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Augmenting ureagenesis in patients with partial carbamyl phosphate synthetase 1 deficiency with N-carbamyl-L-glutamate.
Ah Mew, Nicholas; McCarter, Robert; Daikhin, Yevgeny; Lichter-Konecki, Uta; Nissim, Ilana; Yudkoff, Marc; Tuchman, Mendel.
Affiliation
  • Ah Mew N; Center for Translational Sciences, Children's National Health System, The George Washington University, Washington, DC; Division of Genetics and Metabolism, Children's National Health System, The George Washington University, Washington, DC.
  • McCarter R; Center for Translational Sciences, Children's National Health System, The George Washington University, Washington, DC.
  • Daikhin Y; Department of Pediatrics, Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA.
  • Lichter-Konecki U; Center for Translational Sciences, Children's National Health System, The George Washington University, Washington, DC; Division of Genetics and Metabolism, Children's National Health System, The George Washington University, Washington, DC.
  • Nissim I; Department of Pediatrics, Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA.
  • Yudkoff M; Department of Pediatrics, Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA.
  • Tuchman M; Center for Genetic Medicine Research, Children's National Health System, The George Washington University, Washington, DC.
J Pediatr ; 165(2): 401-403.e3, 2014 Aug.
Article in En | MEDLINE | ID: mdl-24880889
Identical studies using stable isotopes were performed before and after a 3-day trial of oral N-carbamyl-l-glutamate (NCG) in 5 subjects with late-onset carbamyl phosphate synthetase deficiency. NCG augmented ureagenesis and decreased plasma ammonia in 4 of 5 subjects. There was marked improvement in nitrogen metabolism with long-term NCG administration in 1 subject.
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Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Urea / Carbamoyl-Phosphate Synthase I Deficiency Disease / Glutamates / Glutamine Limits: Adolescent / Adult / Child / Child, preschool / Female / Humans / Male Language: En Journal: J Pediatr Year: 2014 Document type: Article Country of publication: United States
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Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Urea / Carbamoyl-Phosphate Synthase I Deficiency Disease / Glutamates / Glutamine Limits: Adolescent / Adult / Child / Child, preschool / Female / Humans / Male Language: En Journal: J Pediatr Year: 2014 Document type: Article Country of publication: United States