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.
Minimal hepatic encephalopathy (MHE) has a negative effect on patients' daily functioning. Thus far, no study has investigated the effect of treatment-related improvement in cognitive functions on health-related quality of life (HRQOL). We measured psychometric performance by number and figure connection tests parts A and B, picture completion, and block design tests and HRQOL by the Sickness Impact Profile (SIP) of 90 patients with cirrhosis on inclusion into the study and 3 months later. A Z score less than ؊2 on the neuropsychological (NP) tests was considered abnormal. Sixty-one (67.7%) patients had MHE. They were randomly assigned in a 1:1 ratio to receive treatment (lactulose) for 3 months (n ؍ 31) or no treatment (n ؍ 30) in a nonblinded design. H epatic encephalopathy (HE) is a spectrum of neuropsychiatric abnormalities seen in patients with liver dysfunction diagnosed after exclusion of other known brain diseases. The Working Party at the 11th World Congress of Gastroenterology, Vienna, under the Organization Mondiale de Gastroentrologie proposed a multiaxial definition of HE that defined both the type of hepatic abnormality (type A, B, or C) and the duration and characteristics of neurological manifestations (episodic, persistent, or minimal HE) in chronic liver disease. 1 HE has been considered a continuous dimension that could be measured with 1 index to summarize several neurological domains, such as cognition, emotion, behavior, and biologic rhythms. Minimal hepatic encephalopathy (MHE) represents a portion of this dimension and is the mildest form of HE. Whereas patients with HE have impaired intellectual functioning, personality changes, altered level of consciousness, and neuromuscular dysfunction, patients with MHE have no recognizable clinical symptoms of HE but do have mild cognitive and psychomotor deficits. In the absence of a "gold standard" for determining MHE, neuropsychological (NP) and neurophysiological methods have been the most trusted and widely used tests to diagnose this condition. 1,2 MHE is considered clinically relevant for at least 3 reasons. First, it impairs patients' daily functioning and
We conclude that SHE is common in cirrhosis. The natural history of SHE is worse in patients with advanced cirrhosis and SHE probably predisposes the cirrhotic patient to overt hepatic encephalopathy.
__________________________ E xtrahepatic portal venous obstruction (EHPVO) is a common cause of portal hypertension in the developing countries, and constitutes up to 40% of all patients with portal hypertension. 1 2 EHPVO is a common cause of major upper-gastrointestinal bleeding among children.2-4 The most common presentation in children is well-tolerated variceal bleeding and splenomegaly. In adults, EHPVO is often recognised when evaluating for other disorders or with uncommon presentations such as jaundice, pruritus, acute cholecystitis-like syndrome, ascites and so on, resulting from prolonged portal hypertension. [5][6][7] The portal vein in EHPVO is transformed into a cavernoma, which is a bunch of multiple collateral veins around the obstructed portion of portal vein (fig 1). Marked improvements in the management of variceal bleeding in patients with EHPVO have resulted in an improved survival, thus presenting with unusual symptoms in adulthood.The reasons for EHPVO are obscure in approximately half of the patients. Omphalitis and intraabdominal sepsis are the common causes in neonates and children. Adults develop EHPVO due to increased blood coagulability, local inflammation, intra-abdominal sepsis, myeloproliferative disorders, underlying cirrhosis, or tumours in the liver, bile ducts or pancreas.
7-10Gibson et al 11 first reported the relationship between EHPVO and jaundice in 1965. Since then, several cases of obstructive jaundice due to common bile duct (CBD) obstruction caused by cavernomatous transformation of portal vein (portal cavernoma) have been described. Williams et al 12 were the first to report cholangiographic changes caused by choledochal varices. We, for the first time, describe abnormalities on endoscopic retrograde cholangiography (ERC) in a prospective study.13 These abnormalities were similar to those of primary sclerosing cholangitis and the term ''pseudosclerosing cholangitis'' was then coined to describe them. However, unlike primary sclerosing cholangitis, biliary strictures in patients with EHPVO were smooth rather than irregular (figs 2, 3A). Since then, several case series have described biliary abnormalities among patients with portal hypertension on ERC.14-22
The epidemiological and clinical features of NCPF have more similarity to IPH than has previously been documented. The development of spontaneous shunts tends to protect these patients from variceal bleeding.
Hepatic encephalopathy (HE) is a frequent and serious complication of both chronic liver disease and acute liver failure. HE manifests as a wide spectrum of neuropsychiatric abnormalities, from subclinical changes (mild cognitive impairment) to marked disorientation, confusion and coma. The clinical and economic burden of HE is considerable, and it contributes greatly to impaired quality of life, morbidity and mortality. This review will critically discuss the latest classification of HE, as well as the pathogenesis and pathophysiological pathways underlying the neurological decline in patients with end-stage liver disease. In addition, management strategies, diagnostic approaches, currently available therapeutic options and novel treatment strategies are discussed.
Psychometric hepatic encephalopathy score is a useful tool for the diagnosis of minimal hepatic encephalopathy in an outpatient setting. Both psychometric hepatic encephalopathy score and Child-Turcotte-Pugh score have prognostic value on survival.
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