Decreased resting energy expenditure in patients with Duchenne muscular dystrophy.

Abstract

BACKGROUND:

Skeletal muscle metabolism is a major determinant of resting energy expenditure (REE). Although the severe muscle loss that characterizes Duchenne muscular dystrophy (DMD) may alter REE, this has not been extensively investigated.

METHODS:

We studied REE in 77 patients with DMD ranging in age from 10 to 37 years using a portable indirect calorimeter, together with several clinical parameters (age, height, body weight (BW), body mass index (BMI), vital capacity (VC), creatine kinase, creatinine, albumin, cholinesterase, prealbumin), and assessed their influence on REE. In addition, in 12 patients maintaining a stable body weight, the ratio of energy intake to REE was calculated and defined as an alternative index for the physical activity level (aPAL).

RESULTS:

REE (kcal/day, mean±SD) in DMD patients was 1123 (10-11 years), 1186±188 (12-14 years), 1146±214 (15-17 years), 1006±136 (18-29 years) and 1023±97 (≥30 years), each of these values being significantly lower than the corresponding control (p<0.0001). VC (p<0.001) was the parameter most strongly associated with REE, followed by BMI (p<0.01) and BW (p<0.05). The calculated aPAL values were 1.61 (10-11 years), 1.19 (12-14 years), 1.16 (15-17 years), and 1.57 (18-29 years).

CONCLUSION:

The REE in DMD patients was significantly lower than the normal value in every age group, and strongly associated with VC. Both the low REE and PAL values during the early teens, resulting in a low energy requirement, might be related to the obesity that frequently occurs in this age group. In contrast, the high PAL value in the late stage of the disease, possibly due to the presence of respiratory failure, may lead to a high energy requirement, and thus become one of the risk factors for development of malnutrition.

• Authors: Mami Shimizu-Fujiwara, Hirofumi Komaki, Eiji Nakagawa, Madoka Mori-Yoshimura, Yasushi Oya, Toshiyuki Fujisaki, Yasuko Tokita, Norika Kubota, Rie Shimazaki, Kimiko Sato, Tomoko Ishikawa, Katsumasa Goto, Hitoshi Mochizuki, Satoko Takanoha, Katsuhisa Ogata, Mitsuru Kawai, Masaaki Konagaya, Tatsushi Miyazaki, Katsunori Tatara, Kenji Sugai, Masayuki Sasaki
• Journal: Brain & development (Brain Dev) Vol. 34 Issue 3 Pg. 206-12 (Mar 2012) ISSN: 1872-7131 [Electronic] Netherlands
• PMID: 21632191 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: Copyright © 2011 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

Topics

• Adolescent
• Adult
• Body Mass Index
• Body Weight
• Calorimetry, Indirect
• Child
• Energy Intake
• Energy Metabolism (physiology)
• Humans
• Male
• Muscle, Skeletal (metabolism)
• Muscular Dystrophy, Duchenne (metabolism)
• Rest
• Young Adult