Can progression of autosomal dominant or autosomal recessive polycystic kidney disease be prevented? Semin Nephrol 2001 Sep;21(5):430-40
Date
09/18/2001Pubmed ID
11559884DOI
10.1053/snep.2001.24937Scopus ID
2-s2.0-0034844158 (requires institutional sign-in at Scopus site) 31 CitationsAbstract
Data from animal and human studies suggest that the rate of progression of renal insufficiency can be retarded with careful control of blood pressure, institution of a low-protein diet, and the use of lipid-lowering agents. These therapeutic interventions become important when managing patients with renal insufficiency secondary to autosomal dominant polycystic kidney disease (PKD) and autosomal recessive polycystic kidney disease, in which end-stage renal disease is present in nearly 17,000 individuals per year. Several dietary and pharmacologic intervention strategies including blood pressure control, dietary modification, and the use of antioxidants as well as lipid-lowering agents have been studied in humans and animals with PKD in an effort to slow the rate of renal progression. This article reviews the current understanding of the effectiveness of these conventional therapies, as well as novel therapies that specifically target the mediators of cyst formation in PKD using tyrosine kinase inhibitors and gene therapy in an effort to identify potential strategies for retarding cyst formation and parenchymal injury in PKD. Current pharmacologic and dietary strategies fail to show any consistent benefits in preserving renal function and reducing renal injury in human PKD. The therapeutic potential for exciting new gene therapies and pharmacologic agents designed to target the pathophysiologic pathways involved in cyst formation are promising. Randomized, controlled trials in children and adults with early PKD are necessary to evaluate the effectiveness of these therapeutic interventions.
Author List
Davis ID, MacRae Dell K, Sweeney WE, Avner EDAuthor
Ellis D. Avner MD Professor in the Pediatrics department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
AnimalsAntihypertensive Agents
Antioxidants
Dietary Proteins
Disease Progression
ErbB Receptors
Genetic Therapy
Humans
Hypolipidemic Agents
Kidney Failure, Chronic
Polycystic Kidney, Autosomal Dominant
Polycystic Kidney, Autosomal Recessive
