Search

Liver transplantation currently represents a therapeutic option for patients with Wilson disease presenting with end-stage liver disease or acute liver failure. Indeed, it has been associated with excellent postoperative survival curves in view of young age at transplant and absence of recurrence. Attention has shifted over the past decades to a wise expansion of indications for liver transplantation. Evidence has emerged supporting the transplantation of carefully selected patients with primarily neuropsychiatric symptoms and compensated cirrhosis. The rationale behind this approach is the potential for surgery to improve copper homeostasis and consequently ameliorate neuropsychiatric symptoms. However, several questions remain unanswered, such as how to establish thresholds for assessing pretransplant neuropsychiatric impairment, how to standardize preoperative neurological assessments, and how to define postoperative outcomes for patients meeting these specific criteria. Furthermore, a disease-specific approach will be proposed both for the liver transplant evaluation of candidates with Wilson disease and for patient care during the transplant waiting period, highlighting the peculiarities of this systemic disease., (Copyright © 2024 American Association for the Study of Liver Diseases.)

Background: Pediatric acute liver failure (PALF) can require emergent liver transplantation (LT, >25%) or lead to death (~15%). Existing models cannot predict clinical trajectory or survival with native liver (SNL). We aimed to create a predictive model for PALF clinical outcomes based on admission variables., Methods: A retrospective, single-center PALF cohort (April 2003 to January 2022) was identified using International Classification of Disease codes, selected using National Institutes of Health PALF Study Group (PALFSG) criteria, and grouped by clinical outcome (SNL, LT, or death). Significant admission variables were advanced for feature selection using least absolute shrinkage and selection operator regression with bootstrapping (5000×). A predictive model of SNL versus LT or death was created using logistic regression and validated using PALFSG data., Results: Our single-center cohort included 147 patients (58% SNL, 32% LT, 10% expired), while the PALFSG validation cohort included 492 patients (50% SNL, 35% LT, 15% expired). Admission variables associated with SNL included albumin (odds ratio [OR], 16; P < 0.01), ammonia (OR, 2.37; P < 0.01), and total bilirubin (OR, 2.25; P < 0.001). A model using these variables predicted SNL versus LT or death with high accuracy (accuracy [0.75 training, 0.70 validation], area under the curve [0.83 training, 0.78 validation]). A scaled score (CHLA-acute liver failure score) was created that predicted SNL versus LT or death with greater accuracy (C statistic 0.83) than Pediatric End-Stage Liver Disease (C statistic 0.76) and admission liver injury unit (C statistic 0.76) scores., Conclusions: The CHLA-acute liver failure score predicts SNL versus LT or mortality in PALF using admission laboratories with high accuracy. This novel, externally validated model offers an objective guide for urgent referral to a pediatric LT center., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Drug-induced liver injury has become the main cause of acute liver failure (ALF). Injury may be dose-dependent or occur idiosyncratically. We report the case of a 37-year-old female patient admitted with grade 4 hepatic encephalopathy. Morosil use was initiated 30 days prior, and after 2 weeks, the case evolved with jaundice and diarrhea interspersed with constipation when the medication was discontinued. Morosil is a dry extract of the juice of red Moro oranges (Citrus species), grown in eastern Sicily (Italy). It is an important source of anthocyanidin pigments, which act as antioxidants and are used in the care and prevention of obesity, insulin resistance, hepatic steatosis, and cardiovascular diseases. According to the clinical picture and tests performed, the diagnosis of ALF was confirmed, by the criteria of Clichy and King's College. The patient was listed for liver transplantation as a priority, with the model for end stage liver disease (MELD) score: 41. Orthotopic liver transplantation was uneventfully performed using the Piggy-back technique. There are few reports in the literature of acute liver injury by other Citrus species, and we found no case reports of ALF related to the use of Morosil., (Copyright © 2023. Published by Elsevier Inc.)

Background: In children, acute liver failure (ALF) is a severe condition associated with high mortality if an emergency liver transplantation (LT) is impossible. Clinical laboratory parameters and different scores or criteria are used to predict ALF evolution in children. We aimed to assess the role of coagulation factors as predictive markers of poor outcomes in children with ALF., Methods: The prospective study included 40 children with ALF, diagnosed based on the Pediatric ALF study group criteria. Patients with emergency LT or deceased were considered with poor outcomes. For all patients, we analyzed clinical and laboratory parameters (including plasma level of factor V (FV), factor VII (FVII), and INR). We calculated the PELD (Pediatric End-stage Liver Disease) and MELD (Model for End-stage Liver Disease) scores, King's College Hospital (KCH), and Clichy criteria. We analyzed their performance in predicting a poor outcome., Results: FV and FVII levels were significantly lower in children with poor outcomes than survivors (18.92 ± 19.95% vs. 10.72 ± 10.21%, p = 0.00139, respectively 46.51 ± 26.05% vs. 10.72 ± 10.21%, p = 0.00014). These parameters varied with ALF etiology, being the lowest in metabolic and infectious causes. The maximum value of INR (INR-max) was higher in children with poor outcomes than survivors (7.05 ± 3.20 vs. 2.96 ± 1.82, p = 0.000007), as it also was for the PELD/MELD score (30.06 ± 15.55 vs. 15.77 ± 9.64, p = 0.00092). FVII, FV, and INR-max had an excellent performance in predicting the poor outcome with an area under the ROC curve of 0.894, 0.816, and 0.861, respectively. KCH criteria had a higher sensitivity than Clichy criteria (92.86% vs. 50%) but lower specificity (53.85% vs. 95%)., Conclusions: Our results support the role of coagulation factors (INR, FV, and FVII) as predictive markers for the fatal evolution of children with ALF and underlined the need for monitoring along with the usual liver function tests in children with ALF.

Introduction: Indeterminate acute liver failure (IND-ALF) is a rare clinical syndrome with a high mortality rate. Lacking a known etiology makes rapid evaluation and treatment difficult, with liver transplantation often considered as the only therapeutic option. Our aim was to identify genetic variants from whole exome sequencing data that might be associated with IND-ALF clinical outcomes., Methods: Bioinformatics analysis was performed on whole exome sequencing data for 22 patients with IND-ALF. A 2-tier approach was used to identify significant single-nucleotide polymorphisms (SNPs) associated with IND-ALF clinical outcomes. Tier 1 identified the SNPs with a higher relative risk in the IND-ALF population compared with those identified in control populations. Tier 2 determined the SNPs connected to transplant-free survival and associated with model for end-stage liver disease serum sodium and Acute Liver Failure Study Group prognostic scores., Results: Thirty-one SNPs were found associated with a higher relative risk in the IND-ALF population compared with those in controls, of which 11 belong to the human leukocyte antigen (HLA) class II genes but none for the class I. Further analysis showed that 5 SNPs: rs796202376, rs139189937, and rs113473719 of HLA-DRB5; rs9272712 of HLA-DQA1; and rs747397929 of IDO1 were associated with a higher probability of IND-ALF transplant-free survival. Using 3 selected SNPs, a model for the polygenic risk score was developed to predict IND-ALF prognoses, which are comparable with those by model for end-stage liver disease serum sodium and Acute Liver Failure Study Group prognostic scores., Discussion: Certain gene variants in HLA-DRB5, HLA-DQA1, and IDO1 were found associated with IND-ALF transplant-free survival. Once validated, these identified SNPs may help elucidate the mechanism of IND-ALF and assist in its diagnosis and management., (Copyright © 2022 Written work prepared by employees of the Federal Government as part of their official duties is, under the U.S. Copyright Act, a “work of the United States Government” for which copyright protection under Title 17 of the United States Code is not available. As such, copyright does not extend to the contributions of employees of the Federal Government.)

