One Minute Education
Platelet count is the best predictor of the severity of portal hypertension, which has early onset but is underdiagnosed in patients with ARPKD. Seventy percent of patients with ARPKD have biliary abnormalities. Kidney and liver disease are independent, and variability in severity is not explainable by type of PKHD1 mutation.
For more information:
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4162098/
