BACKGROUND: We assessed the long-term outcomes of @entity1 with @entity2876 with @entity600 (BASM). METHODS: We retrospectively assessed outcomes of 255 @entity1 who underwent the Kasai procedure (KP) at our hospital between 1972 and 2014. Clinical outcomes of 11 @entity1 with BASM (group A: nine with @entity4032 , two with @entity4032 ) and 244 @entity1 with isolated BA (group B) were compared. RESULTS: The incidence of @entity255 and @entity7185 ( @entity7185 ) was significantly higher in group A than in group B. Of the 11 group A @entity1 , three died of severe @entity299 during early infancy. Seven became @entity1087 free following KP, with three @entity1 subsequently requiring liver transplantation (LTx). Four survived with their native livers for 2, 5, 22, and 23years, respectively. Overall 20-year survival rates were 63.6% and 66.5% and 20-year native liver survival rates were 29.0% and 47.3% in groups A and B, respectively. No significant difference in cumulative survival rates was observed between both groups. CONCLUSIONS: Long-term outcomes in BASM @entity1 without lethal @entity299 were comparable to @entity1 with isolated BA. Careful follow-up may be required in @entity1 with BASM because of a potentially higher risk of secondary complications such as @entity7185 .

Long-term outcomes of @entity2876 with XXXX .

@entity1 :: ('9606', 'Species') :: ['patients'] @entity1087 :: ('MESH:D007565', 'Disease') :: ['jaundice'] @entity7185 :: ('MESH:D020065', 'Disease') :: ['hepatopulmonary syndrome', 'HPS'] @entity299 :: ('MESH:D006331', 'Disease') :: ['cardiac defects'] @entity600 :: ('MESH:D000014', 'Disease') :: ['splenic malformation'] @entity2876 :: ('MESH:D001656', 'Disease') :: ['biliary atresia'] @entity4032 :: ('MESH:D059446', 'Disease') :: ['asplenia', 'polysplenia'] @entity255 :: ('MESH:D002761', 'Disease') :: ['early cholangitis']
@entity600 :: (MESH:D000014,Disease) :: ['splenic malformation']