Objective: Drug-induced liver injury (DILI) is a leading cause of acute liver failure (ALF). Predictors for orthotopic liver transplantation (OLT) or death in drug-induced ALF (DI-ALF) are scarce., Methods: In total 33 of 346 patients recruited for our prospective study on potentially hepatotoxic drugs had ALF. DILI diagnosis was based on Roussel Uclaf Causality Assessment Method (RUCAM) score and expert opinion. Area under the receiver operating characteristic (AUROC) curve, Youden's index and positive and negative likelihood ratios were calculated to identify the best performing predictive markers and scores for OLT or death., Results: Poor outcome was associated with lower baseline platelet counts and cholinesterase (CHE) levels, higher International Normalized Ratio (INR) levels and Model for End-Stage Liver Disease (MELD) scores. Yet, AUROC reached a maximum of only 0.71-0.75 for either of those laboratory markers or the MELD score. Notably however, combinations of those scores were highly discriminatory, in particular INR/(CHE*platelet count) and MELD/(CHE*platelet count), showing an AUROC of 0.91, a positive likelihood ratios of 13.78 and a negative likelihood ratios of 0.08., Conclusion: While baseline MELD score, INR, CHE, and platelet counts had limited potential to discriminate between DI-ALF with survival or poor outcome, their combinations were highly associated with OLT or death in patients with DI-ALF., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Background and Aims: The 13 C-methacetin breath test (MBT) is a noninvasive, quantitative hepatic metabolic function test. The aim of this prospective, multicenter study was to determine the utility of initial and serial 13 C-MBT in predicting 21-day outcomes in adults with acute liver failure (ALF) and non-acetaminophen acute liver injury (ALI)., Approach and Results: The 13 C-MBT BreathID device (Exalenz Biosciences, Ltd.) provided the percent dose recovery (PDR) for a duration of 60 minutes after administration of 13 C-methacetin solution as the change in exhaled 13 CO 2 / 12 CO 2 compared with pre-ingestion ratio on study days 1, 2, 3, 5, and 7. Results were correlated with 21-day transplant-free survival and other prognostic indices. A total of 280 subjects were screened for enrollment between May 2016 and August 2019. Median age of the 62 enrolled patients with adequate data was 43 years, 79% were Caucasian, 76% had ALF with the remaining 24% having ALI. The mean PDR peak on day 1 or day 2 was significantly lower in nonsurvivors compared with transplant-free survivors (2.3%/hour vs. 9.1%/hour; P < 0.0001). In addition, serial PDR peaks were consistently lower in nonsurvivors versus survivors (P < 0.0001). The area under the receiver operating characteristic curve (AUROC) of the 13 C-MBT in the combined cohort was 0.88 (95% CI: 0.79-0.97) and higher than that provided by King's College (AUROC = 0.70) and Model for End-Stage Liver Disease scores (AUROC = 0.83). The 13 C-MBT was well tolerated with only two gastrointestinal adverse events reported., Conclusions: The 13 C-MBT is a promising tool to estimate the likelihood of hepatic recovery in patients with ALF and ALI. Use of the PDR peak data from the 13 C-MBT point-of-care test may assist with medical decision making and help avoid unnecessary transplantation in critically ill patients with ALF and ALI., (© 2021 by the American Association for the Study of Liver Diseases.)

BACKGROUND Wilson's disease (WD) manifesting as acute liver failure (ALF) is a life-threatening condition, and spontaneous recovery is rare. Diagnostic scores like the alkaline phosphatase elevation/total bilirubin elevation ratio and aspartate aminotransferase/alanine aminotransferase ratio can distinguish WD from other ALF etiologies. Liver transplantation plays a major role in treating these patients, and the revised Wilson Index is useful in patient selection for this procedure. The aim of this study was to evaluate diagnostic scores, treatments, and outcomes of a large cohort of patients with WD-ALF. MATERIAL AND METHODS Twenty adult patients of a historical cohort admitted from January 2001 to December 2017 were prospectively observed. Demographic, clinical, laboratory, and radiology data, and treatment, time on the waiting list for liver transplantation, and outcomes were recorded. RESULTS No diagnostic laboratory scores were 100% positive in patients with WD-ALF. Cut-off values for the alkaline phosphatase/total bilirubin ratio and aspartate aminotransferase/alanine aminotransferase ratio were met by 65.0% and 80.0% of patients, respectively. All patients met at least 1 criterion for high risk of death (Nazer or revised Wilson Index) and qualified for liver transplantation. In 9 patients, albumin dialysis was used before surgery. Survival after liver transplantation was 85.0% and 74.4% after 1 month and 1 year, respectively. CONCLUSIONS Further research on a novel diagnostic score in WD-ALF is warranted. Adult patients suspected to have WD as the cause of ALF should be treated in the referral liver transplantation unit. Liver transplantation makes long-term survival possible for patients with this critical illness.

Aim: Liver transplantation (LT) has been used as a definitive management for children with end-stage liver disease or acute liver failure. Living-donor LT (LDLT) has been a common type of LT performed in Asian countries, including Japan, where deceased donors are rarely available. However, the neurological complications (NCs) associated with LDLT remain unknown. The purpose of this study was to clarify the characteristics of NCs in children after LDLT., Methods: This study is a retrospective observational study carried out at a tertiary children's hospital in Japan. We studied children who had undergone LDLT between January 2001 and January 2020., Results: We examined 602 cases of LT, of which 559 were LDLT cases (92.9%). NCs after LT were present in 21 cases (3.8%). The most common neurological symptoms were seizure (n = 17), whereas disturbance of consciousness without seizure was observed in four cases. The frequency of NCs for each of the indications was 12.2% for fulminant hepatic failure, 6.5% for metabolic liver disease, and 0.7% for cholestatic liver disease., Interpretation: We report the characteristics of NCs after LDLT in children. The frequency of NCs after LT was high in cases of fulminant hepatic failure and metabolic diseases, who might have had neurological symptoms or impaired consciousness before LT., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2021 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.)

