Prospective study on the potential of RAAS blockade to halt renal disease in Alport syndrome patients with heterozygous mutations

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• 作者：Johanna Stock ; Johannes Kuenanz ; Niklas Glonke ; Joseph Sonntag…
• 关键词：Chronic kidney disease ; Renal insufficiency ; Fibrosis ; Collagen ; Alport syndrome ; Thin basement membrane disease ; Familial benign hematuria
• 刊名：Pediatric Nephrology
• 出版年：2017
• 出版时间：January 2017
• 年：2017
• 卷：32
• 期：1
• 页码：131-137
• 全文大小：
• 刊物类别：Medicine
• 刊物主题：Pediatrics; Nephrology; Urology;
• 出版者：Springer Berlin Heidelberg
• ISSN：1432-198X
• 卷排序：32

文摘

BackgroundPatients with autosomal or X-linked Alport syndrome (AS) with heterozygous mutations in type IV collagen genes have a 1–20 % risk of progressing to end-stage renal disease during their lifetime. We evaluated the long-term renal outcome of patients at risk of progressive disease (chronic kidney disease stages 1–4) with/without nephroprotective therapy.