Regular Article| Volume 95, ISSUE 11, P898-903, November 2001

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Lung function in children with Duchenne's muscular dystrophy

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      Duchenne's muscular dystrophy (DMD), characterized by gradually developing muscular weakness, leads to respiratory symptoms and reduced lung function. We aimed to assess lung function in 25 patients with DMD in relationship to age and muscular function. The 25 boys, mean age 13 years, comprized patients in southern Norway with DMD, taking part in an epidemiological follow-up study. None had chronic respiratory disease. Lung function was measured by maximum expiratory flow–volume loops and whole body plethysmography, and repeated after 1 year (n=14). Lung function was reduced compared to predicted values for healthy children. Forced expiratory volume in 1 sec (FEV1)% predicted and forced vital capacity (FVC)% predicted correlated (significantly) inversely to age. FEV1and FVC decreased annually 5·61 and 4·2% of predicted, respectively. Absolute values of FVC (litres) and FEV1(l sec−1) increased until mean age 14 years, decreasing thereafter. Values in % predicted decreased steadily throughout the age range (6–19 years). Lung function correlated closely to upper limb muscle function.




        • McDonald CM
        • Abresch RT
        • Carter GT
        Profiles of neuromuscular diseases. Duchenne muscular dystrophy.
        Am J Phys Med Rehabil. 1995; 74: S70-S92
        • Emery A EH
        Diagnostic Criteria for Neuromuscular Disorders. European Neuromuscular Centre, Baarn1994
        • Griggs RC
        • Donohoe KM
        • Utell MJ
        Evaluation of pulmonary function in neuromuscular disease.
        Arch Neurol. 1981; 38: 9-12
        • Smith AD
        • Koreska J
        • Moseley CF
        Progression of scoliosis in Duchenne muscular dystrophy.
        J Bone Joint Surg. 1989; 71: 1066-1074
        • Hahn A
        • Bach JR
        • Delaubier A
        • Renardel-Irani A
        • Guillou C
        • Rideau Y
        Clinical implications of maximal respiratory pressure determinations for individuals with Duchenne muscular dystrophy.
        Arch Phys Med Rehabil. 1997; 78: 1-6
        • Inkley SR
        • Oldenburg FC
        • Vignos Jr, PJ
        Pulmonary function in Duchenne muscular dystrophy related to stage of disease.
        Am J Med. 1974; 56: 297-306
        • Melacini P
        • Vianello A
        • Villanova C
        • Fanin M
        • Miorin M
        • Angelini C
        Cardiac and respiratory involvement in advanced stage Duchenne muscular dystrophy.
        Neuromuscul Disord. 1996; 6: 367-376
        • Rideau Y
        • Delaubier A
        • Guillou C
        • Renardel-Irani A
        Treatment of respiratory insufficiency in Duchenne's muscular dystrophy: nasal ventilation in the initial stages.
        Monaldi Arch Chest Dis. 1995; 50: 235-238
        • Lyager S
        • Steffensen B
        • Juhl B
        Indicators for an impending need of artificial respiration in patients with muscular dystrophy.
        Ugeskr Laeger. 1993; 155: 1963-1967
        • Lynn DJ
        • Woda RP
        • Mendell JR
        Respiratory dysfunction in muscular dystrophy and other myopathies.
        Clin Chest Med. 1994; 15: 661-674
        • Tangsrud SE
        • Halvorsen S
        Child neuromuscular disease in southern Norway. Prevalence, age and distribution of diagnosis with special reference to “non-Duchenne muscular dystrophy”.
        Clin Genet. 1988; 34: 145-152
        • Tangsrud SE
        • Halvorsen S
        Child neuromuscular disease in southern Norway. The prevalence and incidence of Duchenne muscular dystrophy.
        Acta Paediatr Scand. 1989; 78: 100-103
        • Quanjer PH
        Standardized lung function testing.
        Bull Eur Physiopatol Respir. 1983; 19: 1-95
        • Zapletal A
        • Samanek M
        • Paul T
        Lung function in children and adolescents. Methods, reference values.
        Prog Respir Res. 1987; 22: 113-218
        • Brooke MH
        • Fenichel GM
        • Griggs RC
        Clinical investigation in Duchenne dystrophy: 2. Determination of the “power” of therapeutic trials based on the natural history.
        Muscle Nerve. 1983; 6: 91-103
        • Brooke MH
        • Fenichel GM
        • Griggs RC
        Clinical investigation of Duchenne muscular dystrophy.
        Arch Neurol. 1987; 44: 812-817
        • Gozal D
        Pulmonary manifestations of neuromuscular disease with special reference to Duchenne muscular dystrophy and spinal muscular atrophy.
        Pediatr Pulmonol. 2000; 29: 141-150
        • Vincken WG
        • Elleker MG
        • Cosio MG
        Flow–volume loop changes reflecting respiratory muscle weakness in chronic neuromuscular disorders.
        Am J Med. 1987; 83: 673-680
        • Griggs RC
        The use of pulmonary function testing as a quantitative measurement for therapeutic trials.
        Muscle Nerve. 1990; 13: S30-S34
        • Galasko C SB
        • Williamson JB
        • Delaney CM
        Lung function in Duchenne muscular dystrophy.
        Eur Spine J. 1995; 4: 263-267
        • Miller F
        • Moseley CF
        • Koreska J
        • Levison H
        Pulmonary function and scoliosis in Duchenne dystrophy.
        J Pediatr Orthop. 1988; 8: 133-137
        • Kennedy JD
        • Staples AJ
        • Brook PD
        Effect of spinal surgery on lung function in Duchenne muscular dystrophy.
        Thorax. 1995; 50: 1173-1178