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(Chest. 1972;61:41-47.)
© 1972 American College of Chest Physicians

Pulmonary Function in Progressive Muscular Dystrophy

E. J. Hapke M.D.1; J. C. Meek M.D.1; and J. Jacobs M.D.1

1 Sully Hospital, the Thoracic Centre of Wales, Great Britain

Serial pulmonary function studies performed over a four-year period in children with progressive muscular dystrophy revealed a restrictive type of functional impairment, the severity of which was unrelated to the grade of each child's general functional ability. CO2 retention was not observed in any of the children during the period of observation, including the two who died from pulmonary causes within three months of their last examination. The results of our study suggest that primary pulmonary insufficiency and failure are associated only with severe general disability marking the ultimate stage of the disease.




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