Citrulline in the management of patients with urea cycle disorders.

ABSTRACT:

Background

Treatment recommendations for urea cycle disorders (UCDs) include supplementation with amino acids involved in the urea cycle (arginine and/or citrulline, depending on the enzyme deficiency), to maximize ammonia excretion through the urea cycle, but limited data are available regarding the use of citrulline. This study retrospectively reviewed clinical and biological data from patients with UCDs treated with citrulline and/or arginine at a reference center since 1990. The aim was to describe the prescription, impact, and safety of these therapies. Data collection included patient background, treatment details, changes in biochemical parameters (plasma ammonia and amino acids concentrations), decompensations, and patient outcomes.

Results

Overall, 79 patients (medi

SUBMITTER: Imbard A 

PROVIDER: S-EPMC10362745 | biostudies-literature | 2023 Jul

REPOSITORIES: biostudies-literature