Research Article| Volume 12, ISSUE 6, P576-583, August 2002

Dose-dependent effect of individualized respiratory muscle training in children with Duchenne muscular dystrophy


      The aim of this study was to evaluate the effects of low intensity, home inspiratory muscle training on respiratory muscle endurance in children with Duchenne muscular dystrophy, using a double-blind protocol. The originality aspect of this study is the use of a reproducible method of endurance and of the same method for evaluation and training. We studied eight trained children (mean age 14.7±4.5 years) and eight control children (mean age, 12.6±1.8 years). For 6 weeks, children breathed twice a day for 10 min through a valve with either 30% (training group) or less than 5% (control group) of their maximum inspiratory pressure (Pimax). The results showed (1) a 46% improvement in the time limit after training in the training group and no change in the control group and (2) a significant correlation between the total time of respiratory muscle training and the percentage of endurance improvement in the training group. We conclude that specific training improves respiratory muscle endurance in Duchenne muscular dystrophy and the effectiveness of training appears to be dependent on the quantity of training.


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        • Joanne L.D
        • Woda R.P
        • Mendell J.R
        Respiratory dysfunction in muscular dystrophy and other myopathies.
        Clin Chest Med. 1994; 15: 661-674
        • Rideau Y
        • Jankowski L.W
        • Grillet J
        Respiratory function in the muscular dystrophies.
        Muscle Nerve. 1981; 4: 155-164
        • Wanke T
        • Toifl K
        • Merkle M
        • Formanek D
        • Lahrmann H
        • Zwick H
        Inspiratory muscle training in patients with Duchenne muscular dystrophy.
        Chest. 1994; 105: 475-482
        • Martin A.J
        • Stern L
        • Yeates J
        • Lepp D
        • Little J
        Respiratory muscle training in Duchenne muscular dystrophy.
        Dev Med Child Neurol. 1986; 28: 314-318
        • Di Marco A.F
        • Kelling J.S
        • Di Marco M
        • Jacobs I
        • Shields R
        • Altose M.D
        The effects of inspiratory resistive training on respiratory muscle function in patients with muscular dystrophy.
        Muscle Nerve. 1985; 8: 284-290
        • Houser C.R
        • Johnson D.M
        Breathing exercises for children with pseudohypertrophic muscular dystrophy.
        Phys Ther. 1971; 51: 751-759
        • Smith P.E.M
        • Coakley J.H
        • Edwards R.H.T
        Respiratory muscle training in Duchenne muscular dystrophy.
        Muscle Nerve. 1988; 7: 784-785
        • Vilozni D
        • Bar-Yishay E
        • Gur I
        • Shapira Y
        • Meyer S
        • Godfrey S
        Computerized respiratory muscle training in children with Duchenne muscular dystrophy.
        Neuromuscul Disord. 1994; 4: 249-255
        • Stern L.M
        • Martin A.J
        • Jones N
        • Garrett R
        • Yeates J
        Training inspiratory resistance in Duchenne dystrophy using adapted computer games.
        Dev Med Child Neurol. 1989; 31: 494-500
        • Matecki S
        • Topin N
        • Hayot M
        • et al.
        A standardized method for the evaluation of the respiratory muscle endurance in patients with Duchenne muscular dystrophy.
        Neuromuscul Disord. 2001; 11: 171-177
        • Smith K
        • Cook D
        • Guyatt G.H
        • Madhavan J
        • Oxman D
        Respiratory muscle training in chronic airflow limitation: a meta-analysis.
        Am Rev Respir Dis. 1992; 145: 533-539
        • American Thoracic Society/European Respiratory Society
        Respiratory mechanics in infants: physiologic evaluation in health and disease.
        Am Rev Respir Dis. 1993; 147: 474-496
        • Zapletal A
        • Samanek M
        • Paul T
        Lung function in children and adolescents.
        in: Herzog H Progress in Respiration Research. Karger, Basel1987
        • Black L.F
        • Hyatt R.E
        Maximal respiratory pressures: normals values and relationship to age and sex.
        Am Rev Respir Dis. 1969; 99: 696-702
        • Roussos C
        • Fixley M
        • Gross D
        • Macklem P.T
        Fatigue of inspiratory muscles and their synergic behavior.
        J Appl Physiol. 1979; 46: 897-904
        • Nickerson B.G
        • Keens T.G
        Measuring ventilatory muscle endurance in humans as sustainable inspiratory pressure.
        J Appl Physiol. 1982; 52: 768-772
        • Winkler G
        • Zifko U
        • Nader A
        • et al.
        Dose-dependent effects of inspiratory muscle training in neuromuscular disorders.
        Muscle Nerve. 2000; 23: 1257-1260
        • Estrup C
        • Lyager S
        • Noeraa N
        • Olsen C
        Effect of respiratory muscle training in patients with neuromuscular diseases and in normals.
        Respiration. 1986; 50: 36-43
        • Clanton T.L
        • Dixon G.F
        • Drake J
        • Gadek J.E
        Effects of breathing pattern on inspiratory muscle endurance in humans.
        J Appl Physiol. 1985; 9: 1834-1841
        • Martyn J.B
        • Moreno R.H
        • Pare P.D
        • Pardy R.L
        Measurement of inspiratory muscle performance with incremental threshold loading.
        Am Rev Respir. 1987; 135: 919-923
        • Belman M.J
        • Botnick W.C
        • Nathan S.D
        • Chon K.H
        Ventilatory load characteristics during ventilatory muscle training.
        Am J Respir Crit Care Med. 1994; 149: 925-929
        • Petrof B.J
        The molecular basis of activity-induced muscle injury in Duchenne muscular dystrophy.
        Mol Cell Biochem. 1998; 179: 111-123
        • Larson J.L
        • Kim M.J
        • Sharp J.T
        • Larson A.D
        Inspiratory muscle training with a pressure threshold breathing device in patients with chronic obstructive pulmonary disease.
        Am Rev Respir Dis. 1988; 138: 689-696
        • Preusser B.A
        • Winningham M.L
        • Clanton T.L
        High- vs low-intensity inspiratory muscle interval training in patients with COPD.
        Chest. 1994; 106: 110-117
        • Leith D.E
        • Bradley M
        Ventilatory muscle strength and endurance training.
        J Appl Physiol. 1976; 41: 508-516