http://www.ncbi.nlm.nih.gov/pubmed/25708058

Malhotra S, Sibal A, Bhatia V, Kapoor A, Gopalan S, Seth S, Jerath N, Wadhawan M, Gupta S. Living Related Liver Transplantation for Biliary Atresia in the Last 5 years: Experience from the First Liver Transplant Program in India. Indian J Pediatr. 2015 Oct; 82(10):884-9.

Abstract

OBJECTIVE:
To study the clinical, biochemical profile and outcome of patients with biliary atresia (BA) who underwent living related liver transplantation (LRLT) at authors' institute in the last 5 y (2008-2013).

METHODS:
Case records of the 20 patients diagnosed with biliary atresia who had undergone living related liver transplantation at authors' centre in the last 5 y were analysed.

RESULTS:
Eighteen patients with BA with a failed Kasai procedure and 2 without a prior Kasai's portoenterostomy received a liver transplant. At a median follow up of 2 y and 6 mo, both the patient and graft survival rates were 90 %. The median age of the recipients at the time of LRLT was 8 mo and 12 (60 %) of the transplanted children were less than or equal to 1 y of age. The male-female ratio was 1.8:1. The median weight was 7.3 kg (5.8-48 kg); two thirds were less than 10 kg. The median pre-transplant total serum bilirubin (TSB) and international normalized ratio (INR) were 12.98 (0.5-48.3) mg/dl and 1.3 (1.0-3.9) respectively. All patients received a living related graft and there was no donor mortality. The median duration of postoperative ventilation was 14 h. The post-operative complications were infection (30 %), vascular complications (20 %) and acute rejection (20 %). The median duration of postoperative hospital stay was 21 d (17-42). Two patients died of combined hepatic and portal vein thrombosis in the early postoperative period. Late rejection was encountered in 1 patient and another developed chronic kidney disease necessitating a renal transplant. There were no late vascular occlusions or development of post transplant lymphoproliferative disease.

CONCLUSIONS:
Thus, LRLT for BA with or without a prior portoenterostomy, is a feasible and successful treatment modality with good outcomes attained despite the challenges of age and size. This treatment modality is now well established in India.