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Focal nodular hyperplasia (FNH) is a benign nodular lesion, but because of its feature of portal tract vessel abnormality, it may induce portal hypertension. A 27-year-old woman was admitted with a fever. A large nodule with satellite lesions was found in the liver and cotton wool-like feature of arteries were detected on angiography. Technetium galactosyl serum albumin scintigraphy and diagnostic laparoscopy showed that the tumor site was functional, while the surrounding area was a non-functional fibrotic area. A biopsy specimen indicated that the nodular lesion was an FNH-like lesion. She experienced several instances of variceal rupture and suffered liver failure, receiving liver transplantation. The excised liver showed a centrally scarred area in the nodule, indicating that the diagnosis was FNH. We herein report this case as a rare case of FNH that progressed to liver failure., (© 2021. Japanese Society of Gastroenterology.)

Recurrent autoimmune hepatitis (rAIH) occurs in patients who undergo liver transplantation (LT) for AIH and de novo AIH (dAIH) is seen in patients who are transplanted for etiologies other than AIH. Whether these are distinct diseases with a similar phenotype remains understudied. The aim of this study was to identify clinical and immunologic factors affecting outcome in patients with dAIH and rAIH. A retrospective review of 387 LT patients from 1997 to 2014 was carried out, and they were followed until 2018. Patients with rAIH or dAIH were identified based on the pre-transplant diagnosis of AIH (or not) and characteristic histology. Liver biopsies were stained with H&E, B-cell marker CD20, and plasma cell marker CD138. Out of 387 patients, 31 were transplanted for AIH, and 8/31 developed rAIH. Of the remaining 356 patients, eight developed dAIH. Compared to the dAIH group, rAIH occurred in older patients, had an earlier onset in the allograft, and had higher IgG and serum ALT levels. It was most commonly seen in African American (AA) patients (87%). rAIH patients had significantly higher CD20 and CD138 positivity in liver biopsies. In addition, they had increased rejection episodes prior to the onset of recurrence, increased graft loss, and mortality. rAIH is a more aggressive disease, and has a preponderance of B cells and plasma cells in the liver tissue as compared to dAIH. The concurrent association with increased graft loss and patient mortality in rAIH warrants further investigations into B cell-targeted therapies., (© 2021 Wiley Periodicals LLC.)

Anaplastic large cell lymphoma (ALCL) accounts for 10-15% of childhood non-Hodgkin lymphoma cases; it is generally chemo-sensitive and is one of the most curable pediatric cancers. We report here a case of pediatric ALCL complicated with acute liver failure due to the aggravation of pre-existing biliary hepatopathy by lymphoma-associated hemophagocytic lymphohistiocytosis (HLH). Although the initial treatment response against ALCL was very good, poor and irreversible liver function due to biliary cirrhosis worsening by lymphoma-associated HLH prevented the patient from receiving further consolidation chemotherapies. To make matters worse, his condition was accompanied with intrahepatic fungal pseudoaneurysm and invasive fungal infection. Thus, we decided to perform an urgent living-donor liver transplantation from his father to correct the patient's liver function and make it possible to proceed with further ALCL therapy. After the living-donor liver transplantation, the patient successfully received consolidation therapy with brentuximab vedotin. To our knowledge, this may be an early reported case of a pediatric patient undergoing liver transplantation during treatment for ALCL. In most patients with HLH-associated ALCL, liver function improves when ALCL is controlled. However, acute liver failure is occasionally observed in HLH cases with pre-existing liver dysfunction. In such cases, liver transplantation should be considered to correct liver dysfunctions if the disease control of HLH is satisfactory.

We describe the case of a 4-year-old male with a past medical history significant for nephrotic syndrome, short-bowel syndrome and fulminant hepatic failure status post (s/p) liver transplant (LT) who developed early post-transplant allograft dysfunction (hyperbilirubinemia, coagulopathy) and septic shock requiring central extracorporeal membrane oxygenation (ECMO). He remained on ECMO for 85 hours before he was decannulated without event and later underwent repeat LT. This case highlights the potential of central ECMO to provide the circulatory output necessary to reverse distributive shock physiology in patients with sepsis and hepatic dysfunction following LT. Furthermore, this is the first documented example of central ECMO as a bridge to recovery for repeat LT.

