title: Premature changes in trabecular and cortical microarchitecture result in decreased bone strength in hemophilia.
abstract: Low bone density is a growing concern in aging men with hemophilia and may result in high-morbidity fragility fractures. Using high-resolution peripheral quantitative computed tomography (HR-pQCT), we demonstrate low trabecular and cortical bone density contributing to lower volumetric bone mineral density (BMD) at both distal radius and tibia in patients with hemophilia compared with age- and sex-matched controls. The low trabecular bone density found in hemophilia is attributed to significantly decreased trabecular number and increased separation; the lower cortical bone density results from thinner cortices, whereas cortical porosity is maintained. Microfinite element analysis from three-dimensional HR-pQCT images demonstrates that these microarchitectural deficits seen in patients with hemophilia translate into significantly lower estimated failure load (biomechanical bone strength) at the distal tibia and radius when compared with controls. In addition, an inverse association of joint score with BMD and failure load suggests the negative role of hemophilic arthropathy in bone density loss.
authors: Department of Medicine, University of Calgary, Calgary, AB, Canada; Southern Alberta Rare Blood and Bleeding Disorders Comprehensive Care Program, Foothills Medical Centre, Calgary, AB, Canada; and.
authors: Adrienne
authors: http://orcid.org/0000-0003-2032-8997
authors: A
authors: Lee
authors: Department of Radiology, University of Calgary, Calgary, AB, Canada.
authors: Steven K
authors: SK
authors: Boyd
authors: Department of Medicine, University of Calgary, Calgary, AB, Canada;
authors: Gregory
authors: G
authors: Kline
authors: Department of Medicine, University of Calgary, Calgary, AB, Canada; Southern Alberta Rare Blood and Bleeding Disorders Comprehensive Care Program, Foothills Medical Centre, Calgary, AB, Canada; and.
authors: Man-Chiu
authors: http://orcid.org/0000-0002-7012-2420
authors: MC
authors: Poon
mesh_terms: D015502:Absorptiometry, Photon; D000328:Adult; D001696:Biomechanical Phenomena; D015519:Bone Density; D001851:Bone Diseases, Metabolic / Q000000981:diagnostic imaging / Q000453:epidemiology / Q000209:etiology*; D016022:Case-Control Studies; D019245:Compressive Strength; D005260:Female; D006467:Hemophilia A / Q000150:complications* / Q000000981:diagnostic imaging / Q000453:epidemiology; D006801:Humans; D008297:Male; D016062:Porosity; D011884:Radius / Q000000981:diagnostic imaging; D013977:Tibia / Q000000981:diagnostic imaging; D013997:Time Factors