Acute metabolic decompensation after liver transplant in a patient with maple syrup urine disease

Acute metabolic decompensation after liver transplant in a patient with maple syrup urine disease

Abstract Maple syrup urine disease (MSUD) is an inborn error of metabolism characterized by the accumulation of branched‐chain amino acids (leucine, isoleucine, and valine) caused by a defect in the branched‐chain alpha‐keto acid dehydrogenase complex. Liver transplant is an effective therapy for MS...

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Bibliographic Details
Main Authors: Shao Ching Tu, Marium Khan, Katie Wolfe, Sakil S. Kulkarni, Elizabeth Toolan, Dorothy K. Grange
Format: Article
Language:English
Published: Wiley 2025-01-01
Series:JIMD Reports
Subjects:
decompensation
dehydration
encephalopathy
hyperleucinosis
liver transplant
MSUD
Online Access:https://doi.org/10.1002/jmd2.12460
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