The first consensus report of the working party of the Asian Pacific Association for the Study of the Liver (APASL) set up in 2004 on acute-on-chronic liver failure (ACLF) was published in 2009. With international groups volunteering to join, the “APASL ACLF Research Consortium (AARC)” was formed in 2012, which continued to collect prospective ACLF patient data. Based on the prospective data analysis of nearly 1400 patients, the AARC consensus was published in 2014. In the past nearly four-and-a-half years, the AARC database has been enriched to about 5200 cases by major hepatology centers across Asia. The data published during the interim period were carefully analyzed and areas of contention and new developments in the field of ACLF were prioritized in a systematic manner. The AARC database was also approached for answering some of the issues where published data were limited, such as liver failure grading, its impact on the ‘Golden Therapeutic Window’, extrahepatic organ dysfunction and failure, development of sepsis, distinctive features of acute decompensation from ACLF and pediatric ACLF and the issues were analyzed. These initiatives concluded in a two-day meeting in October 2018 at New Delhi with finalization of the new AARC consensus. Only those statements, which were based on evidence using the Grade System and were unanimously recommended, were accepted. Finalized statements were again circulated to all the experts and subsequently presented at the AARC investigators meeting at the AASLD in November 2018. The suggestions from the experts were used to revise and finalize the consensus. After detailed deliberations and data analysis, the original definition of ACLF was found to withstand the test of time and be able to identify a homogenous group of patients presenting with liver failure. New management options including the algorithms for the management of coagulation disorders, renal replacement therapy, sepsis, variceal bleed, antivirals and criteria for liver transplantation for ACLF patients were proposed. The final consensus statements along with the relevant background information and areas requiring future studies are presented here.

NCPH is a heterogeneous group of liver disorders of vascular origin, leading to PHT with near normal HVPG. NCPF/IPH is a disorder of young adults or middle aged women, whereas EHPVO is a disorder of childhood. Early age acute or recurrent infections in an individual with thrombotic predisposition constitute the likely pathogenesis. Both disorders present with clinically significant PHT with preserved liver functions. Diagnosis is easy and can often be made clinically with support from imaging modalities. Management centers on control and prophylaxis of variceal bleeding. In EHPVO, there are additional concerns of growth faltering, portal biliopathy, MHE and parenchymal dysfunction. Surgical shunts are indicated in patients with failure of endotherapy, bleeding from sites not amenable to endotherapy, symptomatic hypersplenism or symptomatic biliopathy. Persistent growth failure, symptomatic and recurrent hepatic encephalopathy, impaired quality of life or massive splenomegaly that interferes with daily activities are other surgical indications. Rex-shunt or MLPVB is the recommended shunt for EHPVO, but needs proper pre-operative radiological assessment and surgical expertise. Both disorders have otherwise a fairly good prognosis, but need regular and careful surveillance. Hepatic schistosomiasis, CHF and NRH have similar presentation and comparable prognosis.

No abstract

Pediatric hepatocellular carcinoma (HCC) is the second common malignant liver tumor in children after hepatoblastoma. It differs from the adult HCC in the etiological predisposition, biological behavior and lower frequency of cirrhosis. Perinatally acquired hepatitis-B virus, hepatorenal tyrosinemia, progressive familial intrahepatic cholestasis, glycogen storage disease, Alagille’s syndrome and congenital portosystemic shunts are important predisposing factors. Majority of children (87%) are older than 5 years of age. Following mass immunization against hepatitis-B, there has been a drastic fall in the incidence of new cases of pediatric HCC in the Asia-Pacific region. Management is targeted on complete surgical removal either by resection or liver transplantation. There is a trend towards improving survival of children transplanted for HCC beyond Milan criteria. Chemotherapeutic regimens do not offer good results but may be helpful for down-staging of advanced HCC. Surveillance of children with chronic liver diseases with ultrasound and alpha-fetoprotein may be helpful in timely detection, intervention and overall improvement in outcome of HCC.

The article Acute-on-chronic liver failure: consensus recommendations of the Asian Pacific association for the study of the liver (APASL): an update, written by [Shiv Sarin], was originally published electronically on the publisher’s internet portal (currently SpringerLink) on June 06, 2019 without open access.

Acute kidney injury developed in 22.6% of children with acute-on-chronic liver failure. Bilirubin more than 17.7 mg/dL and presence of systemic inflammatory response syndrome were high risk factors for acute kidney injury. Development of acute kidney injury in a child with acute-on-chronic liver failure suggests poor outcome and need for early intervention.

This review gives a detailed summary of these two vascular conditions of liver-IPH and EHPVO. Further research is needed to understand the pathogenesis and natural history of these disorders.

In countries where deceased organ donation is sparse, emergency living donor liver transplantation (LDLT) is the only lifesaving option in select patients with acute liver failure (ALF). The aim of the current study is living liver donor safety and recipient outcomes following LDLT for ALF. A total of 410 patients underwent LDLT between March 2011 and February 2018, out of which 61 (14.9%) were for ALF. All satisfied the King’s College criteria (KCC). Median admission to transplant time was 48 hours (range, 24‐80.5 hours), and median living donor evaluation time was 18 hours (14‐20 hours). Median Model for End‐Stage Liver Disease score was 37 (32‐40) with more than two‐thirds having grade 3 or 4 encephalopathy and 70% being on mechanical ventilation. The most common etiology was viral (37%). Median jaundice‐to‐encephalopathy time was 15 (9‐29) days. Preoperative culture was positive in 47.5%. There was no difference in the complication rate among emergency and elective living liver donors (13.1% versus 21.2%; P = 0.19). There was no donor mortality. For patients who met the KCC but did not undergo LT, survival was 22.8% (29/127). The 5‐year post‐LT actuarial survival was 65.57% with a median follow‐up of 35 months. On multivariate analysis, postoperative worsening of cerebral edema (CE; hazard ratio [HR], 2.53; 95% confidence interval [CI], 1.01‐6.31), systemic inflammatory response syndrome (SIRS; HR, 16.7; 95% CI, 2.05‐136.7), preoperative culture positivity (HR, 6.54; 95% CI, 2.24‐19.07), and a longer anhepatic phase duration (HR, 1.01; 95% CI, 1.00‐1.02) predicted poor outcomes. In conclusion, emergency LDLT is lifesaving in selected patients with ALF. Outcomes of emergency living liver donation were comparable to that of elective donors. Postoperative worsening of CE, preoperative SIRS, and sepsis predicted outcome after LDLT for ALF.