King's College Criteria[64]O'Grady JG, Alexander GJ, Hayllar KM, et al. Early indicators of prognosis in fulminant hepatic failure. Gastroenterology. 1989 Aug;97(2):339-45.
http://www.ncbi.nlm.nih.gov/pubmed/2490426?tool=bestpractice.com
The most widely accepted prognostic tool for patients who present with ALF. They were developed through a retrospective analysis of 588 consecutive patients with ALF who were admitted to the King's College Hospital Liver Unit between 1973 and 1987.[64]O'Grady JG, Alexander GJ, Hayllar KM, et al. Early indicators of prognosis in fulminant hepatic failure. Gastroenterology. 1989 Aug;97(2):339-45.
http://www.ncbi.nlm.nih.gov/pubmed/2490426?tool=bestpractice.com
Prognostic factors associated with mortality were identified and assessed for predictive value. Note that the international normalised ratio (INR) level used in this prognostic tool differs from the INR level used as a diagnostic feature of ALF.
Although fulfilment of these criteria has a high specificity for mortality, the sensitivity and negative predictive value remain low. Therefore, not fulfilling the criteria does not ensure survival.[81]Pauwels A, Mostefa-Kara N, Florent C, et al. Emergency liver transplantation for acute liver failure. Evaluation of London and Clichy criteria. J Hepatol. 1993 Jan;17(1):124-7.
http://www.ncbi.nlm.nih.gov/pubmed/8445211?tool=bestpractice.com
[82]Anand AC, Nightingale P, Neuberger JM. Early indicators of prognosis in fulminant hepatic failure: an assessment of the King's criteria. J Hepatol. 1997 Jan;26(1):62-8.
http://www.ncbi.nlm.nih.gov/pubmed/9148024?tool=bestpractice.com
[83]Shakil AO, Kramer D, Mazariegos GV, et al. Acute liver failure: clinical features, outcome analysis, and applicability of prognostic criteria. Liver Transpl. 2000 Mar;6(2):163-9.
http://onlinelibrary.wiley.com/doi/10.1002/lt.500060218/pdf
http://www.ncbi.nlm.nih.gov/pubmed/10719014?tool=bestpractice.com
[84]Bailey B, Amre DK, Gaudreault P. Fulminant hepatic failure secondary to acetaminophen poisoning: a systematic review and meta-analysis of prognostic criteria determining the need for liver transplantation. Crit Care Med. 2003 Jan;31(1):299-305.
http://www.ncbi.nlm.nih.gov/pubmed/12545033?tool=bestpractice.com
[85]McPhail MJ, Wendon JA, Bernal W. Meta-analysis of performance of Kings's College Hospital Criteria in prediction of outcome in non-paracetamol-induced acute liver failure. J Hepatol. 2010 Sep;53(3):492-9.
http://www.ncbi.nlm.nih.gov/pubmed/20580460?tool=bestpractice.com
The King’s College Criteria has a sensitivity of 68% to 69% and a specificity of 82% to 92%.[8]American Association for the Study of Liver Diseases. AASLD position paper: the management of acute liver failure: update 2011. Nov 2011 [internet publication].
https://www.aasld.org/practice-guidelines/management-acute-liver-failure
Although the King’s College Criteria have been validated in adult cohorts with ALF, data suggest they may not reliably predict outcomes in the paediatric population.[86]Sundaram V, Shneider BL, Dhawan A, et al. King's College Hospital Criteria for non-acetaminophen induced acute liver failure in an international cohort of children. J Pediatr. 2013 Feb;162(2):319-23.e1.
