Autosomal dominant polycystic kidney disease (ADPKD) has an incidence of 1/1000 and accounts for about 5% of patients with end-stage renal disease (ESRD) requiring renal replacement therapy. Clinical manifestations are rare before adulthood, but penetrance is essentially complete; all patients ≥ 80 years have some signs.

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ADPKD is inherited in an autosomal dominant manner. About 95% of individuals with ADPKD have an affected parent, but at least 10% of families can be traced to a <i>de novo</i> pathogenic variant. Each child of an affected individual has a 50% chance of inheriting the pathogenic variant. Once the pat …

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