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埃塞俄比亚迟发性皮肤卟啉症

Porphyria cutanea tarda in Ethiopia.

作者信息

Tsega E, Besrat A, Landells J W, Seyoum E

出版信息

Trans R Soc Trop Med Hyg. 1981;75(2):201-6. doi: 10.1016/0035-9203(81)90317-5.

Abstract

The clinical, biochemical and histological features of 75 Ethiopians with porphyria cutanea tarda (PCT) are described. PCT in Ethiopia is definitely related to alcohol abuse and there is no clinical evidence for hereditary predisposition. Significant elevation of transaminases and bromsulphthalein retention, moderate to marked hepatic siderosis and inflammation with little or no fibrosis, suggestive of mild or likely reversible parenchymal changes, characterize the liver affection in these patients. A similar study of 18 patients with hyperpigmentation of the face and hands but without blisters, an unexplained but common feature of liver disease in Ethiopia, revealed that serum iron and urinary uroporphyrin levels were normal in eight but significantly elevated in ten (56%). Neither elevated serum iron and hepatic siderosis nor increased urinary uroporphyrin completely explains the hyperpigmentation in this group of patients.

摘要

本文描述了75例迟发性皮肤卟啉病(PCT)埃塞俄比亚患者的临床、生化和组织学特征。埃塞俄比亚的PCT肯定与酒精滥用有关,且无遗传易感性的临床证据。转氨酶显著升高和磺溴酞钠潴留、中度至重度肝铁质沉着症以及炎症伴轻微或无纤维化,提示这些患者肝脏病变为轻度或可能可逆的实质改变。对18例面部和手部色素沉着但无水泡(埃塞俄比亚肝病的一种无法解释但常见特征)患者的类似研究显示,8例患者血清铁和尿卟啉水平正常,10例(56%)显著升高。血清铁升高、肝铁质沉着症以及尿卟啉增加均不能完全解释该组患者的色素沉着情况。

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