Objectives: Paracetamol overdose is common and can lead to fulminant hepatic failure. In cases that are not improving with standard medical therapy with N‐acetylcysteine, some patients may require liver transplant. The Australia and New Zealand (ANZ) referral criteria for transfer to a liver unit have not been extensively studied for its predictive value. The aim of this study was to evaluate the ANZ referral criteria for predicting mortality in paracetamol overdose. Methods: This study involves a retrospective analysis of patients who developed hepatotoxicity post‐paracetamol overdose presenting to an Australian health service with a liver transplant unit between 2010 and 2019 and were treated with N‐acetylcysteine. The primary outcome was death or transplant. Results: Out of 983 paracetamol overdose presentations, 81 (8.2%) cases developed hepatotoxicity. Of these, 17 cases (21%) met the composite endpoint of death or transplant. The ANZ referral criteria is highly sensitive at predicting the primary endpoint of death or transplant at time of referral 100% (95% confidence interval 81–100) but had low specificity at 30% (95% confidence interval 19–42). Conclusions: The ANZ referral criteria were highly sensitive for predicting the outcome of mortality and transplant. This is important for screening patients who may become unstable and difficult to transfer at a later stage of their admission. [ABSTRACT FROM AUTHOR]

The occurrence of hepatotoxicity following etanercept (tumor necrosis factor-alpha antagonist) prescription, has been studied well. However, an acute hepatic failure leading to liver transplant as an adverse effect of this drug has not been reported in the literature. In this article, we are going to present a case of acute liver failure followed by liver transplantation, in a 32 years old man with a previous history of ankylosing spondylitis after etanercept administration. On pathologic examination of the explanted liver, extensive confluent necrosis in all liver segments, as well as prominent infiltration of a mixed population of inflammatory cells in portal tracts, were noted. This study urges the importance of close follow-up of patients receiving etanercept regarding liver complications. Further studies are required to assess the prevalence, risk factors, and outcome of these cases.

Background: Therapeutic plasma exchange (TPE) has been utilized in various liver disorders. There is limited data on the efficacy of TPE in patients with acute liver failure (ALF). Methods: Study group consisted of patients who underwent TPE for ALF due to yellow phosphorous poisoning (YPP) between 2015 and 2019. Demographic data and biochemical parameters were recorded before and after TPE. Overall survival and transplant-free survival (based on King's College Hospital Criteria [KCHC]) were analyzed. Results: Forty-three patients underwent TPE for ALF due to YPP. Most of them were young males. Overall survival was 34 (79.06%). In our study population, 20 patients fulfilled KCHC (Group A) and 23 did not fulfill KCHC (Group B). Both the groups showed significant improvement in alanine aminotransferase, aspartate aminotransferase, and international normalized ratio (INR) after TPE (p < 0.05). In Group B, there was significant improvement in ammonia after TPE (p < 0.05) and all 23 patients (100%) survived after TPE. In Group A, 4 underwent liver transplantation (LT), 7 survived without LT, and the remaining 9 died without LT. Mean survival after completing TPE was 41.2 ± 44.5 days in Group A and 90 days in Group B. This difference was statistically significant (p = 0.001). There was statistically significant difference in post-TPE values of INR (p = 0.012) and ammonia (p = 0.011) between non-survivors and survivors. Adverse events such as hypotension (11.62%) and minor allergic reaction (4.65%) were managed conservatively. Conclusion: TPE is an effective procedure in ALF due to YPP, not fulfilling KCHC for LT. In KCHC fulfilled group, though it shows LT-free survival benefit, there is requirement of prospective, large volume, multi-center study to assess its efficacy. [ABSTRACT FROM AUTHOR]

The paper describes current approaches to liver transplantation in children. It gives data on needs for liver transplantation, defines indications for its use, and considers main methods for grafting the liver from a living-related donor, the issues of immunosuppression, and legal and deontological problems. It also presents the results of 31 transplantations of the left lateral section of the liver from a living-related donor, which have been done at the A.A. Shalimov National Institute of Surgery and Transplantology, Academy of Medical Sciences of Ukraine.

