Hospital for Special Surgery, New York, NY.
National Jewish Health, Denver, CO.
Spine (Phila Pa 1976). 2019 Aug 1;44(15):1057-1063. doi: 10.1097/BRS.0000000000003012.
Retrospective clinical study of individuals with osteogenesis imperfecta (OI).
To assess the relationship between severity of scoliosis and pulmonary function, and to assess the relationship between restrictive lung disease and self-reported quality of life in individuals with OI.
OI is a heritable connective tissue disorder characterized by osteopenia and a predisposition to fracture. Respiratory insufficiency is a leading cause of mortality. Literature on pulmonary function in this population has shown a negative correlation between percent-predicted vital capacity and severity of scoliosis. However, it has been suggested that decreased pulmonary function in OI may be due to intrinsic pulmonary disease, in addition to the impact of vertebral compression fractures and scoliosis.
Anterior-posterior spine radiographs and pulmonary function tests from 30 individuals with OI were reviewed. Radiographs were evaluated for scoliosis, defined as a curve ≥ 10°. If more than one curve was present, the largest curve was used. Pulmonary function was defined as the forced expiratory volume in 1 second (FEV1)/forced vital capacity (FVC) ratio. Restrictive pulmonary disease was defined as FEV1/FVC > 80%, while obstructive disease was defined as FEV1/FVC < 70%. Bivariate correlation analysis was performed, using Spearman rho correlation coefficient (P < 0.05). Quality of life was assessed by SF-36.
The mean age was 27.6 years (range: 12-42 yrs). 57.6% were female. OI type IV was the most common (46.7%), followed by OI type III (33.3%), OI type I (10%), OI type IX (6.67% each), and OI type VIII (3.33%). Pulmonary comorbidity was present in 40% of individuals, while 6.67% had a cardiac comorbidity. The correlation between scoliosis and pulmonary function was weak and not significant (R = -0.059, P = 0.747).
Pulmonary function is not significantly correlated with scoliosis, supporting the hypothesis that decreased pulmonary function is intrinsic to OI and/or chest wall deformities, rather than secondary to scoliosis.
成骨不全症（OI）个体的回顾性临床研究。
评估脊柱侧弯严重程度与肺功能之间的关系，并评估 OI 个体中限制性肺疾病与自我报告的生活质量之间的关系。
OI 是一种遗传性结缔组织疾病，其特征为骨质疏松症和骨折易感性增加。呼吸功能不全是导致死亡的主要原因。该人群的肺功能文献表明，预测肺活量的百分比与脊柱侧弯的严重程度呈负相关。然而，有人认为 OI 中肺功能下降可能不仅是由于椎体压缩性骨折和脊柱侧弯的影响，还可能是由于内在的肺部疾病所致。
对 30 例 OI 患者的前后位脊柱 X 线片和肺功能检查结果进行了回顾性分析。对 X 线片进行了脊柱侧弯评估，定义为曲线≥10°。如果存在多个曲线，则使用最大曲线。肺功能定义为 1 秒用力呼气量（FEV1）/用力肺活量（FVC）比值。限制性肺疾病定义为 FEV1/FVC>80%，而阻塞性疾病定义为 FEV1/FVC<70%。使用 Spearman rho 相关系数（P<0.05）进行了双变量相关分析。使用 SF-36 评估生活质量。
平均年龄为 27.6 岁（范围：12-42 岁）。57.6%为女性。最常见的 OI 类型是 IV 型（46.7%），其次是 III 型（33.3%）、I 型（10%）、IX 型（各 6.67%）和 VIII 型（各 3.33%）。40%的个体存在肺部合并症，而 6.67%的个体存在心脏合并症。脊柱侧弯与肺功能之间的相关性较弱且无统计学意义（R=-0.059，P=0.747）。
肺功能与脊柱侧弯无明显相关性，支持肺功能下降是 OI 内在的和/或胸廓畸形所致，而不是继发于脊柱侧弯的假说。
4 级。
