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Effect of ABO-incompatible liver transplantation on patients with severe hepatitis B (SHB) remains unclear. Herein, we summarized 22 cases with SHB in whom were performed emergency liver transplantation from ABO-incompatible donors. The immunosuppressive protocol consisted basiliximab, tacrolimus, steroids and mycophenolate mofetil. The mean MELD score was 35.2 ± 7.1. Major complications included rejection, infections, biliary complications, hepatic artery thrombosis or stenosis and portal vein thrombosis. Patient survival rates were 40.9%, 78.9% and 82.3% in 1 year, 29.2%, 66.8% and 72.9% in 3 years, and 21.9%, 60.1% and 62.5% in 5 years for ABO-incompatible, ABO-compatible and ABO-identical groups. Graft survival rates were 39%, 78.9% and 82.3% in 1 year, 27.8%, 66.4% and 71.1% in 3 years, and 20.9%, 57.9% and 61.0% in 5 years for incompatible, compatible and identical ABO graft-recipient match. The 1-, 3-, 5-year graft and patient survival rates of ABO-incompatible were distinctly lower than that of ABO-compatible group (P < 0.05). Our results suggested that ABO-incompatible liver transplantation might be a life-saving procedure for patients with SHB as a promising alternative operation when ABO-compatible donors are not available and at least bridges the second opportunity for liver retransplantation., (© 2015 Steunstichting ESOT.)

Factors predicting survival after liver transplantation (LT) for irreversible acute liver failure (ALF) are rare. The aim of this study was to identify prognostic preoperative factors of patients with ALF that predict mortality after LT to avoid futile transplantation. From chart review, we identified 57 patients receiving transplants for ALF from 12/2000 to 09/2010. Recipient and donor data were analyzed and correlated with in-hospital mortality and patient survival by univariable/multivariable logistic regression and Cox proportional hazards. The survival rates at 30 days and 12 months were 77.2% and 64.9%, respectively. The in-hospital mortality rate was 29.8%. Follow-up of patients discharged from the hospital alive showed 30-day and 12-month survivals of 100% and 92.5%, respectively. Multivariable analysis of factors known preoperatively showed that the lowest pH of the recipient before LT (P = 0.03) was independently associated with in-hospital mortality, and the recipient's BMI (P = 0.03) and the lowest pH before LT (P = 0.03) were independently associated with patient survival. A pH of 7.26 was the calculated cutoff (ROC) for increased in-hospital mortality. Donor factors did not affect patient survival. Patients with ALF and a pH ≤ 7.26 have the worst outcome after liver transplantation. Therefore, emergency liver transplantation should be critically discussed for each individual., (© 2014 Steunstichting ESOT.)

Macrophage activation syndrome (MAS) is a rare, potentially fatal condition, which most frequently complicates rheumatological conditions and is often associated with liver dysfunction. In this case report of a patient with MAS, acute liver failure developed despite conventional immunosuppressive therapy. Liver transplantation resulted in rapid recovery and the patient has remained well for six years. A recent diagnosis of Adult Onset Still's Disease (AOSD) provides additional supporting evidence that the initial presentation was caused by MAS. While transplantation in the context of systemic disease remains controversial, this first reported case of successful adult liver transplantation for acute liver failure caused by MAS raises an interesting clinical dilemma., (© 2013 Steunstichting ESOT. Published by John Wiley & Sons Ltd.)

Living donor liver transplantation (LDLT) is a well-established strategy to decrease the mortality in the waiting list and recent studies have demonstrated its value even in patients with low MELD score. However, LDLT is still under a high level of scrutiny because of its technical complexity and ethical challenges as demonstrated by a decline in the number of procedures performed in the last decade in Western Countries. Many aspects make LDLT different from deceased donor liver transplantation, including timing of transplantation, procedure-related complications as well as immunological factors that may affect graft outcomes. Our review suggests that in selected cases, LDLT offers significant advantages over deceased donor liver transplantation and should be used more liberally., (© 2012 The Authors Transplant International © 2012 European Society for Organ Transplantation.)