Occurrence of hepatotoxicity following prescription of etanercept, a tumor necrosis factor-alpha (TNF-α) antagonist, is a relatively well-known issue. On the other hand, acute hepatic failure which could lead to liver transplantation is extremely rare to the best of our knowledge, and there is no previously published case in the literature. In this article, we presented a case of acute liver failure followed by liver transplantaion, in a 32-year old man with a previous history of ankylosing spondylitis after etanercept usage. On pathologic examination of the explanted liver of the patient, extensive confluent necrosis in all liver segments with prominent infiltration of a mixed population of inflammatory cells in portal tracts was noticed. In conclusion, close follow-up of patients who are receiving etanercept is crucial, since its liver complications could be severe enough to subject the patients for liver transplantation. [ABSTRACT FROM AUTHOR]

BACKGROUND: In order to solve the problem that the clinical application of liver transplantation is limited by the shortage of supply sources of liver, scientists from all over the world are actively exploring and have successively studied and developed technical means such as artificial liver, tissue-engineered liver and xenotransplantation. It can be used as a corrective or alternative treatment for liver failure. OBJECTIVE: To expound the development history, research status and future expectation of repair and alternative treatment for liver failure. METHODS: Relevant articles published from 2000 to 2019 were searched in the Web of Science and WanFang databases. The keywords were "active liver, liver tissue engineering, hepatic failure" in English and Chinese, respectively. RESULTS AND CONCLUSION: For advanced liver diseases such as liver failure, there are some repair and alternative treatments, including in situ liver transplantation, cell transplantation, artificial liver system, and tissue-engineered liver. To date, a number of hospitals and institutions in China have independently developed artificial liver devices. Although bioartificial liver and mixed artificial liver have shown good prospects in the treatment of liver failure, most of them are still in the experimental stage of animals. Further investigations on the development of bioreactors to enhance cell survival in scaffolds are necessary. Tissue-engineered liver constructed by biological materials and seed cells can to some extent simulate the physiological functions of liver synthesis, detoxification, metabolism, and secretion, which can be transplanted into the body to treat end-stage liver disease. It is a research hotspot in the field of tissue function. Efforts to solve the clotting problem and to promote the vascular formation of scaffold are warranted in this field. Xenograft is the best way to solve the serious shortage of human organ donors, but there is still a long way for this technique away from clinical application. [ABSTRACT FROM AUTHOR]

Background: A novel index, total liver LU15, has been identified as a surrogate marker for liver function. We evaluated the ability of preoperative remnant liver LU15 values to predict postoperative hepatic failure. Methods: Preoperative risk factors for postoperative hepatic failure and remnant liver LU15 were evaluated in 123 patients undergoing liver resection for several diseases from September 1st, 2007 to December 1st, 2016. We calculated the remnant liver LU15 value from the total liver LU15 value and the functional remnant liver ratio. Risk factors for postoperative hepatic failure was determined by univariate and multivariate analysis. Results: Hepatic failure grade B/C developed postoperatively in six patients of seven patients within Makuuchi criteria / without criteria for remnant liver LU15. Operative time (p = 0.0242) and criteria for remnant liver LU15 (p = 0.0001) were prognostic factors for hepatic failure according to the univariate analysis. And criteria for remnant liver LU15 (p = 0.0009) was only prognostic factor by multivariate analysis. Conclusion: Based on the findings form this pilot study, it appears that patients with a remnant liver LU15 value of 13 or less may have a high risk of postoperative hepatic failure. [ABSTRACT FROM AUTHOR]

