波兰患有成骨不全症儿童的人体测量学：单中心回顾性队列研究。
Anthropometrics of Polish children with osteogenesis imperfecta: a single-centre retrospective cohort study.
机构信息
Department of Pediatrics, Newborn Pathology and Bone Metabolic Diseases, University of Lodz, Sporna 36/50, 91-738, Lodz, Poland.
出版信息
BMC Pediatr. 2022 Oct 6;22(1):577. doi: 10.1186/s12887-022-03621-7.
BACKGROUND
Osteogenesis imperfecta (OI) causes a number of abnormalities in somatic development. The predominant symptoms are reduced bone mass and an increased risk of fractures as well as bone deformities and short stature. Due to the lack of causal treatment options, bisphosphonates are considered the gold standard of therapy. The aim of our study is to present selected anthropometric parameters (body weight, height, BMI) in children with type I and III of OI.
METHODS
We performed a retrospective analysis of medical records of patients with osteogenesis imperfecta type I and III confirmed by genetic testing. The study group included individuals admitted to the Department in 2020. We analysed the anthropometric parameters of 108 children (receiving and not receiving bisphosphonates treatment).
RESULTS
In the group of children with OI type I admitted for follow-up (group 1), the median weight percentile was 37, while in the group 2 it was 17. In the patients with OI type III (group 3), the median weight percentile was 0.1. The median height percentile in group 1 was 21, in group 2 it was 5, whereas in group 3 = 0.1. The differences in anthropometric measurements of the patients with OI type I and OI type III were statistically significant (p < 0.001). Among the analysed patients, an abnormal BMI was found in 41.67% of whom 37.78% were underweight, 48.89% were overweight and 13.33% were obese.
CONCLUSION
Considering prevalence of the disease, it is not only low stature but also abnormal BMI, and especially excessive body weight, that play an important role in the somatic development disorder.
背景
成骨不全症（OI）会导致身体发育出现多种异常。主要症状是骨量减少、骨折风险增加以及骨骼畸形和身材矮小。由于缺乏因果治疗选择，双膦酸盐被认为是治疗的金标准。我们研究的目的是介绍 I 型和 III 型成骨不全症患儿的一些选定人体测量参数（体重、身高、BMI）。
方法
我们对经基因检测确诊为 I 型和 III 型成骨不全症的患者的病历进行了回顾性分析。研究组包括 2020 年在我院就诊的患者。我们分析了 108 名儿童（接受和未接受双膦酸盐治疗）的人体测量参数。
结果
在接受随访的 I 型成骨不全症患儿组（第 1 组）中，体重中位数为 37%，而在第 2 组中为 17%。在 III 型成骨不全症患儿组（第 3 组）中，体重中位数为 0.1%。第 1 组的身高中位数为 21%，第 2 组为 5%，而第 3 组为 0.1%。I 型和 III 型成骨不全症患儿的人体测量测量值差异具有统计学意义（p<0.001）。在分析的患者中，41.67%存在异常 BMI，其中 37.78%体重不足，48.89%超重，13.33%肥胖。
结论
考虑到疾病的流行程度，不仅身材矮小，而且异常 BMI，特别是体重过度增加，在躯体发育障碍中起着重要作用。
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