Kim Soyeon, Kwon Soon Hyo
Division of Nephrology, Department of Internal Medicine, Soonchunhyang University Seoul Hospital, Soonchunhyang University College of Medicine, Seoul, Republic of Korea.
Kidney Res Clin Pract. 2025 Jul;44(4):583-587. doi: 10.23876/j.krcp.24.143. Epub 2024 Nov 12.
Kidney transplantation is recognized as an effective treatment for end-stage renal disease in Alport syndrome, demonstrating outcomes comparable to or even superior to those in other causes of renal failure. When considering living related donor kidney transplantation for Alport syndrome patients, it is crucial to consider genetic factors during the donor selection process. In addition to a comprehensive health check, genetic testing is strongly recommended for potential donors at risk of carrying mutations in COL4A3- COL4A5 before undergoing kidney transplantation. Individuals carrying these mutations face an inherent risk of kidney disease and due to the possibility of further deterioration in renal function after nephrectomy for transplantation, they are not suitable as priority donors. Posttransplant anti-glomerular basement membrane nephritis is rare but can lead to graft loss, especially in males with X-linked Alport syndrome.
肾移植被认为是治疗Alport综合征终末期肾病的有效方法，其治疗效果与其他肾衰竭病因相当，甚至更佳。在考虑为Alport综合征患者进行亲属活体供肾移植时，供体选择过程中考虑遗传因素至关重要。除全面的健康检查外，强烈建议对有携带COL4A3 - COL4A5突变风险的潜在供体在肾移植前进行基因检测。携带这些突变的个体面临患肾病的固有风险，并且由于移植肾切除术后肾功能可能进一步恶化，他们不适合作为优先供体。移植后抗肾小球基底膜肾炎虽罕见，但可导致移植肾丢失，尤其在患有X连锁Alport综合征的男性中。
