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Autosomal dominant polycystic kidney disease (ADPKD) is caused by loss-of-function or deleterious mutations in the PKD1 or PKD2 genes and is seen with a prevalence of 1: 400 to 1: 1000. 1 Patients develop kidney and liver cysts that accumulate and expand to crowd out the normal organ parenchyma and lead to kidney failure in half of patients by age 60 years. 2 To find targeted therapies, we need to better understand the function of the PKD1 and PKD2 gene products, polycystin-1 (PC1) and polycystin-2 (PC2). Autosomal dominant polycystic liver disease (ADPLD), also known as isolated polycystic liver disease (PCLD), is a clinical characterization of patients on the same phenotypic and mechanistic spectrum as ADPKD with regard to their liver cysts, but lacking clinically relevant kidney cysts. 3 Autopsy studies suggest that PCLD has a similar prevalence to that of ADPKD. 4 Approximately 50% of cases of this …