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3504621
http://www.ncbi.nlm.nih.gov/pubmed/22906509?tool=bestpractice.com
Overall, these criteria are instrumental in selecting patients who have a high risk of mortality with ALF. However, they have limitations, and reliance upon prognostic scoring systems to determine candidacy for liver transplantation is not recommended by the American Association for the Study of Liver Diseases.[8]American Association for the Study of Liver Diseases. AASLD position paper: the management of acute liver failure: update 2011. Nov 2011 [internet publication].
https://www.aasld.org/practice-guidelines/management-acute-liver-failure
ALF secondary to paracetamol overdose:
Non-paracetamol associated ALF:
INR >6.5 (PT >100 seconds), or
any 3 of the following: age <10 or >40 years; aetiology non-A, non-B hepatitis, or idiosyncratic drug reaction; duration of jaundice before hepatic encephalopathy >7 days; INR >3.5 (PT >50 seconds); serum bilirubin >300 micromol/L (>17.6 mg/dL).
Clichy criteria[62]Bernuau J, Samuel D, Durand F, et al. Criteria for emergency liver transplantation in patients with acute viral hepatitis and factor V below 50% of normal: a prospective study. Hepatology. 1991;14:49A.
Based on a French prospective study of patients presenting with acute viral hepatitis, in which patients identified as having the lowest survival without liver transplantation included those with hepatic encephalopathy and low factor V levels.[62]Bernuau J, Samuel D, Durand F, et al. Criteria for emergency liver transplantation in patients with acute viral hepatitis and factor V below 50% of normal: a prospective study. Hepatology. 1991;14:49A. These criteria predicted mortality with a positive predictive value of 82% and negative predictive value of 98% in this cohort. However, subsequent studies have reported much lower predictive values which were inferior to the King's College Criteria in other populations, including paracetamol and non-paracetamol ALF.[81]Pauwels A, Mostefa-Kara N, Florent C, et al. Emergency liver transplantation for acute liver failure. Evaluation of London and Clichy criteria. J Hepatol. 1993 Jan;17(1):124-7.
http://www.ncbi.nlm.nih.gov/pubmed/8445211?tool=bestpractice.com
[87]Izumi S, Langley PG, Wendon J, et al. Coagulation factor V levels as a prognostic indicator in fulminant hepatic failure. Hepatology. 1996 Jun;23(6):1507-11.
http://www.ncbi.nlm.nih.gov/pubmed/8675171?tool=bestpractice.com
Presence of hepatic encephalopathy and factor V level:
Model for End-Stage Liver Disease (MELD)[88]Kamath PS, Wiesner RH, Malinchoc M, et al. A model to predict survival in patients with end-stage liver disease. Hepatology. 2001 Feb;33(2):464-70.
http://www.ncbi.nlm.nih.gov/pubmed/11172350?tool=bestpractice.com
[89]Wiesner R, Edwards E, Freeman R, et al. Model for end-stage liver disease (MELD) and allocation of donor livers. Gastroenterology. 2003 Jan;124(1):91-6.
http://www.ncbi.nlm.nih.gov/pubmed/12512033?tool=bestpractice.com
Adopted by the United Network for Organ Sharing and the Organ Procurement and Transplantation Network organisation, the MELD score is well established as a validated predictive model of short-term mortality in patients with cirrhosis and is currently utilised in the allocation of donor organs in patients awaiting liver transplantation in the US.[88]Kamath PS, Wiesner RH, Malinchoc M, et al. A model to predict survival in patients with end-stage liver disease. Hepatology. 2001 Feb;33(2):464-70.
http://www.ncbi.nlm.nih.gov/pubmed/11172350?tool=bestpractice.com
[89]Wiesner R, Edwards E, Freeman R, et al. Model for end-stage liver disease (MELD) and allocation of donor livers. Gastroenterology. 2003 Jan;124(1):91-6.
http://www.ncbi.nlm.nih.gov/pubmed/12512033?tool=bestpractice.com
Several retrospective studies have reported the MELD score to have similar predictive value to the King's College Criteria for mortality associated with ALF.[90]Kremers WK, van IJperen M, Kim WR, et al. MELD score as a predictor of pretransplant and posttransplant survival in OPTN/UNOS status 1 patients. Hepatology. 2004 Mar;39(3):764-9.
