Polycystic kidney disease: inheritance, pathophysiology, prognosis, and treatment

Both autosomal dominant and recessive polycystic kidney disease are conditions with severe associated morbidity and mortality. Recent advances in the understanding of the genetic and molecular pathogenesis of both ADPKD and ARPKD have resulted in new, targeted therapies designed to disrupt cell sign...

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Bibliographic Details
Published in:International journal of nephrology and renovascular disease 2010-01, Vol.3 (default), p.69-83
Main Authors: Halvorson, Christian R, Bremmer, Matthew S, Jacobs, Stephen C
Format: Article
Language:English
Subjects:
Cellular signal transduction
Chronic kidney failure
Development and progression
Genetic aspects
Hypertension
Kidney diseases
Kidney failure
Morbidity
Mortality
Pathogenesis
Polycystic kidney disease
Prognosis
Review
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Summary:Both autosomal dominant and recessive polycystic kidney disease are conditions with severe associated morbidity and mortality. Recent advances in the understanding of the genetic and molecular pathogenesis of both ADPKD and ARPKD have resulted in new, targeted therapies designed to disrupt cell signaling pathways responsible for the abnormal cell proliferation, dedifferentiation, apoptosis, and fluid secretion characteristic of the disease. Herein we review the current understanding of the pathophysiology of these conditions, as well as the current treatments derived from our understanding of the mechanisms of these diseases.
ISSN:1178-7058
1178-7058
DOI:10.2147/IJNRD.S6939