RT Journal Article
SR Electronic
T1 Albuterol increases lean body mass in ambulatory boys with Duchenne or Becker muscular dystrophy
JF Neurology
JO Neurology
FD Lippincott Williams & Wilkins
SP 137
OP 143
DO 10.1212/01.WNL.0000287070.00149.a9
VO 70
IS 2
A1 C. L. Skura
A1 E. G. Fowler
A1 G. T. Wetzel
A1 M. Graves
A1 M. J. Spencer
YR 2008
UL http://n.neurology.org/content/70/2/137.abstract
AB Background: Albuterol is a beta-2 agonist that has been demonstrated to increase muscle strength in studies in animals and humans. Based on a pilot study of extended-release albuterol Repetabs in children with dystrophinopathies, the authors conducted a randomized, double-blind, placebo-controlled study with a crossover design. Methods: Fourteen boys with Duchenne or Becker muscular dystrophy, 6 to 11 years old, completed two treatment periods (albuterol and placebo), 12 weeks each, separated by a 12-week washout period. As the albuterol Repetab formulation was no longer available, an alternate extended release albuterol was used (Volmax, 12 mg per day). Outcome measurements included 1) lean body mass, 2) fat mass, 3) isometric knee extensor and flexor moments, 4) manual muscle testing, and 5) timed functional tests. Results: Lean body mass was significantly higher for subjects following albuterol treatment compared to placebo treatment, while fat mass was significantly lower. No differences were found in isometric knee moments or manual muscle tests. Time to run/walk 30 feet was improved following albuterol. Conclusions: Short-term treatment with extended release albuterol may increase lean body mass, decrease fat mass, and improve functional measures in patients with dystrophinopathies. However, the significant change in strength of specific muscle groups found in the pilot study was not observed in the present study. These findings may be attributed to differences in the drug release and kinetics between Repetab and Volmax formulations as they affect the concentration of available beta-2 receptors on the muscle cell surface differently. 2A=beta-2 agonists; BMD=Becker muscular dystrophy; DMD=Duchenne muscular dystrophy; LBM=lean body mass; MMT=manual muscle test; PODCI=Pediatric Outcomes Data Collection Instrument.