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

Caldovic, Ljubica; Morizono, Hiroki; Daikhin, Yevgeny; Nissim, Itzhak; McCarter, Robert J; Yudkoff, Marc; Tuchman, Mendel · J Pediatr · 2004

case_report · Level V

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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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