Neonatal hyperammonemia: the N-carbamoyl-L-glutamic acid test.

Guffon, Nathalie; Schiff, Manuel; Cheillan, David; Wermuth, Bendicht; Häberle, Johannes; Vianey-Saban, Christine · J Pediatr · 2005

case_report · Level V

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Abstract

In a prospective study, patients with a suspected urea cycle defect underwent oral N-carbamoyl-L-glutamic acid loading testing. In patients with subsequently confirmed N-acetylglutamate synthase deficiency, hyperammonemia normalized within 8 hours. This test may be useful in the early diagnosis of patients with suspected urea cycle disorders.

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