NUTRITIONAL CONSIDERATIONS AND MANAGEMENT OF THE CHILD WITH LIVER-DISEASE

Nutritional management of the infant and child with liver disease is h ighly dependent upon the type of liver disease. Acute liver disease, s uch as that secondary to viral hepatitis, requires no specific nutriti onal therapy with the exception that branched-chain amino acid supplem ents may be indicated in the management of hepatic encephalopathy. Nut ritional management of the child with chronic liver disease depends up on whether or not cholestasis is present, since in that condition, lar ge amounts of fat-soluble Vitamin supplements and medium-chain triglyc erides are usually required for optimum growth. However, anicteric cir rhotic liver disease also presents nutritional challenges because of h ypermetabolism, enteropathy, and increased protein oxidation. Certain inborn errors of metabolism that result in liver disease (including ga lactosemia, hepatorenal tyrosinemia, hereditary fructose intolerance, and Wilson's disease) have specific nutritional requirements. And, fin ally, the advent of pediatric liver transplantation has placed new emp hasis on the importance of optimum nutritional management of the child with chronic liver disease, since improvement of nutritional status i n the pretransplant period maximizes success of the transplant. This r eview will focus on the pathogenesis of malnutrition in childhood live r 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 m etabolism enumerated above. Specific recommendations for nutritional m anagement of the child awaiting liver transplantation will be provided . (C) Elsevier Science Inc. 1997.