Adrenal insufficiency, which is related to hemodynamic instability and increased mortality, has been reported in patients with advanced liver disease regardless of the presence of septic conditions. In this regard, the hepatoadrenal syndrome has been recently proposed as adrenal insufficiency in critically ill patients with liver disease. We describe here a 67-year-old female patient with hepatic failure and adrenal insufficiency. The patient showed stable vital signs and no evidence of sepsis preoperatively. Despite hydrocortisone replacement and inotropics administration, severe intraoperative hemodynamic instability was observed. Hydrocortisone administration was continued postoperatively, nevertheless inotropics could not be tapered. On postoperative day 11, the patient died due to pneumonia and septic shock. Hepatoadrenal syndrome may have played a key role in her severe hemodynamic fluctuation and poor outcome, reinforcing the importance of adrenal function in the liver transplantation surgery.

Introduction. The aim of this study is to evaluate the risk factors for acute kidney injury (AKI) and 30-day mortality after liver transplantation. Material and methods. This is a retrospective cohort of consecutive adults undergoing orthotopic liver transplantation (OLT) at a referral hospital in Brazil, from January 2013 to January 2014. Risk factors for AKI and death were investigated. Results. A total 134 patients were included, with median age of 56 years. AKI was found in 46.7% of patients in the first 72 h after OLT. Risk factors for AKI were: viral hepatitis (OR 2.9, 95% CI = 1.2-7), warm ischemia time (OR 1.1, 95% CI = 1.01-1.2) and serum lactate (OR 1.3, 95%CI = 1.02-1.89). The length of intensive care unit (ICU) stay was longer in AKI group: 4 (3-7) days vs. 3 (2-4) days (p = 0.001), as well as overall hospitalization stay: 16 (9-26) days vs. 10 (8- 14) days (p = 0.001). The 30-day mortality was 15%. AKI was an independent risk factor for mortality (OR 4.3, 95% CI = 1.3-14.6). MELD-Na ≥ 22 was a predictor for hemodialysis need (OR 8.4, 95%CI = 1.5-46.5). Chronic kidney disease (CKD) was found in 36 patients (56.2% of AKI patients). Conclusions. Viral hepatitis, longer warm ischemia time and high levels of serum lactate are risk factors for AKI after OLT. AKI is a risk factor for death and can lead to CKD in a high percentage of patients after OLT. A high MELD-Na score is a predictor for hemodialysis need. [ABSTRACT FROM AUTHOR]

Artificial and bioartificial liver support systems (LSSs) appear to be safe and effective in the treatment of acute and acute-on-chronic hepatic failure (AHF and AOCHF); however, individually published studies and previous meta-analyses have revealed inconclusive results. The aim of the present meta-analysis was to derive a more precise estimation of the benefits and disadvantages of artificial and bioartificial LSSs for patients with AHF and AOCHF. A literature search was conducted in the PubMed, Embase, Web of Science and Chinese Biomedical (CBM) databases for publications prior to March 1, 2013. Crude relative risks (RRs) or standardized mean differences (SMDs) with 95% confidence intervals (95% CI) were calculated using either the fixed effects or random effects models. Nineteen randomized controlled trials (RCTs) were included, which comprised a total of 566 patients with AHF and 371 patients with AOCHF. The meta-analysis showed that artificial LSS therapy significantly reduced mortality in patients with AOCHF; however, it had no apparent effect on total mortality in patients with AHF. The results also indicated that the use of bioartificial LSSs was correlated with decreased mortality in patients with AHF. A significant reduction in the bridging to liver transplantation was observed in patients with AOCHF following artificial LSS therapy; however, similar results were not observed in patients with AHF. Patients with AHF and those with AOCHF showed significant reductions in total bilirubin levels following artificial LSS therapy. There were no significantly increased risks of hepatic encephalopathy or bleeding in either the patients with AHF or AOCHF following a rtificial o r b ioartificial LSS therapies. Univariate and multivariate meta-regression analyses confirmed that none of the factors explained the heterogeneity. The present meta-analysis indicated that artificial LSSs reduce mortality in patients with AOCHF, while the use of bioartificial LSSs was correlated with reduced mortality in patients with AHF. [ABSTRACT FROM AUTHOR]

