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Liver transplantation for argininosuccinic aciduria: Clinical, biochemical, and metabolic outcome
Author(s) -
Newnham Tanya,
Hardikar Winita,
Allen Katrina,
Wellard R. Mark,
Hamilton Charles,
Angus Peter,
Jones Robert,
Boneh Avihu
Publication year - 2008
Publication title -
liver transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.814
H-Index - 150
eISSN - 1527-6473
pISSN - 1527-6465
DOI - 10.1002/lt.21297
Subject(s) - medicine , liver transplantation , cirrhosis , transplantation , gastroenterology
We report successful liver transplantation in a young adult with argininosuccinic aciduria but without cirrhosis. Plasma amino acid profile normalized and brain magnetic resonance spectroscopy indicated improved metabolism after transplantation. The general well‐being of the patient and his quality of life improved. We suggest that orthotopic liver transplantation should be considered for patients with argininosuccinic aciduria even in the absence of cirrhosis, with the aim of correcting (at least in part) central nervous system metabolism, thereby preventing further neurological deterioration. Liver Transpl 14:41–45, 2008. © 2007 AASLD.