Liver transplantation is considered the ultimate solution for patients with end-stage chronic liver disease or acute liver failure. Patients with liver transplant need special care starting from preoperative preparation, surgical intervention ending with postoperative care. Transplanted patients have to receive immunosuppressive therapy to prevent rejection. Such a state of immune suppression could predispose to different types of infections in liver transplant recipients. Currently, the world is suffering a pandemic caused by a new strain of the coronavirus family called COVID-19. Certain infection control precautions are needed to protect immunocompromised and vulnerable patients, including liver transplant candidates and recipients from acquiring COVID-19 infection. Restricting non-transplant elective surgical procedures, managing transplant patients in separate outpatient clinics, and in-patient wards can prevent transmission of infection both to patients and healthcare workers. Telemedicine can help in the triage of patients to screen for symptoms of COVID-19 before their regular appointment. Management of immunosuppressive therapy and drug-drug interactions in liver transplant recipients infected with COVID-19 should be cautiously practiced to prevent rejection and effectively treat the underlying infection. In this report, we are trying to summarize available evidence about different aspects of the management of liver transplant candidates and recipients in the era of COVID-19., (Copyright © 2020 Pan-Arab Association of Gastroenterology. Published by Elsevier B.V. All rights reserved.)

Background: Liver transplantation (LT) is an important treatment for acute liver failure and end-stage liver disease. Due to the limited supply of livers, there are still thousands of candidates waiting for transplantation in Turkey. We aimed to analyze LT waiting list access by demographics and etiology, particularly the diagnosis of hepatocellular carcinoma (HCC), which has been prioritized for LT in recent years., Materials and Methods: Between 2011 and 2018, all patients listed for LT in our center were retrospectively reviewed. Demographic features, etiology of liver disease, waiting time, Model for End-Stage Liver Disease (MELD) score, and survival data were recorded. Differences between the LT group and deceased patients on the waiting list were evaluated., Results: During this period, 266 patients were included in the LT waiting list. Only 119 patients (44.7%) underwent LT (men, 94; women, 25; mean age, 53 years), whereas 103 (38%) died (men, 60; women, 43; mean age, 53 years) in the waiting period. Seventeen patients were status 1A or 1B and of these, 7 patients died from fulminant hepatic failure. MELD score was significantly higher in deceased group (28 ± 7 vs 25 ± 6; P = .014). The frequency of HCC was significantly higher in LT group (29% vs 11%; P = .002). Overall survival of the patients in the waiting list with and without liver transplantation were 63% and 41%, respectively., Conclusions: HCC is one of the leading etiologies that is considered for cadaveric LT from the waiting list in our center. These patients had slightly lower MELD scores compared to deceased patients with shorter waiting times. We recommend early referral and close monitoring of the patients who are LT candidates., (Copyright © 2019 The Authors. Published by Elsevier Inc. All rights reserved.)

Introduction and Aim: 1. Study of liver explants - Etiologic types of end-stage chronic liver disease (ESCLD) and acute liver failure (ALF) in adults and children. 2. Assessment of donor steatosis and incidental granulomas. 3. Post-transplant liver biopsies., Material and Methods: Specimens of 180 explant hepatectomies, 173 donor wedge and 30 core liver biopsies, and 58 post transplant liver biopsies received in our department from April 2013 to March 2017., Results: 1. Most common causes of ESCLD in adults were: alcohol related (30.32%), hepatitis virus related (18.71%) and non-alcoholic steatohepatitis related (18.06%); and in children ≤ 12 years were: biliary atresia (27.27%), autoimmune disease (18.18%) and Wilson's disease (18.18%). Most common causes of ALF in adults and children were anti-tubercular therapy induced and idiopathic respectively. 2. Prevalence rate of moderate steatosis (between 30-60%) was 4.28%. Incidental granulomas were seen in 5 cases. 3. Most common diagnoses of post-transplant biopsies in adults included acute cellular rejection (ACR) (36.17%), recurrence of viral disease (8.51%) and moderate non-specific portal triaditis (8.51%). Among children ≤ 12 years, most common diagnoses included unremarkable liver parenchyma, ACR and ischemia/reperfusion injury., Conclusion: 1. Alcohol- and hepatitis- virus related ESCLD, and biliary atresia are leading indications for liver transplantation in adults and children respectively. 2. Prevalence of 4.28% of moderate steatosis, is much lower than that documented in western literature. Only 5 cases of incidental granulomas is unexpectedly low in a country endemic for tuberculosis. 3. Most common diagnoses of post-transplant liver biopsies in adults has been acute rejection, which is similar to the findings from much larger published series.

Introduction: Identification of predictive factors of mortality in a liver transplant (LT) program optimizes patient selection and allocation of organs., Objective: To determine survival rates and predictive factors of mortality after LT in the National Liver Transplant Program of Uruguay., Methods: A retrospective study was conducted analyzing data prospectively collected into a multidisciplinary database. All patients transplanted since the beginning of the program on July 2009 to April 2017 were included (n = 148). Twenty-nine factors were analyzed through the univariate Kaplan-Meier model. A Cox regression model was used in the multivariate analysis to identify the independent prognostic factors for survival., Results: Overall survival was 92%, 87%, and 78% at discharge, 1 year, and 3 years, respectively. The Kaplan-Meier survival curves were significantly lower in: recipients aged >60 years, Model for End-Stage Liver Disease score >21, LT due to hepatocellular carcinoma (HCC) and acute liver failure (ALF), donors with comorbidities, intraoperative blood loss beyond the median (>2350 mL), red blood cell transfusion requirement beyond the median (>1254 mL), intraoperative complications, delay of extubation, invasive bacterial, and fungal infection after LT and stay in critical care unit >4 days. The Cox regression model (likelihood ratio test, P = 1.976 e -06 ) identified the following independent prognostic factors for survival: LT for HCC (hazard ratio [HR] 4.511; P = .001) and ALF (HR 6.346; P = .004), donors with comorbidities (HR 2.354; P = .041), intraoperative complications (HR 2.707; P = .027), and invasive fungal infections (HR 3.281; P = .025)., Conclusion: The survival rates of LT patients as well as the mortality-associated factors are similar to those reported in the international literature., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Liver transplantation is the accepted treatment for patients with acute liver failure and liver-based metabolic disorders. However, donor organ shortage and lifelong need for immunosuppression are the main limitations to liver transplantation. In addition, loss of the native liver as a target organ for future gene therapy for metabolic disorders limits the futuristic treatment options, resulting in the need for alternative therapeutic strategies. A potential alternative to liver transplantation is allogeneic hepatocyte transplantation. Over the last two decades, hepatocyte transplantation has made the transition from bench to bedside. Standardized techniques have been established for isolation, culture, and cryopreservation of human hepatocytes. Clinical hepatocyte transplantation safety and short-term efficacy have been proven; however, some major hurdles-mainly concerning shortage of donor organs, low cell engraftment, and lack of a long-lasting effect-need to be overcome to widen its clinical applications. Current research is aimed at addressing these problems, with the ultimate goal of increasing hepatocyte transplantation efficacy in clinical applications.

