ArticleMetabolic brain disease2009
The first use of N-carbamylglutamate in a patient with decompensated maple syrup urine disease.
Article in Metabolic brain disease, 2009. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers.
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Who cites it
11 citing papers in PubMed, 20 citations in OpenAlex.
- Supplementation of Arginine or N-Carbamylglutamate Affects Jejunum Development, Global Arginine Bioavailability Ratio, and Stress-Related Indices in Young Rex Rabbits.Animals : an open access journal from MDPI · 2025Article
- DietaryFrontiers in physiology · 2023Article
- Hyperleucinosis during infections in maple syrup urine disease post liver transplantation.Molecular genetics and metabolism reports · 2021Article
- Determination ofMolecules (Basel, Switzerland) · 2019Article
- Roles of dietary supplementation with arginine or N-carbamylglutamate in modulating the inflammation, antioxidant property, and mRNA expression of antioxidant-relative signaling molecules in the spleen of rats under oxidative stress.Animal nutrition (Zhongguo xu mu shou yi xue hui) · 2018Article
- Arginine,Animal nutrition (Zhongguo xu mu shou yi xue hui) · 2016Article
- Efficacy of N-carbamoyl-L-glutamic acid for the treatment of inherited metabolic disorders.Expert review of endocrinology & metabolism · 2016Article
- Hyperammonemia in review: pathophysiology, diagnosis, and treatment.Pediatric nephrology (Berlin, Germany) · 2012Review
- N-carbamylglutamate enhances pregnancy outcome in rats through activation of the PI3K/PKB/mTOR signaling pathway.PloS one · 2012Article
- New developments in the treatment of hyperammonemia: emerging use of carglumic acid.International journal of general medicine · 2011Article
- Role of carglumic acid in the treatment of acute hyperammonemia due to N-acetylglutamate synthase deficiency.Therapeutics and clinical risk management · 2011Article
Corrections and comments
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Authors and funding
8 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Maple syrup urine disease (MSUD) is a defect in the catabolism of the branched-chain amino acids; leucine, isoleucine, and valine. Affected patients may also develop hyperammonaemia of unknown etiology. This report describes a four-year-old girl with MSUD, who presented with decompensated hyperleucinaemia with hyperammonaemia. The oral administration of the N-acetylglutamate analogue, N-carbamylglutamate (NCG), 200 mg/kg/day as a loading dose, and 100 mg/kg/day as a maintenance dose, in combination with standard therapy resulted in a significant decrease of plasma ammonia levels. This observation suggests that NCG may be an important adjunct to standard therapy in the management of decompensated MSUD patients with high leucine and ammonia levels. Supportive evidence from either randomized controlled trials or a large prospective cohort study is needed to confirm this interesting finding.
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