血清生长因子浓度和超声信息含量对Ⅲ型成骨不全患儿骨关节系统结构状态研究的评估

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详细

论证。在对成骨不全症患者进行治疗时，必须保证对长骨的结构状态和新陈代谢进行动态监测。在我们现有的文献中，几乎没有关于使用超声波检查来评估成骨不全症儿童骨骼系统的数据。 血清中TGF-β（转化生长因子β）超家族成员的表达增加已被描述为一系列先天性骨组织疾病，但尚未考虑用于III型成骨不全症患儿。

本研究旨在研究III型成骨不全症儿童血清中生长因子的浓度与健康儿童的对比情况，同时确定超声检查在评估III型成骨不全症儿童骨关节系统状况方面的信息量，并证实超声波用于该病症的可行性。

材料和方法。研究对象为患有III型成骨不全症的3-7岁儿童（12人）。血清中的骨矿物质代谢参数由Hitachi/BM 902分析仪（日本）测定，生长因子及其受体的含量由Thermofisher分析仪 （美国）通过酶免疫测定法测定。超声波检查在AVISUS日立（日本）仪器上进行。使用Attestat程序（I.P. Gaidyshev）进行统计处理。在拒绝正态分布的情况下，定量数据以样本的中位数和四分位数（Me [Q1；Q3]）表示。在正态分布下，定量数据以M±σ表示，p<0.05。

结果。成骨不全患者的骨矿化程度和骨转换率均高于健康同龄人，胶原含量较低。成纤维细胞生长因子FGF-basic的变化最大；血管内皮生长因子受体VEGF-R3的含量减少，而血管内皮生长因子和VEGF-R2的含量却成倍增加。超声波检查可以发现股骨和胫骨的骨骺和骨骺以及髋关节和膝关节有畸形和多处骨折。

结论。负责激活破骨细胞生成的生长因子分泌失衡。负责抑制破骨细胞和活化成骨细胞的生长因子含量符合正常或有轻微变化。超声波检查在详细评估成骨不全症患者的骨骼和关节装置方面具有很高的信息量，因此这种非侵入性技术可推荐在该疾病中广泛应用。

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作者简介

Svetlana N. Luneva

National Ilizarov Medical Research Center for Traumatology and Orthopaedics

Email: luneva_s@mail.ru
ORCID iD: 0000-0002-0578-1964
SPIN 代码: 9572-2655

PhD, Dr. Sci. (Biol.), Professor

俄罗斯联邦, Kurgan

Tatyana I. Menshchikova

National Ilizarov Medical Research Center for Traumatology and Orthopaedics

编辑信件的主要联系方式.
Email: tat-mench@mail.ru
ORCID iD: 0000-0002-5244-7539
SPIN 代码: 2820-9120

PhD, Dr. Sci. (Biol.)

俄罗斯联邦, Kurgan

Anna M. Aranovich

National Ilizarov Medical Research Center for Traumatology and Orthopaedics

Email: aranovich_anna@mail.ru
ORCID iD: 0000-0002-7806-7083
SPIN 代码: 7277-6339

MD, PhD, Dr. Sci. (Med.), Professor

俄罗斯联邦, Kurgan

Evgeniia P. Vykhovanets

National Ilizarov Medical Research Center for Traumatology and Orthopaedics

Email: vykhovanets.eva@mail.ru
ORCID iD: 0000-0002-7661-9817
SPIN 代码: 7087-3146

MD, PhD, Cand. Sci. (Med.)

俄罗斯联邦, Kurgan

Kseniya P. Matveeva

National Ilizarov Medical Research Center for Traumatology and Orthopaedics

Email: k_paveleva1996@mail.ru
ORCID iD: 0009-0003-0246-2946

MD, PhD student

俄罗斯联邦, Kurgan

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2. Fig. 2. Sonogram of the hip joint of patient K (7 years old) with type III osteogenesis imperfecta. Standard scanning reveals a spherical femoral head with uneven and heterogeneous contour of the subchondral plate

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3. Fig. 1. Sonograms of pathologic fractures of the femur and tibia of patient N., 7 years old, with type III osteogenesis imperfecta: a, disruption of the cortical plate contour integrity is visualized at the apex of the femur deformity. Small clumps measuring 1–1.2 mm are located between the ends of the fragments. The acoustic density in the fracture zone is 77.3 units (rectangle); b, tibial metaphysis; discontinuous contour is visualized. An area with multiple fractures is shown. The acoustic densities are 79.4, 88.4, and 100.5 units (rectangle)

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4. Fig. 3. Sonograms of the knee joint of patient K (7 years old) with type III osteogenesis imperfecta: a, the angle of the patellar bed at maximal flexion is smoothed, with uneven hyaline cartilage, 1.5–2.2–2.8 mm thick; b, uneven contour of the subchondral plate of the tibia with small clumpy inclusions

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