Bone microstructural and strength changes over one year in children with osteogenesis imperfecta are comparable to age- and sex-matched healthy controls.

Abstract

Osteogenesis imperfecta (OI) is characterized by bone fragility with frequent fractures, especially in children. Some studies have used peripheral-quantitative computed tomography (pQCT) to examine bone density in children with OI and one cross-sectional study used high-resolution-pQCT (HR-pQCT) in nine children with OI. We compared bone density, microstructure, and strength changes over one year at the metaphysis and diaphysis of the radius and tibia, as well as lumbar spine bone mass and area, in 20 children with OI under bisphosphonate treatment and 20 age- and sex-matched controls. For the double-stack metaphyseal scans, we developed an algorithm to correct stack misalignment. For the lumbar spine, we used dual-energy x-ray absorptiometry (DXA). At the tibial metaphysis, children with OI had lower baseline total and trabecular volumetric bone mineral density (vBMD), trabecular and cortical microstructure, and strength. At the radial metaphysis, baseline trabecular microstructure and strength were lower in children with OI. At the tibial and radial diaphysis, children with OI had lower bone area and strength. At the tibial and radial metaphysis, 1-yr increases in most measurements were similar for both groups. Cortical vBMD and trabecular separation (radius) only increased in the OI group. Trabecular vBMD, volume fraction (tibia), number (tibia), and total vBMD (radius) only increased in the control group. At the tibia and radius diaphysis, cortical vBMD increased in the OI group. Tibial Ct. Po decreased in children with OI. At the lumbar spine, the OI group had lower bone mass, density, and area, but similar longitudinal changes. In summary, baseline trabecular bone density, trabecular and cortical microstructure, and strength were lower in metaphyseal regions of children with OI. At diaphysis, children with OI had lower bone area and strength. While longitudinal changes over one year were generally comparable, certain outcomes demonstrated differences. These data are essential for powering future clinical trials.