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

Journal of Inherited Metabolic Disease
B A BarshopW L Nyhan

Abstract

In patients with non-ketotic hyperglycinaemia and two patients with urea cycle disorders treated with varying doses of sodium benzoate there was 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 +/- 7.0 vs 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.

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Citations

Jul 15, 1991·Biochemical Pharmacology·D M CyrG C Tremblay
Jun 18, 2009·Diabetes, Obesity & Metabolism·V Walker
Sep 10, 2005·Journal of Inherited Metabolic Disease·J L K Van HoveJ Jaeken
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Aug 1, 1998·Journal of Inherited Metabolic Disease·F Feillet, J V Leonard
Feb 22, 2000·Journal of Inherited Metabolic Disease·J M NeubergerR Burghard

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