Increased Urinary Excretion of Putrescine in Hyperargininaemia

Overview

Journal J Inherit Metab Dis

Publisher Wiley

Date 1987 Jan 1

PMID 3126359

Citations 1

Authors

T Kato

M Sano

N Mizutani

C Hayakawa

Affiliations

Soon will be listed here.

Abstract

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.

Citing Articles

Homocitrullinuria and homoargininuria in hyperargininaemia.

Kato T, Sano M, Mizutani N, Hayakawa C J Inherit Metab Dis. 1988; 11(3):261-5.

PMID: 2466169 DOI: 10.1007/BF01800367.

References

GOODMAN M, ZIEVE L, Konstantinides F, Cerra F . Mechanism of arginine protection against ammonia intoxication in the rat. Am J Physiol. 1984; 247(3 Pt 1):G290-5. DOI: 10.1152/ajpgi.1984.247.3.G290. View

Hommes F, Kitchings L, Eller A . The uptake of ornithine and lysine by rat liver mitochondria. Biochem Med. 1983; 30(3):313-21. DOI: 10.1016/0006-2944(83)90022-4. View

Snyderman S, Sansaricq C, Chen W, NORTON P, PHANSALKAR S . Argininemia. J Pediatr. 1977; 90(4):563-8. DOI: 10.1016/s0022-3476(77)80367-3. View

Russell D . Increased polyamine concentrations in the urine of human cancer patients. Nat New Biol. 1971; 233(39):144-5. DOI: 10.1038/newbio233144a0. View

Goldstein A, Hoogenraad N, Johnson J, Fukanaga K, SWIERCZEWSKI E, Cann H . Metabolic and genetic studies of a family with ornithine transcarbamylase deficiency. Pediatr Res. 1974; 8(1):5-12. DOI: 10.1203/00006450-197401000-00002. View

Kang S, Wong P, Melyn M . Hyperargininemia: effect of ornithine and lysine supplementation. J Pediatr. 1983; 103(5):763-5. DOI: 10.1016/s0022-3476(83)80481-8. View

Freedland R, Crozier G, HICKS B, Meijer A . Arginine uptake by isolated rat liver mitochondria. Biochim Biophys Acta. 1984; 802(3):407-12. DOI: 10.1016/0304-4165(84)90357-x. View

Qureshi I, Letarte J, Ouellet R, Lelievre M, Laberge C . Ammonia metabolism in a family affected by hyperargininemia. Diabete Metab. 1981; 7(1):5-11. View

Shih V, Jones T, Levy H, Madigan P . Arginase deficiency in Macaca fascicularis. I. Arginase activity and arginine concentration in erythrocytes and liver. Pediatr Res. 1972; 6(6):548-51. DOI: 10.1203/00006450-197206000-00003. View

10.

Mizutani N, Maehara M, Hayakawa C, Kato T, Watanabe K, Suzuki S . Hyperargininemia: clinical course and treatment with sodium benzoate and phenylacetic acid. Brain Dev. 1983; 5(6):555-63. DOI: 10.1016/s0387-7604(83)80059-x. View

11.

Cederbaum S, Moedjono S, SHAW K, Carter M, Naylor E, WALZER M . Treatment of hyperargininaemia due to arginase deficiency with a chemically defined diet. J Inherit Metab Dis. 1982; 5(2):95-9. DOI: 10.1007/BF01800000. View

12.

Marton L, Russell D, Levy C . Measurement of putrescine, spermidine, and spermine in physiological fluids by use of an amino acid analyzer. Clin Chem. 1973; 19(8):923-6. View

13.

Bachmann C, COLOMBO J . Diagnostic value of orotic acid excretion in heritable disorders of the urea cycle and in hyperammonemia due to organic acidurias. Eur J Pediatr. 1980; 134(2):109-13. DOI: 10.1007/BF01846026. View