Definition of Fish odor syndrome

Fish-odor syndrome: An inborn error of metabolism that is associated with an offensive body odor whose scent is similar to the smell of rotting fish. Fish-odor syndrome is due to the excessive excretion of trimethylamine (TMA) in urine, sweat, and breath. This syndrome is caused by a mutation in the gene for the enzyme flavin-containing monooxygenase-3 (FMO3). Dietary modifications to reduce the consumption of triethylamine precursors may provide some relief of symptoms. Also known as trimethylaminuria.


Last Editorial Review: 3/19/2012

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