What are the guideline sources, guidelines and references for Acute liver failure ?
Guideline sources The following summarized guidelines for the evaluation and management of acute liver failure are prepared by our editorial team based on guidelines from the Society of Critical Care Medicine (SCCM 2023; 2020), the American Society for Apheresis (ASFA 2019), the European Association for the Study of the Liver (EASL 2017; 2012), and the American Gastroenterological Association (AGA 2017). 1 2 3 4 5 6 7 7 7 8 8 Definition ALF is a disease characterized by an acute impairment of liver function, leading to encephalopathy and coagulopathy. Hyper-acute, acute, and sub-ALF are defined as occurring within 7 days, 7-28 days, and 28 days-6 months of the onset of symptoms, respectively. 7 Epidemiology https://web.pathway.md/diseases/reccyeTojFmEAip8l 1/9 6/23/23, 1:58 AM Acute liver failure Pathway Causes of ALF include acetaminophen poisoning, viral hepatitis (hepatitis A, B, C, D, and E, HSV, Ebstein-Barr virus, CMV), AIH, idiosyncratic toxins (tetracycline, halothane, isoniazid, anabolic steroids, phytopharmaceuticals), Wilson's disease, fatty liver of pregnancy, and Budd-Chiari syndrome. 7 Pathophysiology The annual incidence of ALF is estimated at approximately 0.55-0.62 cases per 100,000 person- years. 8 Disease course In patients with ALF, apoptosis and necrosis of parenchymal hepatocytes impairs normal liver function, resulting in coagulopathy, hepatic encephalopathy, multiple organ failure, and death. 7 Prognosis and risk of recurrence The hospital survival rates of ALF patients treated with liver transplantation and without liver transplantation are 80-86% and 35-48%, respectively. 8 Calculator King's College Criteria for aceta Guidelines 1. Screening and diagnosis Definitions and clinical presentation: recognize that severe acute liver injury is defined as a syndrome characterized by markers of liver damage (elevated serum transaminases) and impaired liver function (jaundice and INR > 1.5) usually preceding clinical encephalopathy. B Show 5 more 2. Classification and risk stratification Prognosis: As per AGA 2017 guidelines, consider using the MELD score, rather than King's College Criteria, as a prognostic scoring system in patients presenting with ALF. C As per EASL 2017 guidelines, obtain prognostic assessment both in the transplant center and at the site of first presentation to allow making decisions in relation to patient transfer to a specialist center at the earliest opportunity. B https://web.pathway.md/diseases/reccyeTojFmEAip8l 2/9 6/23/23, 1:58 AM Acute liver failure Pathway Show 2 more 3. Diagnostic investigations Evaluation for viral hepatitis: As per AGA 2017 guidelines: Consider testing and treating HSV in patients presenting with ALF. C Consider avoiding routine testing for VZV in immunocompetent patients with ALF. D As per EASL 2017 guidelines, screen for viral etiologies and co-factor effects in all patients with ALF. B Evaluation for autoimmune hepatitis: As per AGA 2017 guidelines, consider autoantibody testing in patients presenting with ALF. C As per EASL 2017 guidelines, suspect autoimmune etiology in patients presenting with other autoimmune disorders, elevated globulin fraction and autoantibodies, recognizing that these features, however, may be absent and liver biopsy may be required. B Evaluation for drug-induced liver injury: As per SCCM 2020 guidelines, screen patients with ALF for drug-induced causes of liver failure. Discontinue drugs proven or highly suspected to be the cause of ALF. E As per EASL 2017 guidelines, recognize that DILI, especially acetaminophen toxicity is the most frequent cause of severe acute liver injury and ALF. Obtain a toxicology screen and determine acetaminophen level in every patient at admission, although levels will frequently be negative. Initiate N-acetyl cysteine therapy if the patient already has coagulopathy and increased serum transaminases. B Show 2 more Evaluation for malignancy: exclude malignant infiltration by imaging or liver biopsy in patients with a history of cancer or significant hepatomegaly. B Evaluation for Wilson's disease: As per AGA 2017 guidelines, avoid obtaining routine testing for Wilson's disease in patients presenting with ALF. D As per EASL 2017 guidelines, suspect