Identification of benzoylcarnitine in the urine of a patient of hyperammonemia.
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.
Sakuma
T
T
Department of Pediatrics, Nagoya City University Medical School.
Asai
K
K
Ichiki
T
T
Sugiyama
N
N
Kidouchi
K
K
Wada
Y
Y
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
D001565:Benzoates; C050750:benzoylcarnitine; D000641:Ammonia; D019817:Benzoic Acid; D002222:Carbamoyl-Phosphate Synthase (Ammonia); D002331:Carnitine