BoneKEy reports最新文献

Cortical bone is not compact; rather it is penetrated by many Haversian and Volkmann canals for blood supply. The lining of these canals are the intracortical bone surfaces available for bone remodeling. Increasing intracortical bone remodeling increases cortical porosity. However, cortical bone loss occurs more slowly than trabecular loss due to the fact that less surface per unit of bone matrix volume is available for bone remodeling. Nevertheless, most of the bone loss over time is cortical because cortical bone constitutes 80% of the skeleton, and the relative proportion of trabecular bone diminishes with advancing age. Higher serum levels of bone turnover markers are associated with higher cortical porosity of the distal tibia and the proximal femur. Greater porosity of the distal radius is associated with higher odds for forearm fracture, and greater porosity of the proximal femur is associated with higher odds for non-vertebral fracture in postmenopausal women. Measurement of cortical porosity contributes to fracture risk independent of areal bone mineral density and Fracture Risk Assessment Tool. On the other hand, antiresorptive treatment reduces porosity at the distal radius and at the proximal femoral shaft. Thus, porosity is a substantial determinant of the bone fragility that underlies the risk of fractures and may be a target for fracture prevention.

Ageing and associated skeletal diseases pose a significant challenge for health care systems worldwide. Age-related fractures have a serious impact on personal, social and economic wellbeing. A significant proportion of physiological loading is carried by the cortical shell. Its role in the fracture resistance and strength of whole bones in the ageing skeleton is of utmost importance. Even though a large body of knowledge has been accumulated on this topic on the macroscale, the underlying micromechanical material behaviour and the scale transition of bone's mechanical properties are yet to be uncovered. Therefore, this review aims at providing an overview of the state-of-the-art of the post-yield and failure properties of cortical bone at the extracellular matrix and the tissue level.

Hypophosphatemic rickets and short stature are observed in nephropathic cystinosis, an orphan autosomal recessive lysosomal storage disease due to a deficiency of cystinosin (CTNS gene). Although bone impairment is not common, it nevertheless appears to be more and more discussed by experts, even though the exact underlying pathophysiology is unclear. Four hypotheses are currently discussed to explain such impairment: copper deficiency, bone consequences of severe hypophosphatemic rickets during infancy, cysteamine toxicity and abnormal thyroid metabolism. In murine models, the invalidation of the CTNS gene is associated neither with renal phosphate wasting nor with renal failure, but causes severe osteopenia and growth retardation, thus raising the hypothesis of a specific underlying bone defect in cystinosis. Moreover, the in vitro ability of mesenchymal stromal cells isolated from bone marrow to differentiate along the osteoblastic lineage is reduced in patients with cystinosis as compared with cells obtained from healthy controls, this cellular abnormality being reverted after cysteamine treatment. From our experience of three pediatric patients with cystinosis and severe bone deformations having undergone a thorough biochemical evaluation, as well as a bone biopsy, we conclude that even though copper deficiency, high-doses cysteamine regimens and abnormal thyroid metabolism may worsen the bone picture in cystinosis patients, the exact pathophysiology of such impairment remains to be defined. The role of chronic hypoparathyroidism due to chronic phosphate wasting could also be discussed. In the future, larger and prospective studies should focus on this topic because of the potential major impact on patients' quality of life.