Chronic sodium benzoate therapy in children with inborn errors of urea synthesis: effect on carnitine metabolism and ammonia nitrogen removal.

Published

Journal Article

Sodium benzoate (SB) therapy is known to increase ammonia (NH3) nitrogen elimination via conjugation with glycine and excretion as urinary hippurate. In 16 children with inborn errors of urea synthesis we studied two issues: (1) the effect of chronic SB administration upon carnitine metabolism and (2) the efficacy of chronic SB therapy as measured by the molar ratio of hippurate excretion to SB intake. Measurements were performed during elective hospitalizations when the patients were in stable metabolic condition. We found that chronic SB therapy is not associated with a constant level of hippurate elimination and that interindividual and intraindividual variability may result in irregular removal of NH3 nitrogen. This variability may be due to various factors including the formation of small quantities of benzoylcarnitine, which was detected in the plasma of three of four patients receiving SB and carnitine therapy and in one of two patients on SB therapy without carnitine supplementation. The ratios of acyl to free carnitine were elevated in both plasma and urine in patients not receiving carnitine supplementation, but were normal in patients receiving supplementation.

Full Text

Duke Authors

Cited Authors

  • Feoli-Fonseca, JC; Lambert, M; Mitchell, G; Melançon, SB; Dallaire, L; Millington, DS; Qureshi, IA

Published Date

  • February 1996

Published In

Volume / Issue

  • 57 / 1

Start / End Page

  • 31 - 36

PubMed ID

  • 8812724

Pubmed Central ID

  • 8812724

International Standard Serial Number (ISSN)

  • 1077-3150

Language

  • eng

Conference Location

  • United States