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. 1999 Aug;75(886):451–452. doi: 10.1136/pgmj.75.886.451

Fish odour syndrome

H Rehman 1
PMCID: PMC1741321  PMID: 10646019

Abstract

Fish odour syndrome (trimethylaminuria) is a metabolic syndrome caused by abnormal excretion of trimethylamine in the breath, urine, sweat, saliva and vaginal secretions. Trimethylamine is derived from the intestinal bacterial degradation of foods rich in choline and carnitine and is normally oxidised by the liver to odourless trimethylamine N-oxide which is then excreted in the urine. Impaired oxidation of trimethylamine is thought to be the cause of the fish odour syndrome and is responsible for the smell of rotting fish. Certain foods rich in choline exacerbate the condition and the patients have a variety of psychological problems. Recognition of the condition is important as dietary adjustments reduce the excretion of trimethylamine and may reduce the odour. Occasionally, a short course of metronidazole, neomycin and lactulose may suppress production of trimethylamine by reducing the activity of gut microflora.


Keywords: fish odour syndrome; trimethylaminuria

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Selected References

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  1. Al-Waiz M., Ayesh R., Mitchell S. C., Idle J. R., Smith R. L. A genetic polymorphism of the N-oxidation of trimethylamine in humans. Clin Pharmacol Ther. 1987 Nov;42(5):588–594. doi: 10.1038/clpt.1987.201. [DOI] [PubMed] [Google Scholar]
  2. Ayesh R., Mitchell S. C., Zhang A., Smith R. L. The fish odour syndrome: biochemical, familial, and clinical aspects. BMJ. 1993 Sep 11;307(6905):655–657. doi: 10.1136/bmj.307.6905.655. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. Brand J. M., Galask R. P. Trimethylamine: the substance mainly responsible for the fishy odor often associated with bacterial vaginosis. Obstet Gynecol. 1986 Nov;68(5):682–685. [PubMed] [Google Scholar]
  4. Fenwick G. R., Butler E. J., Brewster M. A. Are brassica vegetables aggravating factors in trimethylaminuria (fish odour syndrome)? Lancet. 1983 Oct 15;2(8355):916–916. doi: 10.1016/s0140-6736(83)90903-0. [DOI] [PubMed] [Google Scholar]
  5. Fernández M. S., Gutiérrez C., Vila J. J., López A., Ibáez V., Sangüesa C., Lluna J., Barrios J. E. Congenital intrahepatic portocaval shunt associated with trimethylaminuria. Pediatr Surg Int. 1997 Feb;12(2-3):196–197. [PubMed] [Google Scholar]
  6. Growdon J. H., Cohen E. L., Wurtman R. J. Huntington's disease: clinical and chemical effects of choline administration. Ann Neurol. 1977 May;1(5):418–422. doi: 10.1002/ana.410010503. [DOI] [PubMed] [Google Scholar]
  7. Hadidi H. F., Cholerton S., Atkinson S., Irshaid Y. M., Rawashdeh N. M., Idle J. R. The N-oxidation of trimethylamine in a Jordanian population. Br J Clin Pharmacol. 1995 Feb;39(2):179–181. doi: 10.1111/j.1365-2125.1995.tb04427.x. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. Higgins T., Chaykin S., Hammond K. B., Humbert J. R. Trimethylamine N-oxide synthesis: a human variant. Biochem Med. 1972 Aug;6(4):392–396. doi: 10.1016/0006-2944(72)90025-7. [DOI] [PubMed] [Google Scholar]
  9. Humbert J. A., Hammond K. B., Hathaway W. E. Trimethylaminuria: the fish-odour syndrome. Lancet. 1970 Oct 10;2(7676):770–771. doi: 10.1016/s0140-6736(70)90241-2. [DOI] [PubMed] [Google Scholar]
  10. Marks R., Dudley F., Wan A. Trimethylamine metabolism in liver disease. Lancet. 1978 May 20;1(8073):1106–1107. doi: 10.1016/s0140-6736(78)90958-3. [DOI] [PubMed] [Google Scholar]
  11. McConnell H. W., Mitchell S. C., Smith R. L., Brewster M. Trimethylaminuria associated with seizures and behavioural disturbance: a case report. Seizure. 1997 Aug;6(4):317–321. doi: 10.1016/s1059-1311(97)80080-5. [DOI] [PubMed] [Google Scholar]
  12. Mitchell S. C., Zhang A. Q., Barrett T., Ayesh R., Smith R. L. Studies on the discontinuous N-oxidation of trimethylamine among Jordanian, Ecuadorian and New Guinean populations. Pharmacogenetics. 1997 Feb;7(1):45–50. doi: 10.1097/00008571-199702000-00006. [DOI] [PubMed] [Google Scholar]
  13. Pike M. G., King G. S., Pettit B. R., Leonard J. V., Atherton D. J. Lactulose in trimethylaminuria, the fish-odour syndrome. Helv Paediatr Acta. 1989 Feb;43(4):345–348. [PubMed] [Google Scholar]
  14. Rothschild J. G., Hansen R. C. Fish odor syndrome: trimethylaminuria with milk as chief dietary factor. Pediatr Dermatol. 1985 Nov;3(1):38–39. doi: 10.1111/j.1525-1470.1985.tb00483.x. [DOI] [PubMed] [Google Scholar]
  15. Shelley E. D., Shelley W. B. The fish odor syndrome. Trimethylaminuria. JAMA. 1984 Jan 13;251(2):253–255. [PubMed] [Google Scholar]
  16. Wills M. R., Savory J. Biochemistry of renal failure. Ann Clin Lab Sci. 1981 Jul-Aug;11(4):292–299. [PubMed] [Google Scholar]
  17. Zhang A. Q., Mitchell S. C., Smith R. L. Discontinuous distribution of N-oxidation of dietary-derived trimethylamine in a British population. Xenobiotica. 1996 Sep;26(9):957–961. doi: 10.3109/00498259609052497. [DOI] [PubMed] [Google Scholar]
  18. Zhang A. Q., Mitchell S., Smith R. Fish odour syndrome: verification of carrier detection test. J Inherit Metab Dis. 1995;18(6):669–674. doi: 10.1007/BF02436755. [DOI] [PubMed] [Google Scholar]
  19. de la HUERGA J., POPPER H. Urinary excretion of choline metabolites following choline administration in normals and patients with hepatobiliary diseases. J Clin Invest. 1951 May;30(5):463–470. doi: 10.1172/JCI102463. [DOI] [PMC free article] [PubMed] [Google Scholar]

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