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Pulmonary manifestations of neuromuscular disease with special reference to Duchenne muscular dystrophy and spinal muscular atrophy
Author(s) -
Gozal David
Publication year - 2000
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/(sici)1099-0496(200002)29:2<141::aid-ppul9>3.0.co;2-y
Subject(s) - medicine , spinal muscular atrophy , duchenne muscular dystrophy , neuromuscular disease , muscular dystrophy , weakness , pulmonary function testing , respiratory failure , physical medicine and rehabilitation , physical therapy , intensive care medicine , disease , anesthesia , surgery
Deterioration of respiratory function in patients with neuromuscular disorders is primarily responsible for the high mortality associated with these diseases. A review of Duchenne muscular dystrophy and spinal muscular atrophy, the leading neuromuscular disorders affecting children, will be followed by a critical analysis of the various pathophysiological mechanisms underlying respiratory manifestations in these patients. Among such mechanisms, the role of muscular weakness in preservation of lung function, mucociliary clearance, gas exchange at rest and during exercise, and respiratory control during wakefulness and sleep will be examined in detail. In addition, the potential benefits of respiratory muscle training and of early diagnosis and clinical intervention will be delineated. This review underscores the importance of periodic assessment of pulmonary function during wakefulness and sleep in children affected by neuromuscular diseases as an essential component of multidisciplinary care aimed at improving long‐term morbidity, survival, and quality of life. Pediatr Pulmonol. 2000; 29:141–150. © 2000 Wiley‐Liss, Inc.