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


METHODS
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.


CONCLUSIONS
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.
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.
Darlene L
DL
Machado
Elaine C
EC
Silva
Maria B D
MB
Resende
Celso R F
CR
Carvalho
Edmar
E
Zanoteli
Umbertina C
UC
Reed
D000293:Adolescent; D000704:Analysis of Variance; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D005541:Forced Expiratory Volume / Q000187:drug effects / Q000502:physiology; D006801:Humans; D008168:Lung / Q000187:drug effects* / Q000503:physiopathology; D008297:Male; D020388:Muscular Dystrophy, Duchenne / Q000188:drug therapy* / Q000503:physiopathology*; D011239:Prednisolone / Q000627:therapeutic use; D011282:Pregnenediones / Q000627:therapeutic use; D012129:Respiratory Function Tests; D016896:Treatment Outcome; D014797:Vital Capacity / Q000187:drug effects / Q000502:physiology; D055815:Young Adult
D000893:Anti-Inflammatory Agents; D011282:Pregnenediones; D011239:Prednisolone; C021988:deflazacort