Living donor liver transplantation (LDLT) is now an established technique for treating children with end-stage liver disease. Few data exist about liver transplantation (LT) for exclusively young infants, especially infants of <3 months of age. We report our single-center experience with 12 patients in which LDLT was performed during the first 3 months of life and compare the results with those of older infants who underwent LT. All of the patients were treated at the National Center of Child Health and Development, Tokyo, Japan. Between November 2005 to November 2016, 436 children underwent LT. Twelve of these patients underwent LT in the first 3 months of life (median age, 41 days; median weight, 4.0 kg). The indications for transplantation were fulminant hepatic failure (n = 11) and metabolic liver disease (n = 1). All the patients received the left lateral segment (LLS) in situ to mitigate the problem of graft-to-recipient size discrepancy. A reduced LLS graft was used in 11 patients and a segment 2 monosegment graft was used in 1 patient. We compared the results with those of infants who were 4-6 months of age (n = 67) and 7-12 months of age (n = 110) who were treated in the same study period. There were significant differences in the Pediatric End-Stage Liver Disease score and the conversion rate of tacrolimus to cyclosporine in younger infants. Furthermore, the incidence of biliary complications, bloodstream infection, and cytomegalovirus infection tended to be higher, whereas the incidence of acute cellular rejection tended to be lower in younger infants. The overall cumulative 10-year patient and graft survival rates in recipients of <3 months of age were both 90.9%. LDLT during the first 3 months of life appears to be a feasible option with excellent patient and graft survival. Liver Transplantation 23 1051-1057 2017 AASLD., (© 2017 by the American Association for the Study of Liver Diseases.)

Objectives: Autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) are progressive immune-mediated inflammatory diseases that may require liver transplant (LT). Outcomes in children undergoing LT for these diseases are poorly studied in the pediatric end-stage liver disease era. We aimed to characterize the outcome of LT in children with AIH and PSC., Methods: Children 18 years or younger with PSC or AIH who had a first, isolated LT from 2002 to 2012 were identified from the United Network for Organ Sharing database. Graft and patient outcomes were studied., Results: A total of 174 children with AIH and 113 with PSC were transplanted in the study period. One-year patient survival was 95.4% for AIH and 97.3% for PSC. Five-year patient survival was 91.4% for AIH and 92.9% for PSC. Patient survival was not significantly different between the 2 groups. Forty-four (25.2%) children with AIH were listed as status 1 for transplant (fulminant hepatic failure at presentation or acute-on-chronic disease). Patients transplanted as status 1 had significantly lower patient survival compared with patients transplanted with AIH and end-stage liver disease. The one- and five-year graft survival rates were not significantly different between patients with AIH and PSC., Conclusion: Children with AIH transplanted as status 1 had significantly lower patient survival rates but similar graft survival rates to children with chronic AIH. Children transplanted for AIH versus PSC showed no significant differences in patient or graft survival at both 1 and 5 years.

Orthotopic liver transplantation remains the only proven cure for end-stage liver failure. Despite significant advances in the field, the clinical demand for donor organs far outweighs the supply. Hepatocyte transplantation has been proposed as an alternative approach to whole liver transplant in select diseases. Several international centers have reported experimental trials of human hepatocyte transplantation in acute liver failure and liver-based metabolic disorders. This chapter provides an introduction to hepatocyte transplantation from both a technical and clinical perspective. We will also focus on the special needs of pediatric patients, since historically the majority of clinical hepatocyte transplants have involved infants and children.

Approximately 10% of children with autoimmune hepatitis (AIH) and 30% of those with sclerosing cholangitis (SC) require liver transplantation (LT). LT is indicated in patients who present with fulminant hepatic failure (ie, with encephalopathy) and in those who develop end-stage liver disease despite treatment. After LT, recurrent AIH is reported in approximately 30% of patients and recurrent SC in up to 50%. Diagnosis of recurrence is based on biochemical abnormalities, seropositivity for autoantibodies, interface hepatitis on histology, steroid dependence, and, for SC, presence of cholangiopathy. Recurrence of SC after LT is often associated with poorly controlled inflammatory bowel disease (IBD). Recurrence may even appear years after LT; therefore, steroid-based immunosuppression should be maintained at a higher dose than that used for patients transplanted for nonautoimmune liver diseases. Although the impact of recurrent disease on graft function is controversial, it seems that in pediatric LT recipients recurrence of AIH or SC is associated with compromised graft survival. Exacerbation of preexistent IBD may be observed after LT for SC or AIH, and IBD appears to have a more aggressive course than before LT. In addition, IBD can develop de novo following LT. Liver Transplantation 22 1275-1283 2016 AASLD., (© 2016 by the American Association for the Study of Liver Diseases.)

Liver transplantation (LT) in Australia and New Zealand began in 1985. Over this time until December 2014, LT took place in 3700 adults and 800 children. LT is regulated with 1 unit, supported by the government, per state or region. Currently approximately 270 transplants take place per year. Organ donation rates are moderate in Australia (17 per 1 million of population) but very low in New Zealand (11 per 1 million of population). All the units share organ donors for fulminant hepatic failure cases (status 1). Recipient listing criteria and organ allocation criteria are commonly agreed to via National and Trans-Tasman agreements, which are published online. Current survival rates indicate approximately 94% 1-year survival with median survival in adults of approximately 20 years, whereas 75% of children are alive at 20 years. All units collaborate in research projects via the Australia and New Zealand Liver Transplant Registry and have published highly cited articles particularly on the prevention of hepatitis B virus recurrence. Outcomes for indigenous populations have also been analyzed. In conclusion, LT in Australia and New Zealand is well developed with transparent processes related to criteria for listing and organ allocation together with publication of outcomes. Liver Transplantation 22 830-838 2016 AASLD., (© 2016 American Association for the Study of Liver Diseases.)

