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Resting energy expenditure in Duchenne patients using home mechanical ventilation

¹ Service de pneumologie et de réanimation, GH Pitié-Salpêtrière, Assistance Publique - Hôpitaux de Paris, ² UPRES EA2397, Université Paris VI Pierre et Marie Curie, Paris, and ³ Services de Réanimation Médicale, Physiologie - Explorations Fonctionnelles, Maladies Infectieuses et Tropicales-Nutrition Clinique, et Centre d'Innovation Technologique Hôpital Raymond Poincaré, AP-HP, Garches, France

CORRESPONDENCE: J. Gonzalez-Bermejo, Service de Pneumologie et de Réanimation, Groupe Hospitalier Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, 47-83, Bd de l'Hôpital, 75651 Paris Cedex 13, France. Fax: 33 142176708. E-mail: jesus.gonzalez@psl.ap-hop-paris.fr

Keywords: Chronic respiratory insufficiency, Duchenne muscular dystrophy, home mechanical ventilation, nutritional status, resting energy expenditure

Received: March 13, 2004
Accepted December 6, 2004

Nutritional status is both important and difficult to assess in patients with Duchenne muscular dystrophy (DMD), particularly in those requiring mechanical ventilation (MV). The current authors evaluated body composition (bio-impedancemetry), resting energy expenditure (REE; indirect calorimetry) and energy intake in 20 adult patients with DMD using home MV (nocturnal: n = 13; continuous: n = 7) and 12 age-matched healthy controls.

The patients were smaller in height than the controls and had a lower body weight. Most of the reduction in body mass index was accounted for by a reduction in fat free mass (FFM). REE (kJ) was significantly reduced in the patients (4559±853 kJ·24 h^–1 versus 7407±1312 kJ·24 h^–1), but the difference disappeared after correction for FFM.

REE and FFM were correlated in both the controls and patients, but less strongly in the latter, the lower strength of the association being due to the patients using continuous MV (REE and FFM uncorrelated). The food intake of the patients was 1.2±0.4 greater than their REE.

This study shows that patients with advanced forms of Duchenne muscular dystrophy have balanced energy intakes and resting energy expenditure.