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印度儿童肝硬化:一种色氨酸代谢的遗传性疾病?

Indian childhood cirrhosis: an inherited disorder of tryptophan metabolism?

作者信息

Sur A M, Bhatti A

出版信息

Br Med J. 1978 Aug 19;2(6136):529-31. doi: 10.1136/bmj.2.6136.529.

Abstract

Urine samples from members of 29 families of patients with Indian childhood cirrhosis (ICC) and nine families with related disorders gave positive reactions when tested with ferric chloride. Column chromatography showed that this was due to the presence of abnormally large amounts of tryptophan metabolites, notably 3-hydroxyanthranilic acid. Affected pedigrees had a significantly greater prevalence of peptic ulcer, adult cirrhosis, diabetes mellitus, migraine, and Parkinsonism than a control population. ICC may result from an inborn error of tryptophan metabolism in susceptible ethnic groups.

摘要

对29个患有印度儿童肝硬化(ICC)的家庭以及9个患有相关疾病的家庭的成员的尿液样本进行氯化铁测试时,结果呈阳性。柱色谱分析表明,这是由于存在异常大量的色氨酸代谢物,尤其是3-羟基邻氨基苯甲酸。与对照组相比,受影响的家系中消化性溃疡、成人肝硬化、糖尿病、偏头痛和帕金森病的患病率明显更高。ICC可能是由于易感种族中色氨酸代谢的先天性缺陷所致。

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