Abstract

Duchenne muscular dystrophy (DMD) patients are at risk of significant weight change relating to several factors. However, the relationship between body weight and calorie requirement during each stage of the disease is not known. The purpose of this study was to explore the appropriate nutritional management of DMD patients. Retrospective data from 28 DMD patients (mean age 20.6 ± 3.5 years) was assessed withrespect to body weight (BW), resting energy expenditure (REE), and assessment of respiratory and circulatory functions, which were conducted during annual examinations for at least 3 years. Based on the annual change in BW, 76 events were obtained from 28 patients and classified into 3 groups: (1) gain in BW of >10% (n = 5); (2) loss in BW of >10% (n = 10); and (3) no change (n = 61). REE was measured by indirect calorimetry, and the change in REE was evaluated for each group. Clinical features, including respiratory and circulatory functions, were also evaluated. Prior to this study, REE in groups 1 (BW gain), 2 (BW loss), and 3 (no change) were not significantly different (1 115,7, 1 077,5, and 1 144,2 kcal/d, respectively). Analyses of annual change in REE among each group also failed to show significant differences. The study revealed that calorie requirements did not change in group 2 (BW loss). This group also developed severe clinical complications, reduced dietary intake due to infection (4/10), cerebral infarction (2/10), and heart failure (2/10); however, no such complications developed in the other groups. The loss of BW led to a further BW decrease in the absence of appropriate nutritional intervention, due to higher calorie requirements than predicted from BW (3/10). To conclude, the daily calorie requirement did not change with a gain or loss of BW. It is possible that loss of BW lead to severe complication in DMD patients. It may be required for comprehensive care to apply nutritional support as nutritional supplements and respiratory support.

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