The effect of liver transplantation for argininemia-the largest experiences in a single center.

ABSTRACT:

Background

Argininemia, a rare urea cycle disorder resulting from an arginase-1 deficiency, is characterized by a progressive spastic paraplegia. While advances in diagnosis and treatment have increased the management of this condition, not all symptoms are resolved in response to traditional therapies. Interestingly, there exist some rare reports on the use of liver transplantation (LT) for the treatment of argininemia.

Methods

We conducted a retrospective study of eleven patients with argininemia receiving a LT as performed at our center over the period from January 2015 to November 2019. These patients were included due to their poor responses to protein restriction diets and alternative therapies of nitrogen scavengers. Detailed information on coagulation, liver function

SUBMITTER: Cui B 

PROVIDER: S-EPMC9085954 | biostudies-literature | 2022 Apr

REPOSITORIES: biostudies-literature