Nutrition for children with cholestatic liver disease.

Cholestatic liver disease (CLD) in children negatively affects nutritional status, growth and development, which all lead to an increased risk of morbidity and mortality. This is illustrated by the fact that the clinical outcome of children with CLD awaiting a liver transplantation is in part predicted by their nutritional status, which is integrated in the pediatric end-stage liver disease model. Preservation of the nutritional status becomes more relevant as the number of patients waiting for liver transplantation increases and the waiting time for a donor organ becomes prolonged. Nutritional strategies are available to optimize feeding of children with CLD. Patients with CLD, however, form a heterogeneous group and the clinical manifestations of their disease vary. This makes a tailor-made approach for these children crucial. Not all aspects of nutrient metabolism and absorption in children with CLD are well understood and studied. Experiments with stable isotope-labeled triglycerides and fatty acids have provided essential information about fat absorption under physiological and cholestatic conditions in animal models and humans. We expect that in the future, tests using other isotope-labeled macronutrients, i.e. carbohydrates and proteins, can be used to further assess nutritional status of children with CLD, thereby creating tailor-made nutritional therapies.