LA MALADIE DE BYLER

It is also called familial fibrogenic cholestasis, it is a rare intrahepatic cholestasis. It is the second cause of familial cholestasis after the ductular paucity. It occurs in both male and female subjects. Its early manifestations appear at the first year of life. We reported here a case of two patients one and four years old. They were admited to hospital in a cholestasic icteric state with hepatomegaly. The diagnosis was established by familial character, absence of cholestero-lipidic retention, negativity of other biological examinations to detect other causes mainly metabolic or viral and specially the characteristic course of the disease with the presence of an icteric phase intermittent by a remission state.