Management of paucity of interlobular bile ducts

Two types of paucity of interlobular bile ducts (PIBD) may be observed in children with chronic cholestasis. The syndromatic type (80 cases) is the most frequent. The non-syndromatic type (32 cases) is the most severe and half of the patients in this group died from liver failure in the first years of life. The main therapeutic point is that patients with PIBD should not be operated on. The only treatment available is symptomatic medical treatment: the main helpful possibilities concern feeding of medium-chain triglycerides, large supplementation of liposoluble vitamins (A, D, E and K) given intramuscularly, phenobarbital and cholestyramine when there is severe pruritus. The place of the liver transplantation will be clarified in the near future.