title: 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).
  
  
  CONCLUSIONS
  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.affiliation: Department of Child Neurology, National Center Hospital of Neurology and Psychiatry, National Center of Neurology and Psychiatry, Japan.
authors.forename: Mami
authors.initials: M
authors.lastname: Shimizu-Fujiwara
authors.forename: Hirofumi
authors.initials: H
authors.lastname: Komaki
authors.forename: Eiji
authors.initials: E
authors.lastname: Nakagawa
authors.forename: Madoka
authors.initials: M
authors.lastname: Mori-Yoshimura
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Oya
authors.forename: Toshiyuki
authors.initials: T
authors.lastname: Fujisaki
authors.forename: Yasuko
authors.initials: Y
authors.lastname: Tokita
authors.forename: Norika
authors.initials: N
authors.lastname: Kubota
authors.forename: Rie
authors.initials: R
authors.lastname: Shimazaki
authors.forename: Kimiko
authors.initials: K
authors.lastname: Sato
authors.forename: Tomoko
authors.initials: T
authors.lastname: Ishikawa
authors.forename: Katsumasa
authors.initials: K
authors.lastname: Goto
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Mochizuki
authors.forename: Satoko
authors.initials: S
authors.lastname: Takanoha
authors.forename: Katsuhisa
authors.initials: K
authors.lastname: Ogata
authors.forename: Mitsuru
authors.initials: M
authors.lastname: Kawai
authors.forename: Masaaki
authors.initials: M
authors.lastname: Konagaya
authors.forename: Tatsushi
authors.initials: T
authors.lastname: Miyazaki
authors.forename: Katsunori
authors.initials: K
authors.lastname: Tatara
authors.forename: Kenji
authors.initials: K
authors.lastname: Sugai
authors.forename: Masayuki
authors.initials: M
authors.lastname: Sasaki
mesh_terms: D000293:Adolescent; D000328:Adult; D015992:Body Mass Index; D001835:Body Weight; D002153:Calorimetry, Indirect; D002648:Child; D002149:Energy Intake; D004734:Energy Metabolism / Q000502:physiology*; D006801:Humans; D008297:Male; D018482:Muscle, Skeletal / Q000378:metabolism; D020388:Muscular Dystrophy, Duchenne / Q000378:metabolism*; D012146:Rest; D055815:Young Adult