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Hibernomas are uncommon benign soft-tissue tumors which are composed of brown fat. They are usually seen in adults in the fourth and fifth decades and the common locations are thigh, shoulder, and back. Only few cases in the posterior neck region have been reported in literature. Hibernoma is very uncommon in the pediatric age group and those in the neck region are extremely rare. We present a hibernoma presenting as a swelling on the posterior aspect of base of the neck in a 5-month-old male infant. Considering the rare occurrence in the pediatric age group and difficulty in diagnosis, the clinical features, radiological features, pathological characteristics, differential diagnosis, and management of this rare tumor of childhood are discussed herewith.

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., Article Highlights Updated on the basis of AARC data of >3300 cases enrolled into AARC registry prospectivelyACLF is distinct form Acute Decompensation of cirrhosisNewer sections on DILI-ACLF, AIH-ACLF, PVT/HVOTO–ACLFReversibility of Chronic Liver Disease in ACLFPortal and systemic hemodynamics and their relevance in ACLFAcute Portal Hypertension and Variceal progression in ACLFAARC score as a guide for treatment strategies in ACLFACLF in Children-first consensus on pediatric ACLF

Recurrent pyogenic cholangitis (RPC) is a disease characterized by multiple strictures of the biliary tree, impaired biliary drainage, formation of intrahepatic biliary pigment stones and recurrent bouts of cholangitis. We report the case of a 39-year-old businessman with diagnosed chronic calcific pancreatitis, who presented to us with recurrent episodes of cholangitis, leading to portal pyaemia, and progressive liver failure, which could not be controlled despite adequate biliary drainage. The patient rapidly developed progressive liver failure and sepsis-related coagulation failure. He was also found to have idiopathic CD4+ T cell lymphocytopenia (ICL), which resulted in refractory sepsis and formation of metastatic abscesses in the lung and spleen. ICL is now recognised in patients with recurrent and difficult to treat opportunistic infections. The combination of RPC, sepsis and liver failure in the setting of an acquired immunosuppressed state makes this a unique management scenario.

Endogenous heparinoids or heparin-like effects (HLEs) can cause coagulation failure in patients with cirrhosis and sepsis. We performed a prospective study of the association between HLE and bleeding events, sepsis, and outcomes of patients with severe alcohol-associated hepatitis.Our final analysis comprised 78 patients with severe alcohol-associated hepatitis (44.3 ± 11.7 years; all male; discriminant function32) who presented without sepsis at a single center in India from August 2015 through August 2016. Blood samples were collected at days 0, 3, and 7 after presentation and assessed by a global coagulation assay; by SONOCLOT (global and heparinase treated); and in assays for factor VIII, von Willebrand factor, protein C, and antithrombin. Patients were followed for sepsis, bleeding and outcome. The primary outcome was association of HLE with survival 28 days after presentation.HLEs were observed in 32 patients (41%) at day 0, 27 patients (34.6%) at day 3, and 28 patients (35.9%) patients at day 7. Factors associated with mortality at day 0 were factor VIII activity160% (hazard ratio [HR], 3.1; 95% CI, 1.4-9.5; P = .026), level of protein C34% (HR, 0.7; 95% CI, 0.5-0.8; P = .037), antithrombin activity28% (HR, 0.7; 95% CI, 0.3-1.1; P = .008) and international normalized ratio2.6 (HR, 2.3; 95% CI, 1.8-9.7; P = .010). In multivariate analyses, only factor VIII activity (HR, 2.3; 95% CI, 1.6-7.8; P = .046), international normalized ratio (1.9; 95% CI, 1.2-4.3; P = .039), level of protein C (HR, 0.9; 95% CI, 0.7-1.1; P = .052) and model for end-stage liver disease score (HR, 3.2; 95% CI, 1.9-10.2; P = .042) were associated with mortality. Episodes of epistaxis, hemorrhoid bleeding, hemoperitoneum, and pulmonary hemorrhage occurred in 10.2%, 12.3%, 3.4%, and 4.5% of patients respectively. The presence of HLE at day 0 increased the risk of sepsis (HR, 2.5; 95% CI, 2.2-4.3; P = .002), bleeding (HR, 1.4; 95% CI, 1.2-5.3; P = .004) and death (HR, 1.2; 95% CI, 1.4-1.7; P = .044).In a prospective study of patients with severe alcohol-associated hepatitis, we associated HLE with coagulation abnormalities, risk of sepsis, and mortality. Clinicaltrials.govNCT02307409.

