{"title":"Renal transplantation in Alport syndrome.","authors":"Soyeon Kim, Soon Hyo Kwon","doi":"10.23876/j.krcp.24.143","DOIUrl":null,"url":null,"abstract":"<p><p>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.</p>","PeriodicalId":17716,"journal":{"name":"Kidney Research and Clinical Practice","volume":" ","pages":""},"PeriodicalIF":2.9000,"publicationDate":"2024-11-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Kidney Research and Clinical Practice","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.23876/j.krcp.24.143","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"UROLOGY & NEPHROLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
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.
肾移植是公认的治疗阿尔波特综合征终末期肾病的有效方法，其疗效可与其他原因导致的肾衰竭相媲美，甚至更胜一筹。在考虑为阿尔波特综合征患者进行亲属活体肾移植时，选择供体过程中考虑遗传因素至关重要。除了全面的健康检查外，强烈建议有携带 COL4A3-COL4A5 基因突变风险的潜在捐献者在接受肾移植前进行基因检测。携带这些基因突变的个体面临肾脏疾病的固有风险，由于肾切除术后肾功能可能进一步恶化，因此他们不适合作为优先捐献者。移植后抗肾小球基底膜肾炎虽然罕见，但可导致移植物丧失，尤其是在患有 X 连锁阿尔波特综合征的男性患者中。
期刊介绍：
Kidney Research and Clinical Practice (formerly The Korean Journal of Nephrology; ISSN 1975-9460, launched in 1982), the official journal of the Korean Society of Nephrology, is an international, peer-reviewed journal published in English. Its ISO abbreviation is Kidney Res Clin Pract. To provide an efficient venue for dissemination of knowledge and discussion of topics related to basic renal science and clinical practice, the journal offers open access (free submission and free access) and considers articles on all aspects of clinical nephrology and hypertension as well as related molecular genetics, anatomy, pathology, physiology, pharmacology, and immunology. In particular, the journal focuses on translational renal research that helps bridging laboratory discovery with the diagnosis and treatment of human kidney disease. Topics covered include basic science with possible clinical applicability and papers on the pathophysiological basis of disease processes of the kidney. Original researches from areas of intervention nephrology or dialysis access are also welcomed. Major article types considered for publication include original research and reviews on current topics of interest. Accepted manuscripts are granted free online open-access immediately after publication, which permits its users to read, download, copy, distribute, print, search, or link to the full texts of its articles to facilitate access to a broad readership. Circulation number of print copies is 1,600.
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