Modelling polycystic liver disease progression using age-adjusted liver volumes and targeted mutational analysis

•ADPKD-related PLD and isolated ADPLD may lead to equally severe hepatomegaly.•Age at first PLD-related hospitalization may serve as a novel clinically applicable endpoint to assess PLD severity.•Genetic confirmation is predictive for risk of hospitalization in both isolated and non-isolated PLD.•Age-adjusted liver volumetry may improve disease prognostication at early stages.