Sela B A, Trau H, Spira A
Institute of Chemical Pathology, Chaim Sheba Medical Center, Tel Hashomer.
Harefuah. 1993 Feb 1;124(3):138-9, 183.
Fish-odor syndrome is due to a rare metabolic defect in which there is massive excretion of the volatile tertiary amine, trimethylamine (TMA) in the urine. The typical fish-like odor of TMA also appears on the breath or in the sweat of affected individuals. In normal man TMA is oxidized to its odorless N-oxide derivative. TMA is of dietary origin, formed by intestinal bacterial degradation of the choline in egg yolk, liver, soybeans, etc., or by reduction of TMA-oxide present in high concentrations in marine fish. 2 sisters with the tentative diagnosis of trimethylaminuria are presented.
鱼腥味综合征是由一种罕见的代谢缺陷引起的，即尿液中大量排泄挥发性叔胺三甲胺（TMA）。TMA特有的鱼腥味也会出现在受影响个体的呼吸或汗液中。在正常人中，TMA会被氧化为无味的N-氧化物衍生物。TMA来源于饮食，由肠道细菌对蛋黄、肝脏、大豆等中的胆碱进行降解形成，或由海鱼中高浓度存在的氧化三甲胺还原形成。本文介绍了2例初步诊断为三甲胺尿症的姐妹病例。
