Excretion of hippuric acid during sodium benzoate therapy in patients with hyperglycinaemia or hyperammonaemia
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.
KeywordsPublic Health Sodium Internal Medicine Urea Plasma Concentration
Unable to display preview. Download preview PDF.
- Batshaw, M. L. Sodium benzoate and arginine: Alternative pathway therapy in inborn errors of urea synthesis. In Brewer, G. J. (ed.),Orphan Drugs and Orphan Diseases: Clinical Realities and Public Policy, Liss, New York, 1983, pp. 69–83Google Scholar
- Batshaw, M. L., Brusilow, S., Waber, L., Blom, W., Brubakk, A. M., Burton, B. K., Cann, H., Kerr, D., Mamunes, P., Matalon, R., Myerberg, D. and Schafer, I. A. Treatment of inborn errors of urea synthesis. Activation of alternative pathways of waste nitrogen synthesis and excretion.N. Engl. J. Med. 306 (1982) 1387–1392PubMedGoogle Scholar
- Brandt, I. K. Hippurate synthesis in the newborn rat.Am. J. Dis. Child. 100 (1960) 538–539Google Scholar
- Brusilow, S., Danney, M., Waber, L. J., Batshaw, M., Burton, B., Levitsky, L., Roth, K., McKeethren, C. and Ward, J. Treatment of episodic hyperammonemia in children with inborn errors of urea synthesis.N. Engl. J. Med. 110 (1984) 1630–1634Google Scholar
- Gram, T. E. Metabolism of drugs. In Craig, C. R. and Stitzel, R. E. (eds.),Modern Pharmacology, Little, Boston, 2nd edition, 1986, pp. 50ffGoogle Scholar
- Mitch, W. E. and Brusilow, S. Benzoate-induced changes in glycine and urea metabolism in patients with chronic renal failure.J. Pharm. Exp. Ther. 222 (1982) 572–575Google Scholar
- Simkin, J. L. and White, K. The formation of hippuric acid. The influence of benzoate administration on tissue glycine levels.Biochemistry 65 (1957) 574–581Google Scholar
- Spackman, D. H., Stein, W. H. and Moore, S. Automatic recording apparatus for use in the chromatography of amino acids.Anal. Chem. 30 (1958) 1190–1206Google Scholar
- Sweetman, L. Qualitative and quantitative analysis of organic acids in physiologic fluids for diagnosis of the organic acidurias. In Nyhan, W. L.Abnormalities in Amino Acid Metabolism in Clinical Medicine, Appleton-Century-Crofts, E. Norwalk, Conn., 1984, pp. 419–453Google Scholar
- Takeda, S., Kuroda, Y., Toshima, K., Watanabe, T., Naito, E. and Miyao, M. Effect of long-term administration of sodium benzoate to a patient with partial ornithine transcarbamylase deficiency.Clin. Pediatr. 22 (1983) 205–208Google Scholar