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与急性间歇性卟啉症相关的肾小管间质性肾炎。

Tubulointerstitial nephritis associated with acute intermittent porphyria.

作者信息

Onozato M L, Tojo A, Kamijo A, Taniguchi S, Kimura K, Goto A, Fujita T

机构信息

Department of Internal Medicine, University of Tokyo, Japan.

出版信息

Clin Nephrol. 2001 Feb;55(2):171-4.

Abstract

Progressive renal impairment associated with acute intermittent porphyria is not well recognized and the mechanism of renal damage remains unclear. We report a case of a 51-year-old female with acute intermittent porphyria and long-term follow-up who developed proteinuria and renal insufficiency. Her biopsy showed marked tubulointerstitial damage with mitochondrial abnormalities. Urinary excretion of lipid peroxidation was increased compared to healthy controls. The porphyrin precursors may increase lipid peroxidation products and damage mitochondria leading to tubulointerstitial nephritis.

摘要

与急性间歇性卟啉症相关的进行性肾功能损害尚未得到充分认识,肾脏损伤的机制仍不清楚。我们报告一例51岁患有急性间歇性卟啉症的女性患者,经长期随访出现蛋白尿和肾功能不全。她的活检显示有明显的肾小管间质损伤及线粒体异常。与健康对照相比,其尿中脂质过氧化产物排泄增加。卟啉前体可能会增加脂质过氧化产物并损害线粒体,从而导致肾小管间质性肾炎。

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