Autosomal dominant polycystic kidney disease (ADPKD) is the third most common systemic hereditary disease. Only hypercholesterolemia and dominant otosclerosis affect a larger population of patients. Its prevalence is estimated to be between 1 in 400 and 1 in 1000. ADPKD accounts for 10% to 15% of end-stage renal disease cases requiring dialysis. The severity of ADPKD is variable and ranges from asymptomatic to renal failure. This variability in expression and disease is evidence that this disease has multiple interrelated genetic components. Over 50% of the patients never progress to ESRD or transplantation. Further, the sequelae of ADPKD are not limited to the kidney. We discuss a case presenting with subarachnoid hemorrhage and also the most common clinical manifestations of ADPKD and their diagnosis and treatment, with specific attention given to intracranial aneurysms in ADPKD.
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