This article reviews the historical evolution of the liver transplant organ allocation policy and the indications/contraindications for liver transplant, and provides an overview of the liver transplant evaluation process. The article is intended to help internists determine whether and when referral to a liver transplant center is indicated, and to help internists to counsel patients whose initial evaluation at a transplant center is pending., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Introduction: Sparse data are available about the effect of therapy methods on antibody levels in patients with liver failure. The aim of this study was to determine serum immunoglobulin concentrations in patients with chronic hepatic failure (CHF), acute- (ALF), or acute-on-chronic liver failure (ACLF) and to evaluate the impact of MARS treatment or liver transplantation (LT) on antibody levels., Materials and Methods: We followed ten patients with ALF, twelve with ACLF and 18 with CHF. Eight patients with ALF and seven with ACLF underwent MARS therapy, whereas the rest received LT. 13 healthy volunteers served as controls. Serum antibody concentrations were measured using ELISA-technique., Results: Median serum levels of IgA, IgG and IgM were significantly increased in patients with CHF compared to ALF or controls (P<0.02, P<0.01, and P<0.01). IgM and IgG concentrations were also significantly elevated in patients with CHF compared to ACLF (IgM, 3.7 vs. 1 g/L, P<0.001; IgG, 8.7 vs. 3.1 g/L, P=0.004). Immediately after LT a significant decrease of IgA (6.9 vs. 3.1 g/L, P=0.004), IgG (8.7 vs. 5.1 g/L, P=0.02) and IgM (3.7 vs. 1.8 g/L, P=0.001) was detected in patients with CHF and antibody levels further decreased the days after LT reaching levels comparable to healthy individuals. MARS treatment had no apparent effect on the immunoglobulin profile in patients with ALF or ACLF., Conclusion: We provide evidence that LT reverses hypergammaglobulinemia in patients suffering from CHF within one day, which could be explained to a reconstituted hepatic antibody clearance, whereas MARS treatment has no immediate effect on immunoglobulin levels.

Background: In a liver transplant (LT) center, treatments with Prometheus were evaluated. The main outcome considered was 1 and 6 months survival., Methods: During the study period, 74 patients underwent treatment with Prometheus; 64 were enrolled, with a mean age of 51 ± 13 years; 47 men underwent 212 treatments (mean, 3.02 per patient). The parameters evaluated were age, sex, laboratorial (liver enzymes, ammonia) and clinical (model for end-stage liver disease and Child-Turcotte-Pugh score) data., Results: Death was verified in 23 patients (35.9%) during the hospitalization period, 20 patients (31.3%) were submitted to liver transplantation, and 21 were discharged. LT was performed in 4 patients with acute liver failure (ALF, 23.7%), in 7 patients with acute on chronic liver failure (AoCLF, 43.7%), and in 6 patients with liver disease after LT (30%). Seven patients who underwent LT died (35%). In the multivariate analysis, older age (P = .015), higher international normalized ratio (INR) (P = .019), and acute liver failure (P = .039) were independently associated with an adverse 1-month clinical outcome. On the other hand, older age (P = .011) and acute kidney injury (P = .031) at presentation were both related to worse 6-month outcome. For patients with ALF and AoCLF we did not observe the same differences., Conclusions: In this cohort, older age was the most important parameter defining 1- and 6-month survival, although higher INR and presence of ALF were important for 1-month survival and AKI for 6-month survival. No difference was observed between patients who underwent LT or did not have LT., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Background: The impact of ethnicity on outcomes after orthotopic liver transplantation (OLT) is unclear. The British Indo-Asian population has a high incidence of liver disease but its contribution to the national deceased donor pool is small. We evaluated access to and outcomes of OLT in Indo-Asians., Methods: We compared 182 Indo-Asians with white patients undergoing OLT. Matching criteria were transplantation year, liver disease, age, sex. Donor and recipient characteristics, postoperative outcomes, including patient and graft survival, OLT era (early, 1987-2001; late, 2002-2011) were compared. Survival was also analyzed by underlying disease-acute liver failure (ALF) and chronic liver failure., Results: Indo-Asians had higher diabetes incidence. There were no differences in waiting time for transplantation, despite smaller body size and more uncommon blood groups (B, AB) among Indo-Asians. In the early era, patient survival for Indo-Asians with ALF was worse when compared to whites. In the late era, graft and patient survival at 1, 2, and 5 years were similar between groups., Conclusion: This study demonstrates that Indo-Asian patients have equal access to OLT and comparable outcomes to whites in the United Kingdom. Survival has improved among Indo-Asian patients; this may be attributable to careful patient selection in case of ALF, though improvement of patient management may have contributed.

Objectives: Liver transplant is an established curative therapy for children with chronic end-stage liver disease or acute liver failure. In this study, we aimed to evaluate pediatric liver transplant in terms of outcomes, complications, and long-term followup results., Materials and Methods: Pediatric patients who had liver transplant in our institution were included. We retrospectively evaluated demographic features including body weight, Child-Pugh score, etiology of liver disease, graft source, perioperative outcomes, perioperative complications, postoperative complications, and long-term results. Outcomes of treatment of complications and revision transplant were evaluated., Results: Between September 2001 and December 2013, there were 188 pediatric liver transplants performed in our institution. Most grafts (90.9%) were obtained from living-related donors. There were 13 patients (6.9%) who had an intervention because of a hemorrhage postoperatively. Biliary leakage was observed in 33 patients (17.5%) and biliary stricture during follow-up was observed in 32 patients (17%). Thrombosis rates in the hepatic artery and portal vein were 12.3% and 0.5%. Revision transplant was performed in 11 patients (5.8%); reason for revision transplant was rejection in 50% patients. The remaining children were alive with good graft functioning after treatment of complications and revision transplant. The overall 5- and 10-year survival rates were 82.3% and 78.9%., Conclusions: The overall outcomes of pediatric liver transplant at our center are very promising. With improved care of younger children and the combined efforts of the parents and medical team, the number of the children receiving transplants will increase in the future.

Objectives: Liver transplantation is the treatment of choice for acute and chronic liver failure, for selected cases of tumors, and for conditions resulting from errors in metabolism. This paper reports the experience of a medical center in Latin America., Methods: Were conducted 305 orthotopic liver transplantations on 284 patients between 2004 and 2010. Of these patients, 241 were adults undergoing their first transplantation., Results: The average age of patients was 52 years old, and 62% of the individuals were male. The most common indication was alcoholic cirrhosis. The rate of patient survival after 1 and 5 years was 82 and 72% respectively. The rate of liver graft survival after 1 and 5 years was 78 and 68% respectively. The main cause of death was sepsis. Complications in the hepatic artery were documented for 5% of the patients. Additionally, 14.5% of the patients had complications in the biliary tract. Infections were found in 41% of the individuals. Acute rejection was observed in 30% of the subjects, and chronic rejection in 3%., Conclusion: In conclusion, liver transplantation at our medical center in Colombia offers good mid-term results, with a complication rate similar to that reported by other centers around the world.

