Cholestasis, Progressive Familial Intrahepatic 2

Overview

Type of disease: Rare conditions

Progressive familial intrahepatic cholestasis 2 is a rare condition and is one of many forms of cholestasis. Cholestasis is a rare disease where a person’s liver can not move the bile it makes to the small intestine. The liver, an organ, is responsible for producing bile. Bile is a compound that helps people digest fats. Once the bile has been made, it is supposed to go to the small intestine, another organ, to digest the fats there. However, in people with cholestasis, the bile can not move to the small intestine because there is either a physical block or because the bile is stuck in the liver cells. Symptoms of cholestasis are itchiness, jaundice (yellowing of the skin), pale stool, and dark urine. People with progressive familial intraheptic cholestasis 2 are not able to move the bile from the cells in the liver that produce it to the small intestine to digest fats. Talk with your doctor to find the best treatment for you if you have been diagnosed with progressive familial intraheptic cholestasis 2.

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