Clinical practice recommendations for the diagnosis and management of Alport syndrome in children, adolescents, and young adults-an update for 2020

Clinical practice recommendations for the diagnosis and management of Alport syndrome in children, adolescents, and young adults-an update for 2020
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DOI:
10.1007/s00467-020-04819-6
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发表时间:
2020-11-06
影响因子:
3
通讯作者:
Gross, Oliver
Gross, Oliver
中科院分区:
医学3区
文献类型:
--
作者:
Kashtan, Clifford E.;Gross, Oliver

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2013年,我们在本杂志上发表了一组治疗Alport综合征的临床实践建议。我们建议推迟血管紧张素转换酶抑制的开始,直到出现显性蛋白尿或在某些情况下出现微量白蛋白尿。过去7年发生的事态发展促使我们修订这些建议。我们现在建议在诊断为X连锁Alport综合征的男性和常染色体隐性遗传Alport综合征的男性和女性患者中开始治疗。我们进一步建议在X连锁Alport综合征女性患者和常染色体显性遗传Alport综合征男性和女性患者出现微量白蛋白尿时开始治疗。本文介绍了这些修订的基本原理以及旨在确保Alport综合征早期诊断的诊断策略的建议。
In 2013, we published a set of clinical practice recommendations for the treatment of Alport syndrome in this journal. We recommended delaying the initiation of angiotensin-converting enzyme inhibition until the onset of overt proteinuria or, in some cases, microalbuminuria. Developments that have occurred over the past 7 years have prompted us to revise these recommendations. We now recommend the initiation of treatment at the time of diagnosis in males with X-linked Alport syndrome and in males and females with autosomal recessive Alport syndrome. We further recommend starting treatment at the onset of microalbuminuria in females with X-linked Alport syndrome and in males and females with autosomal dominant Alport syndrome. This article presents the rationale for these revisions as well as recommendations for diagnostic tactics intended to ensure the early diagnosis of Alport syndrome.