Congenital hepatic fibrosis (CHF) is a rare genetic disease that causes malformation and fibrosis (scarring) of the liver. Occurring in roughly one out of every 20,000 births, CHF can lead to an enlarged liver, impaired blood flow to the intestines (portal hypertension), infection of the bile ducts and liver failure. In severe cases, a liver transplant is usually needed to treat the disease. In a study published in the Journal of Hepatology, researchers centered at Tokyo Medical and Dental University (TMDU) describe a new experimental model of CHF, which allowed them to uncover molecular players that may contribute to the illness.
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