Acute liver failure (ALF) results from the acute and rapid loss of hepatocyte function and frequently exhibits a fulminant course, characterized by high mortality in the absence of immediate state-of-the-art intensive care and/or emergency liver transplantation (ELT). The role of hepatocyte-mediated liver regeneration during acute and chronic liver injury has been extensively investigated, and recent studies suggest that hepatocytes are not exclusively responsible for the regeneration of the injured liver during fulminant liver injury. Liver progenitor cells (LPC) (or resident liver stem cells) are quiescent in the healthy liver, but may be activated under conditions where the regenerative capacity of mature hepatocytes is severely impaired. This review aims to provide an overview of the role of the LPC population during ALF, and the role of putative cytokines, growth factors, mitogens, and hormones in the LPC response. We will highlight the potential interaction among cellular compartments during ALF, and discuss the possible prognostic value of the LPC response on ALF outcomes. [ABSTRACT FROM AUTHOR]

Adrenal insufficiency, which is related to hemodynamic instability and increased mortality, has been reported in patients with advanced liver disease regardless of the presence of septic conditions. In this regard, the hepatoadrenal syndrome has been recently proposed as adrenal insufficiency in critically ill patients with liver disease. We describe here a 67-year-old female patient with hepatic failure and adrenal insufficiency. The patient showed stable vital signs and no evidence of sepsis preoperatively. Despite hydrocortisone replacement and inotropics administration, severe intraoperative hemodynamic instability was observed. Hydrocortisone administration was continued postoperatively, nevertheless inotropics could not be tapered. On postoperative day 11, the patient died due to pneumonia and septic shock. Hepatoadrenal syndrome may have played a key role in her severe hemodynamic fluctuation and poor outcome, reinforcing the importance of adrenal function in the liver transplantation surgery. (Korean J Anesthesiol 2013; 64: 536-540) [ABSTRACT FROM AUTHOR]

Background: Liver transplantation is the treatment of choice for both acute and chronic hepatic failure. GSTs family is one of the most important genes in phase II detoxification interfering with the xenobiotics and free radical metabolism. GSTO2 (N142D) is a member of this family the polymorphism of which may influence the metabolism of active components and free radicals and may contribute to hepatic failure. Objective: To investigate the association between GSTO2 genetic polymorphism and the susceptibility of hepatic failure that would lead to liver transplantation. Methods: This case-control study included 330 healthy people and 302 patients with liver transplantation as a result of hepatic failure. To determine the variants of GSTO2, we used polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) method. Results: There was a significant association between D allele and hepatic failure, thus, people with DD genotype are more susceptible to develop heaptic failure leading to liver transplantation (OR=1.8, 95% CI: 1.10-2.95, p=0.02). We also observed that male sex increases the chance of hepatic failure (OR=2.69, 95% CI: 1.95-3.71, p=0.001). Conclusion: D allele may reduce the detoxification ability of liver so people with mutant D allele are more prone to develop hepatic failure. [ABSTRACT FROM AUTHOR]