Wilson's disease in patients with Coombs-negative hemolytic anemia and high bilirubin to ALP ratio. B Evaluation for Budd-Chiari syndrome: suspect Budd-Chiari syndrome in patients presenting with ALF with gross ascites. Confirm the diagnosis based on imaging techniques. B Evaluation for other etiologies: Screen for systemic diseases presenting as ALF. B Assess clinical context to identify less common causes of ALF. B Screening for hepatic encephalopathy: obtain intensive screening at the first sign of hepatic encephalopathy as the clinical appearance of hepatic encephalopathy is crucial for the diagnosis https://web.pathway.md/diseases/reccyeTojFmEAip8l 3/9 6/23/23, 1:58 AM Acute liver failure Pathway of ALF but mental alterations may be initially subtle. B Viscoelastic testing: Consider obtaining viscoelastic testing (thromboelastography/rotational thromboelastography) over measuring INR, platelet, and fibrinogen in critically ill patients with ALF. C Obtain viscoelastic testing (thromboelastography/rotational thromboelastography) over measuring INR, platelet, and fibrinogen in critically ill patients with ALF undergoing procedures. B 4. Diagnostic procedures Liver biopsy: As per AGA 2017 guidelines, consider avoiding the routine use of liver biopsy in patients presenting with ALF. D As per EASL 2017 guidelines, recognizing that indications for liver biopsy in ALF are limited. Perform liver biopsy preferably by a transjugular route, in a center experienced in its use, and with access to a histopathologist with liver experience. Exclude underlying chronic liver disease, malignancies or alcohol-induced liver disease if possible, recognizing that this does not provide prognostic information. B 5. Respiratory support Supplemental oxygen: Provide supportive care with supplemental oxygen in the management of patients with hepatopulmonary syndrome, pending possible liver transplantation. E Consider using high-flow nasal cannula over noninvasive ventilation in hypoxic critically ill patients with ALF. C Mechanical ventilation: As per SCCM 2020 guidelines: Consider using a low tidal volume strategy in patients with ALF and ARDS. C Avoid using high PEEP in patients with ALF and ARDS. D As per EASL 2017 guidelines, administer standard sedation and use lung-protective ventilation techniques in patients with ALF. B Show 2 more 6. Medical management Setting of care: obtain prognostic assessment both in the transplant center and at the site of first presentation to allow making decisions in relation to patient transfer to a specialist center at the earliest opportunity. B Show 3 more https://web.pathway.md/diseases/reccyeTojFmEAip8l 4/9 6/23/23, 1:58 AM Acute liver failure Pathway General principles (goals of care): consider targeting a mean arterial pressure of 65 mmHg in patients with ALF, with concomitant assessment of perfusion. C Show 2 more General principles (precautions): adjust the doses of medications undergoing hepatic metabolism based on the patient's residual hepatic function. Consult a clinical pharmacist when available. E Fluid resuscitation: As per SCCM 2020 guidelines: Do not use hydroxyethyl starch for initial fluid resuscitation of patients with ALF. D Avoid using gelatin solutions for initial fluid resuscitation of patients with ALF. D As per EASL 2017 guidelines: Initiate crystalloid volume resuscitation as most patients are volume depleted at presentation. B Recognize that volume overload is as detrimental as underfilling. B Albumin: consider administering albumin over other fluids for resuscitation of patients with ALF, especially if serum albumin is < 3 mg/dL. C Vasopressors: As per SCCM 2020 guidelines: Administer norepinephrine as first-line vasopressor in patients with ALF remaining hypotensive despite fluid resuscitation, or in patients with profound hypotension and tissue hypoperfusion even if fluid resuscitation is ongoing. B Consider adding low-dose vasopressin to norepinephrine in patients with ALF remaining hypotensive despite fluid resuscitation. C As per EASL 2017 guidelines, administer vasopressive agents guided by appropriate monitoring techniques in patients with persistent hypotension. B Administer norepinephrine as the vasopressor of choice. B Inotropes: consider administering inotropic agents in patients with