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不同类型卟啉病患者的尿卟啉排泄情况。薄层色谱研究。

Urinary porphyrin excretion in various types of porphyria. Thin-layer chromatographic study.

作者信息

Perrot H, Boucherat M, Gardella C

出版信息

Dermatologica. 1980;161(3):167-78. doi: 10.1159/000250353.

Abstract

A new method of thin-layer chromatography was used for the study of urinary porphyrins in 42 porphyric patients (27 cases of porphyria cutanea tarda (PCT), 5 cases of porphyria variegata, 4 cases of acute intermittent porphyria, 2 cases of hereditary coproporphyria and 4 cases of erythropoietic protoporphyria), 21 of their clinically normal relatives and 5 controls. The results are compared to previously published data and discussed for the diagnosis of the porphyrias. If urinary porphyrin pattern seems sufficiently pathognomonic for PCT, it appears often unable to allow exact diagnosis of the acute porphyrias; faecal studies and sometimes enzymatic determinations are necessary.

摘要

一种新的薄层色谱法用于研究42例卟啉病患者(27例迟发性皮肤卟啉病(PCT)、5例混合型卟啉病、4例急性间歇性卟啉病、2例遗传性粪卟啉病和4例红细胞生成性原卟啉病)、21例临床正常的亲属以及5例对照者的尿卟啉。将结果与先前发表的数据进行比较,并就卟啉病的诊断进行讨论。尿卟啉模式对PCT似乎具有足够的诊断特异性,但往往无法对急性卟啉病做出准确诊断;粪便研究以及有时的酶测定是必要的。

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