An international team of researchers studying the effects of progressive familial intrahepatic cholestasis (PFIC) in children have found that half of the children had received a liver transplant by age 10, reports Medical Xpress. They also found that surgical biliary diversion can be an effective treatment for those with PFIC2.
PFIC is a very rare genetic condition that prevents bile from draining from the liver. It tends to become apparent soon after birth (around 6 months), but there are exceptions where children can develop their first symptoms several years later. The disease is often associated with significant liver scarring and liver failure by the age of 10. Symptoms of the condition may include an enlarged liver and/or spleen, fatigue, nausea, vomiting, poor weight gain and growth, intense itching caused by bile salt build-up, and jaundice. PFIC can also result in complications, such as liver failure, liver cancer, gallstones, and difficulty absorbing certain nutrients. The condition is usually treated through a combination of drugs and surgery. Medicines may be used to replace nutrients and reduce itching, and a surgical procedure called a partial external biliary diversion, which uses a short segment of the intestine as a passage for bile, may be used to remove bile from the liver in patients with little liver scarring. A liver transplant is also a possible treatment for patients who have more severe liver scarring.
