Journal of Inherited Metabolic Disease

, Volume 12, Issue 1, pp 72–79 | Cite as

Excretion of hippuric acid during sodium benzoate therapy in patients with hyperglycinaemia or hyperammonaemia

  • B. A. Barshop
  • J. Breuer
  • J. Holm
  • J. Leslie
  • W. L. Nyhan


In patients with non-ketotic hyperglycinaemia and two patients with urea cycle disorders treated with varying doses of sodium benzoate there was a linear correlation between intake of benzoate and excretion of hippurate. Patients with non-ketotic hyperglycinaemia excreted significantly more benzoate in the form of hippurate than patients with urea cycle disorders (74 ± 7.0vs 41 ± 3.6%). The plasma concentration of glycine decreased following benzoate treatment only in the patients with non-ketotic hyperglycinaemia. The observed difference between the two groups in the excretion of hippurate seems to support the concept that glycine availability may be limiting in benzoate therapy for some patients.


Public Health Sodium Internal Medicine Urea Plasma Concentration 
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Copyright information

© SSIEM and Kluwer Academic Publishers 1989

Authors and Affiliations

  • B. A. Barshop
    • 1
  • J. Breuer
    • 1
  • J. Holm
    • 1
  • J. Leslie
    • 1
  • W. L. Nyhan
    • 1
  1. 1.Department of Pediatrics, and the Center for Molecular GeneticsUniversity of California San DiegoLa JollaUSA

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