一例原发疾病不明的女性异体肾移植受者发生抗肾小球基底膜新月形肾小球肾炎:Alport病的回顾性分子证实

M. Vankalakunti, A. Parekh, A. K. Thimmegowda, Rajanna Sreedhara
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引用次数: 0

摘要

抗肾小球基底膜(抗gbm)新月形肾小球肾炎在同种异体移植中发展是一种罕见的现象。由于缺乏正常的胶原网络COL4α3、COL4α4和COL4α5三聚体,接受肾移植的Alport综合征患者有发生抗gbm肾小球肾炎的风险。这类患者肾移植计划面临的两个独特挑战包括:理想的供体选择;患抗gbm肾炎的风险我们报告一例肾移植后抗gbm新月形肾小球肾炎,患者为女性,原发肾脏病不明,肾移植后近2年出现血尿和蛋白尿,诊断为Alport病。在我们的病例中,同种异体移植的结果是不利的,患者在诊断的6个月内达到了终末期肾病。患者仍在进行连续的动态腹膜透析,目前活跃在已故供体移植等待名单上。
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A Case of Anti-Glomerular Basement Membrane Crescentic Glomerulonephritis in a Female Renal Allograft Recipient with Unknown Native Disease: Retrospective Molecular Confirmation of Alport Disease
Anti-Glomerular Basement Membrane (anti-GBM) crescentic glomerulonephritis developing in an allograft is a rare phenomenon. A patient with Alport syndrome receiving a renal transplant is at risk of developing anti-GBM glomerulonephritis, due to the absence of normal COL4α3, COL4α4 and COL4α5 trimer of the collagen network. Two unique challenges with planning kidney transplant in such a patient include- ideal donor selection; and risk of developing anti-GBM nephritis. We report a case of post-transplant anti-GBM crescentic glomerulonephritis in a female recipient with unknown native kidney disease who was diagnosed with Alport disease when she presented with hematuria and proteinuria nearly 2 years postrenal transplant. Allograft outcome in our case was unfavourable, patient reaching end-stage kidney disease within 6-month of diagnosis. The patient remains on continuous ambulatory peritoneal dialysis and currently active on the deceased donor transplant waiting list.
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