■椎体压缩性骨折(VFs)是骨质疏松症患者中常见且严重的发现。在儿童中,VF具有重塑和恢复其原始配置的独特潜力。自发性椎体重塑(即,无辅助用药)已在继发性骨质疏松症中报道。在这里,我们描述了先前未报道的成骨不全症(OI)伴多发性椎骨骨折的青少年自发性椎骨重塑。
■一名17岁女性在5岁时被诊断为I型OI,原因是COL1A1中的一种新型移码变异(NM_000088.4:c.540delC;p.Met181TrpfsTer84)。由于父母对药物的保留,她从未接受过双膦酸盐或任何其他骨活性疗法.脊柱侧位X射线显示骨骼透明且无VF。然而,以前在6岁时在外部机构拍摄的脊柱X射线显示T5-7的VF(Genant半定量方法I-II级)。脊柱的两个侧面X光片,相隔11年,证明在青春期没有骨活性治疗的情况下发生了实质性的自发性椎体重塑。
■脊椎重塑是通过稳定骨密度(BMD)和儿童剩余的生长能力来解释的。我们假设自发重塑可能发生在较温和形式的OI中,青春期可能是这一现象的关键中介。在所有患有OI和椎骨骨折的儿童中,尽管如此,我们还是推荐双膦酸盐治疗,因为它可以改善骨量,BMD,椎骨形状,身体活动,降低骨折率。
UNASSIGNED: Vertebral compression fractures (VFs) are a common and severe finding in patients with osteoporosis. In children, VFs have the unique potential to reshape and regain their original configuration. Spontaneous vertebral body reshaping (i.e., medication-unassisted) has been reported in secondary osteoporosis. Here we describe a previously unreported spontaneous vertebral reshaping in an adolescent with osteogenesis imperfecta (OI) with multiple vertebral fractures.
UNASSIGNED: A 17-year-old female was diagnosed with OI type I at 5 years of age caused by a novel frameshift variant in COL1A1 (NM_000088.4: c.540delC; p.Met181TrpfsTer84). Due to parental reservations about medication, she had never received bisphosphonate or any other bone active therapy. A lateral spine X-ray demonstrated transparent bones and no VF. However, previous spine X-rays taken at age of 6 years at an external institution showed VFs in T5-7 (Genant semiquantitative method grade I-II). The two lateral spine x-rays, taken 11 years apart, demonstrate that substantial spontaneous vertebral reshaping occurred without bone active therapy during puberty.
UNASSIGNED: Vertebral reshaping is explained by the stabilization of bone mineral density (BMD) and the remaining growth capacity the children. We hypothesize that spontaneous reshaping may occur in milder forms of OI, and that puberty may be a key mediator of the phenomenon. In all children with OI and vertebral fractures, we nevertheless recommend bisphosphonate therapy since it improves bone mass, BMD, vertebral shape, physical activity and reduces fracture rates.