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Abstract
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.
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References
REFERENCES
- Profiles of neuromuscular diseases. Duchenne muscular dystrophy.Am J Phys Med Rehabil. 1995; 74: S70-S92
- Diagnostic Criteria for Neuromuscular Disorders. European Neuromuscular Centre, Baarn1994
- Evaluation of pulmonary function in neuromuscular disease.Arch Neurol. 1981; 38: 9-12
- Progression of scoliosis in Duchenne muscular dystrophy.J Bone Joint Surg. 1989; 71: 1066-1074
- Clinical implications of maximal respiratory pressure determinations for individuals with Duchenne muscular dystrophy.Arch Phys Med Rehabil. 1997; 78: 1-6
- Pulmonary function in Duchenne muscular dystrophy related to stage of disease.Am J Med. 1974; 56: 297-306
- Cardiac and respiratory involvement in advanced stage Duchenne muscular dystrophy.Neuromuscul Disord. 1996; 6: 367-376
- Treatment of respiratory insufficiency in Duchenne's muscular dystrophy: nasal ventilation in the initial stages.Monaldi Arch Chest Dis. 1995; 50: 235-238
- Indicators for an impending need of artificial respiration in patients with muscular dystrophy.Ugeskr Laeger. 1993; 155: 1963-1967
- Respiratory dysfunction in muscular dystrophy and other myopathies.Clin Chest Med. 1994; 15: 661-674
- 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
- Child neuromuscular disease in southern Norway. The prevalence and incidence of Duchenne muscular dystrophy.Acta Paediatr Scand. 1989; 78: 100-103
- Standardized lung function testing.Bull Eur Physiopatol Respir. 1983; 19: 1-95
- Lung function in children and adolescents. Methods, reference values.Prog Respir Res. 1987; 22: 113-218
- Clinical investigation in Duchenne dystrophy: 2. Determination of the “power” of therapeutic trials based on the natural history.Muscle Nerve. 1983; 6: 91-103
- Clinical investigation of Duchenne muscular dystrophy.Arch Neurol. 1987; 44: 812-817
- Pulmonary manifestations of neuromuscular disease with special reference to Duchenne muscular dystrophy and spinal muscular atrophy.Pediatr Pulmonol. 2000; 29: 141-150
- Flow–volume loop changes reflecting respiratory muscle weakness in chronic neuromuscular disorders.Am J Med. 1987; 83: 673-680
- The use of pulmonary function testing as a quantitative measurement for therapeutic trials.Muscle Nerve. 1990; 13: S30-S34
- Lung function in Duchenne muscular dystrophy.Eur Spine J. 1995; 4: 263-267
- Pulmonary function and scoliosis in Duchenne dystrophy.J Pediatr Orthop. 1988; 8: 133-137
- Effect of spinal surgery on lung function in Duchenne muscular dystrophy.Thorax. 1995; 50: 1173-1178
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Publication history
Accepted:
June 21,
2001
Received:
February 8,
2001
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© 2001 Harcourt Publishers Ltd. Published by Elsevier Inc.
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