Liver transplantation following the Kasai procedure in treatment of XXXX : a single institution analysis.

@entity1 @entity1087 @entity101 @entity548 @entity7185 @entity2876 @entity255
PURPOSE: This study aimed to assess outcomes of liver transplantation (LTx) in @entity1 with @entity2876 ( @entity2876 ). METHODS: The Kasai procedure was performed for 358 @entity1 at Tohoku University Hospital between January 1955 and December 2013; 64 (17.9%) required LTx. These 64 @entity1 were divided into 4 groups according to their age at the time of transplantation: Group 1, aged <2 years (n = 27); Group 2, aged 2-9 years (n = 16); Group 3, aged 10-19 years (n = 11); and Group 4, aged >= 20 years (n = 10). Clinical parameters were evaluated retrospectively. RESULTS: Both living-donor (n = 57) and deceased-donor (n = 7) LTx were performed. Indications were irreversible @entity1087 (n = 53), intractable @entity255 (n = 3), @entity7185 (n = 6), portopulmonary @entity101 (n = 1), and intestinal @entity548 (n = 1). @entity1087 occurred more frequently in Groups 1 and 2 than in Groups 3 and 4 (p = 0.031). Survival rates were 81.5, 100, 90.9, and 80% in Groups 1, 2, 3, and 4, respectively. CONCLUSION: Although the overall LTx survival rate was satisfactory, some adult recipients experienced LTx-related difficulty. Close follow-up, meticulous assessment of physical and social conditions, presence of a multidisciplinary support system, and appropriate time course for LTx are all essential factors in the treatment of @entity2876 .
