Early angiotensin-converting enzyme inhibition in Alport syndrome delays renal failure and improves life expectancy
Early angiotensin-converting enzyme inhibition in Alport syndrome delays renal failure and improves life expectancy
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DOI：
10.1038/ki.2011.407
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发表时间：
2012-03-01
影响因子：
19.6
通讯作者：
Weber, Manfred
中科院分区：
文献类型：
--
作者：
Gross, Oliver;Licht, Christoph;Weber, Manfred
Alport综合征不可避免地导致终末期肾病，并且没有已知的治疗方法可以改善结局。在这里，我们确定了血管紧张素转换酶抑制剂是否可以延迟透析时间和改善Alport家族三代人的预期寿命。患者按治疗开始时的肾功能分类，包括33例血尿或微量白蛋白尿，115例蛋白尿，26例肾功能受损，109例未治疗的亲属。对患者进行了平均持续时间超过20年的随访。未接受治疗的亲属开始透析的中位年龄为22岁。肾功能受损患者的治疗显著延迟了透析的中位年龄25岁，而蛋白尿患者的治疗延迟了透析的中位年龄40岁。值得注意的是，迄今为止，没有血尿或微量白蛋白尿患者进展为肾衰竭。兄弟姐妹对证实了这些结果，表明较年轻患者的早期治疗与较年长的兄弟姐妹的晚期治疗或无治疗相比，显著延迟了13年的透析。治疗显著提高了预期寿命，超过了未治疗队列的中位年龄55岁。因此，Alport综合征可通过血管紧张素转换酶抑制剂治疗以延迟肾衰竭，治疗以时间依赖性方式改善预期寿命。这支持了对无症状患者进行早期诊断和早期肾保护治疗的必要性。Kidney International（2012）81，494-501; doi：10.1038/ki.2011.407; 2011年12月14日在线发表
Alport syndrome inevitably leads to end-stage renal disease and there are no therapies known to improve outcome. Here we determined whether angiotensin-converting enzyme inhibitors can delay time to dialysis and improve life expectancy in three generations of Alport families. Patients were categorized by renal function at the initiation of therapy and included 33 with hematuria or microalbuminuria, 115 with proteinuria, 26 with impaired renal function, and 109 untreated relatives. Patients were followed for a period whose mean duration exceeded two decades. Untreated relatives started dialysis at a median age of 22 years. Treatment of those with impaired renal function significantly delayed dialysis to a median age of 25, while treatment of those with proteinuria delayed dialysis to a median age of 40. Significantly, no patient with hematuria or microalbuminuria advanced to renal failure so far. Sibling pairs confirmed these results, showing that earlier therapy in younger patients significantly delayed dialysis by 13 years compared to later or no therapy in older siblings. Therapy significantly improved life expectancy beyond the median age of 55 years of the no-treatment cohort. Thus, Alport syndrome is treatable with angiotensin-converting enzyme inhibition to delay renal failure and therapy improves life expectancy in a time-dependent manner. This supports the need for early diagnosis and early nephroprotective therapy in oligosymptomatic patients. Kidney International (2012) 81, 494-501; doi:10.1038/ki.2011.407; published online 14 December 2011