Bone Abstracts

Dominant osteogenesis imperfecta (OI) is a bone disease mainly caused by collagen type I mutations and characterized by bone fragility and growth delay. Nowadays no definitive cure is available. A zebrafish OI model (Chihuahua) carrying an heterozygous G574D substitution in the α1 chain of collagen type I was generated by ENU mutagenesis and is available in our laboratory. Control (WT) and mutant (Chi+/−) fish growth was followed up from day 1 post fertilization to ...

Background: Bone involvement in patients with β-thalassemia is well known, but only few studies have analyzed bone microarchitecture and the prevalence of intervertebral disc calcifications (IDCs) in these patients. The aim of our study was to evaluate the bone quality in a group of patients with β-thalassemia in terms of geometry and microarchitecture properties; moreover, we evaluated the presence of IDCs in these patients.Material and method...

Bone tissue homeostasis requires the coordinated activity of osteoblasts, the bone forming cells, of osteoclasts, the bone resorbing cells, and of osteocytes, generally referred as the bone mechano-sensors. In this contest, osteoblasts are the mesenchymal cells secreting the extracellular matrix components on which hydroxyapatite crystals are then deposited. The most abundant protein of this organic matrix is type I collagen, a heterotrimeric secretory protein, synthesized as ...

Introduction: The clinical diagnosis of mild forms of hypophosphatasia [HPP], a rare genetic bone disease, is often made in adulthood, on the basis of persistently low serum levels of alkaline phosphatase [ALP], often coupled with signs of poor bone/teeth mineralization.Case report: A 50-year-old male on treatment with vitamin D supplementation because of osteoporosis of lumbar spine (T-score –3.2) and femoral neck (T-score –2.4), was referred ...

Introduction: The effect of vitamin D± calcium supplementation in children has traditionally been assessed using growth parameters, biochemical markers and radiographs.Objectives: To evaluate the utility of Bone Health Index (BHI), obtained using automated hand radiogrammetry, in assessing treatment effect in children participating in a vitamin D supplementation trial.Methods: In this double-blind placebo-controlled trial, Afg...

Besides its well known effect on longitudinal bone growth, GH plays a role in the maintenance of adult bone mass. As aging progresses GH levels decline, bone mass decreases and mesenchymal precursors show a reduced osteogenic differentiation capacity resulting in an increase in bone marrow adipocytes. We investigated the effect of GH on MSC differentiation and the possible involvement of microRNA in this process. Human MSC derived from trabecular specimens, waste material of o...

Background: Osteoporosis treatment involves several therapeutic tools, including long-term drug therapy. Subjects with chronic disorders are more likely to be non-adherent and/or non-persistent to treatment than those with other diseases. Adherence is the extent to which patients take medication as prescribed by their physicians, whereas persistence is the time from treatment initiation to discontinuation. Lack of persistence is common among subjects using oral anti-osteoporot...

Bone disease is the most frequent complication in multiple myeloma (MM) resulting in pain, bone fractures, spinal cord compression and hypercalcemia. Within the bone marrow microenvironment (BMM), MM cells interact with bone cells to enhance bone resorption activity and compromise new bone formation mechanism; in turn, BMM provides a survival and drug resistance framework by interaction of MM cells with bone marrow components. Exosomes are important mediators of crosstalk betw...

Osteoclast-poor RANKL-dependent autosomal recessive osteopetrosis (ARO) is a rare bone disease characterized by an increase in bone density due to the failure of bone resorption by impaired osteoclast formation. Haematopoietic stem cell transplantation is not an effective therapy for this ARO form, since in bone RANKL is produced mainly by cells of mesenchymal origin. Whether also these cells, besides the osteoclast, are in some way affected by RANKL deficiency is not known. T...

Bone is a complex tissue constituted by a mineral phase, hydroxyapatite, and an organic phase, mainly represented by collagen type I. Specialized cells are responsible for bone formation and remodeling. Osteoblasts represent the bone forming cells, osteocyte are the orchestrator of bone remodeling through regulation of the other bone cells activity, by functioning as endocrine cells and by acting as mechanosensor, and osteoclasts, the bone resorbing cells. Mesenchymal osteopro...