✦ LIBER ✦

Liver transplantation for argininosuccinic aciduria: Clinical, biochemical, and metabolic outcome

✍ Scribed by Tanya Newnham; Winita Hardikar; Katrina Allen; R. Mark Wellard; Charles Hamilton; Peter Angus; Robert Jones; Avihu Boneh

Publisher: John Wiley and Sons
Year: 2007
Tongue: English
Weight: 83 KB
Volume: 14
Category: Article
ISSN: 1527-6465
DOI: 10.1002/lt.21297

No coin nor oath required. For personal study only.

✦ Synopsis

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.

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