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薄基底膜肾病与肾移植

Thin basement membrane nephropathy and renal transplantation.

作者信息

Ierino Francesco L, Kanellis John

机构信息

Department of Nephrology, Austin Health, Heidelberg, Victoria, Australia.

出版信息

Semin Nephrol. 2005 May;25(3):184-7. doi: 10.1016/j.semnephrol.2005.01.012.

Abstract

The clinical implications of thin basement membrane nephropathy (TBMN) in renal transplantation must be considered from the perspectives of both the allograft recipient and the donor. Most individuals with TBMN have a benign course, but some develop end-stage renal failure (ESRF) and undergo transplantation. ESRF in patients with TBMN often results from the presence of additional glomerular or interstitial lesions and some of these, such as immunoglobulin (Ig)A disease, may recur in the renal allograft and affect outcome. In addition, individuals with TBMN always must be distinguished from those with glomerular membrane thinning due to Alport syndrome. This is not only to enable appropriate genetic counseling but also to anticipate the possible complication of posttransplant anti-glomerular basement membrane disease. From the perspective of the live renal donor, donation from individuals with TBMN (or carriers of X-linked Alport syndrome with thinned membranes) remains controversial because the risks remain unknown. Any effects of the thinned membranes themselves on allograft function are unclear. Further advances in our understanding of the clinical, pathologic, and molecular features of TBMN should result in improved assessment of potential live donors and help stratify those at risk for renal impairment.

摘要

必须从同种异体肾移植受者和供者两个角度来考虑薄基底膜肾病(TBMN)在肾移植中的临床意义。大多数TBMN患者病情呈良性,但部分患者会发展为终末期肾衰竭(ESRF)并接受肾移植。TBMN患者发生ESRF往往是由于存在其他肾小球或间质病变,其中一些病变,如免疫球蛋白(Ig)A病,可能会在移植肾中复发并影响移植效果。此外,TBMN患者始终必须与因Alport综合征导致肾小球基底膜变薄的患者相鉴别。这不仅是为了进行适当的遗传咨询,也是为了预测移植后抗肾小球基底膜病可能出现的并发症。从活体肾供者的角度来看,TBMN患者(或X连锁Alport综合征且基底膜变薄的携带者)进行捐赠仍存在争议,因为风险尚不清楚。基底膜变薄本身对移植肾功能的任何影响尚不明确。我们对TBMN临床、病理和分子特征认识的进一步进展,应能改善对潜在活体供者的评估,并有助于对有肾功能损害风险的人群进行分层。

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