Acute liver failure (ALF) is a clinical syndrome associated with significant morbidity and mortality with a highly unpredictable outcome. We retrospectively analyzed 71 ALF patients (53 males; mean age = 27.5 ± 15.6 years) that underwent transjugular liver biopsy (TJLB) at our institution. The aims of this study are (i) to report our experience with TJLB in these patients, and (ii) to examine the role of liver histology in predicting their outcome. We also compared the histopathological findings between TJLB and explanted liver specimens in 31 patients who underwent liver transplantation (LT). Biopsy specimens were satisfactory for histopathological analyses in 69 (97.1%) patients, confirmed the clinical diagnosis in 56 (81.2%) patients, and altered the diagnosis in 13 (18.8%) patients. Minor complications were encountered in four (5.6%) patients. Percentage of hepatocyte necrosis was the only histological parameter that has significant discriminatory prognostic value, with no survivors having >75% necrosis without LT. In conclusions, TJLB is a safe technique for obtaining liver tissue in both adult and pediatric patients with ALF. Histological characteristics, mainly etiological diagnosis and degree of hepatocyte necrosis may assist in clinical decision-making for need of LT in these patients., (© 2012 The Authors. Transplant International © 2012 European Society for Organ Transplantation.)

Nowadays, most paediatric liver transplant recipients receive a split or other technical variant graft from adult deceased or live donors, because of a lack of available age- and size matched paediatric donors. Few data are available, especially for liver grafts obtained from very young children (<6 years). We analysed all paediatric liver transplantations between 1989 and 2009. Recipients were divided into five groups (1-5) depending on donor age (<1, ≥1 to <6, ≥6 to <16, ≥16 to <45, ≥45 years). Overall, 413 paediatric liver transplantations from deceased donors were performed; 1- and 5-year graft survival rates were 75%, 80%, 78%, 81%, 74% and 75%, 64%, 70%, 67%, 46%, and 1- and 5-year patient survival rates were 88%, 91%, 90%, 89%, 78% and 88%, 84%, 84%, 83%, 63% for groups 1-5, respectively, without significant difference. Eight children received organs from donors younger than 1 year and 45 children received organs from donors between 1 and 6 years of age. Overall, vascular complications occurred in 13.2% of patients receiving organs from donors younger than 6 years. Analysis of our data revealed that the usage of liver grafts from donors younger than 6 years is a safe procedure. The outcome was comparable with grafts from older donors with excellent graft and patient survival, even for donors younger than 1 year., (© 2011 The Authors. Transplant International © 2011 European Society for Organ Transplantation.)

We reviewed 57 pediatric patients admitted with acute liver failure to Kyoto University Hospital in Japan over a period of 15 years to compare the etiology and the long-term outcome of infants and children after living donor liver transplantation (LDLT). Patients were divided into two groups according to age at the time of liver transplantation, infants group (<1 year, n = 20), and children group (1-18 years, n = 37). The overall survival rates were 73.6%, 69.5% and 67.2% at 1, 5, and 10 years after LDLT respectively. Age of recipients at the time of LDLT had a strong impact on their outcome, Children had significantly better outcome than infants (P = 0.001). Surgical complications were comparable between both groups. Infants had higher rates of acute cellular rejection (ACR), which was associated with features of hepatitis in many cases. Refractory ACR was the leading cause of death in eight out of 12 infants, while it resulted in loss of one child only. Cox's proportional hazard regression model was used to examine potential risk factors for graft loss and it shows that age <1 year was associated with high risk of graft loss [hazard ratio (HR) = 11.393; CI = 1.961-76.1763] (P < 0.05). In conclusion, Infants had poorer prognosis than children and refractory ACR was the leading cause of death. Using additional immunosuppressant for cases with severe and atypical rejections is recommended.

Summary: Perioperative infections remain an important problem for patients undergoing liver transplantation (LT). For prevention of these infections, perioperative prophylaxis has become the standard procedure. Yet, either guidelines or data on current practice are lacking. The aim of the study was to gain insight into prophylactic antimicrobial strategies used in Europe. A survey questionnaire was sent out to all LT centers that are member of the European Liver and Intestine Transplant Association. In the survey questionnaire, we asked for details on the prophylactic antimicrobial regimen used in LT recipients. The response rate was 48%. Antibiotic prophylaxis for elective LT was provided by a first-line betalactam antibiotic or co-trimoxazole in 25%. Seventy-three per cent of those centers surveyed gave an extended spectrum, and one center used a 6-month rotation strategy. Antifungal prophylaxis was administered in 35% of centers in all LT recipients, in 53% of centers in patients at risk, and in 12% of centers not at all. Cytomegalovirus prophylaxis was never administered in 10%. In 12% of the centers surveyed, all the patients received cytomegalovirus prophylaxis, and another 78% of the centers gave it only to risk groups. In Europe, there is a considerable variation in the different antibiotic, antifungal and cytomegalovirus prophylactic strategies used for LT. These findings underscore the need for randomized controlled trials to determine the optimal prophylactic antimicrobial regimen.

