Long-term management of Alport syndrome in pediatric patients

Abstract

Clifford E Kashtan Department of Pediatrics, Division of Nephrology, University of Minnesota Medical School, Minneapolis, MN, USA Abstract: Alport syndrome, an important inherited cause of end-stage renal disease, has long been considered an untreatable disorder. That view is changing as a result of treatment studies in experimental animals and human populations. This review describes current management of Alport kidney disease, which is based on early diagnosis and institution of therapy with antagonists of the renin-angiotensin-aldosterone system. Keywords: Alport syndrome, glomerular basement membrane, type IV collagen, familial hematuria