Summary
A 4-year-old boy with hyperargininaemia had an increased urinary excretion of putrescine which was exaggerated with oral ornithine supplementation. It seems unlikely that putrescine was overproduced within the gut, because a loading with a single oral dose of ornithine showed that the intestinal ornithine absorption in the patient was normal. An acceleration of extramitochondrial ornithine metabolism due to impaired mitochondrial ornithine uptake may have caused the hyperexcretion of putrescine.
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References
Bachmann, C. and Colombo, J. P. Diagnostic value of orotic acid excretion in heritable disorders of the urea cycle and in hyperammonemia due to organic acidurias.Eur. J. Pediatr. 134 (1980) 109–113
Cederbaum, S. D., Moedjono, S. J., Shaw, K. N. F., Carter, M., Naylor, E. and Walzer, M. Treatment of hyperargininaemia due to arginase deficiency with a chemically defined diet.J. Inher. Metab. Dis. 5 (1982) 95–99
Freedland, R. A., Crozier, G. L., Hicks, B. L. and Meijer, A. J. Arginine uptake by isolated rat liver mitochondria.Biochim. Biophys. Acta 802 (1984) 407–412
Goldstein, A. S., Hoogenraad, N. J., Johnson, J. D., Fukanaga, K., Swierczewski, E., Cann, H. M. and Sunshine, P. Metabolic and genetic studies of a family with ornithine transcarbamylase deficiency.Pediatr. Res. 8 (1974) 5–12
Goodman, M. W., Zieve, L., Konstantinides, F. N. and Cerra, F. B. Mechanism of arginine protection against ammonia intoxication in the rat.Am. J. Physiol. 247 (1984) G290-G295
Hommes, F. A., Kitchings, L. and Eller, A. G. The uptake of ornithine and lysine by rat liver mitochondria.Biochem. Med. 30 (1983) 313–321
Kang, S., Wong, P. W. K. and Melyn, M. A. Hyperargininemia: Effect of ornithine and lysine supplementation.J. Pediatr. 103 (1983) 763–765
Marton, L. J., Russell, D. H. and Levy, C. C. Measurement of putrescine, spermidine, and spermine in physiological fluids by use of an amino acid analyzer.Clin. Chem. 19 (1973) 923–926
Mizutani, N., Maehara, M., Hayakawa, C., Kato, T., Watanabe, K. and Suzuki, S. Hyperargininemia: Clinical course and treatment with sodium benzoate and phenylacetic acid.Brain Dev. 5 (1983) 555–563
qureshi, I. A., Letarte, J., Ouellet, R., Leliévre, M. and Laberge, C. Ammonia metabolism in a family affected by hyperargininemia.Diabet. Metab. 7 (1981) 5–11
Russell, D. H. Increased polyamine concentrations in the urine of human cancer patients.Nature 233 (1971) 144–145
Segal, S. and Thier, S. O. Cystinuria. In Stanbury, J. B., Wyngaarden, J. B., Fredrickson, D. S., Goldstein, J. L. and Brown, M. S. (eds.),The Metabolic Basis of Inherited Disease, McGraw-Hill, New York, 1983, pp. 1774–1791
Shih, V. E. Urea cycle disorders and other congenital hyperammonemic syndromes. In Stanbury, J. B., Wyngaarden, J. B. and Fredrickson, D. S. (eds.),The Metabolic Basis of Inherited Disease, McGraw-Hill, New York, 1978, pp. 362–386
Shih, V. E., Jones, T. G., Levy, H. L. and Madigan, P. M. Arginase deficiency in macaca fascicularis. 1. Arginase activity and arginine concentration in erythrocytes and in liver.Pediatr. Res. 6 (1972) 548–551
Snyderman, S. E., Sansaricq, C., Chen, W. J., Norton, P. M. and Phansalkar, S. V. Argininemia.J. Pediatr. 90 (1977) 563–568
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Kato, T., Sano, M., Mizutani, N. et al. Increased urinary excretion of putrescine in hyperargininaemia. J Inherit Metab Dis 10, 391–396 (1987). https://doi.org/10.1007/BF01799982
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DOI: https://doi.org/10.1007/BF01799982