hypoxic hepatitis. B Corticosteroids: As per SCCM 2020 guidelines, consider administering stress-dose corticosteroids for the management of septic shock in patients with ALF. C As per EASL 2017 guidelines: Initiate hydrocortisone therapy to decrease vasopressor requirements, recognizing that it does not reduce mortality. B Consider initiating early treatment with corticosteroids in patients with suspected autoimmune etiology. B N-acetylcysteine: As per AGA 2017 guidelines, initiate N-acetyl cysteine therapy in patients presenting with acetaminophen-associated ALF. B https://web.pathway.md/diseases/reccyeTojFmEAip8l 5/9 6/23/23, 1:58 AM Acute liver failure Pathway As per EASL 2017 guidelines, initiate N-acetylcysteine in patients with coagulopathy and increased serum transaminases. B Management of metabolic derangements: pay stringent attention to detail and normalization of biochemical abnormalities in patients with ALF. B Show 2 more Management of coagulopathy: do not administer routine FFP and other coagulation factors, limit their administration to specific situations, such as insertion of ICP monitors or active bleeding. D Show 2 more Management of infection: obtain regular periodic surveillance cultures in all patients with ALF. B Show 3 more Management of portopulmonary hypertension: consider treating portopulmonary hypertension with agents approved for pulmonary arterial hypertension in patients with mean pulmonary artery pressure > 35 mmHg. C Management of vascular complications: consider administering LMWH or vitamin K antagonists over offering conservative management in patients with portal venous thrombosis or pulmonary embolus. C Management of hepatic encephalopathy: As per SCCM 2023 guidelines, consider administering hypertonic saline in critically ill patients with ALF at risk of developing intracranial hypertension. C Show 3 more As per AGA 2017 guidelines, consider avoiding the empiric use of treatments to reduce ICP in patients presenting with ALF. D As per EASL 2017 guidelines, evaluate patients with low grade encephalopathy frequently for signs of worsening encephalopathy. B Show 5 more 7. Inpatient care Clinical and laboratory monitoring: As per SCCM 2020 guidelines: Consider placing an arterial catheter for BP monitoring in patients with ALF and shock. C Consider obtaining invasive hemodynamic monitoring to guide therapy in patients with ALF and clinically impaired perfusion. C As per EASL 2017 guidelines: Obtain frequent senior clinical review (BID minimum) and assess physiological parameters, blood results and metabolic status in patients with ALF. B Assess hourly urine output as a marker of renal function, alongside creatinine. B https://web.pathway.md/diseases/reccyeTojFmEAip8l 6/9 6/23/23, 1:58 AM Acute liver failure Pathway 8. Nonpharmacologic interventions Nutrition: As per SCCM 2020 guidelines, avoid using a low protein goal in patients with ALF. Consider targeting protein goals comparable to critically ill patients without liver failure (such as 1.2-2.0 g protein/kg dry or ideal body weight per day). D Show 2 more As per EASL 2017 guidelines, initiate enteral or parenteral nutrition because patients with ALF have increased resting energy expenditure. B Show 4 more 9. Therapeutic procedures Plasmapheresis: As per SCCM 2023 guidelines, consider performing plasma exchange, when available, in critically ill patients with ALF developing hyperammonemia. C As per EASL 2017 guidelines, consider performing plasma exchange to improve transplant-free survival and to modulate immune dysfunction in patients with ALF. B Consider performing plasma exchange in patients treated early who will not ultimately undergo liver transplantation. B Renal replacement therapy: As per SCCM 2020 guidelines, consider performing early RRT in patients with ALF and AKI. C As per EASL 2017 guidelines, recognize that lactate elevation is related to increased production and decreased clearance, and remains a poor prognostic marker. Perform RRT to correct acidosis and metabolic disturbances. B Show 3 more Pleurodesis: place a chest tube with an attempt to pleurodesis for hepatic hydrothorax in patients unfit for TIPS or as a palliative intent. E Extracorporeal liver support: As per SCCM 2023 guidelines, consider initiating extracorporeal liver support