Murakawa Y, Miyagawa-Hayashino A, Ogura Y, Egawa H, Okamoto S, Soejima Y, Kurosawa M, Sumiyoshi S, Uemoto S, Haga H. Liver transplantation for severe hepatitis in patients with common variable immunodeficiency. Pediatr Transplantation 2011. © 2011 John Wiley & Sons A/S. Abstract: CVID is a heterogeneous group of primary immunodeficiency diseases characterized by hypogammaglobulinemia, recurrent bacterial infections, and frequent autoimmune manifestations. The post-transplant course of liver transplant recipients with CVID is rarely described. We report two patients with CVID complicated by severe enteropathy who underwent living donor liver transplantation for liver failure because of severe hepatitis. The post-transplant course was complicated by recurrent acute rejection, leading to ductopenic rejection in one and recurrent hepatitis in the other. We reviewed the tissue samples histologically and immunohistochemically. Native livers showed submassive hepatocyte necrosis in one and cirrhotic liver with active hepatitis in the other, both with infiltration of CD8+ T cells accompanied by endothelialitis and bile duct damage; the intestine contained increased numbers of intraepithelial CD8+ T cells with apoptosis of epithelial cells. The liver allograft exhibited acute rejection, with prominent CD8+ T cells infiltrating the bile duct or endothelium. In the allograft following the diagnosis of post-transplant recurrent hepatitis, CD8+ T cells comprised the majority of infiltrating cells in portal areas spilling over into hepatic parenchyma. Our cases suggest that T cells contribute to the pathogenesis of CVID in native organs as well as allografts and may constitute evidence of T-cell deregulation in the pathogenesis of CVID. [ABSTRACT FROM AUTHOR]

The article presents a study which examines the role of angiopoietin-2, an antagonistic ligand of the endothelial Tie2 receptor, in acute liver failure (ALF). Angiopoietin-2 has been associated with several features of multiple organ dysfunction syndrome. The significance of Tie2 agonists as an endothelium-targeted therapy to improve the outcome in ALF is outlined.

Background: Standard evaluation of patients undergoing hepatic surgery has been through radiological and quantitative determination of liver function. As more complex and extensive surgery is now being performed, often in the presence of cirrhosis/fibrosis or following administration of chemotherapy, it is questioned whether additional assessment may be required prior to embarking on such surgery. The aim of this review was to determine the current knowledge base in relation to the performance of quantitative assessment of hepatic function both pre- and post-operatively in patients undergoing hepatic resectional surgery and liver transplantation.Methods: An electronic search was performed of the medical literature using the MEDLINE database to identify relevant articles with cross-referencing of all identified papers to ensure full literature capture.Results and Conclusions: The review has identified a number of different methods of dynamically assessing hepatic function, the most frequently performed being through the use of indocyanine green clearance. With the recent and further anticipated developments in hepatic resectional surgery, it is likely that quantitative assessment will become more widely practiced in order to reduce post-operative hepatic failure and improve outcome. [ABSTRACT FROM AUTHOR]

Serum aminotransferase elevations are a commonly known adverse effect of 3- hydroxy-3-methylglutaryl coenzyme A reductase inhibitor (statin) therapy. However, hepatotoxic events have not been widely published with ezetimibe or the combination agent simvastatin-ezetimibe. We describe a 70-year-old Hispanic woman who developed fulminant hepatic failure necessitating liver transplantation 10 weeks after conversion from simvastatin 40 mg/day to simvastatin 10 mg-ezetimibe 40 mg/day. The patient's lipid panel had been maintained with simvastatin for 18 months before the conversion without evidence of hepatotoxicity. A routine laboratory work-up 10 weeks after conversion revealed elevated serum aminotransferase levels. Simvastatinezetimibe and escitalopram (which she was taking for depression) were discontinued, and other potential causes of hepatotoxicity were excluded. A repeat work-up revealed further elevations in aminotransferase levels, and liver biopsy revealed evidence of moderate-to-severe drug toxicity. She underwent liver transplantation with an uneventful postoperative course. Her aminotransferase levels returned to normal by postoperative day 23, and her 2-year follow-up showed no adverse events. Ezetimibe undergoes extensive glucuronidation by uridine diphosphate glucoronosyltransferases (UGT) in the intestine and liver and may have inhibited the glucuronidation of simvastatin hydroxy acid, resulting in increased simvastatin exposure and subsequent hepatotoxicity. To our knowledge, this is the first case report of simvastatin-ezetimibe-induced liver failure that resulted in liver transplantation. We postulate that the mechanism of the simvastatinezetimibe- induced hepatotoxicity is the increased simvastatin exposure by ezetimibe inhibition of UGT enzymes. Clinicians should be aware of potential hepatotoxicity with simvastatin-ezetimibe especially in elderly patients and should carefully monitor serum aminotransferase levels when starting therapy and titrating the dosage. [ABSTRACT FROM AUTHOR]

