Bone Abstracts

Background: Gorham Disease is a rare condition characterised by massive osteolysis. The pathophysiology is related to angio/lymphatic proliferation within bone. No genetic transmission has been identified and onset occurs in patients of all ages. Surgical fixation of the spine may be unsuccessful due to progressive osteolysis of bone surrounding the metalwork, or of the bone graft.Presenting problem: An 11 year-old boy presented with a 2 year history of ...

Aims: To review changes in the advice regarding vitamin D deficiency in the UK and compare these with other national guidance.Methods: All Vitamin D guidance in the Royal College of Paediatrics and Child Health 2003 guide (Medicines for Children) and in the eight editions since the British National Formulary for Children (BNFc) was first published in 2005 was reviewed.Results: Dosage and indications of prevalence are shown in the T...

Purpose: To evaluate whether calcium and vitamin D intake is associated with 25-hydroxyvitamin D (25(OH)D) and parathyroid hormone (PTH) serum concentrations or is associated with either the phalangeal dual energy X-ray absorptiometry (pDXA) or the quantitative bone ultrasound (QUS) at the phalanges and the calcaneus in independent elderly men from southwestern Spain.Methods: Serum PTH and 25(OH)D were measured in 199 healthy elderly men (mean age: 73.31...

Peripheral computed tomography (pQCT) can estimate volumetric bone mineral density (vBMD) and distinguish trabecular from cortical bone. Few comprehensive studies have examined correlates of vBMD in Spanish premenopausal women.The aim was to study the association between bone microarchitecture, muscle mass and strength in premenopausal Spanish women and to evaluate the role of calcium intake. vBMD was assessed in 164 premenopausal women at the distal rad...

Introduction: The matrilin-3 polymorphism T298M has been described to be associated with hand osteoarthritis in the carpometacarpal joint 1 (CMC1) and with spinal disc degeneration. In vitro studies revealed that expression levels, processing and secretion of matrilin-3 T298M were similar to the wt protein. Structural analysis indicated an impact on the formation of collagen II/IX/XI heterofibrils, whereas the binding to collagen was not affected. We have now generate...

Objectives: Vitamin D has an important effect on bone but there are no trials that directly address the boosting of serum levels of 25-hydroxyvitamin D (25OHD) in bone microarchitecture in Juvenile-onset Systemic Lupus patients (JoSLE). The aim of this study was to evaluate the effect of vitamin D supplementation on bone microarchitecture parameters using HR-pQCT in JoSLE patients.Methods: This study was a randomized double-blind placebo-controlled 24-we...

Interleukin-34 (IL-34) is a newly discovered cytokine which regulates, like macrophage-colony stimulating factor (M-CSF), the differentiation/activation of the myeloid lineage. IL-34 and M-CSF are homodimers known to bind to the M-CSF receptor (M-CSFR) in a competitive manner. In this system, IL-34 can substitute for the M-CSF to induce osteoclastic differentiation. The aim of the present work was to study the functional relationships of these cytokines on cells expressing the...

Introduction: Collagen and glycosaminoglycans (GAGs) such as hyaluronan (HA) and chondroitin sulfate (CS) are basic elements of bone structure and collagen-GAG composites are currently developed for a wide range of applications. Here, we report on the molecular and cellular effects of GAGs and their sulfated derivatives on osteocytes, which are fundamental orchestrators of bone remodeling.Materials and methods: The effects of native and sulfate-modified ...

Osteosarcoma, the main malignant primary bone tumor, affects a ‘young’ population composed of children and young adults. Current treatment consists of tumor resection associated with chemotherapy. Unfortunately in many cases, a lack of response to anti-tumor drugs is observed, leading to development of metastases and to the patient’s death. Because TGF-β promotes metastases from many solid epithelial tumors, we investigated the effect of inhibitory Smad7 ov...

Fanconi anemia (FA) is a rare genetic disease associated with a progressive decline in hematopoietic stem cells leading to bone marrow failure. FA is also characterized by various developmental defects including short stature and skeletal malformations of the upper and lower limbs. Indeed, more than half of children affected with FA have radial-ray abnormalities with a tendency to early osteoporosis and osteopenia. However, the underlying mechanisms leading to bone defects in ...