Epidemiology of autosomal dominant polycystic liver disease in Olmsted county
2020
Abstract Background Isolated Autosomal Dominant Polycystic Liver Disease (ADPLD) is generally considered a rare disease. However, the frequency of truncating mutations to ADPLD genes in large, population sequencing databases is 1:496. With the increasing use of abdominal imaging, incidental detection of hepatic cysts and ADPLD has become more frequent. The present study was performed to ascertain the incidence and point prevalence of ADPLD in Olmsted County, Minnesota and how these are impacted by the increasing utilization of abdominal imaging. Research Design and Methods The Rochester Epidemiology Project and Radiology databases of Mayo Clinic and Olmsted Medical Center were searched to identify all subjects meeting diagnostic criteria for definite, likely or possible ADPLD. Annual incidence rates were calculated using incident cases during 1980-2016 as numerator and age- and sex-specific estimates of the population of Olmsted County as denominator. Point prevalence was calculated using prevalence cases as numerator and age- and sex-specific estimates of the population of Olmsted County on January 1, 2010, as denominator. Results Incidence rate and point prevalence of combined definite and likely ADPLD were 1.01 per 100,000 person-years and 9.5 per 100,000 population, respectively. Only 15 of 35 definite and likely incident ADPLD cases had received a diagnostic code and only 8 had clinically significant hepatomegaly. Incident rates were much higher when adding possible cases, mainly identified through Radiology databases, particularly in recent years and in older patients due to the increased utilization of imaging studies. Conclusion Clinically significant isolated ADPLD is a rare disease with a prevalence
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