Autosomal Dominant Polycystic Kidney Disease

Abstract

Over 50% of people affected with autosomal dominant polycystic kidney disease (ADPKD) will develop kidney failure, making ADPKD the 4th most common cause of end-stage kidney disease. ADPKD is a systemic condition affecting the kidneys, liver, heart, vasculature, and other organ systems. A minority of patients may have severe complications such as massive hepatomegaly from a polycystic liver or rupture of an intracranial aneurysm. Recent advances in the understanding of genetics, prognosis, and treatment of this condition have allowed delivery of personalized treatment capable of changing the natural history of the disease. This review focuses on diagnosis, determining risk of kidney failure, treatment, blood pressure management, and preimplantation genetic testing related to ADPKD.