Fracture Rates and Fracture Sites in Patients With Osteogenesis Imperfecta: A Nationwide Register-Based Cohort Study
Fracture Rates and Fracture Sites in Patients With Osteogenesis Imperfecta: A Nationwide Register-Based Cohort Study
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DOI：
10.1002/jbmr.2920
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发表时间：
2017-01-01
影响因子：
6.2
通讯作者：
Brixen, Kim
中科院分区：
文献类型：
--
作者：
Folkestad, Lars;Hald, Jannie Dahl;Brixen, Kim
成骨不全症 (OI) 是一种遗传性、临床异质性结缔组织疾病。丹麦的成骨不全症患病率为 10 万分之 10.6。该疾病的一个特点是频繁的骨折，通常是由轻微的创伤引起的。本研究的目的是将成骨不全患者一生中的骨折率与普通人群的参考人群进行比较。本研究是一项丹麦全国性、基于人群的队列研究，使用登记数据。我们通过丹麦国家患者登记册确定了成骨不全队列中的 644 名患者（55.6% 女性），并从民事登记系统中随机选择了 3361 名患者（55.2% 女性）。在中位17.9年（四分位距[IQR]，12.4至18.0年）的观察期内，共有416名成骨不全患者总共经历了1566处骨折，总计10137人年。相比之下，参考人群中有 709 人在随访期间总共经历了 1018 次骨折。男性和女性成骨不全患者一生中骨折率都会增加。与参考人群相比，0至19岁参与者的骨折率为10.7，20至54岁参与者为17.2，55岁及以上参与者为4.1。 0 至 19 岁成骨不全男性的骨折率最高（每 1000 人年 257 例骨折）。骨折似乎遵循与一般人群相同的模式，在幼儿和青少年时期达到高峰（发病率 [IR] 233.9/1000 人年），成年期骨折较少（IR 84.5/1000 人年），老年女性骨折率增加（IR 111.9/1000 人年）。这是关于成骨不全患者骨折流行病学的最大的基于登记的全国性研究。骨折风险似乎在儿童和青少年时期最大，并且与普通人群相比，成骨不全患者的骨折相对风险随着年龄的增长而下降。 (c) 2016 年美国骨与矿物质研究学会。
Osteogenesis imperfecta (OI) is a hereditary, clinically heterogeneous, connective tissue disorder. The population prevalence of OI in Denmark is 10.6 in 100,000. A hallmark of the disease is frequent fractures that are often precipitated by minimal trauma. The aim of the current study was to compare the fracture rates across the lifespan of patients with OI with that of a reference population from the general population. The present study was a Danish nationwide, population-based, cohort study using register data. We identified 644 (55.6% females) patients in the OI cohort through the Danish National Patient Register and 3361 (55.2% females) persons, randomly selected from the Civil Registry System. A total of 416 patients with OI experienced a total of 1566 fractures during the observation period of median 17.9 years (interquartile range [IQR], 12.4 to 18.0 years), summing to 10137 person years. In comparison, 709 persons in the reference population experienced a total of 1018 fractures during follow-up. Both male and female patients with OI had an increased fracture rate throughout their life. The fracture rate ratio for participants aged 0 to 19 years was 10.7, for participants aged 20 to 54 years 17.2, and for participants aged 55 years and over 4.1 when compared to the reference population. The highest fracture rate was seen in males with OI aged 0 to 19 years (257 fractures per 1000 person-years). The fractures appear to follow the same pattern as in the general population, with a peak during the toddler and adolescent years (incidence rate [IR] 233.9 per 1000 person years), fewer fractures during adulthood (IR 84.5 per 1000 person years), and increased fracture rates in older women (IR 111.9 per 1000 person years). This is the largest register-based nationwide study on the fracture epidemiology of patients with OI. The risk of fractures seems largest in the childhood and adolescent years, and the relative risk of fracture declines with age in patients with OI compared to the general population. (c) 2016 American Society for Bone and Mineral Research.