Futagawa Natsuko, Hasegawa Kosei, Miyahara Hiroyuki, Tanaka Hiroyuki, Tsukahara Hirokazu
Department of Pediatrics, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan.
Department of Pediatrics, Okayama University Hospital, Okayama, Japan.
Front Pediatr. 2024 Dec 3;12:1500023. doi: 10.3389/fped.2024.1500023. eCollection 2024.
Osteogenesis imperfecta (OI) is a congenital skeletal disorder characterized by bone fragility. Bisphosphonates (BISs) have become the mainstream treatment in children with OI. However, an optimal treatment protocol has not yet been established, while BIS treatment tends to be administered to normalize bone mineral density (BMD). Bone quality is an important component of bone strength. The trabecular bone score (TBS) is a quantitative measure of the microstructure that affects bone quality. This study investigated the TBS during BIS treatment in children with OI.
Twenty-nine children with OI were enrolled and classified into two groups: mild (type 1) and moderate to severe (types 3 and 4). Dual-energy x-ray absorptiometry images were retrospectively analyzed for TBS calculation. The relationship between the areal BMD (aBMD), its Z-score, height-adjusted BMD (BMD) Z-score, TBS, and TBS Z-score with the treatment duration was assessed for each group.
In the mild group, the aBMD, its Z-score, and BMDHAZ Z-score showed a significant positive correlation with treatment duration (r = 0.68, 0.68, 0.72, respectively, < 0.01). The TBS Z-score tended to increase with treatment duration, albeit without reaching significance. In the moderate to severe group, the TBS Z-score showed a significant positive correlation with treatment duration (r = 0.48, < 0.01), in contrast to the aBMD Z-score, which did not increase. Finally, the BMDHAZ Z-score only showed a weak positive correlation with treatment duration (r = 0.37, < 0.01).
Because BIS affect the BMD and TBS differently based on the severity of OI, treatment goals may need to be stratified by disease severity.
成骨不全症（OI）是一种以骨骼脆弱为特征的先天性骨骼疾病。双膦酸盐（BISs）已成为治疗儿童OI的主流方法。然而，尚未建立最佳治疗方案，而BIS治疗往往旨在使骨矿物质密度（BMD）正常化。骨质量是骨强度的重要组成部分。小梁骨评分（TBS）是影响骨质量的微观结构的定量指标。本研究调查了OI患儿接受BIS治疗期间的TBS。
招募了29例OI患儿，并分为两组：轻度（1型）和中度至重度（3型和4型）。回顾性分析双能X线吸收测定图像以计算TBS。评估每组的面积BMD（aBMD）、其Z评分、身高调整后的BMD（BMD）Z评分、TBS和TBS Z评分与治疗持续时间之间的关系。
在轻度组中，aBMD、其Z评分和BMDHAZ Z评分与治疗持续时间呈显著正相关（r分别为0.68、0.68、0.72，P<0.01）。TBS Z评分虽未达到显著水平，但倾向于随治疗持续时间增加。在中度至重度组中，TBS Z评分与治疗持续时间呈显著正相关（r=0.48，P<0.01），而aBMD Z评分没有增加。最后，BMDHAZ Z评分仅与治疗持续时间呈弱正相关（r=0.37，P<0.01）。
由于BIS根据OI的严重程度对BMD和TBS的影响不同，治疗目标可能需要根据疾病严重程度进行分层。
