Assessment of the serum concentration of growth factors and the informativeness of ultrasonography in studying the structural conditions of the osteoarticular system in children with type III osteogenesis imperfecta

Abstract

BACKGROUND: The treatment of patients with osteogenesis imperfecta requires dynamic monitoring of the structural state and metabolism of the long bones. In the available literature, practically no data are available on the use of ultrasonography to assess the skeletal system in children with osteogenesis imperfecta. Increased expression of the members of the transforming growth factor-β superfamily in the serum has been described in several congenital bone diseases; however, this has not yet been examined in children with type III osteogenesis imperfecta.

AIM: To examine the serum concentrations of growth factors in children with type III osteogenesis imperfecta relative to healthy children and evaluate the informativeness of ultrasonography for assessing the state of the osteoarticular system in type III osteogenesis imperfecta and justify the feasibility of its use in this pathology.

MATERIALS AND METHODS: Children aged 3–7 years with type III osteogenesis imperfecta (n = 12) were examined. In the blood serum, bone-mineral metabolism parameters were determined on a Hitachi/BM 902 analyzer (Japan), and the contents of growth factors and their receptors were determined by enzyme-linked immunosorbent assay on a Thermo Fisher Scientific analyzer (USA). Ultrasound examinations were performed using an AVISUS Hitachi device (Japan). Statistical processing was carried out using the Attestat program (I.P. Gaidyshev). Quantitative data are presented as medians and quartiles (Me [Q₁; Q₃]) for samples with non-normal distribution. In cases with normal distribution, quantitative data are presented as M ± σ, p < 0.05.

RESULTS: In patients with osteogenesis imperfecta, the degree of bone tissue mineralization and bone turnover rates were higher and the collagen content was lower than those of their healthy peers. Fibroblast growth factor-basic underwent the greatest changes; a decrease in the content of the vascular endothelial growth factor (VEGF)-R3 receptor was accompanied by multiple increases in VEGF and VEGF-R2. Ultrasonography identified areas of deformation and multiple fractures in the area of the diaphyses and metaphyses of the femur, tibia, hip, and knee joints.

CONCLUSIONS: Predominance was noted toward the production of growth factors responsible for the activation of osteoclastogenesis. The content of growth factors responsible for osteoclast inhibition and osteoblast activation is normal or slightly changed. Ultrasonography has demonstrated high informativeness in a detailed assessment of the osteoarticular system in patients with osteogenesis imperfecta, which allows us to recommend this noninvasive technique for wider use in this disease.

Keywords

osteogenesis imperfecta type III / growth factors of osteotropic hormones / bone ultrasound

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