Chest
Clinical InvestigationsPulmonary Function in Progressive Muscular Dystrophy
Section snippets
MATERIAL
The study involved 23 dystrophic children, 21 hoys and two girls, ranging in age from 7 to 17 years. The diagnosis of progressive muscular dystrophy was made in all cases several years prior to this study, initially on grounds of clinical and epidemiologic evidence, later with support by appropriate biochemical studies and muscle biopsies. Table 1 presents pertinent clinical data on these children and information on the course of their disease during the four-year period of observation. At the
METHODS
During the four-year period of observation the dystrophic children were studied at the following intervals: Initially, at six, 12, and 18 months, two and four years after the first study. Each examination included the following lung function tests.
- (1)
Lung volumes: Functional residual capacity (FRC) was determined by the closed-circuit helium dilution method,5 the other compartments of total lung capacity were measured from a spirogram.
- (2)
Forced vital capacity (FVC) was determined with a low
RESULTS
Table 2 presents the initial findings on lung volumes and spirometric studies in the dystrophic group. In the majority of children vital capacities were reduced below the range of normal, while the residual volumes were within normal limits. Functional residual capacity was below the normal mean in 19 children, and 12 of these had volumes below the normal range. The average values for the dystrophic group were 62 percent of predicted for vital capacity, 113 percent for residual volume and 80
DISCUSSION
Except for absence of airway obstruction in the dystrophic children, our study revealed abnormalities in all other parameters of pulmonary function tested. In spite of the great variation observed among individuals, a distinct pattern of functional impairment emerged for the group of dystrophic children. The changes in lung volumes are of restrictive type, characterized by a reduction in inspiratory capacity, downward displacement of the resting pulmonary midposition and preservation of the
ACKNOWLEDGMENTS
Thanks are due to all those who helped in this investigation: Dr. Patricia Murray and her stall, Marquette University, Milwaukee, Wisconsin, for help with the statistical analysis; Miss Verna Davies, Sully Hospital, for evaluation of functional ability and muscle performance; Mrs. Mary Ninan, Miss Diane Harding, Miss Julia Dabbs, and Mrs. Doris Barron, of the Respiratory Function Laboratory, Sully Hospital, for technical assistance and much inconvenience; Miss Patricia Edwards, Sully Hospital,
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