Acute Metabolic Crises in Maple Syrup Urine Disease After Liver Transplantation from a Related Heterozygous Living Donor

Maple syrup urine disease (MSUD) is an autosomal recessive disorder associated with impaired metabolism of branched-chain amino acids (BCAA) leucine, isoleucine, and valine. Children with MSUD suffer from bouts of metabolic decompensation, which may lead to neurological damage. Liver transplantation...

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Vydáno v:JIMD Rep
Hlavní autoři: Al-Shamsi, Aisha, Baker, Alastair, Dhawan, Anil, Hertecant, Jozef
Médium: Artigo
Jazyk:Inglês
Vydáno: Springer Berlin Heidelberg 2016
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Article
On-line přístup:https://ncbi.nlm.nih.gov/pmc/articles/PMC5110448/
https://ncbi.nlm.nih.gov/pubmed/27117295
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1007/8904_2016_532
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