Shiraz University of Medical Sciences, Shiraz, Iran.
Gastroentrology Research Center, Neonatology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
BMC Pediatr. 2021 Jan 14;21(1):37. doi: 10.1186/s12887-020-02491-1.
Osteogenesis imperfecta(OI) is a frequent bone fragility disorder in children. The purpose of this study was to assess the BMD and Vitamin D level in children with OI in southern Iran.
This case-control study was conducted on 23 children, clinically diagnosed as osteogenesis imperfecta and 23 age- and gender-matched healthy controls. Demographic and anthropometric data, biochemical parameters, puberty, sun exposure and physical activity were assessed. Bone mineral density (BMD) was measured by Dual-energy X-ray absorptiometry (DXA). Data analysis was done by SPSS22.
Forty-three point four percent of OI patients and fifty-six point five percent of control group had vitamin D deficiency (P = 0.376). Thirteen OI patients (56%) had low bone mass for chronological age in lumbar area (P < 0.001). Fracture episodes during treatment was significantly influenced by time of Pamidronate start, courses of Pamidronate injection, puberty and sun exposure (P values = 0.015, 0.030, 0.044 and 0.032, respectively). Fracture episodes during treatment had significantly increased in patients who had received Pamidronate more than 3 years compared with those received less than 3 years(P values = 0.047).
This study showed that vitamin D deficiency is prevalent amongst OI children in southern Iran. More than half of the OI children had low bone mass for chronological age in lumbar area, despite receiving bisphosphonate therapy. The present results revealed that early initiation of Pamidronate and number of Pamidronate courses are associated with lower fracture rate. However, treatment period more than 3 years can have adverse effect on fracture rates.
成骨不全症（OI）是儿童中常见的骨脆弱性疾病。本研究旨在评估伊朗南部 OI 儿童的骨密度（BMD）和维生素 D 水平。
这项病例对照研究纳入了 23 名临床诊断为成骨不全症的儿童和 23 名年龄和性别匹配的健康对照。评估了人口统计学和人体测量学数据、生化参数、青春期、阳光暴露和身体活动。通过双能 X 射线吸收法（DXA）测量骨矿物质密度（BMD）。数据采用 SPSS22 进行分析。
43.4%的 OI 患者和 56.5%的对照组存在维生素 D 缺乏（P=0.376）。13 名 OI 患者（56%）腰椎区域的骨量低于年龄骨量（P<0.001）。在治疗期间发生骨折的次数显著受到帕米膦酸盐起始时间、帕米膦酸盐注射次数、青春期和阳光暴露的影响（P 值分别为 0.015、0.030、0.044 和 0.032）。与接受帕米膦酸盐治疗<3 年的患者相比，接受帕米膦酸盐治疗>3 年的患者治疗期间发生骨折的次数显著增加（P 值=0.047）。
本研究表明，伊朗南部 OI 儿童中维生素 D 缺乏较为普遍。尽管接受了双膦酸盐治疗，但超过一半的 OI 儿童腰椎区域的骨量低于年龄骨量。本研究结果表明，早期开始帕米膦酸盐治疗和帕米膦酸盐疗程数与较低的骨折率相关。然而，治疗期超过 3 年可能对骨折率产生不利影响。
