Cholestatic liver disease causes severe risk of malnutrition which includes protein-energy malnutrition and specific nutritional deficiencies. The nutritional status can be assessed based on anthropometric measurements, which can be misleading because of ascitis and peripheral edema. Biochemical determinations of lipid-soluble vitamin status are important to evaluate requirements. Based on nutritional status assessment, nutritional therapy should be planned according to a well-defined schedule. The basic principle of nutritional management is to correct the nutritional status as well as to reduce the risk of nutritional deficiencies. Children with cholestasis usually need extra energy supply that can be obtained by increasing energy density of feeds or addition of glucose polymers and lipids. For catch-up growth, usually, protein intake should be increased. Lipid-soluble vitamin supplementation deserves special attention and it is not easy to correct poor vitamin E status. For some children, parenteral administration of vitamin K is needed. Since recently, a water-soluble vitamin E (d-α-tocopheryl polyethylene glycol 1000 succinate) given by oral route is used with a good therapeutic effect. As the liver disease progresses to liver failure in many chronic cholestatic diseases, nutritional therapy can often be regarded as ‘bridging’ for liver transplantation to improve prognosis. Thus, invasive nutritional support is justified in severe liver disease which usually includes nocturnal nasogastric tube feeding, or even parenteral nutrition.

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