ORIGINAL COMMUNICATION Body composition and energy expenditure in Duchenne muscular dystrophy MC Zanardi 1 , A Tagliabue 1 *, S Orcesi 2 , A Berardinelli 2 , C Uggetti 3 and A Pichiecchio 3 1 Applied Health Sciences Department, University of Pavia, Pavia, Italy; 2 Child Neurology Department, Regional Referring Centre for Neuromuscular Disorders in Childhood and Adolescence, Neurological Foundation C. Mondino, Pavia, Italy; and 3 Neuroradiology Department, Neurological Foundation C. Mondino, Pavia, Italy Objective: To investigate the relationship between resting energy expenditure (REE) and body composition in Duchenne Muscular Dystrophy (DMD). Design: An observational study. Setting: University Research Centre. Subjects: Nine Duchenne children (age range 6 – 12 y), mean relative weight 128%, agreed to undergo the investigation and all of them completed the study; Interventions: Assessment of body composition (total body fat and skeletal muscle mass) by magnetic resonance imaging and resting energy expenditure by indirect calorimetry. Main outcome measures: Fat mass (FM; kg and percentage weight), fat-free mass (FFM; kg and percentage weight), muscle mass (kg and percentage weight), resting energy expenditure (kJ=kg body weight and kJ=kg fat-free mass). Results: In Duchenne children fat mass averages 32% and total skeletal muscle mass 20% of body weight. Resting energy expenditure per kg of body weight falls within the normal range for children of the same age range, while when expressed per kg of FFM is significantly higher than reference values. No relationship was found between REE and total skeletal muscle mass. Conclusions: Our results do not demonstrate a low REE in DMD boys; on the contrary REE per kg of FFM is higher than normal, probably due to the altered FFM composition. We suggest that the development of obesity in DMD children is not primarily due to a low REE but to other causes such as a reduction in physical activity and or overfeeding. Sponsorship: Italian Telethon Foundation (grant no. 1137C, 1999). European Journal of Clinical Nutrition (2003) 57, 273 – 278. doi:10.1038=sj.ejcn.1601524 Keywords: muscular dystrophy; muscle mass; energy expenditure; magnetic resonance imaging Introduction Nutritional disorders often worsen the physical disability of patients affected by muscular dystrophy: 44 – 54% of dys- trophic children are obese by the age of 13 and around the same percentage are underfed by the age of 18 (Willig et al, 1993, McDonald et al, 1995). Obesity increases the handicap of muscle wasting, accentuates skeletal deformity and endangers the results of orthopaedic surgery (Willig et al, 1993); moreover, it makes patients’ respiratory function worse in the terminal phase of the disease. When patients lose their independence, the fact that they are overweight can become an additional problem for carers, particularly in maintaining a daily hygiene programme. Little is known about the genesis of obesity in Duchenne dystrophy. Prob- ably, in this as in other conditions associated with obesity, gain in body weight over a number of years could be due to an unbalance between energy intake and expenditure (Blun- dell & Cooling, 2000; Kopelman, 2000). Energy expenditure is determined by the amount and composition of the metabolically active fat-free mass (FFM) and also by physical activity. In Duchenne muscular dystro- *Correspondence: A Tagliabue, Applied Health Sciences Dpt, Human Nutrition Research Center, University of Pavia, Via Bassi 21, 27100 Pavia, Italy. E-mail: anna.tagliabue@unipv.it Guarantor: A Tagliabue. Contributors: MCZ and AT were responsible for the overall research project, data collection, statistical analysis and report writing. SO and AB were responsible for patient recruitment, data collection and interpretation of findings. CU and AP were responsible for magnetic resonance imaging measurements and interpretations of findings. Received 12 September 2001; revised 29 April 2002; accepted 7 May 2002 European Journal of Clinical Nutrition (2003) 57, 273–278 ß 2003 Nature Publishing Group All rights reserved 0954–3007/03 $25.00 www.nature.com/ejcn European Journal of Clinical Nutrition (2003) 57, 273–278 ß 2003 Nature Publishing Group All rights reserved 0954–3007/03 $25.00 www.nature.com/ejcn