To analyze the outcomes between identical and compatible liver transplantation (OLT) for fulminant hepatic failure (FHF) from September 1984 to November 2005. The patients were divided in three groups; group 1 (identical), group 2 (compatible) and group 3 (incompatible), according to the donor-recipient blood type matching. We analyzed several outcomes regarding mortality, patient and graft survival, incidence of acute graft rejection during the first postoperative month (30 days), incidence of biliary complications and indications of re-transplantation. We also analyzed the relationship of Coomb's positive test with postoperative hemolysis to all the above mentioned factors. During the study period, 168 males and 112 females underwent their first OLT for FHF, with 37.1% overall mortality and 42.1% overall graft failure rate. The results between group 1 (203 patients) and group 2 (73 patients) were comparable. A statistically significant difference was recorded in 1 year and overall graft survival between group 1 and group 2 (P = 0.049 and log-rank = 0.035 respectively). Coomb's positive test did not influence the outcomes. OLT in FHF can be safely carried out whether the donor organs are identical or compatible. Hemolysis (Coomb's positive test) after identical or compatible OLT does not influence the outcomes.

Uncontrollable intracranial pressure elevation in hyperacute liver failure often proves fatal if no suitable liver for transplantation is found in due time. Both ABO-compatible and auxiliary partial orthotopic liver transplantation have been described to control such scenario. However, each method is associated with downsides in terms of immunobiology, organ availability and effects on the overall waiting list.

To report our experience with 17 children who underwent a liver transplantation (LT) for acute liver failure (ALF). All LT procedures (deceased and living donor) were offered. Since 2003 Molecular Adsorbents Recycling System (MARS) was proposed as bridging procedure. We monitored the perioperative course and the short- and long-term outcomes. All children developed pretransplant hepatic encephalopathy (mostly grades II and III); six needed ventilator support and three haemodialysis. Median PELD/MELD score was 30. MARS was used in five children with poor pretransplant prognostic factors: all five survived the LT without sequelae. We performed 13 deceased donor LT (seven whole, five split and onr reduced) and four left lateral LDLT. Postoperative complications were observed in 10 children, requiring re-operation in seven. Two children developed irreversible neurological disorders. After a median follow up of 45 months, 16 children are still alive. About 1- and 5-year cumulative patient survival rates are 94% with a corresponding graft survival of 88% and 81%, respectively. The combination of experienced paediatric ICU management, the application of new liver support devices, and the capacity to offer both living and deceased donor transplant alternatives in a timely fashion represent the best formula to achieve optimal results in children with ALF.

The introduction of living donor liver transplantation (LDLT) has been one of the most remarkable steps in the field of liver transplantation (LT), able to significantly expand the scarce donor pool in countries in which the growing demands of organs are not met by the shortage of available cadaveric grafts. Although the benefits of this procedure are enormous, the physical and psychological sacrifice of the donors is immense, and the expectations for a good outcome for themselves, as well as for the recipients, are high. We report a current overview of the latest trends in live liver donation in its different aspects (i.e. donor's selection, evaluation, operation, morbidity, mortality, ethics and psychology). This review is based on our center's personal experience with almost 200 LDLTs and a detailed analysis of the international literature of the last 7 years about this topic. Knowing in detail how to approach to the different aspects of living liver donation may be helpful in further improve donor's safety and even recipient's outcome.

There is no agreement regarding the treatment of early allograft rejection (EAR) in adult living donor liver transplantation (LDLT). A protocol biopsy was performed in 62 adult LDLT recipients. Twenty-one patients (33.9%) had histological evidence of EAR. Of these, 14 patients had biochemical abnormalities and seven patients had no associated biochemical abnormalities. None of the seven patients with subclinical EAR (11.3% of the entire study population) were treated, and no subsequent rejection was observed. Gender mismatch (female-to-male) was the single independent risk factor for histological EAR [odds ratio (OR) = 13.458; 95% confidence interval (CI), 1.836-98.649] and the cumulative probability for a subsequent rejection was higher in patients with EAR (OR = 11.085; 95% CI, 1.221-100.654). However, the actuarial 1 year patient and graft survival rate in patients with EAR (81.0% and 85.5%) were similar to those without EAR (92.7% and 97.25%; P = 0.127 and 0.302, respectively). The presence of an initial biochemical abnormality was an independent risk factor for both a decreased patient survival (OR = 5.827; 95% CI, 1.095-31.017; P = 0.039) and graft loss (OR = 20.646; 95% CI, 2.044-208.524; P = 0.010). Subsequent rejection developed more frequently in patients with EAR. However, the survival is not determined by the presence of EAR but by the presence of a biochemical abnormality.

