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Case Reports
. 2004 Oct;145(4):552-4.
doi: 10.1016/j.jpeds.2004.06.047.

Restoration of ureagenesis in N-acetylglutamate synthase deficiency by N-carbamylglutamate

Ljubica Caldovic  1 , Hiroki Morizono, Yevgeny Daikhin, Itzhak Nissim, Robert J McCarter, Marc Yudkoff, Mendel Tuchman
Affiliations
Case Reports

Restoration of ureagenesis in N-acetylglutamate synthase deficiency by N-carbamylglutamate

Ljubica Caldovic et al. J Pediatr. 2004 Oct.

Abstract

In a patient with N-acetylglutamate synthase (NAGS) deficiency, incorporation of an isotopic label from ammonium chloride into urea was markedly reduced before treatment with N-carbamyl-L-glutamate (NCLG) and completely normalized following treatment. Blood ammonia rose following ammonium tracer ingestion before treatment but remained low following treatment. Serum urea concentration doubled following the treatment.

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