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Journal article
Glutamine and hyperammonemic crises in patients with urea cycle disorders
Molecular genetics and metabolism, v 117(1), pp 27-32
Jan 2016
PMID: 26586473
Featured in Collection : UN Sustainable Development Goals @ Drexel
Abstract
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.
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.
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) glutamine values >900μmol/L had higher baseline ammonia levels (mean [SD]: 39.6 [26.2]μmol/L) than patients with baseline glutamine ≤900μmol/L (26.6 [18.0]μmol/L). Glutamine values >900μmol/L during the study were associated with an approximately 2-fold higher HAC risk (odds ratio [OR]=1.98; p=0.173). However, glutamine lost predictive significance (OR=1.47; p=0.439) when concomitant ammonia was taken into account, whereas the predictive value of baseline ammonia ≥1.0 upper limit of normal (ULN) was highly statistically significant (OR=4.96; p=0.013). There was no significant effect of glutamine >900μmol/L on time to first HAC crisis (hazard ratio [HR]=1.14; p=0.813), but there was a significant effect of baseline ammonia ≥1.0 ULN (HR=4.62; p=0.0011).
The findings in this UCD population suggest that glutamine is a weaker predictor of HACs than ammonia and that the utility of the predictive value of glutamine will need to take into account concurrent ammonia levels.
•Glutamine is a weaker predictor of HA crises in UCD patients than is ammonia.•Blood glutamine varies several-fold less over 24-h than does ammonia.•Morning ammonia after an overnight fast is a strong predictor of HA crises.
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Details
- Title
- Glutamine and hyperammonemic crises in patients with urea cycle disorders
- Creators
- B. Lee - Baylor College of MedicineG.A. Diaz - Icahn School of Medicine at Mount SinaiW. Rhead - Medical College of WisconsinU. Lichter-Konecki - Drexel UniversityA. Feigenbaum - University of California, San DiegoS.A. Berry - University of MinnesotaC. Le Mons - National Urea Cycle Disorders FoundationJ. Bartley - Miller Children's & Women's HospitalN. Longo - University of UtahS.C. Nagamani - Baylor College of MedicineW. Berquist - Stanford UniversityR.C. Gallagher - University of CA, San Francisco, USAC.O. Harding - Oregon Health & Science UniversityS.E. McCandless - Case Western Reserve UniversityW. Smith - Maine Medical CenterA. Schulze - Hospital for Sick ChildrenM. Marino - Oregon Health & Science UniversityR. Rowell - MED Technical Consulting, Inc., Union City, CA, USAD.F. Coakley - Horizon TherapeuticsM. Mokhtarani - Horizon TherapeuticsB.F. Scharschmidt - Horizon Therapeutics
- Publication Details
- Molecular genetics and metabolism, v 117(1), pp 27-32
- Publisher
- Elsevier
- Resource Type
- Journal article
- Language
- English
- Academic Unit
- Pediatrics
- Web of Science ID
- WOS:000368220400005
- Scopus ID
- 2-s2.0-84951762877
- Other Identifier
- 991019167696604721
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- Collaboration types
- Domestic collaboration
- International collaboration
- Web of Science research areas
- Endocrinology & Metabolism
- Genetics & Heredity
- Medicine, Research & Experimental