http://www.ncbi.nlm.nih.gov/pubmed/14999695?tool=bestpractice.com
[91]Zaman MB, Hoti E, Qasim A, et al. MELD score as a prognostic model for listing acute liver failure patients for liver transplantation. Transplant Proc. 2006 Sep;38(7):2097-8.
http://www.ncbi.nlm.nih.gov/pubmed/16980011?tool=bestpractice.com
[92]Katoonizadeh A, Decaestecker J, Wilmer A, et al. MELD score to predict outcome in adult patients with non-acetaminophen-induced acute liver failure. Liver Int. 2007 Apr;27(3):329-34.
http://www.ncbi.nlm.nih.gov/pubmed/17355453?tool=bestpractice.com
[93]Yantorno SE, Kremers WK, Ruf AE, et al. MELD is superior to King's College and Clichy's criteria to assess prognosis in fulminant hepatic failure. Liver Transpl. 2007 Jun;13(6):822-8.
http://onlinelibrary.wiley.com/doi/10.1002/lt.21104/full
http://www.ncbi.nlm.nih.gov/pubmed/17539002?tool=bestpractice.com
Prospective data from the US Acute Liver Failure Study Group revealed that a MELD score ≥30 in patients with paracetamol overdose had a high negative predictive value of 82%, such that patients with MELD scores <30 had a high probability of survival. In non-paracetamol ALF, a MELD score ≥30 had a positive predictive value of 81%, yet these values were not more accurate than the King's College Criteria.[8]American Association for the Study of Liver Diseases. AASLD position paper: the management of acute liver failure: update 2011. Nov 2011 [internet publication].
https://www.aasld.org/practice-guidelines/management-acute-liver-failure
[94]Rossaro L, Chambers CC, Polson J, et al. Performance of MELD in predicting outcome in acute liver failure (Abstract S1492). Gastroenterology. 2005;128(suppl 2):A-705. Based on findings from a large meta-analysis, the MELD score could have a role in predicting hospital mortality, particularly in non-paracetamol ALF.[95]McPhail MJ, Farne H, Senvar N, et al. Ability of King's College criteria and Model for End-stage Liver Disease scores to predict mortality of patients with acute liver failure: a meta-analysis. Clin Gastroenterol Hepatol. 2016 Apr;14(4):516-25.e5.
http://www.cghjournal.org/article/S1542-3565(15)01403-2/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/26499930?tool=bestpractice.com
Acute Physiology and Chronic Health Evaluation (APACHE) II[96]Knaus WA, Draper EA, Wagner DP, et al. APACHE II: a severity of disease classification system. Crit Care Med. 1985 Oct;13(10):818-29.
http://www.ncbi.nlm.nih.gov/pubmed/3928249?tool=bestpractice.com
The APACHE II scoring system was developed to predict mortality in patients of all disease categories admitted to intensive care units. The score comprises 12 common physiological and laboratory parameters, adjusted for patient age and underlying chronic health problems.[96]Knaus WA, Draper EA, Wagner DP, et al. APACHE II: a severity of disease classification system. Crit Care Med. 1985 Oct;13(10):818-29.
http://www.ncbi.nlm.nih.gov/pubmed/3928249?tool=bestpractice.com
One prospective study in patients with paracetamol overdose found that an APACHE II score >15 was associated with high mortality and provided similar predictive value to the King's College Criteria, while another study found a score of ≥20 to be more predictive of mortality and need for liver transplant.[25]Larson AM, Polson J, Fontana RJ, et al. Acetaminophen-induced acute liver failure: results of a United States multicenter, prospective study. Hepatology. 2005 Dec;42(6):1364-72.
http://www.ncbi.nlm.nih.gov/pubmed/16317692?tool=bestpractice.com
[97]Mitchell I, Bihari D, Chang R, et al. Earlier identification of patients at risk from acetaminophen-induced acute liver failure. Crit Care Med. 1998 Feb;26(2):279-84.
