Patients with a urea cycle disorder (UCD) require regular monitoring of their labs because their ammonia levels may become dangerously high.1,2 Measuring ammonia, however, can be challenging and may result in falsely elevated ammonia if blood is not drawn correctly.2,3
Ammonia testing is routinely available at most hospitals. Learn more about the correct procedures for accurate ammonia testing.
This test is vital for patients with a UCD
Review important information you can share with your patients and practice
1. Pei KJ, Pei KJ, Rajikan RB, et al. Growth and nutritional status of children with urea cycle defects (UCD): a 6-months follow up study in Institute of Pediatric, Hospital Kuala Lumpur. Int J Clin Nutr. 2014; 2(3):41-52. 2. Häberle J, Boddaert N, Burlina A, et al. Suggested guidelines for the diagnosis and management of urea cycle disorders. Orphanet J Rare Dis. 2012;7:32. 3. Barsotti RJ. Measurement of ammonia in blood. J Pediatr. 2001;138 (suppl 1):S11-S20. 4. Hawke L. Ammonia (Plasma, Blood). Association of Clinical Biochemistry; 2012. 5. Ah Mew N, Simpson KL, Gropman AL, Lanpher BC, Chapman KA, Summar ML. Urea Cycle Disorders Overview. In: Adam MP, Mirzaa GM, Pagon RA, et al., eds. GeneReviews®. Seattle (WA): University of Washington, Seattle; April 29, 2003. 6. Lee B, Diaz GA, Rhead W, et al. Glutamine and hyperammonemic crises in patients with urea cycle disorders. Mol Genet Metab. 2016;117(1):27-32. 7. Mokhtarani M, Diaz GA, Rhead W, et al. Elevated phenylacetic acid levels do not correlate with adverse events in patients with urea cycle disorders or hepatic encephalopathy and can be predicted based on the plasma PAA to PAGN ratio. Mol Genet Metab. 2013;110(4):446-453. 8. Mokhtarani M, Diaz GA, Rhead W, et al. Urinary phenylacetylglutamine as dosing biomarker for patients with urea cycle disorders. Mol Genet Metab. 2012;107(3):308-314.12. 9. Häberle J, Burlina A, Chakrapani A, et al. Suggested guidelines for the diagnosis and management of urea cycle disorders: first revision. J Inherit Metab Dis. 2019;42(6):1192-1230.