An 11-year-old boy presented with hepatic failure secondary to parvovirus B19 infection, requiring urgent liver transplantation. His recovery was complicated by primary Epstein–Barr virus and cytomegalovirus infections. He subsequently developed aplastic anaemia that has been refractory to antithymocyte globulin and cyclosporine therapy and may now require bone marrow transplantation. We present this case to emphasize parvovirus as a rare cause of hepatic failure and of aplastic anaemia as a complication of the virus. [ABSTRACT FROM AUTHOR]

Parvovirus B19 has rarely been associated with acute liver failure (ALF), which has a high mortality. Plasma exchange that usually acts as a bridge to liver transplantation removes toxins, antibodies, cytokines, and can correct coagulopathy while maintaining a euvolemic state. Pediatric data regarding its use are scarce. We report a case of 16-year-old girl with acute liver failure in stage 4 encephalopathy with coagulopathy due to parvovirus B19 who was successfully managed with high-volume therapeutic plasma exchange (TPE). We tried to use it as a treatment modality due to nonavailability of in-hospital transplant facilities. Parvovirus B19 may be an underdiagnosed cause of acute viral hepatitis. Therapeutic plasma exchange can act as a bridge to liver transplant (LT) or bridge to recovery especially in self-limiting illnesses such as viral hepatitis. [ABSTRACT FROM AUTHOR]

There is a need to develop artificial means of liver replacement and/or assistance with the aim of either supporting patients with borderline functional liver cell mass until their liver regenerates, or until a donor liver becomes available for transplantation. Selective plasma filtration is a novel approach to blood purification therapy designed to reduce the level of circulating toxins of hepatic and renal failure, mediators of inflammation and inhibitors of hepatic regeneration. The results of preclinical studies indicate that treatment of pigs with experimentally-induced fulminant hepatic failure is safe and effective in extending survival time and arresting brain swelling. In addition, the amount of ammonia, aromatic amino acids, IL6, TNFα and C3a removed during the 6-h treatment in the present study was higher by 34% to 175% than the total plasma content of those substances at the start of therapy. [ABSTRACT FROM AUTHOR]

Pediatric liver transplantations are becoming increasingly more common. Recent advances in the surgical and anesthetic management of these cases have greatly improved the survival rate. In order to successfully manage the anesthesia in these patients, one needs to have a thorough understanding of the pathophysiology of end-stage liver disease and the subsequent anesthetic implications. It is also necessary to appreciate the stages of the surgical procedure, as each stage presents different dilemmas to the anesthesiologist. This article will review the pathophysiology of liver failure in pediatric patients and outline the particular issues related to each stage of liver transplantation, allowing for the anticipation and management of the derangements that occur during surgery. [ABSTRACT FROM AUTHOR]

Background/Aims: Indication of liver transplantation in acute liver failure following amatoxin intoxication is still uncertain. Methods: One hundred and ninety-eight patients were studied retrospectively. The laboratory parameters alanineaminotransferase, serum bilirubin, serum creatinine and prothrombin index were analyzed over time. Predictors of fatal outcome and survival were determined by receiver-operating-characteristic and sensitivity–specificity analysis. Results: Twenty-three patients died in the median 6.1 days (range, 2.7–13.9 days) after ingestion. Using a single parameter as predictor of fatal outcome the area under the receiver-operating-characteristic curve of prothrombin index (0.96) and serum creatinine (0.93) were both significantly greater (P<0.05) compared with serum bilirubin (0.82) and alanineaminotransferase (0.69). Prediction of fatal outcome had an optimum, if a prothrombin index less than 25% was combined with a serum creatinine greater than 106μmol/l from day 3 after ingestion onwards (sensitivity 100%, 95% confidence interval 87–100; specificity 98%, 95% confidence interval 94–100). The median time period between the first occurrence of this predictor in non-survivors and death was 63h (range, 3–230h). Conclusions: A decision model of liver transplantation following amatoxin intoxication using prothrombin index in combination with serum creatinine from day 3 to 10 after ingestion enables an early and reliable assessment of outcome. [Copyright &y& Elsevier]

