威尔逊病患者用d -青霉胺治疗后的肾病综合征

A. Kostadinova, M. Mihaylov, I. Ivanova, R. Robeva
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引用次数: 2

摘要

Wilson病是一种铜平衡的遗传性常染色体隐性遗传病,主要由运铜的p型atp酶缺失或功能降低导致铜在肝脏和大脑中积累。铜是一种必需的微量元素,但在威尔逊氏病中,它积累到中毒的地步。d -青霉胺是治疗威尔森氏病的经典药物。其主要作用是促进尿中铜的排泄。使用d -青霉胺治疗威尔逊氏病是已知的复杂的各种肾小球疾病的发展。在这篇报告中,我们描述了一位31岁男性威尔逊病患者在接受d -青霉胺治疗2年后出现肾病综合征,停药后病情迅速好转。我们提出这个病例是为了引起人们对威尔森氏病患者在d -青霉胺治疗下的罕见并发症肾病综合征及其可能的潜在原因的关注。定期监测Wilson病患者的治疗和临床状况是非常必要的,我们建议每月监测一次。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Nephrotic syndrome after treatment with D-penicillamine in a patient with Wilson’s disease
Abstract Wilson’s disease is an inherited autosomal recessive disorder of copper balance leading to accumulation of copper mainly in liver and brain result from absent or reduced function of copper-transporting P-type ATPase. Copper is an essential trace element but in Wilson’s disease it accumulate to the point of toxicity. D-penicillamine is a classic drug for treatment of Wilson’s disease. Its major effect is to promote the urinary copper excretion. The use of D-penicillamine in the therapy of Wilson’s disease is known to be complicated by the development of various glomerular diseases. In this report we describe the development of nephrotic syndrome after 2 years treatment with D-penicillamine in a 31-year-old male undergoing treatment for Wilson’s disease, with a prompt regression at the discontinuation of the drug. We present this case to draw attention to the rare complication as nephrotic syndrome in patients with Wilson’s disease under D-penicillamine treatment and possible underlying causes. It is strongly necessary the therapy and clinical condition of patients with Wilson’s disease to be monitoring regularly - we recommended monthly.
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