HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Full Text Full Text (PDF) Data Supplement All Versions of this Article: 01.WNL.0000287070.00149.a9v1 70/2/137    most recent Correspondence: Submit a response Alert me when this article is cited Alert me when Correspondence are posted Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Skura, C. L. Articles by Spencer, M. J. Search for Related Content PubMed PubMed Citation Articles by Skura, C. L. Articles by Spencer, M. J. Related Collections All Neuromuscular Disease Muscle disease All Pediatric

Albuterol increases lean body mass in ambulatory boys with Duchenne or Becker muscular dystrophy

From the UCLA Department of Orthopaedic Surgery, Tarjan Center for Disabilities at UCLA (E.G.F.), UCLA Department of Pediatrics (G.T.W., M.J.S.), and UCLA Department of Neurology (M.G., M.J.S.), University of California (C.L.S.), Los Angeles.

Address correspondence and reprint requests to Dr. Melissa J. Spencer, Department of Neurology, David Geffen School of Medicine at UCLA, 635 Charles Young Drive South, NRB1 Room 401, Los Angeles, CA 90095-7334 mspencer{at}mednet.ucla.edu

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.

Abbreviations: β2A = 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.

Supplemental data at www.neurology.org

e-Pub ahead of print on October 17, 2007, at www.neurology.org.

Disclosure: Funding for this trial was provided by the Muscular Dystrophy Association of America and the Parent Project for Muscular Dystrophy. These organizations did not have a role in the design or conduct of the study; collection, management, analysis, or interpretation of the data; or preparation, review, or approval of the manuscript. No other conflicts of interest are reported.

Received February 11, 2007. Accepted in final form June 26, 2007.