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Excretion of hippuric acid during sodium benzoate therapy in patients with hyperglycinaemia or hyperammonaemia
Author(s) -
Barshop B. A.,
Breuer J.,
Holm J.,
Leslie J.,
Nyhan W. L.
Publication year - 1989
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1007/bf01805533
Subject(s) - hippuric acid , sodium benzoate , excretion , urea cycle , medicine , chemistry , endocrinology , urea , urine , glycine , biochemistry , arginine , amino acid
Summary 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 ± 7.0 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.