Persistance of a large spontaneous splenorenal shunt (SRS) may result in graft failure in adult living donor liver transplantation (LDLT) because it reduces the effective portal perfusion to the partial liver graft by diversion of hepatotrophic portal flow into this hepatofugal pathway. We performed a prospective study to evaluate the efficacy of ligation of left renal vein (LRV) to prevent portal flow steal and the safety of this procedure to the renal function in adult LDLT patients with SRS. Between October 2001 and January 2005, 44 cirrhotic patients with large SRS underwent LDLT with ligation of LRV. Each patient received pre- and postoperative computed tomography and Doppler USG to assess the changes of collaterals and portal flow, as well as serial renal and liver function tests. Portal flow after ligation of LRV was statistically and significantly increased when compared with pre-operative value (P = 0.001). Whereas four patients (9.1%) demonstrated sustained, elevated serum creatinine levels after operation, the renal function tests returned to normal in 40 patients. All patients recovered with satisfactory regeneration of the partial liver graft and there was no procedure-related permanent renal dysfunction. In conclusion, ligation of LRV to prevent a 'portal steal phenomenon' seems to be a safe and effective graft salvage procedure for large spontaneous SRS (>10-mm diameter) in adult LDLT.

Fulminant hepatic failure (FHF) is a devastating disease. Liver transplantation is the definitive treatment. However, a third of these patients die due to brain edema before a donor becomes available. Cerebrospinal fluid (CSF) drainage and decompressive craniectomy have been used to treat brain edema in brain trauma and hemispheric stroke. However, their role in brain edema associated with FHF has not been examined. In this study we evaluated the potential effects of CSF drainage and decompressive craniectomy on survival in FHF using an experimental model in rats. In CSF drainage experiments all animals had ventriculostomy placed. Five days later FHF was induced with d-galactosamine. Those FHF rats that progressed into comatose stages either received CSF aspiration or did not. In separate experiments the study rats had either a decompressive craniectomy or a sham procedure. FHF was induced 5 days later. We found that both CSF drainage and decompressive craniectomy significantly increased survival of FHF rats compared with the controls: 53.2 +/- 1.1 vs. 48.7 +/- 1.5 h (P = 0.031), and 69.4 +/- 3.9 vs. 53.7 +/- 3.2 h (P = 0.009), respectively. In conclusion, these findings suggest that CSF drainage and decompressive craniectomy may increase the window of opportunity for liver transplantation.

We report the first case of auxiliary partial orthotopic liver transplantation (APOLT) in a patient with isoniazid (INH)-related fulminant hepatic failure (FHF) with the aim to determine the ability of the native liver (NL) to recover after this particular toxic event. A 10-year-old boy with INH-related FHF underwent APOLT after left hepatectomy on the NL. Neurological status and liver function rapidly improved, but, on postoperative day 22, urgent re-transplantation was needed for graft-hepatic artery thrombosis (HAT) and the NL's incapacity to sustain adequate liver function. Histological examination of the NL showed signs evident of its regeneration, however. In conclusion, though we faced the clinical failure of the NL functionally to sustain the patient in the presence of the graft HAT 3, weeks after APOLT, such a failure may be interpreted as time related. In fact, the histological picture in this particular case may suggest the potential for NL recovery after INH-related FHF.

The Model for End-stage Liver Disease (MELD) provides a score able to predict short-term mortality in patients awaiting liver transplantation (LT). In the early 2002, United Network for Organ Sharing (UNOS) has proposed to replace the conventional statuses 3, 2B, and 2A with a modified MELD score. However, the accuracy of the MELD model to predict post-transplantation outcome is fairly elusive. In the present study we investigated the predictive value of the MELD score for short-term patient and graft mortality in comparison with conventional UNOS status. Sixty-nine patients listed at UNOS status 3 (n = 5), 2B (n = 55) or 2A (n = 9) who underwent LT were enrolled according to strict criteria. No donor-related parameters affected 3-month patient survival. Through univariate Cox regression, pretransplantation international normalized ratio (P = 0.049) and activated partial thromboplastin time (P = 0.032) were significantly associated with 3-month patient survival, although not in the subsequent multivariate analysis. The overall MELD score was 17 +/- 6.63 (median: 16, range: 4-34), increasing from UNOS Status 3 to 2A (r(2) = 0.171, P = 0.0001). No significant difference occurred in the median MELD score between patients who underwent a second LT and those who did not (P =0.458). The inter-rate agreement between UNOS status and MELD score after categorization for clinical urgency showed a fair agreement (kappa = 0.244). The 3-month patient and graft mortality was 15.94% and 20.29% respectively. The concordance statistic did not find significance between UNOS status and MELD score for 3-month patient (P = 0.283) or graft mortality (P = 0.957), although the MELD score revealed a major sensitivity for short-term patient mortality (0.637; 95%CI: 0.513-0.75). These findings suggest the need to implement MELD model accuracy for both inter-rate agreement with UNOS Status and patient outcome.