The Model for End-Stage Liver Disease (MELD) has been the single best predictor of outcome of the progression of cirrhosis. Acute-on-chronic liver failure (ACLF) has been proposed as an alternative path in the natural history of cirrhosis. ACLF occurs in patients with chronic liver disease and is characterized by a precipitating event, resulting in acute deterioration in liver function, multiorgan system failure, and high short-term mortality. In this review, the natural course of patients with ACLF, especially as it relates to management of cirrhotic patients on the transplant waiting list, and its impact on liver transplantation outcomes are defined., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Background: We sought to compare liver transplant waiting list access by demographics and geography relative to the pool of potential liver transplant candidates across the United States using a novel metric of access to care, termed a liver wait-listing ratio (LWR)., Methods: We calculated LWRs from national liver transplant registration data and liver mortality data from the Scientific Registry of Transplant Recipients and the National Center for Healthcare Statistics from 1999 to 2006 to identify variation by diagnosis, demographics, geography, and era., Results: Among patients with ALF and CLF, African Americans had significantly lower access to the waiting list compared with whites (acute: 0.201 versus 0.280; pre-MELD 0.201 versus 0.290; MELD era: 0.201 versus 0.274; all, P<0.0001) (chronic: 0.084 versus 0.163; pre-MELD 0.085 versus 0.179; MELD 0.084 versus 0.154; all, P<0.0001). Hispanics and whites had similar LWR in both eras (both P>0.05). In the MELD era, female subjects had greater access to the waiting list compared with male subjects (acute: 0.428 versus 0.154; chronic: 0.158 versus 0.140; all, P<0.0001). LWRs varied by three-fold by state (pre-MELD acute: 0.122-0.418, chronic: 0.092-0.247; MELD acute: 0.121-0.428, chronic: 0.092-0.243)., Conclusions: The marked inequity in early access to liver transplantation underscores the need for local and national policy initiatives to affect this disparity.

Background and Aim: Clinical course and mortality of acute liver failure (ALF) are determined by its causes. Traditionally, fulminant hepatitis B infection (HBV) was thought to be the predominant etiology of ALF in Germany. However, recent studies, conducted in American and European cohorts pointed to drug-induced liver injury (DILI) as the major cause. Aim of this study was to identify currently predominant etiologies of ALF in a monocenter study at a leading transplant center in Germany., Patients and Methods: The data of 161 patients admitted with ALF from 1/2002 to 12/2012 were analyzed retrospectively. All patients fulfilled the criteria of the "Acute Liver Failure Study Group Germany" (international normalized ratio (INR) ≥ 1.5, hepatic encephalopathy ≥ stage 1)., Results: DILI was the leading cause of ALF in this cohort. About 20 % of ALF patients with DILI died or received liver transplantats. Mortality rate was highest in ALF patients with unknown etiology and those without specific therapy available., Conclusions: In Europe ALF etiologies exhibit a North-South divide. In Germany the most common cause for ALF is idiosyncratic pharmacological intoxication followed by acute hepatitis B., (© Georg Thieme Verlag KG Stuttgart · New York.)

Living donor liver transplantation (LDLT) for patients with high model for end-stage liver disease score and acute liver failure patients have little or not gained any substantial following among Western centers because of the "donor high risk-low recipient benefit scenario" that puts the donor at a significant risk against the survival odds for a recipient who is receiving a partial graft and considered marginal by Western standards. In most Asian countries, there is sometimes no other source of live graft but a willing live liver donor. There are individual Asian center reports that conclude that LDLT has comparable outcome to deceased donor liver transplant. However, the outcomes of a large number of patients after undergoing adult LDLT for high model for end-stage liver disease scores and acute liver failure at a single center have not been investigated. Here in, we present our experience with such subgroup of patients undergoing LDLT.

Objectives: We assessed the anesthetic management and short-term morbidity and mortality in pediatrics patients who underwent an orthotopic liver transplant for fulminant hepatic failure or end-stage liver disease in a university hospital., Material and Methods: We retrospectively analyzed the records of children who underwent orthotopic liver transplant from May 2002 to May 2012. Patients were categorized into 2 groups: group fulminant hepatic failure (n=22) and group end-stage liver disease (n=19). Perioperative data related to anesthetic management and intraoperative events were collected along with information related to postoperative course and survival to hospital discharge., Results: Mean age and weight for groups fulminant hepatic failure and end-stage liver disease were 8.6 ± 2.7 years and 10.8 ± 3.8 years (P = .04) and 29.2 ± 11.9 kg and 33.7 ± 16.9 kg (P = .46). There were no differences between the groups regarding length of anhepatic phase (65 ± 21 min vs 73 ± 18 min, P = .13) and operation time (9.1 ± 1.6 h vs 9.5 ± 1.8 h, P = .23). When compared with the patients in group fulminant hepatic failure, those in group end-stage liver disease more commonly had a Glasgow Coma score of 7 or less (32% vs 6%, P = .04). Compared with patients in group fulminant hepatic failure, those in group end-stage liver disease were more frequently extubated in the operating room (31.8% versus 89.5% P < .001). Postoperative duration of mechanical ventilation (2.78 ± 4.02 d vs 2.85 ± 10.21 d, P = .05), and the mortality rates at 1 year after orthotopic liver transplant (7.3% vs 0%, P = .09) were similar between the groups., Conclusions: During pediatric orthotopic liver transplant, those children with fulminant hepatic failure require more intraoperative fluids and more frequent perioperative mechanical ventilation than those with end-stage liver disease.

The first liver transplant in the former Czechoslovakia took place in Brno on 2 February 1983; the patient concerned has lived for more than 30 years with a normally functioning liver and is one of the longest surviving patients after a liver transplant in Europe. The Cardiovascular and Transplantation Surgery Centre (CTSC) in Brno has experienced an increased development in the area of liver transplants since the mid  1990s. At present, about 30 patients a year undergo a transplantation, with 451 liver transplants in total as of 31 December 2012. The primary indication concerns liver cirrhosis of various etiologies, with an increasing number of cases of cirrhosis resulting from hepatitis C. Urgent liver transplants (for acute liver failure or primary dysfunction of first liver graft) amount to 11% of cases. There were 18 retransplants performed as of 31 December 2012, with 50% five year survival. The primary graft dysfunction was present in 7 patients (i.e. 1.5%). The 1 year survival rate of all patients after a liver transplant performed in CTSC is 92%, 5 year survival rate is 80%, and 10 year survival rate is 71%. Currently the parameters such as recipients age, donors age, and transplant waiting time has been statistically increasing; small recipients with a body weight below 70 kg especially have to wait for a significantly longer period of time (waiting median of 178 days). In CTSC Brno 14 combined simultaneous transplants (13 transplants of liver + kidney and one combined simultaneous transplants of liver + heart + kidney in 2005) have been performed as of 31 December 2012.