or standard medical therapy in critically ill patients with ALF. C As per AGA 2017 guidelines, insufficient evidence to make a conclusive recommendation for or against the use of extracorporeal artificial liver support systems in patients with ALF. These systems should only be used within the context of a clinical trial. As per EASL 2017 guidelines, consider using liver support system techniques (biological or adsorbent) only in the context of RCTs. B 10. Surgical interventions Liver transplantation, indications: https://web.pathway.md/diseases/reccyeTojFmEAip8l 7/9 6/23/23, 1:58 AM Acute liver failure Pathway As per EASL 2017 guidelines, assess patients with ALF for emergency liver transplantation by a multidisciplinary team with appropriate experience in this process. B Show 7 more As per EASL 2012 guidelines, perform liver transplantation in patients with ALF due to Wilson's disease if the revised King's score is 11. B Liver transplantation (management of deceased donor liver transplant): Consider administering systemic corticosteroids for deceased liver graft donors. C Consider employing either using goal-directed fluid management or standard fluid management strategies for the deceased organ donor. C Liver transplantation (peri-transplant management): avoid performing selective bowel decontamination in critically ill liver transplant candidates. D Show 6 more 11. Specific circumstances Pediatric patients: Recognize that in pediatric patients: definition of ALF is not dependent upon the presence of encephalopathy some etiologies are specific, notably metabolic disorders transplantation criteria are different compared to adults. B Pregnant patients: As per AGA 2017 guidelines, consider testing for hepatitis E in pregnant women presenting with ALF. C As per EASL 2017 guidelines, initiate prompt delivery of the baby in patients with HELLP and acute fatty liver of pregnancy, especially in patients with elevated lactate levels and hepatic encephalopathy. Offer screening for putative fatty acid defects. B References 1. Rahul Nanchal, Ram Subramanian, Constantine J Karvellas et al. Guidelines for the Management of Adult Acute and Acute-on-Chronic Liver Failure in the ICU Cardiovascular, Endocrine, Hematologic, Pulmonary and Renal Considerations: Executive Summary. Crit Care Med. 2020 Mar;48 3 415 419. Open 2. Wendon, J, Cordoba J et al. EASL Clinical Practical Guidelines on the management of acute (fulminant) liver failure. J Hepatol. 2017 May;66 5 1047 1081. Open 3. Rahul Nanchal, Ram Subramanian, Waleed Alhazzani et al. Guidelines for the Management of Adult Acute and Acute-on-Chronic Liver Failure in the ICU Neurology, Peri-Transplant Medicine, Infectious Disease, and Gastroenterology Considerations. Crit Care Med. 2023 May 1;51 5 657 676. Open 4. Flamm SL, Yang YX, Singh S et al. American Gastroenterological Association Institute Guidelines for the Diagnosis and Management of Acute Liver Failure. Gastroenterology. 2017 Feb;152 3 644 647. Open https://web.pathway.md/diseases/reccyeTojFmEAip8l 8/9 6/23/23, 1:58 AM Acute liver failure Pathway 5. European Association for Study of Liver. EASL Clinical Practice Guidelines: Wilson's disease. J Hepatol. 2012 Mar;56 3 671 85. Open 6. Anand Padmanabhan, Laura Connelly-Smith, Nicole Aqui et al. Guidelines on the Use of Therapeutic Apheresis in Clinical Practice Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Eighth Special Issue. J Clin Apher. 2019 Jun;34 3 171 354. Open 7. Canbay A, Tacke F, Hadem J et al. Acute liver failure: a life-threatening disease. Dtsch Arztebl Int. 2011 Oct;108 42 714 20. Open 8. Thanapirom K, Treeprasertsuk S, Soonthornworasiri N et al. The incidence, etiologies, outcomes, and predictors of mortality of acute liver failure in Thailand: a population-base study. BMC Gastroenterol. 2019 Jan 28;19 1 18. Open 9. Paul Martin, Andrea DiMartini, Sandy Feng et al. Evaluation for liver transplantation in adults: 2013 practice guideline by the American Association for the Study of Liver Diseases and the American Society of Transplantation. Hepatology. 2014 Mar;59 3 1144 65. Open 10. J G O'Grady, G J Alexander, K M Hayllar et al. Early indicators of prognosis in fulminant hepatic failure. Gastroenterology. 1989 Aug;97 2 439 45. Open https://web.pathway.md/diseases/reccyeTojFmEAip8l 9/9