Glutamine and hyperammonemic crises in patients with urea cycle disorders.

ABSTRACT:

Unlabelled

Blood ammonia and glutamine levels are used as biomarkers of control in patients with urea cycle disorders (UCDs). This study was undertaken to evaluate glutamine variability and utility as a predictor of hyperammonemic crises (HACs) in UCD patients.

Methods

The relationships between glutamine and ammonia levels and the incidence and timing of HACs were evaluated in over 100 adult and pediatric UCD patients who participated in clinical trials of glycerol phenylbutyrate.

Results

The median (range) intra-subject 24-hour coefficient of variation for glutamine was 15% (8-29%) as compared with 56% (28%-154%) for ammonia, and the correlation coefficient between glutamine and concurrent ammonia levels varied from 0.17 to 0.29. Patients with baseline (fasting) glut

SUBMITTER: Lee B 

PROVIDER: S-EPMC4915945 | biostudies-literature | 2016 Jan

REPOSITORIES: biostudies-literature