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Open Access Publications from the University of California

Fish odor syndrome: a case report of trimethylaminuria

  • Author(s): Ulman, Catherine A
  • Trevino, Julian J
  • Miller, Marvin
  • Gandhi, Rishi K
  • et al.
Creative Commons 'BY-NC-ND' version 4.0 license

Trimethylaminuria is a rare, autosomal recessive, metabolic disorder that results in accumulation of trimethylamine (TMA), which smells like rotten fish. The chemical is excreted in sweat and urine owing to a deficiency in the enzyme flavin monooxygenase 3 (FMO3). We report a case of trimethylaminuria in a 12-year-old girl. The patient failed treatment with diet and hygiene modification, but achieved symptomatic improvement after a four-month course of metronidazole.

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