期刊
JOURNAL OF CHILD NEUROLOGY
卷 25, 期 9, 页码 1116-1129出版社
SAGE PUBLICATIONS INC
DOI: 10.1177/0883073810371004
关键词
Duchenne muscular dystrophy; corticosteroid therapy; prednisone; deflazacort
资金
- NCRR NIH HHS [UL1 RR024160, RR024160, M01 RR00044] Funding Source: Medline
- NIAMS NIH HHS [AR52274] Funding Source: Medline
- NINDS NIH HHS [NS048843] Funding Source: Medline
- NCBDD CDC HHS [U58/DD0000190] Funding Source: Medline
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.
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