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Background The aim of this study was to assess improvements in long-term survival of pediatric patients after liver transplantation by analyzing outcomes in transplant recipients who survived beyond 1 year after transplantation. There has been a marked increase in the 1-year survival of pediatric patients, from 78% in transplant recipients between 1987 and 1990 to 95% in transplant recipients between 2011 and 2017. The long-term outcomes have not seen a similar trend, creating a disparity that warrants analysis. Methods We analyzed 13 753 pediatric patients who survived for 1 year after receiving orthotopic liver transplantation between 1987 and 2017. The study period was divided into six eras. Outcomes were analyzed using the Kaplan-Meier method for time-to-event analysis, and multivariable Cox regression. Results There were no significant gains in long-term outcomes among 1-year survivors over the past three decades. Log-rank tests for equality of survivor functions between each era and 1987-1990 were not statistically significant. Cause of death analysis revealed that although infections caused 20.6% of deaths in patients transplanted between 1987 and 1990, this number dropped to 5.6% in those transplanted between 2011 and 2017 (p = .01). Malignancy caused 10.6% of deaths in 1987-1990 but caused 22.2% of the deaths in 2011-2017 (p = .04). Conclusion Despite the gratifying gains in short-term survival of pediatric patients, 1-year survivors have no significant improvements in long-term survival after undergoing a liver transplantation. Long-term sequelae of immunosuppression, such as malignancy and infection, continue to be the most common causes of death. This study highlights the necessity for better long-term management of immunosuppression.

APOLT is a suitable technique of liver transplantation in patients with ALF and some types of MLD. Portal venous steal is a problem with this procedure that leads to graft dysfunction and failure. Modulation of the portal flow to the graft and native liver can help in preventing this problem. We discuss the pathophysiology of this complication, review available literature regarding its management, and describe our results using the technique of graded hemiportal banding to achieve adequate perfusion for the graft and native liver.

Gilbert's syndrome is one major cause for unconjugated hyperbilirubinemia in healthy individuals with the prevalence being approximately 3.2-8.6%. It is caused by a mutation in the promoter region of the UGT1A1-gene with a prolonged TAA-repeat coding for the enzyme bilirubin UDP-glucuronosyltransferase (A(TA)(7) TAA allele). After OLT, Gilbert's disease of the transplanted liver can cause unconjugated hyperbilirubinemia. Therefore, we looked for the presence of A(TA)(7) TAA alleles in pediatric liver transplant recipients with unconjugated hyperbilirubinemia. Laboratory results of 106 pediatric liver transplant recipients (aged 0-17 yr) were evaluated for elevated total bilirubin over 2.0 mg/dL (conjugated bilirubin

The aim of this study was to analyze the impact of TAC on medium term (three-yr follow-up) renal function in pediatric liver transplant (OLT) recipients. Glomerular and tubular indices were retrospectively analyzed in 24 consecutive OLT pediatric recipients on TAC. CrCl increased significantly each month post-OLT (p = 0.003), with a trend toward significance between pre-OLT and 36 months (p = 0.17). There was no correlation between CrCl and TAC troughs (p = 0.783). Sixteen percent of patients had CrCl

Children with unresectable HCC have a dismal prognosis and few approved treatment options. TACE is an effective treatment option for adults with HCC, but experience in children is very limited. Retrospective analysis was performed of 8 patients aged 4-17 years (4 male, mean 12.5 years) who underwent TACE for unresectable HCC. Response to TACE was evaluated by change in AFP, RECIST and tumor volume, PRETEXT, and transplantation eligibility by UCSF and Milan criteria. Post-procedure mean follow-up was 8.2 years. Mean overall change in tumor volume for the 8 patients was 51%. Percent change in AFP ranged from a decrease of 100% to an increase of 89.3%, with a mean change of -49.6%. Two patients did not undergo resection or transplantation and died of progressive disease. Six patients underwent orthotopic liver transplantation with mean first TACE-to-transplant interval of 141 days (range 11-514). Following transplantation, 5 patients were alive at the end of the follow-up period and one died of recurrent disease. Based on our initial experience, TACE for children with unresectable HCC appears to be a safe and effective method for managing hepatic tumor burden and for downstaging and bridging to liver transplantation.

HBS is used in the management of liver transplantation, a significant complication of which is biliary stricture. Strictures may be intraparenchymal within segments and main duct (non-anastomotic) or at the biliary-enteric anastomosis (anastomotic). Strictures are definitively diagnosed, and often managed, by PTC. This is invasive, technically challenging, and requires general anesthesia in young children. HBS may allow early detection of these complications and is non-invasive. The aim of this study was to review the scintigraphic pattern of biliary strictures using 99mTcDISIDA HBS following pediatric orthotopic liver transplantation, and to assess its role in the diagnostic algorithm of suspected biliary strictures. All available hepatobiliary studies performed post-transplant in 101 episodes of liver transplantation in 92 pediatric patients were reviewed. Twenty-three (23%) patients had known biliary strictures. Twenty-two patients had adequate studies available for review; five had intrahepatic (non-anastomotic) strictures alone, nine had a stricture of the anastomosis alone, and eight had both intrahepatic and anastomotic strictures. HBS patterns (either segmental or global changes) correlated very highly with clinically significant biliary strictures. All patients with known strictures had abnormal HBS; hence, in patients with abnormal liver function tests post-liver transplant, a normal HBS makes strictures very unlikely. We propose that HBS can thus be used to determine if further investigation is required.

ADH following OLT is a rare entity. Herein, we report a case of Alagille syndrome who developed ADH secondary to OLT, and possible etiological causes are discussed in light of the literature.

This report describes a teenager who developed aplastic anemia (AA) because of non-A-E acute liver failure (ALF) requiring orthotopic liver transplantation (OLT). His AA did not recover spontaneously and he required treatment with ATG 9 months post-OLT. Bone marrow recovery occurred 4 months after immunotherapy and coincided with further intensification of immunusuppression required to treat early chronic rejection of the liver graft. Three years post-OLT he remains well with good bone marrow and liver function. Intensification of immunosuppression can lead to successful resolution of AA associated with non-A-E ALF.

Cash WJ, Knisely AS, Waterhouse C, Iqbal M, Stokes V, Byrne B, McCormick PA. Successful pregnancy after liver transplantation in progressive familial intrahepatic cholestasis, type 1. Pediatr Transplantation 2011: 15: E174–E176. © 2010 John Wiley & Sons A/S. Abstract: A woman who had undergone liver transplantation for genetically documented ATP8B1 disease/progressive familial intrahepatic cholestasis, type 1, successfully conceived, carried, and was delivered of a healthy child. The pregnancy and its management are described; implications are discussed.

Diaphragmatic hernia after OLT is a rare surgical complication. We here report successful diagnosis and treatment of two cases with right-sided diaphragmatic hernia developed after OLT both utilizing left-sided allografts. Combination of factors related to the surgical techniques and patient characteristics might explain the pathophysiology behind the diaphragmatic hernias following liver transplantation. Respiratory as well as non-specific gastrointestinal symptoms may be hints for an overlooked diaphragmatic hernia after liver transplantation. Diaphragmatic hernia should be added to the list of potential complications of liver transplantation for prompt diagnosis and appropriate treatment.