Background: Acute-on-chronic liver failure (ACLF) is a severe clinical condition for which liver transplantation (LT) is the only curative option. However, there are little published data on risk factors and outcomes of LT for ACLF., Methods: The objective of this study was to analyze preoperative, intraoperative, postoperative, and overall survival data on 100 consecutive cases with ACLF in order to try to determine for which patients LT are futile., Results: One hundred consecutive patients with pathology-confirmed ACLF who underwent LT from June 2004 to September 2012 were enrolled. The preoperative data showed that all patients were in a serious condition with a median high model for end-stage liver disease (MELD) score of 32, total bilirubin of 440.20 umol/L, international normalized ratio (INR) of 3.012, and at least one organ dysfunction as assessed by a Sequential Organ Failure Assessment (SOFA) score of ≥9. The patients had either deceased or a living donor LT with an overall mortality of 20%. The 1-, 3-, and 5-year cumulative survival rates were 76.8%, 75.6%, and 74.1%, respectively, and graft 1-, 3-, and 5-y accumulative survival rates were 73.3%, 72.1%, and 70.6%, respectively. However, the area under receiver operating characteristic of SOFA score, MELD score, as well as Child-Pugh score were 0.552, 0.547, and 0.547, respectively., Conclusions: Both deceased and living donor LT are effective therapeutic options for patients with ACLF and the short- and long-term survival rates are encouraging. It is important to conduct more prospective and multi-center studies to define preoperatively which patients would benefit from LT., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Background/aims: ABO-incompatible (ABO-I) liver transplantation (LTx) may be mandatory in urgent conditions such as acute liver failure (ALF) or acute-on-chronic liver failure (ACLF) when deceased donor (DD) is unavailable or living donor (LD) selection is limited. This study specifically addresses the problem of urgent ABO-I LTx in critically ill adult patients having ALF or severely decompensated end-stage liver disease., Methodology: This series included 16 patients, 10 underwent ABO-I LD LTx and 6 underwent 7 ABO-I DD LTx. Multiple sessions of plasmapheresis reduced isoaglutinin titres to 1/16 or below, before and after LTx., Results: Mean follow-up period was 10.37 months (1 to 38). Median for MELD scores was 22.5 (17 to 30). Median survival was 9 months and mean survival was 19.5 months. Hospital mortality was 3 (18.7%). Two patients died due to small for size graft syndrome and cerebrovascular bleeding respectively. Hepatic artery thrombosis developed in 3 patients. Two of them died at postoperative 4th and 9th months. Third patient is still alive with hepatic necrosis problem., Conclusions: ABO-I LTx remains an important and unavoidable therapeutic option in adult patients with ALF or ACLF and urgent need for an allograft without the possibility to allocate a blood group compatible liver graft.

Background: The aim of this study was to evaluate the outcomes of orthotopic liver transplantation (OLT) or combined liver-kidney transplantation (CLKT) for acute-on-chronic liver failure (ACLF) patients with renal dysfunction., Methods: From January 2001 to December 2009, 133 patients underwent OLT for ACLF at our center. Among them, 30 had both ACLF and renal dysfunction. Of the 30 patients, 12 underwent CLKT for end-stage renal disease (ESRD), and the other 18 with hepatorenal syndrome type 1 (HRS1) underwent OLT alone. ACLF was defined according to the Asian Pacific Association for the Study of Liver Consensus Meeting. Clinical data were reviewed for survival outcomes., Results: The median model for end-stage liver disease score (MELD) of patients with ACLF was 28. Among the 133 patients, all of whom received deceased donor liver grafts, 12 also got the kidney grafts from the same deceased donor. The hospital mortality rate was 21.8% for all patients with ACLF. The 5-year survival rates were 72.8% for patients without renal dysfunction and 70% for patients with renal dysfunction. The results of patients with ESRD who underwent CLKT were better than those of subjects without renal dysfunction or patients with HRS1 who underwent OLT alone., Conclusions: OLT alone improved renal function in most patients with HRS1, including those requiring short-term hemodialysis. Simultaneous liver-kidney transplantation was an excellent strategy for patients with both ACLF and ESRD. It provided protection to the kidney allograft for liver-based metabolic diseases affecting the kidney. The rate of acute rejection episodes in kidneys was low., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Background: Acute liver failure (ALF) is a devastating clinical syndrome with a high mortality rate. The MELD score has been implied as a prognostic tool in ALF. Hyponatremia is associated with lethal outcome in ALF. Inclusion of serum sodium (Na) into the MELD score was found to improve its predictive value in cirrhotic patients. Therefore the aim of this study was to determine whether inclusion of serum Na improves the predictive value of MELD in ALF compared to established criteria., Methods: In a prospective single center study (11/2006-12/2010), we recruited 108 consecutive ALF patients (64% females / 36% males), who met the criteria defined by the "Acute Liver Failure Study Group Germany". Upon admission, clinical and laboratory data were collected, King's College Criteria (KCC), Model of End Stage Liver Disease score (MELD), and serum sodium based modifications like the MELD-Na score and the United Kingdom Model of End Stage Liver Disease score (UKELD) were calculated and area under the receiver operating characteristic curve analyses were performed regarding the prediction of spontaneous recovery (SR) or non-spontaneous recovery (NSR; death or transplantation)., Results: Serum bilirubin was of no prognostic value in ALF, and Na also failed to predict NSR in ALF. The classical MELD score was superior to sodium-based modifications and KCC., Conclusions: We validated the prognostic value of MELD-Na and UKELD in ALF. Classic MELD score calculations performed superior to KCC in the prediction of NSR. Serum Na and Na-based modifications of MELD did not further improve its prognostic value.

Recipients of liver transplantation (LT) receive lifelong immunosuppression, which causes side effects. We investigated self-reported oral symptoms and associated risk factors with the following hypothesis: symptoms and signs would differ between LT recipients of different etiology groups and also between LT recipients and a control population. Eighty-four LT recipients (64 with chronic liver disease and 20 with acute liver disease) were recruited for clinical oral and salivary examinations (median follow-up = 5.7 years). A structured questionnaire was used to record subjective oral symptoms. Matched controls (n = 252) came from the National Finnish Health 2000 survey. The prevalence of symptoms was compared between the groups, and the risk factors for oral symptoms were analyzed. Xerostomia was prevalent in 48.4% of the chronic LT recipients and in 42.1% of the acute LT recipients. This subjective feeling of dry mouth was only partly linked to objectively measured hyposalivation. The chronic transplant recipients had significantly lower unstimulated salivary flow rates than the acute transplant recipients (0.34 ± 0.31 versus 0.61 ± 0.49 mL/minute, P = 0.005). Among the chronic transplant recipients, hyposalivation with unstimulated salivary flow was associated with fewer teeth (17.7 ± 8.2 versus 21.9 ± 8.4, P = 0.047) and more dentures (33.3% versus 12.2%, P = not significant). The chronic patients reported significantly more dysphagia than their controls (23.4% versus 11.5%, P = 0.02). Increases in the number of medications increased the symptoms in all groups. In conclusion, dysphagia was significantly more prevalent among the chronic LT recipients versus the controls. The number of medications was a risk factor for dry mouth-related symptoms for both the LT recipients and the controls. The chronic transplant recipients presented with lower salivary flow rates than the acute transplant recipients. Hyposalivation correlated with generally worse oral health among the chronic transplant recipients. These differences between the chronic and acute LT recipients may have been due to differences in their medical conditions due to the different etiologies., (Copyright © 2012 American Association for the Study of Liver Diseases.)

