Lung function monitoring in patients with duchenne muscular dystrophy on steroid therapy

BMC Research Notes

Umbertina C Reed²  Edmar Zanoteli¹  Celso R F Carvalho³  Maria B D Resende²  Elaine C Silva²  Darlene L Machado² 

[1]Neuromuscular Division, Associação de Assistência à Criança Deficiente (AACD), São Paulo, Brazil

[2]Department of Neurology, Medical School of the University of São Paulo, Av. Dr. Enéas de Carvalho Aguiar 255, room 5131, Cerqueira Cesar, São Paulo, 05403900, Brazil

[3]Department of Physical Therapy, Speech Therapy and Occupational Therapy, Medical School of the University of São Paulo, São Paulo, Brazil

关键词: Myopathies;    Respiratory function tests;    Vital forced capacity;    Steroids;    Duchenne muscular dystrophy;   

Others  :  1165944
DOI  :  10.1186/1756-0500-5-435

 received in 2012-07-19, accepted in 2012-07-31,  发布年份 2012

【 摘 要 】

Background

Duchenne muscular dystrophy (DMD) is a sex-linked inherited muscle disease characterized by a progressive loss in muscle strength and respiratory muscle involvement. After 12 years of age, lung function declines at a rate of 6 % to 10.7 % per year in patients with DMD. Steroid therapy has been proposed to delay the loss of motor function and also the respiratory involvement.

Method

In 21 patients with DMD aged between seven and 16 years, the forced vital capacity (FVC) and the forced expiratory volume in one second (FEV₁) were evaluated at three different times during a period of two years.

Results

We observed in this period of evaluation the maintenance of the FVC and the FEV₁ in this group of patients independently of chronological age, age at onset of steroid therapy, and walking capacity.

Conclusion

The steroid therapy has the potential to stabilize or delay the loss of lung function in DMD patients even if they are non-ambulant or older than 10 years, and in those in whom the medication was started after 7 years of age.

【 授权许可】

   
2012 Machado et al.; licensee BioMed Central Ltd.

【 图 表 】

Figure 1.

Figure 2.

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