Background The presence of left ventricular diastolic dysfunction (LVDD) in patients with cirrhosis leads to a restriction of activities and a poor health related quality of life (HRQoL), which should be taken into consideration when treating them for liver and cardiac complications. Aims The prevalence, complications, predictors of HRQoL and survival in cirrhotic patients with LVDD were studied. Methods We report a prospective cohort study of 145 consecutive cirrhotic patients with LVDD who were evaluated for cardiac functional status at enrollment and followed up for hepatic complications, cardiac events, outcome and HRQoL using the Minnesota Living With Heart Failure Questionnaire (MLHFQ) over a period of 2 years. Results In total, 145 (mean age 61 years, 59% male) patients were included. Seventeen patients died with 10.5%, 22.5% and 40% mortality rates in patients with Grades 1, 2 and 3 LVDD respectively over 24 months. The parameters that were significant for predicting mortality on bivariate analysis were MELD, MELDNa, hepatic venous pressure gradient, MLHFQ, and left ventricular (LV) diastolic function (e′ and E/e′ ratio), but only MELD, MELDNa and E/e′ remained significant on multivariate analysis. The E/e′ ratio (8.7 ± 3.3 in survivors vs. 9.1 ± 2.3 in non-survivors) predicted outcome. On univariate analysis, the predictors of poor HRQoL were the Child-Pugh score ≥9.8 (OR 2.6; 95% confidence intervals (CI) 2.3–9.1, P = 0.041), MELD score ≥ 15.7 (OR 2.48; 95% CI 1.4–3.9, P = 0.029), refractory ascites (OR 1.9; 95% CI 1.1–6.1, P = 0.050), and E/e′ ratio ≥7.6 (OR 1.9; 95% CI 1.8–7.1, P = 0.036) The presence of Class II/III (P = 0.046) symptoms of heart failure and MLHFQ≥ 45 (P = 0.042) were predictors of mortality at 24 months. Conclusion The grade of LVDD correlates with liver function, clinical events, risk of renal dysfunction and HRQoL. Evaluation of novel therapies which target symptomatic improvement in LVDD, should be done with suitable outcome measures, including HRQoL assessment.

Sister Mary Joseph nodules represent metastatic cancer of the umbilicus. More than half of these cases are attributable to gastrointestinal malignancies including gastric, colonic, and pancreatic cancer. In addition, gynecologic (ovarian, uterine cancer), unknown primary tumors, and, rarely, bladder or respiratory malignancies may cause umbilical metastasis. We report the case of a Sister Mary Joseph nodule originating from a hilar cholangiocarcinoma. Umbilical nodules should prompt clinical evaluation, as these tumors are usually associated with poor prognosis.

Systemic amyloidosis of amyloid light chain associated protein (AL), also called primary amyloidosis, frequently involves the liver, but rarely causes clinically apparent liver disease. The more common presentation is with acute renal failure. Hepatomegaly and mild elevation of alkaline phosphatase are the most common clinical and biochemical findings, respectively. We report a case of systemic amyloidosis of AL that clinically presented as acute-on-chronic liver failure and resulted in a fatal clinical course in a 56-year-old man.

A 51 year old male, presented to us with progressive abdominal distention and jaundice since 1 month, and a single episode of hematemesis. He had undergone pericardiectomy 10 years back for chronic constrictive pericarditis (CCP) and had completed a course of anti-tubercular therapy following the surgery. Physical examination revealed normal vital signs and mild icterus, moderate ascites, splenomegaly, and mild ankle edema. On endoscopy, he found to have large esophageal varices and endoscopic variceal ligation (EVL) was performed. Thyroid function tests, hepatitis serologies, autoimmune assays (ANA, AMA) ASMA, ferritin, ceruloplasmin, and α1-AT, level were unremarkable. Liver biopsy showed cirrhosis. After extensive evaluation, a diagnosis of cardiac cirrhosis was made.

Aluminium phosphide poisoning is very common in India. It is one of the most fatal poisons. The clinical spectrum of poisoning varies depending upon the dosage and duration of consumption. The main effect of the poison is due to the release of phosphine which inhibits cytochrome oxidase and thereby hampers cellular oxygen utilization. Almost any organ can be affected by aluminium phosphide poisoning. We report a case where the heart was the predominantly affected organ. We describe the clinical symptoms and signs and their correlation with electrocardiographic and histopathological examinations.

The transport of sick neonates to the surgical centers or transportation within the center is an essential requirement of neonatal surgery. Neonatal transport incubators are costly, space occupying, and are not available at many places in the developing countries. We report here a cheap yet effective and easy to make, alternate neonatal carrier device.

A full term male neonate presented with stridor and respiratory distress at birth. Direct laryngoscopy after tracheostomy and patient stabilization revealed a subglottic web with a very small hole at 6 o′clock position. The patient was managed by cauterization of web using Bugbee electrode with resultant normal lumen post operatively.