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Nutrient Requirements and Optimal Nutrition

Mild-to-Moderate Chronic Cholestatic Liver Disease Increases Leucine Oxidation in Children^1,2

Diana R. Mager^*,, Linda J. Wykes, Eve A. Roberts^,**,, Ronald O. Ball^*,, and Paul B. Pencharz^*,,,,3

^* Departments of Nutritional Sciences, Paediatrics, and ^** Pharmacology, University of Toronto, Toronto, Canada; The Research Institute, The Hospital for Sick Children, Toronto, ON, M5G 1X8, Canada; School of Dietetics and Human Nutrition, McGill University, Ste-Anne-de-Bellevue, QC, H9X 3V9, Canada; and Department of Agricultural, Food and Nutritional Sciences, University of Alberta, Edmonton, AB, T6G 2P5, Canada

³ To whom correspondence should be addressed. Email: paul.pencharz{at}sickkids.ca.

Malnutrition is prevalent in children with chronic cholestatic liver disease. Using the noninvasive indicator amino acid oxidation (IAAO) technique, we recently determined that mild-to-moderate chronic cholestatic (MCC) liver disease increases the need for branched-chain amino acids (BCAA) in children. To examine the underlying mechanisms responsible for this increased need for BCAA in liver disease, we measured L-[1-¹³C]-leucine oxidation in the postabsorptive and fed states in 10 children with MCC liver disease (8.8 ± 3.5 y) and in 11 healthy children (9.4 ± 2.2 y). The oxidation of L-[1-¹³C]-leucine to ¹³CO₂ [F¹³CO₂ in µmol/(kg·h)] was determined after a primed, continuous oral administration of the tracer. Total BCAA in diet was provided at 300 mg/(kg·d) to ensure that leucine oxidation was measured when leucine intake was in excess of requirements. In the postabsorptive state, the rate of release of ¹³CO₂ from ¹³C-leucine oxidation (F¹³CO₂) and whole-body leucine oxidation were significantly higher in children with MCC liver disease (P < 0.05). However, F¹³CO₂ and whole-body leucine oxidation did not differ in the fed state. We conclude that the increased need for dietary BCAA in MCC liver disease is mediated in part by increased leucine oxidation in the postabsorptive state.

KEY WORDS: • leucine metabolism • children • liver disease