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Progressive Familial Intrahepatic Cholestasis

Progressive Familial Intrahepatic Cholestasis (PFIC) or Byler disease is a disorder that can cause liver disease and, subsequently, liver failure. In children with Byler disease, cells in the liver cannot release bile, a fluid that helps the body digest food. Without enough of this fluid present, the body can’t break down and absorb fats. The unreleased bile accumulates in the liver cells known as cholestasis. Cholestasis can damage the liver. The condition typically develops before adulthood, and it can progress very rapidly over a few months. PFIC is a rare disorder which affects around 1 in 50,000 – 1 in 100,000 births and it affects males and females equally [1-3].


Sophia Roberts*

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