Purpose: This study aimed to assess outcomes of liver transplantation (LTx) in patients with biliary atresia (BA). Methods: The Kasai procedure was performed for 358 patients at Tohoku University Hospital between January 1955 and December 2013; 64 (17.9 %) required LTx. These 64 patients 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 jaundice (n = 53), intractable cholangitis (n = 3), hepatopulmonary syndrome (n = 6), portopulmonary hypertension (n = 1), and intestinal bleeding (n = 1). Jaundice 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 BA.
- Biliary atresia
- Liver transplantation
- Long-term follow-up
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health