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Oral glutamine and amino acid supplementation inhibit whole-body protein degradation in children with Duchenne muscular dystrophy^1,2,3

¹ From the Centre d'Investigation Clinique 9202 INSERM, Assistance Publique-Hôpitaux de Paris, Hôpital Robert Debré, Paris, France (EM, CE-DV, CD, and RH); the LAPHAP EA3813, Université de Poitiers, Poitiers, France (EM, RH); the Clinique de Pédiatrie, CHR&U de Lille, Hôpital Jeanne de Flandre, Lille, France (FG); the Biochimie, Assistance Publique-Hôpitaux de Paris, Hôpital Robert Debré, Paris, France (OR); the Pharmacie, Assistance Publique-Hôpitaux de Paris, Hôpital Jean-Verdier, Paris, France(J-EF); the Service de Neuropédiatrie, CHR&U de Lille, Hôpital Roger-Salengro, Lille, France (J-MC); and the Clinical Research Center, CHU de Poitiers, Poitiers, France (JG)

Background: Glutamine has been shown to acutely decrease whole-body protein degradation in Duchenne muscular dystrophy (DMD).

Objective: To improve nutritional support in DMD, we tested whether oral supplementation with glutamine for 10 d decreased whole-body protein degradation significantly more than did an isonitrogenous amino acid control mixture.

Design: Twenty-six boys with DMD were included in this randomized, double-blind parallel study; they received an oral supplement of either glutamine (0.5 g · kg^–1 · d^–1) or an isonitrogenous, nonspecific amino acid mixture (0.8 g · kg^–1 · d^–1) for 10 d. The subjects in each group were not clinically different at entry. Leucine and glutamine metabolisms were estimated in the postabsorptive state by using a primed continuous intravenous infusion of [1-¹³C]leucine and [2-¹⁵N]glutamine before and 10 d after supplementation.

Results: A significant effect of time was observed on estimates of whole-body protein degradation. A significant (P < 0.05) decrease in the rate of leucine appearance (an index of whole-body protein degradation) was observed after both glutamine and isonitrogenous amino acid supplementation [ ±SEM: 136 ± 9 to 124 ± 6 µmol · kg fat-free mass (FFM)^–1 · h^–1 for glutamine and 136 ± 6 to 131 ± 8 µmol · kg FFM^–1 · h^–1 for amino acids]. A significant (P < 0.05) decrease in endogenous glutamine due to protein breakdown was also observed (91 ± 6 to 83 ± 4 µmol · kg FFM^–1 · h^–1 for glutamine and 91 ± 4 to 88 ± 5 µmol · kg FFM^–1 · h^–1 for amino acids). The decrease in the estimates of whole-body protein degradation did not differ significantly between the 2 supplemental groups.

Conclusion: Oral glutamine or amino acid supplementation over 10 d equally inhibits whole-body protein degradation in DMD.

Key Words: Duchenne muscular dystrophy • children • randomized controlled clinical trial • supplement • oral administration • stable isotopes • protein metabolism • glutamine • leucine • amino acids

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