Abstract

Protein energy malnutrition leading to growth failure is an inevitable consequence of chronic liver disease in childhood. Although the precise pathophysiology is not understood considerable progress has been made in understanding the mechanisms of fat malabsorption and protein turnover in liver disease. There are many difficulties with the correct assessment of nutritional parameters in children with liver disease related to their abnormal body composition and energy expenditure and care needs to be taken with the interpretation of results. The effects of malnutrition secondary to chronic liver disease are varied and include fat soluble vitamin deficiencies, generalised growth failure, impairment of gastrointestinal function, immunosuppression and hypotonia. It is now recognised that malnutrition is an important risk factor for liver transplantation and increases both mortality and morbidity. Strategies to prevent or reverse malnutrition are now established and include the use of specific infant formulas based on low salt protein and an increased concentration of medium train triglyceride (50-70%). Careful nutritional support in association with generous fat soluble vitamin supplementation may produce dramatic improvement in catch up weight gain but for those children in whom growth failure persists, the only management is liver transplantation.

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