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We performed a review of case records of children diagnosed with hepatic venous outflow tract obstruction at our center in last 10 years. Out of 11 cases identified, 6 had variable blocks in the hepatic venous system and 4 had combined hepatic venous and inferior vena cava (IVC) block. One child with paroxysmal nocturnal hemoglobinuria (PNH) had isolated IVC involvement. Angioplasty was attempted in 3 patients; among them 2 had successful outcome. Seven children with advanced liver disease underwent transplantation, which was successful in six. With availability of modalities like interventional radiology and transplantation, the overall prognosis of hepatic venous outflow tract obstruction seems to be good when managed in a well-equipped center.

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.

To analyze the clinical profile and outcome of pediatric patients who had undergone a liver and/or RT at our center over a five yr period, case records of all the patients who had undergone a liver or RT were analyzed retrospectively. One hundred solid organ transplants were performed at our center between January 2007 and January 2012. These included 50 liver, 44 renal, one sequential liver and renal, and two CLKT. BA was the most common indication for an LT (38%). At a median follow-up of two yr three months, the patient survival was 88%. The most common indication for an RT was chronic glomerulonephritis (54.5%). At a median follow-up of three yr, the survival was 91%. The CLKT were performed for hyperoxaluria. Two yr post LT, a sequential RT was performed for ESRD resulting from transplant associated microangiopathy. All patients received a living related graft. The common post-operative complications were infections, vascular complications, and graft dysfunction. Survival rates for liver and RT at our center are comparable to those in the established centers in the West., (© 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Process: Selected members were requested to prepare guidelines on specific issues, which were reviewed by two other members. These guidelines were then incorporated into a draft statement, which was circulated to all members. On 17th December 2011, Kunwar Viren Oswal round table conference was organized by the Apollo Center for Advanced Pediatrics, Indraprastha Apollo Hospital, New Delhi and the Sub-specialty Chapter of Pediatric Gastroenterology, Indian Academy of Pediatrics. Presentations, ensuing discussions, and opinions expressed by the participants were incorporated into the final draft., Objectives: To formulate comprehensive evidence based guidelines for management of acute liver failure in India., Recommendations: Viral hepatitis is the leading cause of acute liver failure (ALF) in India. Search for metabolic etiology, particularly in infants and neonates, and in apparently idiopathic cases needs to be done. Planning for early transfer is important as the risks involved with patient transport may increase or even preclude transfer at later stages. Management should be in an intensive care setting in select situations. There is currently insufficient evidence to routinely prescribe branched-chain amino acids, non-absorbable antibiotics or lactulose. Group recommends use of N-acetyl cysteine routinely in patients with ALF. Administration of antibiotics is recommended where infection is present or the likelihood of impending sepsis is high. Enteral nutrition is preferred to parenteral nutrition. Protein restriction is not recommended. An international normalized ratio >4 or Factor V concentration of <25% are the best available criteria for listing for liver transplantation. Overall 40-50% of ALF patients survive without transplantation. Survival in patients fulfilling criteria for liver transplantation and not transplanted is 10-20%. Liver transplantation is a definite treatment for ALF with high one-and five-year survival rates.

Abstract: The successful development of pediatric liver transplantation (LT) over the past four decades has dramatically changed the prognosis for children dying of acute and end-stage liver failure. Over the past two decades survival post transplantation has improved because of better preoperative management, optimal nutritional support, innovative surgical techniques, and improvements in immunosuppressive medications. The ensuing improvement in survival rate has extended the range of indications for LT in children to include transplantation for metabolic liver disease and unresectable hepatic tumors. [Copyright &y& Elsevier]

Pediatric Liver Transplant (LT) is now an established procedure for End Stage Liver Disease (ESLD) with biliary atresia being the commonest indication. Intensive pre-transplant evaluation, nutritional buildup and immunization are the fundamental pre-requisites of a successful LT. With improvement in surgical micro-anastomotic techniques and superior immunosuppressive regimens the success rate of pediatric LT is in excess of 90%. Most of the transplants in our country however are Living related, due to which a fairly large number of children expire awaiting a donor liver. There should be a concerted effort to evolve the cadaveric donation program, so that majority of the children are benefitted. [Copyright &y& Elsevier]