Acute Liver Failure in Children

Robert H. Squires, Jr.

Disclosures

Semin Liver Dis. 2008;28(2):153-166. 

In This Article

Abstract and Introduction

Abstract

Acute liver failure (ALF) in children differs from that observed in adults in both the etiologic spectrum and the clinical picture. Children, particularly very young ones, do not demonstrate classical features of encephalopathy and the definition of ALF has been revised to include patients with advanced coagulopathy, regardless of mental status. A significant number of these children will go on to require transplant or die. Etiologies vary by age with metabolic and infectious diseases prominent in the first year of life and acetaminophen overdose and Wilson's disease occurring in adolescents. In almost 50% of cases, however, the child has an indeterminate cause for ALF. Management requires a multidisciplinary approach and is directed at establishing the etiology where possible and monitoring, anticipating, and managing the multisystem complications that occur in children with ALF. Overall, short-term outcomes are better in children than adults but are dependent upon the degree of encephalopathy and diagnosis.

Introduction

Acute liver failure (ALF) is not a diagnosis but a clinical syndrome. ALF was initially characterized in adults with biochemical evidence of severe hepatic dysfunction (e.g., jaundice and coagulopathy) complicated by hepatic encephalopathy that develops within 8 weeks of the onset of the signs and symptoms of liver disease.[1,2,3] Initial studies in children utilized the adult definition of ALF.[4,5,6] However, recognition of hepatic encephalopathy in children is difficult and may not be clinically apparent until the terminal stages of the disease process.[6] Thus, more recent single site reviews of ALF have included children without clinical encephalopathy.[7,8]

The Pediatric Acute Liver Failure (PALF) Study Group was formed in 2000 as a multisite, multinational consortium to prospectively study ALF in children from birth up to 18 years of age. A consensus reached by 21 PALF investigators defined entry criteria of the study: (1) no evidence of a known chronic liver disease; (2) hepatic-based coagulopathy that is not corrected by parenteral administration of vitamin K; (3) hepatic encephalopathy must be present if the uncorrected prothrombin time (PT) or international normalized ratio (INR) was between 15 and 19.9 seconds or 1.5 to 1.9, respectively; and (4) hepatic encephalopathy was not required if the PT or INR was greater than or equal to 2.0 seconds or 2.0, respectively.[9] Other presentations of liver failure such as subfulminate hepatic failure,[10,11] acute-on-chronic liver failure,[12,13] and primary nonfunction following liver transplantation[14] were excluded from the PALF study and will not be covered in this discussion.

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