Document Detail


Change in natural history of Duchenne muscular dystrophy with long-term corticosteroid treatment: implications for management.
MedLine Citation:
PMID:  20581335     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
In 2005, the American Academy of Neurology and the Child Neurology Society published a practice parameter, based primarily on studies that involved 6 to 18 months of treatment, indicating that prednisone has a beneficial effect on muscle strength and function in patients with Duchenne muscular dystrophy and recommended that corticosteroids be offered (prednisone 0.75 mg/kg/d and deflazacort 0.9 mg/kg/d) as treatment. Recent reports emphasize that longer term treatment with corticosteroids (greater than 3 years) produces important sustained benefits in neuromuscular function without causing major side effects. This review highlights these reports and indicates that long-term corticosteroid therapy (1) prolongs ambulation by 2 to 5 years, (2) reduces the need for spinal stabilization surgery, (3) improves cardiopulmonary function, (4) delays the need for noninvasive nasal ventilation, and (5) increases survival and the quality of life of patients with Duchenne muscular dystrophy. Educational, vocational, and other social counseling is now a vital part of management for Duchenne muscular dystrophy.
Authors:
Richard T Moxley; Shree Pandya; Emma Ciafaloni; Deborah J Fox; Kim Campbell
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.; Review     Date:  2010-06-25
Journal Detail:
Title:  Journal of child neurology     Volume:  25     ISSN:  1708-8283     ISO Abbreviation:  J. Child Neurol.     Publication Date:  2010 Sep 
Date Detail:
Created Date:  2010-09-06     Completed Date:  2011-06-01     Revised Date:  2013-04-26    
Medline Journal Info:
Nlm Unique ID:  8606714     Medline TA:  J Child Neurol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1116-29     Citation Subset:  IM    
Affiliation:
Department of Neurology, University of Rochester, Rochester, NY 14642, USA. tracy_forrester@urmc.rochester.edu
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MeSH Terms
Descriptor/Qualifier:
Child
Disease Progression
Drug Administration Schedule
Humans
Male
Muscular Dystrophy, Duchenne / diagnosis*,  drug therapy*,  physiopathology
Prednisone / administration & dosage*,  therapeutic use
Pregnenediones / administration & dosage*,  therapeutic use
Time Factors
Treatment Outcome
Grant Support
ID/Acronym/Agency:
AR52274/AR/NIAMS NIH HHS; M01 RR00044/RR/NCRR NIH HHS; NS048843/NS/NINDS NIH HHS; RR024160/RR/NCRR NIH HHS; U58/DD0000190/DD/NCBDD CDC HHS; UL1 RR024160/RR/NCRR NIH HHS
Chemical
Reg. No./Substance:
0/Pregnenediones; 53-03-2/Prednisone; KR5YZ6AE4B/deflazacort

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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