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肾源性系统性纤维化

Nephrogenic systemic fibrosis.

作者信息

Vosshenrich R, Reimer P

机构信息

Radiologen-Gemeinschaftspraxis, MRT im Friederikenstift, Hannover, Germany.

出版信息

Vasa. 2009 Feb;38(1):31-8. doi: 10.1024/0301-1526.38.1.31.

Abstract

Nephrogenic systemic fibrosis (NSF) is a relative new disease affecting predominantly patients with severely impaired kidney function. Strict adherence to a concordant clinical and pathological definition has allowed for careful separation of this entity from other fibrosing disorders, leading eventually to the realization that gadolinium based contrast agents were closely associated with its onset. Gadodiamide and similarly unstable Gd-CA should therefore not be used in patients with renal insufficiency. The clinical symptoms of NSF vary considerably from patient to patient. NSF should be suspected in renal failure patients presenting any possible NSF symptom appearing in the weeks after Gd-enhanced MRI. Suspected cases should have a skin biopsy taken from an affected region for the histological confirmation or rejection of the diagnosis. There are no established curative treatments to NSF patients. Prevention is the only alternative. Guidelines for the daily routine seem to be the right way. When NSF is identified the primary goal is reversal of underlying renal failure or renal transplantation. In addition, use of physical therapy and pain control is mandatory. One of the best news in late 2007 is the decreasing rate of newly detected NSF cases. In the case this development continues, NSF may become an historical disease in the near future.

摘要

肾源性系统性纤维化(NSF)是一种相对较新的疾病,主要影响肾功能严重受损的患者。严格遵循一致的临床和病理定义有助于将该疾病与其他纤维化疾病仔细区分开来,最终认识到钆基造影剂与其发病密切相关。因此,钆双胺和类似不稳定的钆造影剂不应在肾功能不全患者中使用。NSF的临床症状在患者之间差异很大。对于在钆增强MRI后数周出现任何可能的NSF症状的肾衰竭患者,应怀疑患有NSF。疑似病例应从受影响区域进行皮肤活检,以进行组织学确诊或排除诊断。目前尚无针对NSF患者的确立的治愈性治疗方法。预防是唯一的选择。日常指南似乎是正确的方法。当确诊为NSF时,首要目标是逆转潜在的肾衰竭或进行肾移植。此外,物理治疗和疼痛控制的使用是必不可少的。2007年末的一个好消息是新检测到的NSF病例的发生率在下降。如果这种趋势持续下去,NSF可能在不久的将来成为一种历史疾病。

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