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肾源性系统性纤维化:新难题的综述

Nephrogenic systemic fibrosis: a review of the new conundrum.

作者信息

Goenka Ajit H, Das Chandan J, Sharma Raju

机构信息

Department of Radiodiagnosis, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110029, India.

出版信息

Natl Med J India. 2009 Nov-Dec;22(6):302-6.

Abstract

Nephrogenic systemic fibrosis is an idiopathic, sclerosing condition that occurs only in patients who have impaired renal function. Although its most conspicuous manifestation is in the skin, the condition involves multiple organ systems and is potentially fatal. Its postulated association with gadolinium-based magnetic resonance contrast agents has attracted attention. The distinctive clinical features of this entity include a prodromal systemic inflammatory syndrome followed by a chronic course of fibrosis that has a predilection for the skin and subcutaneous tissues of the extremities. The progressive systemic fibrosing process involves multiple organs and contributes to the morbidity and the increased mortality. Appropriate preventive action, prompt recognition and timely reporting of cases may enable better management of this condition.

摘要

肾源性系统性纤维化是一种仅发生于肾功能受损患者的特发性硬化性疾病。尽管其最显著的表现是在皮肤,但该疾病累及多个器官系统,且可能致命。其与钆基磁共振造影剂的假定关联已引起关注。该疾病的独特临床特征包括前驱性全身炎症综合征,随后是慢性纤维化病程,该病程易累及四肢的皮肤和皮下组织。进行性全身纤维化过程累及多个器官,导致发病率和死亡率增加。采取适当的预防措施、及时识别并及时报告病例可能有助于更好地管理这种疾病。

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