http://www.ncbi.nlm.nih.gov/pubmed/9468165?tool=bestpractice.com
Acute Liver Failure Study Group (ALFSG) Index[98]Rutherford A, King LY, Hynan LS, et al; ALF Study Group. Development of an accurate index for predicting outcomes of patients with acute liver failure. Gastroenterology. 2012 Nov;143(5):1237-43.
http://www.gastrojournal.org/article/S0016-5085%2812%2901155-9/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/22885329?tool=bestpractice.com
[99]Koch DG, Tillman H, Durkalski V, et al. Development of a model to predict transplant-free survival of patients with acute liver failure. Clin Gastroenterol Hepatol. 2016 Aug;14(8):1199-206.e2.
http://www.ncbi.nlm.nih.gov/pubmed/27085756?tool=bestpractice.com
A prognostic index was developed utilising a cohort of 250 patients enrolled in the ALFSG, and then validated in a separate cohort of 250 patients. Variables at the time of initial presentation that were found to have a strong association with mortality or need for liver transplantation included advanced coma grade, bilirubin, INR, elevated phosphorus, and serum levels of M30 antigen, a marker of apoptotic hepatocyte cell death. This index was found to have an overall sensitivity of 85.6% and specificity of 64.7% with no significant difference in performance between paracetamol and non-paracetamol ALF. Although this predictive index was superior to the King’s College Criteria and the MELD score, assessment of M30 antigen requires an enzyme-linked immunosorbent assay (ELISA)-based test and may not be readily available at most centres.[98]Rutherford A, King LY, Hynan LS, et al; ALF Study Group. Development of an accurate index for predicting outcomes of patients with acute liver failure. Gastroenterology. 2012 Nov;143(5):1237-43.
http://www.gastrojournal.org/article/S0016-5085%2812%2901155-9/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/22885329?tool=bestpractice.com
An additional model predictive of transplant-free survival was developed from the ALFSG database, involving 878 patients, and was then validated in a cohort of 885 patients. Variables predictive of transplant-free survival included the degree of hepatic encephalopathy, aetiology of ALF, requirement for vasopressors, bilirubin, and INR. Aetiologies of ALF considered to be favourable in this model included paracetamol (acetaminophen) overdose, pregnancy, ischaemia, or hepatitis A. The model performed with a sensitivity of 37% and specificity of 95% in determining an 80% transplant-free survival within the validation cohort.[99]Koch DG, Tillman H, Durkalski V, et al. Development of a model to predict transplant-free survival of patients with acute liver failure. Clin Gastroenterol Hepatol. 2016 Aug;14(8):1199-206.e2.
http://www.ncbi.nlm.nih.gov/pubmed/27085756?tool=bestpractice.com
West Haven criteria for hepatic encephalopathy[49]Vilstrup H, Amodio P, Bajaj J, et al. Hepatic encephalopathy in chronic liver disease: 2014 practice guideline by the American Association for the Study of Liver Diseases and the European Association for the Study of the Liver. Hepatology. 2014 Aug;60(2):715-35.
https://aasldpubs.onlinelibrary.wiley.com/doi/full/10.1002/hep.27210
http://www.ncbi.nlm.nih.gov/pubmed/25042402?tool=bestpractice.com
Grade 1: subtly impaired awareness, sleep alterations, shortened attention span, impaired addition or subtraction, heightened mood or anxiety, oriented in time and space.
Grade 2: lethargy or apathy, disorientation for time, obvious personality change, inappropriate behaviour, dyspraxia, asterixis.
Grade 3: somnolence to semi-stupor, responsive to vocal stimuli, marked confusion, gross disorientation (disoriented in time and space), bizarre behaviour. Physical findings may include hyper-reflexia, nystagmus, clonus, and rigidity.
Grade 4: coma.