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肾移植治疗肾源性系统性纤维化:病例报告及文献复习。

Renal transplantation for nephrogenic systemic fibrosis: a case report and review of the literature.

机构信息

Department of Renal Unit of Internal Medicine, Hachioji Medical Center of Tokyo Medical University, 1163 Tate-machi, Hachioji-shi, Tokyo 193-0998, Japan.

出版信息

Nephrol Dial Transplant. 2011 Mar;26(3):1099-101. doi: 10.1093/ndt/gfq693. Epub 2010 Nov 15.

DOI:10.1093/ndt/gfq693
PMID:21079195
Abstract

Nephrogenic systemic fibrosis (NSF) is a rare fibrosing disorder described among patients with renal disease. Currently, no standard therapy exists, although therapeutic modalities have included plasmapheresis, extracorporeal photopheresis, sodium thiosulphate, imatinib and renal transplantation. We describe a patient with NSF who was physically debilitated and underwent renal transplantation. After transplantation, the patient's lesions improved clinically, and the patient was ambulatory. Despite developing worsening renal function, her lesions remained unchanged. We conclude that renal transplantation improves symptoms of NSF, and believe that in patients with NSF, careful consideration should be made for early renal transplantation.

摘要

肾源性系统性纤维化(NSF)是一种罕见的纤维性疾病,发生于患有肾脏疾病的患者中。目前尚无标准的治疗方法,尽管已经尝试了包括血浆置换、体外光化学疗法、硫代硫酸钠、伊马替尼和肾移植在内的治疗方法。我们描述了一位患有 NSF 的患者,他身体虚弱并接受了肾移植。移植后,患者的病变在临床上得到了改善,能够行走。尽管肾功能恶化,但她的病变仍未改变。我们得出结论,肾移植可改善 NSF 的症状,并且我们认为对于患有 NSF 的患者,应仔细考虑尽早进行肾移植。

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