摘要
To evaluate cardiopulmonary function, muscle strength, and cardiopulmonary fit ness (VO2peak) in patients with osteogenesis imperfecta (OI). In 17 patients wit h OI type I (mean age 13.3 ±3.9 years) cardiopulmonary function was assessed at rest using spirometry, plethysmography, electrocardiography, and echocardiogr aphy. Exercise capacity was measured using a maximal exercise test on a bicycle ergometer and an expired gas analysis system. Muscle strength in shoulder abduct ors, hip flexors, ankle dorsal flexor, and grip strength were measured. All resu lts were compared with reference values. Cardiopulmonary function at rest was wi thin normal ranges, but when it was compared with normal height for age and sex, vital capacities were reduced. Mean absolute and relative VO2 peak were respect ively-1.17 (±0.67) and-1.41 (±1.52) standard deviations lower compared w ith reference values (P < .01)-. Muscle strength also was significantly reduce d in patients with OI, ranging from-1.24 ±1.40 to-2.88 ±2.67 standard de viations lower compared with reference values. In patients with OI type I, no pu lmonary or cardiac abnormalities at rest were found. The exercise tolerance and muscle strength were significantly reduced in patients with OI, which might acco unt for their increased levels of fatigue during activities of daily living.
To evaluate cardiopulmonary function, muscle strength, and cardiopulmonary fit ness (VO2peak) in patients with osteogenesis imperfecta (OI). In 17 patients wit h OI type I (mean age 13.3 ±3.9 years) cardiopulmonary function was assessed at rest using spirometry, plethysmography, electrocardiography, and echocardiogr aphy. Exercise capacity was measured using a maximal exercise test on a bicycle ergometer and an expired gas analysis system. Muscle strength in shoulder abduct ors, hip flexors, ankle dorsal flexor, and grip strength were measured. All resu lts were compared with reference values. Cardiopulmonary function at rest was wi thin normal ranges, but when it was compared with normal height for age and sex, vital capacities were reduced. Mean absolute and relative VO2 peak were respect ively-1.17 (±0.67) and-1.41 (±1.52) standard deviations lower compared w ith reference values (P < .01)-. Muscle strength also was significantly reduce d in patients with OI, ranging from-1.24 ±1.40 to-2.88 ±2.67 standard de viations lower compared with reference values. In patients with OI type I, no pu lmonary or cardiac abnormalities at rest were found. The exercise tolerance and muscle strength were significantly reduced in patients with OI, which might acco unt for their increased levels of fatigue during activities of daily living.
