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[特发性血色素沉着症与迟发性皮肤卟啉症的关联]

[Association of idiopathic hemochromatosis and tardive cutaneous porphyria].

作者信息

Buysschaert M, Verstraeten L, Novik V, Brasseur A, Donckier J, Hassoun A

机构信息

Département de Médecine Interne, Cliniques Universitaires UCL de Mont-Godinne, Yvoir.

出版信息

Acta Clin Belg. 1991;46(5):333-7. doi: 10.1080/17843286.1991.11718185.

Abstract

Porphyria cutanea tarda is a disorder of porphyrin metabolism that results from a deficiency of uroporphyrinogen decarboxylase, resulting in a characteristic pattern of porphyrin excretion. Elevated serum iron values are frequently observed among patients with porphyria cutanea tarda. The hypothesis has been advanced that a hemochromatosis allele is implicated in the clinical manifestation of porphyria cutanea tarda. We report the case of a patient suffering from both idiopathic hemochromatosis and porphyria cutanea tarda. The data of the medical literature concerning such an association are discussed.

摘要

迟发性皮肤卟啉症是一种卟啉代谢紊乱疾病,由尿卟啉原脱羧酶缺乏引起,导致特征性的卟啉排泄模式。迟发性皮肤卟啉症患者中经常观察到血清铁值升高。有一种假说认为,血色素沉着病等位基因与迟发性皮肤卟啉症的临床表现有关。我们报告了一例同时患有特发性血色素沉着病和迟发性皮肤卟啉症的患者。文中讨论了有关这种关联的医学文献数据。

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