Indian National Association for the Study of the Liver Consensus Statement on Acute Liver Failure (Part 1): Epidemiology, Pathogenesis, Presentation and Prognosis

Abstract

Acute liver failure (ALF) is an infrequent, unpredictable, potentially fatal complication of acute liver injury (ALI) consequent to varied etiologies. Etiologies of ALF as reported in the literature have regional differences, which affects the clinical presentation and natural course. In this part of the consensus article designed to reflect the clinical practices in India, disease burden, epidemiology, clinical presentation, monitoring, and prognostication have been discussed. In India, viral hepatitis is the most frequent cause of ALF, with drug-induced hepatitis due to antituberculosis drugs being the second most frequent cause. The clinical presentation of ALF is characterized by jaundice, coagulopathy, and encephalopathy. It is important to differentiate ALF from other causes of liver failure, including acute on chronic liver failure, subacute liver failure, as well as certain tropical infections which can mimic this presentation. The disease often has a fulminant clinical course with high short-term mortality. Death is usually attributable to cerebral complications, infections, and resultant multiorgan failure. Timely liver transplantation (LT) can change the outcome, and hence, it is vital to provide intensive care to patients until LT can be arranged. It is equally important to assess prognosis to select patients who are suitable for LT. Several prognostic scores have been proposed, and their comparisons show that indigenously developed dynamic scores have an edge over scores described from the Western world. Management of ALF will be described in part 2 of this document.

Keywords: ACLF, acute on chronic liver failure; AFLP, acute fatty liver of pregnancy; AKI, Acute kidney injury; ALF, Acute liver failure; ALFED, Acute Liver Failure Early Dynamic; ALT, alanine transaminase; ANA, antinuclear antibody; AP, Alkaline phosphatase; APTT, activated partial thromboplastin time; ASM, alternative system of medicine; ASMA, antismooth muscle antibody; AST, aspartate transaminase; ATN, Acute tubular necrosis; ATP, adenosine triphosphate; ATT, anti-TB therapy; AUROC, Area under the receiver operating characteristics curve; BCS, Budd-Chiari syndrome; BMI, body mass index; CBF, cerebral blood flow; CBFV, cerebral blood flow volume; CE, cerebral edema; CHBV, chronic HBV; CLD, chronic liver disease; CNS, central nervous system; CPI, clinical prognostic indicator; CSF, cerebrospinal fluid; DAMPs, Damage-associated molecular patterns; DILI, drug-induced liver injury; EBV, Epstein-Barr virus; ETCO2, End tidal CO2; GRADE, Grading of Recommendations Assessment Development and Evaluation; HAV, hepatitis A virus; HBV, Hepatitis B virus; HELLP, hemolysis; HEV, hepatitis E virus; HLH, Hemophagocytic lymphohistiocytosis; HSV, herpes simplex virus; HV, hepatic vein; HVOTO, hepatic venous outflow tract obstruction; IAHG, International Autoimmune Hepatitis Group; ICH, intracerebral hypertension; ICP, intracerebral pressure; ICU, intensive care unit; IFN, interferon; IL, interleukin; IND-ALF, ALF of indeterminate etiology; INDILI, Indian Network for DILI; KCC, King's College Criteria; LC, liver cirrhosis; LDLT, living donor liver transplantation; LT, liver transplantation; MAP, mean arterial pressure; MHN, massive hepatic necrosis; MPT, mitochondrial permeability transition; MUAC, mid-upper arm circumference; NAPQI, n-acetyl-p-benzo-quinone-imine; NPV, negative predictive value; NWI, New Wilson's Index; ONSD, optic nerve sheath diameter; PAMPs, pathogen-associated molecular patterns; PCR, polymerase chain reaction; PELD, Pediatric End-Stage Liver Disease; PPV, positive predictive value; PT, prothrombin time; RAAS, renin–angiotensin–aldosterone system; SHF, subacute hepatic failure; SIRS, systemic inflammatory response syndrome; SNS, sympathetic nervous system; TB, tuberculosis; TCD, transcranial Doppler; TGF, tumor growth factor; TJLB, transjugular liver biopsy; TLR, toll-like receptor; TNF, tumor necrosis factor; TSFT, triceps skin fold thickness; US, ultrasound; USALF, US Acute Liver Failure; VZV, varicella-zoster virus; WD, Wilson disease; Wilson disease (WD); YP, yellow phosphorus; acute liver failure; autoimmune hepatitis (AIH); drug-induced liver injury; elevated liver enzymes, low platelets; sALI, severe acute liver injury; viral hepatitis.