In a study designed to better understand the complications of autosomal recessive polycystic kidney disease, researchers at the NIH analyzed clinical, molecular and imaging data from 73 patients (ages 1-56 years old, average 12.7) with mutations in PKHD1 and kidney and liver involvement. The findings identified platelet count as the best predictor of the severity of portal hypertension, which has early onset but is underdiagnosed in patients with autosomal recessive polycystic kidney disease