A patient who presents emergently with acute liver failure (ALF) has an overall greater likelihood of either dying or requiring emergent transplantation than recovering without transplant.1 Regardless of etiology, the unifying feature of ALF is a compact clinical timeline and a rapid, often precipitous natural history of disease. Whereas spontaneous recovery of liver function is possible with supportive measures, particularly with acetaminophen overdose, there remains a significant risk of spiraling decline after presentation with multiorgan failure, bleeding, and infectious complications often heralded by high-grade encephalopathy with cerebral edema.2
The key message for the emergency critical care practitioner is that first contact with a patient with ALF requires an orchestrated team effort to be triggered to rapidly and efficiently triage and mobilize management resources. Because of the rarity and complexity of ALF, it has been argued that ALF is best managed within the framework of a previously defined protocol, similar to the standards that have gained broad acceptance in stroke and acute coronary syndrome.3 Such team efforts are crucial to give the patient with ALF the best opportunity for transplant-free survival. In the setting of deteriorating status, teams are required to rapidly mobilize those resources, interventions, and caregivers that are crucial to provide stabilization and life support as well as to triage rapidly and for transplantation in a crisis setting when time course may not forgive hesitation.
Recommendations and best evidence for developing this approach are substantially informed by the Acute Liver Failure Study Group (ALFSG) registry from a consortium of transplant centers that continues to prospectively collect data, report their findings, and grade level of evidence.4 This chapter is designed as a practical guide for the emergency critical care practitioner to provide an organizational framework for key clinical interventions.
Often used interchangeably, both the terms ALF and fulminant hepatic failure are defined by the new onset of hepatocellular dysfunction as reflected by coagulopathy (INR >1.5) and encephalopathy in the absence of preexisting liver disease.5 By convention, the further stratification of fulminant hepatic failure is based on the rapidity of encephalopathy onset in the course of illness: less than 2 weeks for acute fulminant and 8 weeks for subfulminant.6 The ALFSG study consortium has extended that time course up to 26 weeks for entry in their multicenter data analysis, and have adopted the preferred eponymous term arguing that this captures the variable pace of illness in ALF, and better encompasses an extended range of patients who share epidemiologic, etiologic, physiologic, and management characteristics.7 In the absence of encephalopathy or coagulopathy, population-based studies of patients at risk of ALF have defined hepatotoxicity by ALT >1,000.8
How to Suspect and Make the Diagnosis
As suggested by the variable terminology, the patient with ALF in the absence of prior liver disease may present with a strikingly distinct duration of symptoms, variable chief complaints, and without a clear-cut ...