Acute hepatic failure is a rare clinical syndrome associated with high mortality. Hepatic failure leads to a well-recognised pattern of clinical signs and symptoms, sometimes with rapid deterioration and progression to multi-organ failure. Early recognition of this syndrome is essential for appropriate treatment; once identified, patients benefit from early interventional support and treatment in the intensive care unit. Aggressive management may allow stabilisation of patients before their transfer to specialist liver units. At present, orthotopic liver transplantation is the only treatment modality that provides significant improvement in outcome. This review examines the aetiology and clinical presentation of acute hepatic failure, providing guidelines regarding patient management. We present a critical appraisal of specific clinical areas, including the management of cardiovascular, cerebral, renal, coagulopathic and infective complications. Liver transplantation is discussed as well as emerging therapies including non-biological and hybrid liver support systems that may provide a "bridge to transplantation". [ABSTRACT FROM AUTHOR]

A case of hepatic complication in a pregnant woman suffering from HELLP syndrome resulting in the need for transplantation is reported, and an algorithm for those instances is suggested. According to the literature, this is the 5th report of hepatic complications in HELLP syndrome necessitating liver transplantation. Since all but one of the transplanted patients survived (as opposed to a high mortality in non-transplanted patients), it is concluded, that a timely decision for transplantation is a safe option in this high risk group. [ABSTRACT FROM AUTHOR]

Introduction: Acetaminophen (APAP) is a widely used medication in pregnancy and is considered safe. Unfortunately, APAP is also among the most commonly reported agents implicated in overdoses during pregnancy. We present a unique case of a pregnant patient with fulminant hepatic failure resulting in a liver transplant from repeated supratherapeutic ingestions of APAP. Case Report: A 22 year pregnant female presented with abdominal pain and hepatotoxicity after taking supratherapeutic amounts of APAP to treat dental pain. The patient denied intentional or acute ingestion of APAP but did admit to taking approximately 8-9 grams of APAP per day for 10-14 days for dental pain. Other cause of hepatotoxicity, including acute fatty liver of pregnancy, were evaluated for and ruled out. She developed fulminant hepatic failure and required liver transplantation which was successful. The pregnancy remained viable through the operation but intrauterine fetal demise occurred 2 weeks later. An MRI of the fetus showed extensive peri-cerebral and intraventricular hemorrhage with extensive periventricular leukomalacia. Discussion: The degree of morbidity from repeated supratherapeutic ingestions of APAP seen in this case is rare and poorly described in a pregnant patient. There are no prior reports describing the need for liver transplant after repeated supratherapeutic ingestions of APAP during pregnancy. Along with the typical cause of hepatotoxicy several unique pregnancy-related causes also had to be evaluated for. This case highlights the significant morbidity that can occur with even unintentional APAP toxicity and the need to educate patients, especially pregnant patients, of the risk of excessive APAP use. [ABSTRACT FROM AUTHOR]

Abstract: Advanced liver disease is a classic example of multisystem failure resulting from a single-organ disease. Historically, even minor surgery on patients with cirrhosis resulted in high mortality. Liver transplantation has dramatically improved the prognosis for these patients. It is important for clinicians to understand the multisystem sequelae of end-stage liver disease in order to assess risk and manage patients appropriately. Recognition of severe disease is crucial; improved perioperative care has not significantly reduced operative mortality. These patients should be referred for transplant assessment or, if surgery for unrelated conditions is considered, should be managed in specialist centres. [Copyright &y& Elsevier]

Severe liver dysfunction in late pregnancy is an unusual but dramatic event because it can progress very rapidly to fulminating disease and also because two lives, that of the mother and foetus, are involved. We report a descriptive study of a pregnant woman presenting with severe liver dysfunction. [Copyright &y& Elsevier]