number 22
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Domino Hepatic Transplantation in Maple Syrup Urine Disease

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To the Editor: Orthotopic liver transplantation has been performed in at least 10 patients who have maple syrup urine disease (MSUD).1,2,3,4 In the first patients, transplantation was for nonmetabolic reasons (hepatic failure with hepatitis A1 and hypervitaminosis A2). In all patients, there was marked improvement in dietary protein tolerance and no evidence of any decompensation episodes during follow-up extending 10 years.3 Because the long-term outcome and effect on neurologic development remain to be identified, orthotopic liver transplantation remains a controversial therapy.1 With recent reports of success, it has been established as an option for patients with MSUD whose condition . . . [Full Text of this Article]



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