Nutritional considerations and management of the child with liver disease.

Nutritional management of the infant and child with liver disease is highly dependent upon the type of liver disease. Acute liver disease, such as that secondary to viral hepatitis, requires no specific nutritional therapy with the exception that branched-chain amino acid supplements may be indicated in the management of hepatic encephalopathy. Nutritional management of the child with chronic liver disease depends upon whether or not cholestasis is present, since in that condition, large amounts of fat-soluble vitamin supplements and medium-chain triglycerides are usually required for optimum growth. However, anicteric cirrhotic liver disease also presents nutritional challenges because of hypermetabolism, enteropathy, and increased protein oxidation. Certain inborn errors of metabolism that result in liver disease (including galactosemia, hepatorenal tyrosinemia, hereditary fructose intolerance, and Wilson's disease) have specific nutritional requirements. And, finally, the advent of pediatric liver transplantation has placed new emphasis on the importance of optimum nutritional management of the child with chronic liver disease, since improvement of nutritional status in the pretransplant period maximizes success of the transplant. This review will focus on the pathogenesis of malnutrition in childhood liver disease and will provide recommendations for nutritional assessment and monitoring as well as nutritional management of cholestatic liver disease, anicteric cirrhotic liver disease, and the inborn errors of metabolism enumerated above. Specific recommendations for nutritional management of the child awaiting liver transplantation will be provided.