Fish odor syndrome: a case report of trimethylaminuria.
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.
Wright State University, Boonshoft School of Medicine.
Catherine A
CA
Ulman
Julian J
JJ
Trevino
Marvin
M
Miller
Rishi K
RK
Gandhi
D001008:Anxiety Disorders / Q000150:complications; D002648:Child; D005260:Female; D006801:Humans; D006920:Hygiene; D006945:Hyperhidrosis / Q000150:complications; D008661:Metabolism, Inborn Errors / Q000175:diagnosis* / Q000178:diet therapy / Q000188:drug therapy / Q000235:genetics / Q000652:urine; D008744:Methylamines / Q000652:urine*; D008795:Metronidazole / Q000627:therapeutic use; D010105:Oxygenases / Q000172:deficiency / Q000235:genetics
D008744:Methylamines; D008795:Metronidazole; D010105:Oxygenases; C020066:dimethylaniline monooxygenase (N-oxide forming)