Chapter 424

Liver failure is defined in adults by the requisite hallmarks of the presence of hepatic encephalopathy and uncorrectable coagulopathy within 8 weeks of the development of clinical jaundice, in the absence of known preexisting chronic liver disease. In children, a modified working definition for pediatric acute liver failure has been achieved by consensus of an expert panel (the Pediatric Acute Liver Failure [PALF] Study Group) as follows:

1. 1. No known evidence of chronic liver disease.

2. Biochemical evidence of acute liver injury.

3. Hepatic-based uncorrectable coagulopathy defined as an international normalized ratio (INR) greater than or equal to 1.5 (approximate to a prothrombin time [PT] ⩾ 15 sec) in the presence of clinical hepatic encephalopathy OR (3a) an INR ⩾ 2.0 (approximate to PT ⩾ 20 sec) without hepatic encephalopathy.1

These modifications to the definition for pediatric acute liver failure notably recognize the difficulty of detecting hepatic encephalopathy, particularly the earlier stages, in infants and small children; markers of liver synthetic failure, such as an uncorrectable coagulopathy, as important indicators of liver failure even in the absence of hepatic encephalopathy; and children presenting in acute liver failure may in fact have a chronic liver condition not previously suspected or known. A clinical coma grade scale has been adapted for infants and children under the age of 4 years (Table 424-1).2

Table 424-1 Assessment of Encephalopathy for Young Children: Birth to Age 3 Years

The approach to the child with acute liver failure should include the following: (1) establishment of an accurate diagnosis; (2) provision of intense, comprehensive medical supportive therapy; (3) anticipation, prevention, and timely treatment of complications, which may preclude the realization of spontaneous recovery; and (4) early referral to a liver transplant center, prior to the onset of complications of multiorgan failure, to assess both for indications and contraindications to liver transplantation. The causes of acute liver failure in children include a variety of age-dependent etiologies (Table 424-2). The diagnostic evaluations for these disorders is discussed elsewhere.

Table 424-2. Conditions Associated with Acute Liver Failure at Different Ages

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