A standardized method for the evaluation of respiratory muscle endurance in patients with Duchenne muscular dystrophy.
The aim of the study was to develop a standardized method using controlled breathing to quantify respiratory muscle endurance in children with Duchenne muscular dystrophy (DMD) and to test its reproducibility. In 10 DMD patients, all between 10 and 14 years (mean age, 11.5 +/- 1.5 years), except for two patients of 20 and 22 years, and 10 healthy children (mean age, 12 +/- 1 years), we measured the maximal time (Tlim) that a threshold load fixed at 35% of the individual maximal inspiratory pressure (Pimax) could be tolerated. We asked the children to maintain their rest breathing pattern until exhaustion using visual feedback and an auditory signal. The mean Tlim in the DMD children was 4.45 +/- 1.45 min and values were reproducible. All healthy children were able to obtain Tlim values greater than 30 min. The respiratory muscles of DMD children are more susceptible to fatigue than those of healthy subjects. This method should be satisfactory for estimating the effect of treatment and for the specific training of respiratory muscles in DMD patients without significant learning disability.
Laboratoire de Physiologie des Interactions, Service Central de Physiologie Clinique, Hôpital Arnaud de Villeneuve, 34295 Cedex 5, Montpellier, France. smatecki@aol.com
S
S
Matecki
N
N
Topin
M
M
Hayot
F
F
Rivier
B
B
Echenne
C
C
Prefaut
M
M
Ramonatxo
D000293:Adolescent; D002648:Child; D004185:Disability Evaluation; D017079:Exercise Tolerance / Q000502:physiology*; D006801:Humans; D008297:Male; D008451:Maximal Voluntary Ventilation / Q000502:physiology; D018908:Muscle Weakness / Q000209:etiology / Q000503:physiopathology*; D020388:Muscular Dystrophy, Duchenne / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology*; D012131:Respiratory Insufficiency / Q000209:etiology / Q000503:physiopathology*; D012132:Respiratory Muscles / Q000473:pathology / Q000503:physiopathology*; D012143:Respiratory Physiological Phenomena