RESUMEN
To analyze the clinical characteristics and the outcomes of living donor liver transplantation in children with Alagille syndrome (AGS). Clinical data of children with AGS who underwent liver transplantation between July 2009 and May 2019 in our unit were retrospectively analyzed. Primary end-points were patient and graft survival. Ten children with AGS underwent living donor liver transplantation at a median age of 28 months (range, 12-84 months). Jaundice was the most common initial symptom and was noted after a median duration of 20 days after birth (range, 7-60 days). Two patients had undergone Kasai porto-enterostomy for misdiagnosis of biliary atresia. The most common indication for transplantation was severe pruritus with poor quality of life. Explant livers in three children showed cirrhosis with early well-differentiated hepatocellular carcinoma. We have 100% patient and graft survival at a mean follow-up of 32 months (range 3-72 months). The median z-score for weight and height at liver transplantation was -2.66 (range: -6.44 to -0.9) and -3.6 (range: -7.96 to -0.93) while at follow-up was -1.7 (range: -3.4 to -0.35) and -2.1 (range: -3.9 to -1.4), respectively. The estimated glomerular filtration rate was normal pretransplant and follow-up. This is the first series of LDLT for Alagille syndrome in the Indian sub-continent. We report excellent post-transplant outcomes in contrast to outcomes reported from Western literature.