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Liver failure is a final common pathway for a broad range of diagnoses. In the patient with liver failure, accurate nutritional assessment may be complicated by fluid shifts and organomegaly. Nutrition therapy in this population can be complex due to metabolic and electrolyte derangements. When chronic liver disease (CLD) is present, malabsorption of fats and fat-soluble vitamins may lead to multiple nutritional deficiencies. Liver failure requiring admission to the pediatric intensive care unit (PICU) can be divided into 2 categories: acute liver failure (ALF) and acute-on-chronic liver failure (ACLF). Even during early stages of disease, impaired nutritional status has been associated with poor clinical outcome and higher morbidity and mortality in the posttransplant period.1-3 This chapter will review some of the underlying causes of malnutrition and provide recommendations for assessment and management of nutrition support for patients with liver disease in the PICU setting.
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The Pediatric Acute Liver Failure (PALF) study group defined the following entry criteria for ALF (Table 16-1).4
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Liver failure has been further classified based on the timing of onset of encephalopathy. Hyperacute liver failure occurs within 1 week of onset, ALF from 1 to 4, weeks and subacute liver failure from 4 to 12 weeks after onset of hepatic disease.1,2,5 These definitions may be difficult to apply in pediatrics due to the complexity of assessing encephalopathy in young children.
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The exact incidence of pediatric ALF is not known. Among all age groups, the incidence of ALF is estimated to be 5.5 per million per year.3,4 Approximately 675 pediatric liver transplants are done in the United States each year, of which 10% to 13% are done in patients with ALF. These numbers do not include children who recover or die without liver transplantation.6,7
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The etiology of ALF varies with geographical location and age. Data from 215 consecutive ALF patients who presented to King’s College Hospital in London showed a bimodal distribution with the highest prevalence in neonates and infants followed by a smaller peak after 15 years of age.4,8 In the first month of life, it is prudent to consider metabolic disorders and herpes simplex type 1 and 2. Early intervention in these cases can prevent morbidity and mortality. In adolescents, drug toxicity predominates as the cause of ALF. In the Western world, the etiology of up ...