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BACKGROUND

Liver failure, or hepatic failure, is a clinical condition that results from significant hepatocyte dysfunction or death. It differs from hepatitis in that patients must have uncorrectable coagulopathy in addition to hepatocyte injury, with or without encephalopathy. Hepatic failure is an acute process and should be differentiated from the acute decompensation of chronic liver disease.

The strict criteria for acute or fulminant liver failure in adults include encephalopathy, coagulopathy, and evidence of hepatic dysfunction without prior evidence of liver disease, occurring within 8 weeks of the first symptoms of illness. Because encephalopathy is uncommon and difficult to identify in infants and young children, most clinicians use uncorrectable coagulopathy and hepatic dysfunction as clinical criteria for liver failure in this age group.

Recognition of hepatic failure and its associated metabolic disturbances (Table 79-1) is crucial so that supportive therapy can be provided until recovery, or liver transplantation. Hepatic failure accounts for up to 15% of pediatric liver transplants in the United States each year.

TABLE 79-1Complications of Acute Hepatic Failure

CLINICAL PRESENTATION

NEONATES AND INFANTS

Neonates and young infants can present with a range of symptoms, depending on the disease. Some infants are quite ill immediately after birth with coagulopathy and acidosis. This presentation is highly suggestive of hypoxic or ischemic injury, neonatal hemochromatosis, neonatal enteroviral infection, or some other intrauterine or perinatal insult. Laboratory tests may reveal elevated transaminases in the high hundreds to thousands (suggestive of ischemia) and hyperbilirubinemia. Hypoglycemia may also be present. Clinical symptoms suggestive of sepsis, including hypotension and poor perfusion, may occur. Encephalopathy is recognizable in only one-third of these infants.1 Infants with neonatal hemochromatosis have intrauterine growth retardation, coagulopathy, hypoalbuminemia, ascites, mild transaminase elevation, and varying degrees of renal insufficiency.

CHILDREN AND ADOLESCENTS

Children with acute hepatic failure also may present with a wide variety of symptoms. Those with infectious hepatitis may have fever, malaise, nausea, jaundice, and right upper quadrant pain. They may have been recently discharged from the emergency department or physician’s office with a diagnosis of hepatitis and elevated transaminases, jaundice, and a normal prothrombin time (PT). Many children improve (especially those with hepatitis A), but some return with rapidly worsening jaundice, and signs of coagulopathy such as petechiae, bruising, or bleeding. Mental status changes with ...

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