OBJECTIVES: The development of intrahepatic @entity1125 (IBC) after Kasai operation in @entity1 with @entity2876 ( @entity2876 ) is recognized as an important problem; however, management strategy for IBC has not been clarified, particularly in the light of the increased use of liver transplantation. METHODS: Forty consecutive @entity2876 @entity1 underwent hepatic portoenterostomy during 18 years from 1983 to 2000. We compared the clinical course and prognosis of the @entity1 who developed IBC with those who did not. RESULTS: Seven of the 40 @entity1 developed IBC. Three @entity1 had type A (non-communicating cyst) and three @entity1 had type C ( @entity384 ) IBC, and the remaining @entity1 had type B (communicating cyst). Of the 7 @entity1 , one @entity1 underwent successful internal intestinal drainage, and one @entity1 died of complications at the time of internal intestinal drainage. Three @entity1 underwent liver transplantation due to either @entity7185 (one case) or @entity1578 (two cases). One @entity1 with IBC with @entity1578 was judged to require transplant, but was found to have @entity120 and was thus not a candidate. The remaining @entity1 has survived without @entity1087 for 21 months postoperatively. Two of 21 @entity1 with good initial bile drainage and without IBC underwent liver transplantation. The percentage of @entity1 undergoing transplant was significantly higher in the group with IBC than in the group without IBC (P < 0.05). CONCLUSIONS: IBC was associated with worsening liver function. Previously, IBC was treated using internal/external drainage, or the @entity1 were observed without treatment, with limited success. We now consider it reasonable to carry out liver transplantation in @entity1 with long-standing IBC.

Intrahepatic @entity1125 in XXXX in the era of liver transplantation.

@entity1 :: ('9606', 'Species') :: ['patients', 'patient'] @entity1087 :: ('MESH:D007565', 'Disease') :: ['jaundice'] @entity384 :: ('MESH:D002311', 'Disease') :: ['multiple cystic dilation'] @entity7185 :: ('MESH:D020065', 'Disease') :: ['hepato-pulmonary syndrome'] @entity120 :: ('MESH:D006976', 'Disease') :: ['pulmonary hypertension'] @entity1578 :: ('MESH:D017093', 'Disease') :: ['liver failure'] @entity2876 :: ('MESH:D001656', 'Disease') :: ['BA', 'biliary atresia'] @entity1125 :: ('MESH:D001661', 'Disease') :: ['biliary cysts']
@entity2876 :: (MESH:D001656,Disease) :: ['biliary atresia']
