TRIMETHYLAMINURIA

By Gary M. White, MD


Trimethylaminuria, AKA Fish Odor Syndrome, is a rare autosomal recessive disorder of metabolism caused by a mutation in flavin-containing monooxygenase 3 (FMO3 gene).

Treatment

Genetic counseling is in order. Referral to a dietician for aid in minimizing consumption of certain foods, e.g., eggs, legumes, fish, and certain meats is recommended. Daily or twice a day shower, using deodorants, and avoiding hot, sweaty environments is helpful. Oral metronidazole can reduce gut flora and be very helpful [Dermatology Online Journal 20(1)]. Psychological counseling may be needed.

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