title: Identification of benzoylcarnitine in the urine of a patient of hyperammonemia.
abstract: Benzoylcarnitine was identified in the urine of a patient with a carbamoyl-phosphate synthase I deficiency for whom sodium benzoate and L-carnitine had been used to treat hyperammonemia. This is a newly identified metabolite of benzoate. Its excretion in the urine was increased day by day at the administration of both sodium benzoate and L-carnitine from 0.10 to 2.25 mmol/g creatinine. Since there is the possibility of a secondary carnitine deficiency and an increase of benzoyl toxicity after long-term therapy with benzoate supplementation and protein restriction, it is important to monitor the urinary excretion of benzoylcarnitine.
authors.affiliation: Department of Pediatrics, Nagoya City University Medical School.
authors.forename: T
authors.initials: T
authors.lastname: Sakuma
authors.forename: K
authors.initials: K
authors.lastname: Asai
authors.forename: T
authors.initials: T
authors.lastname: Ichiki
authors.forename: N
authors.initials: N
authors.lastname: Sugiyama
authors.forename: K
authors.initials: K
authors.lastname: Kidouchi
authors.forename: Y
authors.initials: Y
authors.lastname: Wada
mesh_terms: D000641:Ammonia / Q000097:blood*; D001565:Benzoates / Q000627:therapeutic use*; D019817:Benzoic Acid; D002222:Carbamoyl-Phosphate Synthase (Ammonia) / Q000172:deficiency*; D002331:Carnitine / Q000031:analogs & derivatives* / Q000652:urine; D006801:Humans
chemical_list: D001565:Benzoates; C050750:benzoylcarnitine; D000641:Ammonia; D019817:Benzoic Acid; D002222:Carbamoyl-Phosphate Synthase (Ammonia); D002331:Carnitine