Background and Aim: Although establishing accurate prognosis in acute liver failure (ALF) is of paramount importance, prognostic scoring systems still fail to achieve success. The pediatric end-stage liver disease (PELD) score has been used as a predictor of mortality in children with chronic liver disease listed for liver transplantation (LT); however, experience with the PELD score in ALF is limited. The goal of the present study was to investigate the prognostic accuracy of the PELD score in children with ALF., Patients and Methods: PELD score was calculated based on results of blood tests obtained at hospital admission from June 1999 to January 2009, in 40 consecutive patients younger than 18 years who presented with ALF. Poor outcome was defined as LT or death., Results: Mean (±SD) age of patients was 5.3 ± 4.4 years (range 6 months-17 years); 52.5% were girls (n = 21). Etiologies of ALF were hepatitis A in 42.5% (17), indeterminate in 35% (14), autoimmune hepatitis in 17.5% (type 1 12.5% [n5], type 2 5% [n2]), and toxic in 5% (2). Mean PELD score was 34.92 ± 10.48 (range 6-55). PELD scores obtained on admission were significantly higher among nonsurvivors (39.8 ± 9.5) and recipients of an LT (39 ± 7.1) compared with those who survived without LT (31.3 ± 3) (P < 0.001). A cutoff of 33 in PELD score using receiver operating characteristic curves showed 81% specificity and 86% sensitivity for poor outcome (positive predictive value 92% and negative predictive value 69%; area under curve 0.88 95% confidence interval 0.77-1.0; P < 0.0001)., Conclusions: PELD score obtained upon admission may be of help to establish the optimal timing for LT evaluation and listing. Further validation in larger and more diverse populations is needed.

Background: Bile duct injury (BDI) is a severe complication that may arise during the surgical treatment of benign disease and a few patients will develop end-stage liver disease (ESLD) requiring a liver transplant (LT)., Objective: Analyse the experience using LT as a definitive treatment of BDI in Argentina., Patients and Methods: A national survey regarding the experience of LT for BDI., Results: Sixteen out 18 centres reported a total of 19 patients. The percentage of LT for BDI from the total number of LT per period was: 1990-94 = 3.1%, 1995-99 = 1.6%, 2000-04 = 0.7% and 2005-09 = 0.2% (P < 0.001). The mean age was 45.7 ± 10.3 years (range 26-62) and 10 patients were female. The BDI occurred during cholecystectomy in 16 and 7 had vascular injuries. One patient presented with acute liver failure and the others with chronic ESLD. The median time between BDI and LT was 71 months (range 0.2-157). The mean follow-up was 8.3 years (10 months to 16.4 years). Survival at 1, 3, 5 and 10 years was 73%, 68%, 68% and 45%, respectively., Conclusions: The use of LT for the treatment of BDI declined over the review period. LT plays a role in selected cases in patients with acute liver failure and ESLD., (© 2011 International Hepato-Pancreato-Biliary Association.)

Background/aims: This study reports our preliminary experience of living donor liver transplantation (LDLT) for patients with acute-on-chronic liver failure (AoCLF) caused by hepatitis B., Methodology: 47 patients who demonstrated Ao- CLF caused by hepatitis B with mean (±SD) Model for End-Stage Liver Disease scores of 39.2±5.1 were divided by the transplantation group (n=19) and the non-transplantation group (n=28) according to whether or not undergoing LDLT. At the same time, 30 hepatitis B cirrhosis recipients who underwent LDLT and did not reach the criteria of AoCLF were selected as the control group (n=30). In the transplantation group, veno-venous bypass, molecular adsorbent recirculating system (MARS) and continuous renal replacement therapy (CRRT) were introduced. The intraoperative data, post-transplant complications and mortality were analyzed retrospectively., Results: There were no significant differences in survival rates of 1, 6 and 12 months and the postoperative complications except for pneumonia and diabetes, between the control group and the transplantation group (p>0.05). Recurrence of hepatitis B was not found in the recipients of the control group and the transplantation group., Conclusions: Right-lobe LDLT may be an effective therapeutic option for patients with acute-on-chronic hepatitis B liver failure.

Purpose of Review: Circulatory abnormalities in cirrhosis include hyperdynamic circulation and cirrhotic cardiomyopathy. The extent of circulatory abnormalities is further exaggerated in acute-on-chronic liver failure (ACLF). The mechanism remains unclear and management also needs to be evaluated., Recent Findings: The predominant mechanism of ACLF is thought to be a systemic inflammatory reaction. Cardiovascular-active factors such as tumor necrosis factor and nitric oxide are increased and cortisol is decreased; the former further dilates the vasculature and the latter decreases the sensitivity to vasoconstrictors. The exaggerated vasodilatation further decreases the cardiac afterload. However, no study has yet demonstrated the benefit of vasodilators/vasoconstrictors in the management of ACLF. Standard medical treatment in this setting is associated with high mortality. Patients treated with molecular adsorbent recirculating system (MARS) had improved serum levels of inflammatory mediators such as tumor necrosis factor alpha and interleukin-6, but this was not associated with improved survival. Liver transplantation eventually reverses the cardiovascular abnormalities., Summary: Circulatory abnormalities are exaggerated in ACLF. The predominant mechanism is a systemic inflammatory reaction. Modalities such as MARS improve serum markers of inflammation, but not survival. Liver transplantation is the definitive treatment of the cardiovascular abnormalities of ACLF.

Unlabelled: Liver transplantation (LT) is lifesaving for patients with Wilson disease (WD) presenting with fulminant hepatic failure (FHF) or chronic liver disease (CLD) unresponsive to treatment., Aim: To determine the outcome of LT in pediatric and adult patients with WD., Methods: United Network for Organ Sharing data on LT from 1987 to 2008 were analyzed. Outcomes were compared for patients requiring LT for FHF and CLD after 2002. Multivariate logistic regression was used to determine risk factors for death and graft loss., Results: Of 90,867 patients transplanted between 1987 and 2008, 170 children and 400 adults had WD. The one- and five-yr patient survival of children was 90.1% and 89% compared to 88.3% and 86% for adults, p = 0.53, 0.34. After 2002, 103 (41 children) were transplanted for FHF and 67 (10 children) for CLD. One- and five-yr patient survival was higher in children transplanted for CLD compared to FHF; 100%, 100% vs. 90%, 87.5% respectively, p = 0.30, 0.32. One- and five-yr patient survival was higher in adults transplanted for CLD compared to FHF; 94.7%, 90.1% vs. 90.3%, 89.7%, respectively, p = 0.36, 0.88. Encephalopathy, partial graft, and ventilator use were risk factors for death by logistic regression., Conclusion: LT is an excellent treatment option for patients with WD. Patients transplanted for CLD had higher patient survival rates than patients with FHF., (© 2010 John Wiley & Sons A/S.)