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

Pediatr Nephrol. 2021 Mar;36(3):711-719. doi: 10.1007/s00467-020-04819-6. Epub 2020 Nov 6.


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.

Trial registration: NCT00481130 NCT03019185 NCT02855268 NCT02378805 NCT01485978 NCT03019185 NCT02855268.

Keywords: Alport syndrome; Angiotensin-converting enzyme inhibition; Collagen IV; Hematuria; Microalbuminuria; Proteinuria; Treatment recommendations.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adolescent
  • Albuminuria
  • Child
  • Collagen Type IV / genetics
  • Humans
  • Nephritis, Hereditary* / diagnosis
  • Nephritis, Hereditary* / genetics
  • Nephritis, Hereditary* / therapy
  • Proteinuria
  • Young Adult


  • Collagen Type IV

Associated data