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01-01-2017 | Review

The spectrum of autosomal dominant polycystic kidney disease in children and adolescents

Authors: Bharathi V. Reddy, Arlene B. Chapman

Published in: Pediatric Nephrology | Issue 1/2017

Abstract

Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary renal disorder. It is characterized by the development of renal cysts and kidney enlargement and ultimately leads to renal failure typically in the sixth decade of life. Although most patients are asymptomatic until well into adulthood, renal cysts develop much earlier, often in utero. Significant renal anatomic and cystic expansion typically occurs before clinical manifestations in children and young adults with AKPKD. The cyst burden detected by imaging represents the minority of cyst burden, and renal and cardiovascular abnormalities are the most common manifestations in children with ADPKD. Here we review the molecular pathogenesis of ADPKD, discuss the screening, diagnosis and clinical manifestations of this renal disorder in childhood and adolescents and review treatment options and potential therapies currently being tested.

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Title: The spectrum of autosomal dominant polycystic kidney disease in children and adolescents
Authors: Bharathi V. Reddy
Arlene B. Chapman
Publication date: 01-01-2017
Publisher: Springer Berlin Heidelberg
Published in: Pediatric Nephrology / Issue 1/2017
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI: https://doi.org/10.1007/s00467-016-3364-y

Keynote webinar | Spotlight on medication adherence

Springer Medicine

The spectrum of autosomal dominant polycystic kidney disease in children and adolescents

Abstract

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

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