Treatment and Management of Autosomal Dominant Polycystic Kidney Disease
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ABSTRACT
Autosomal dominant polycystic kidney disease (ADPKD) is the most common life-threatening single-gene disease. It affects up to 15 million people worldwide with 50% risk for end-stage kidney disease, 80% risk for hypertension, 60% risk for painful kidney complications, 20% risk for symptomatic polycystic liver disease and 3% risk for intra-cerebral aneurysm rupture. For a long time, the treatment and management strategies of this disease have not progressed in comparison with the treatment of other kidney diseases. Recently, there have been new therapeutic hopes with identification of specific drugs based on the mechanisms of kidney progression. This chapter reviews the treatment and management of ADPKD progression, and the identification of ADPKD patients with rapidly progressing disease, hypertension, and extrarenal complications.
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