Bone Abstracts

Objectives: X-linked hypophosphataemia (XLH) is a rare inherited form of osteomalacia characterised by low blood phosphate levels which lead to inadequate mineralisation of bone and rickets. Burosumab is an anti-FGF23 fully human monoclonal-antibody, and the first treatment to target the underlying pathophysiology of XLH. Real-world evidence is important in validating the findings of clinical studies. We report relevant real-world biochemical data on children under five years ...

Objectives: X-linked hypophosphataemia (XLH) is a rare inherited form of osteomalacia characterised by low blood phosphate levels which lead to inadequate mineralisation of bone resulting in rickets, skeletal abnormalities, physical impairment, weakness, and pain. Burosumab is an anti-FGF23 fully human monoclonal-antibody, and the first treatment to target the underlying pathophysiology of XLH. Real-world evidence is important in validating the findings of clinical studies. We...

Biographical DetailsDr A Linglart is a Paediatric Endocrinologist working at the Hôpital St Vincent de Paul in Parism, France. She has a special interest in rare diseases....

The osteocyte most likely plays a role in bone remodeling by instructing osteoclasts to remove bone at specific sites. This entire process includes recruitment, proliferation and differentiation of osteoclast precursors. And osteocytes are responsible for detecting and responding to mechanical strain and may send signal to other cells. Therefore, to determine the role for osteocytes and mechanical strain in bone remodeling, we examined the effect of steady or pulsatile shear s...

Introduction: Hydrogels has demonstrated efficacy as carriers for growth factors. Our aim was to investigate the effect of curing-time of modified hyaluronan on bone formation.Methods: Hydrogels with rhBMP-2 were cross-linked for 14 and 3 days, 5 h or 1 min before injection. Preformed gels were injected s.c. in 5 rats, the rats were killed after 5 weeks. Explanted samples were radiographed and scanned by pQCT.Results: Bone formatio...

Objective: Classical homocystinuria due to cystathionine beta-synthase (CBS) deficiency, is a rare autosomal recessively inherited disease characterized by the multiple involvement on different organs. While the most striking cause of morbidity and mortality is thromboembolism, patients develop a marked osteoporosis at early age along with many other skeletal abnormalities. As CBS normally converts homocysteine to cystathionine, the result of CBS deficiency is an accumulation ...

Osteoclasts are multinucleated bone-resorbing cells and can be cultured from different monocytic precursors. It is unknown whether osteoclasts derived from different precursors are phenotypically different. One of the aspects not known is the life span of the different osteoclasts and the effect of IL-1β hereupon. Here, we studied this using time-lapse microscopy. Bone marrow cells were isolated from 6-week-old male mice. Early blasts (CD31hiLy-6C-),...

Calcium supplements are associated with increased cardiovascular risk, but the mechanism by which this occurs is presently uncertain. In a secondary analysis of a trial examining the acute effects of calcium supplements, we found that blood pressure declined over 8 h in the control group, consistent with its diurnal rhythm, and that this decline was smaller in the calcium group [1]. To investigate these effects further, we carried out a randomised controlled cross-over trial o...

Vertebral fractures (VF) defined by morphometric analysis of spine radiographs are the most common complication of osteoporosis. Those that come to medical attention, with symptoms such as back pain and kyphosis are termed clinical vertebral fractures (CVF) and account for significant morbidity and mortality. Although much progress was made in identifying loci for bone mineral density, the genetic determinants of CVF remain unclear. Here we present the initial results from a g...

Aim: To determine the safety, tolerability, pharmacokinetics and pharmacodynamics of the cathepsin K inhibitor MIV-711.Methods: A double-blind, placebo-controlled, randomized study in 27 healthy subjects of both genders. Single ascending doses 20–600 mg were investigated for adverse events, clinical chemistry, vital signs, ECG parameters, pharmacokinetics, and serum levels of CTX-I.Results: MIV-711 was well tolerated with no a...