Transient trimethylaminuria in childhood.
Trimethylaminuria, also called fish-odour syndrome, is an inherited disorder caused by deficient N-oxidation and increased excretion of trimethylamine. This study reports on two unrelated and otherwise healthy children with transient trimethylaminuria, a hitherto unknown abnormality, without N-oxidation deficiency. This demonstrates that a diagnosis of fish-odour syndrome should include the analysis of urinary excretion not only of trimethylamine but also of trimethylamine-N-oxide. Since transient trimethylaminuria may be a common condition and no cause for it can presently be recognized, such patients will require careful follow-up.
Mayatepek
E
E
Division of Metabolic Diseases, University Children's Hospital, Heidelberg, Germany.
Kohlmüller
D
D
D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008661:Metabolism, Inborn Errors / Q000652:urine*; D008744:Methylamines / Q000652:urine*; D009812:Odorants; D013997:Time Factors
D008744:Methylamines