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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本文引用的文献
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PEPTIC ULCER AND GASTRIC SECRETION IN PATIENTS WITH LIVER DISEASE.肝病患者的消化性溃疡与胃液分泌
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Autoimmune antibodies in infantile cirrhosis.婴儿肝硬化中的自身免疫抗体。
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3
Sources of upper alimentary tract hemorrhage in cirrhosis of the liver.肝硬化患者上消化道出血的病因
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4
The incidence of portal cirrhosis and fatty metamorphosis in patients dying with diabetes mellitus.死于糖尿病患者的门脉性肝硬化和脂肪变性的发生率。
N Engl J Med. 1953 Sep 10;249(11):442-5. doi: 10.1056/NEJM195309102491104.
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Indian childhood cirrhosis.印度儿童肝硬化
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Plasma zinc concentration in Indian childhood cirrhosis.印度儿童肝硬化患者的血浆锌浓度
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8
Histopathological evolution of Indian childhood cirrhosis with emphasis on criteria of early diagnosis.印度儿童肝硬化的组织病理学演变，重点关注早期诊断标准。
Indian Pediatr. 1974 Jan;11(1):19-28.
9
Morphological features of Indian childhood cirrhosis: the spectrum of changes and their significance.印度儿童肝硬化的形态学特征：变化范围及其意义。
Indian J Med Res. 1974 Jul;62(7):953-63.
10
Testing the functional capacity of the tryptophan-niacin pathway in man by analysis of urinary metabolites.通过分析尿液代谢物来测试人体色氨酸-烟酸途径的功能能力。
Adv Metab Disord. 1965;2:159-225. doi: 10.1016/b978-1-4831-6750-3.50009-3.
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