Takken Tim, Terlingen Heike C, Helders Paul J M, Pruijs Hans, Van der Ent Cornelis K, Engelbert Raoul H H
Department of Pediatric Physical Therapy & Exercise Physiology, Wilhelmina Children's Hospital, University Medical Center Utrecht, The Netherlands.
J Pediatr. 2004 Dec;145(6):813-8. doi: 10.1016/j.jpeds.2004.08.003.
To evaluate cardiopulmonary function, muscle strength, and cardiopulmonary fitness (VO 2 peak) in patients with osteogenesis imperfecta (OI).
In 17 patients with OI type I (mean age 13.3 +/- 3.9 years) cardiopulmonary function was assessed at rest using spirometry, plethysmography, electrocardiography, and echocardiography. Exercise capacity was measured using a maximal exercise test on a bicycle ergometer and an expired gas analysis system. Muscle strength in shoulder abductors, hip flexors, ankle dorsal flexor, and grip strength were measured. All results were compared with reference values.
Cardiopulmonary function at rest was within normal ranges, but when it was compared with normal height for age and sex, vital capacities were reduced. Mean absolute and relative VO 2 peak were respectively -1.17 (+/- 0.67) and -1.41 (+/- 1.52) standard deviations lower compared with reference values ( P < .01). Muscle strength also was significantly reduced in patients with OI, ranging from -1.24 +/- 1.40 to -2.88 +/- 2.67 standard deviations lower compared with reference values.
In patients with OI type I, no pulmonary or cardiac abnormalities at rest were found. The exercise tolerance and muscle strength were significantly reduced in patients with OI, which might account for their increased levels of fatigue during activities of daily living.
评估成骨不全症（OI）患者的心肺功能、肌肉力量和心肺适能（最大摄氧量）。
对17例I型OI患者（平均年龄13.3±3.9岁），采用肺活量测定法、体积描记法、心电图和超声心动图在静息状态下评估心肺功能。使用自行车测力计和呼出气体分析系统通过最大运动试验测量运动能力。测量肩部外展肌、髋部屈肌、踝背屈肌的肌肉力量和握力。所有结果均与参考值进行比较。
静息状态下的心肺功能在正常范围内，但与同年龄、同性别的正常身高者相比，肺活量降低了。平均绝对和相对最大摄氧量分别比参考值低1.17（±0.67）和1.41（±1.52）个标准差（P<.01）。OI患者的肌肉力量也显著降低，与参考值相比，降低幅度在1.24±1.40至2.88±2.67个标准差之间。
在I型OI患者中，未发现静息时的肺部或心脏异常。OI患者的运动耐力和肌肉力量显著降低，这可能是他们在日常生活活动中疲劳程度增加的原因。
