Endocrinol Nutr. 2009 Jun-Jul;56(6):337-40.

[Primary trimethylaminuria: the fish odor syndrome].

[Article in Spanish]

Montoya Alvarez T, Guardiola PD, Roldán JO, Elviro R, Wevers R, Guijarro G.

Source

Servicio de Endocrinología y Nutrición, Hospital Infanta Elena, Madrid, España. tmonalv@alumni.unav.es

Abstract

Primary trimethylaminuria, or fish odor syndrome, is a congenital metabolic disorder characterized by a failure in the hepatic trimethylamine (TMA) oxidation route to trimethylamine N-oxide (TMANO). TMA is mostly derived from dietary precursors such as choline, carnitine and TMANO. The presence of abnormal amounts of TMA in the urine, sweat, exhaled air and other body secretions confers a very unpleasant body odor resembling that of decaying fish. As a consequence, patients can suffer from serious psychosocial sequelae. We present a case of primary trimethylaminuria with the aim of raising awareness about this condition.

PMID:: 19695515; [PubMed - indexed for MEDLINE]

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[Trimethylaminuria: fish-odor syndrome]. [Harefuah. 1993]
[Trimethylaminuria: fish-odor syndrome].
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Cashman JR, Camp K, Fakharzadeh SS, Fennessey PV, Hines RN, Mamer OA, Mitchell SC, Nguyen GP, Schlenk D, Smith RL, et al. Curr Drug Metab. 2003 Apr; 4(2):151-70.
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