Augmenting ureagenesis in patients with partial carbamyl phosphate synthetase 1 deficiency with N-carbamyl-L-glutamate.
prospective_cohort · Level II
Where this comes from
- Record sourced from PubMed, PMID 24880889.
- Also identified by DOI 10.1016/j.jpeds.2014.04.012 and PMC identifier 4111993.
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Abstract
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.
Medical subject headings
- Carbamoyl-Phosphate Synthase I Deficiency Disease
- Glutamates
- Glutamine
- Urea