Connor J M, Evans C C, Evans D A
Thorax. 1981 Jun;36(6):419-23. doi: 10.1136/thx.36.6.419.
Cardiopulmonary function was evaluated in 21 patients with fibrodysplasia ossificans progressiva. Neither cardiac enlargement nor failure was observed, but six patients had abnormal electrocardiograms. All had marked restrictive spirometry because of chest wall fixation and depended upon diaphragmatic respiration. The severity of chest restriction was independent of sex, age, duration of disease, and extnet of other physical disability. Progression to chronic respiratory failure was not observed. Chest infection in the presence of diminished pulmonary reserve is the major hazard of life in this rare disease and prophylactic measures should be considered.
对21例进行性骨化性纤维发育不良患者的心肺功能进行了评估。未观察到心脏扩大或衰竭，但6例患者心电图异常。由于胸壁固定，所有患者均有明显的限制性肺量测定结果，依赖膈肌呼吸。胸部受限的严重程度与性别、年龄、病程和其他身体残疾程度无关。未观察到进展为慢性呼吸衰竭的情况。在这种罕见疾病中，肺储备减少时发生胸部感染是主要的生命威胁，应考虑采取预防措施。
