Bone geometry, density and microarchitecture in the distal radius and tibia in adults with osteogenesis imperfecta type-1 assessed by HR-pQCT

Osteogenesis Imperfecta (OI) is a hereditary disorder characterized by decreased biosynthesis or impaired morphology of collagen type-1 that leads to decreased bone mass and increased bone fragility. We hypothesized that patients with OI have altered bone microstructure and bone geometry. In this cross-sectional study we compared patients with type I OI to age- and gender-matched healthy controls. A total of 39 (13 men and 26 women) patients with OI, aged 53 (range: 21-77) years, and 39 controls, aged 53 (range: 21-77) years, were included in the study. Twenty-seven of the patients had been treated with bisphosphonates. HR-pQCT at the distal radius and distal tibia and dual-energy X-ray absorptiomentry of total hip, femoral neck, trochanteric region and the lumbar spine (L1-L4) were performed. The patients were shorter than the controls (159 ± 10 cm vs. 170 ± 9 cm, p