Liver allograft re-use is an exceptional way of enlarging the donor pool. We describe here a case of a re-used liver allograft, originating from an insulin-intoxicated donor and transplanted at first into a recipient presenting with hyperacute liver failure due to paracetamol intoxication. Because the original recipient developed an irreversible cerebral oedema, the allograft was re-implanted electively 55.5 h later into a patient with post-viral C cirrhosis and solitary hepatocarcinoma. Both donor and recipient operations were technically successful; liver function after the second use of the graft was normal.

Selection of patients with acute hepatic failure for liver transplantation remains difficult, and there is no definite proof of a survival effect. We therefore did a retrospective study in 75 consecutive patients referred over a 12-year period. In two-thirds we identified a cause, mostly viruses or drugs. Patients were grouped by the Clichy and King's College criteria. In 20 there was no indication for transplantation. Of the 5 with autoimmune hepatitis, 3 died, significantly differing from the other 15 ( P = 0.009). The remaining 55 met our criteria, except 1. All 9 patients with absolute contraindications died. Of the 46 enlisted, 7 died without transplantation. One-year survival after transplantation was 69%, compared with 58% by "intention to treat." For patients enlisted, transplantation reduced mortality by 78% ( P = 0.069). The Clichy and King's College criteria reliably predict survival without transplantation, except in autoimmune hepatitis. Our study strongly suggests that transplantation improves survival.

Methylenedimethoxymethamphetamine (MDMA), more commonly known as ecstasy, is a synthetic amphetamine derivative used by teenagers and young adults in the United States as well as in Western Europe as a "dance drug". Though a number of complications associated with this drug have been reported, there is little information pertaining to hepatoxity as a result of MDMA ingestion. This case report is about an 18-year-old female patient who regularly used ecstasy on weekends over a 2-month period. Within 2 days after accepting a "hit" of the substance at a party, she was admitted to the hospital because of lethargy, vomiting, abdominal pain, stool discoloration, icterus, and darkened urine. On day 7 she developed fulminant hepatic failure with reduced hepatic coagulation factors and grade IV encephalopathy. Orthotopic liver transplantation was carried out 10 days following the ingestion. The patient made a full recovery within 72 h and was released from the hospital 6 weeks later. Histopathological examination of the removed liver revealed a nutritive-toxic liver necrosis. This case demonstrates that the ingestion of ecstasy, even on an infrequent basis, can lead to acute fulminant liver necrosis, and that this life-threatening complication can be treated successfully by liver transplantation.

Thirty children received 35 liver transplants for fulminant or late-onset liver failure between March 1988 and May 1993. Aetiology included non-A non-B hepatitis in 12, Wilson's disease in 8, drug-induced hepatic failure in 6, hepatitis B in 1, hepatitis A in 1, tyrosinaemia in 1 and congenital haemochromatosis in 1. Three patients were retransplanted, one each for hepatic artery thrombosis, non-A non-B graft reinfection, and chronic rejection. Two of these three patients received a third transplant for chronic rejection and hepatic artery thrombosis. One patient in the retransplant group survived. Overall, graft and patient survival at a mean follow-up of 17 months were 49% and 57%, respectively. Mortality was related to vascular complications in three patients (hepatic venous obstruction, portal vein thrombosis and hepatic artery thrombosis). Two patients died of primary sepsis (cerebral aspergillosis and cytomegalovirus (CMV) pneumonitis in association with graft-versus-host disease). Systemic sepsis and multiorgan failure was documented as a cause of death in four children and sepsis in association with chronic rejection in a further three patients. One child died of respiratory failure 4 weeks after transplantation. Mortality in eight children less than 2 years was 75% and this was significantly greater than for older children (P < 0.003, Mantel Cox). Earlier referral, even in the absence of a definitive diagnosis and particularly in children under 2 years is advisable and may improve survival.

Fulminant liver failure due to acute viral hepatitis is the most common emergency indication for liver transplantation. The postoperative course is highly correlated with the type and duration of infection. The complication rate is lowest in fulminant hepatitis B patients and highest in subacute hepatitis C/NANB patients.