What are the causes of Progressive Familial Intrahepatic Cholestasis?
Progressive familial intrahepatic cholestasis is caused by defects in several genes that produce proteins needed for bile formation and the “transportation” or flow of bile throughout the body. Bile is a liquid produced in the liver that removes toxins from the body and helps break down fat in food. The bile ducts are important because they carry bile out of the liver. A blockage of bile in the liver or bile ducts means that toxins in bile remain in the body, damaging liver cells and causing a dangerous buildup of waste in the blood stream. Decreased bile flow also prevents the body from being able to properly absorb fats and vitamins. Every person is born with many genes that make up the body and that are passed on to them (inherited) from their parents. Progressive familial intrahepatic cholestasis is an inherited condition and does not appear unless a person receives the same defective gene from both parents.
