Bone Abstracts

Background and objectives: X-linked hypophosphatemic rickets (XLH) is the most common form of inheritable rickets. The treatment of rickets is monitored via laboratory tests such as alkaline phosphatase (ALP), clinical features, and plain X-rays. The objectives of this study were to describe the MRI features in XLH and to look for correlations between those features and XLH activity.Study design: Twenty-seven patients (younger than 18 years with XLH due ...

Melatonin is a neuro-hormone released primarily from the pineal gland, which has been shown to have bone anabolic effect, although it is still unclear whether its skeletal action is directly mediated by receptors expressed on bone cells or is indirect. In this study, we examined melatonin’s effects on bone cellular activities in vitro and tested whether it modifies angiogenesis and blood flow to bone, which are both essential for bone formation.<p class="abst...

Objectives: Hypophosphatasia (HPP) is a rare hereditary disease, leading to deficits in bone and tooth mineralization, muscular as well as neurological abnormalities due to decreased enzymatic activity of the tissue-nonspecific alkaline phosphatase (TNAP, encoded by the alpl gene). In this project, the zebrafish (Danio rerio) will be established as a new and valuable animal model for HPP research. Consequently, endogenous TNAP expression should be analyzed in different zebrafi...

Background: While the relationship between visceral (VFAT) and subcutaneous (SFAT) fat mass with cardiometabolic risk has been demonstrated in adults, fat mass evolution during teenagehood remains poorly explored and usually assessed with irradiative (CT) or expensive (MRI) techniques. Our aim was to evaluate a novel technique derived from DXA to assess VFAT and SFAT in healthy teenagers.Subjects and methods: Healthy teenagers from the VITADOS study unde...

In light of prolonged life expectancy, the need for biomaterials that govern bone regeneration increases. Improved bone regeneration and osseointegration can be achieved by funtionalizing implant materials. The extracellular matrix (ECM) affects differentiation of bone cells and is critical for bone regeneration. Here we assessed the role of the natural occurring bone ECM glycosaminoglycans (GAGs) hyaluronan (HA) and chondroitin sulfate (CS), and their sulfated derivatives, on...

Introduction: The first clinical applications of FE-strength estimates to classify osteoporotic fractures showed inhomogeneous results. We developed a FE model that correlated well with femur strength in-vitro (R2=0.9, 14 femurs). This work aims to verify if our model can classify osteoporotic fractures in three case-control studies: a retrospective and a prospective study on proximal femur fracture, and a retrospective study on prevalent ...

Autosomal dominant polycystic kidney disease (ADPKD) is the most common monogenic cause of renal failure. Data from adult ADPKD population show increased fibroblast growth factor 23 (FGF23) levels while circulating Klotho levels decrease, with a low TmP/GFR even in patients with normal renal function. Moreover, in ADPKD animal models, cyst lining renal cells were demonstrated to produce FGF23, although the animals displayed FGF23 resistance. No data are available in a paediatr...

Introduction: Lower bone mineral content (BMC) has been reported in HIV-infected children, as well as those on ritonavir-boosted lopinavir (LPV/r)-based antiretroviral therapy (ART). Older studies of children with HIV report increased bone resorption, but data with current antiretroviral regimens are limited.Methods: This analysis presents data from the CHANGES Bone Study (Johannesburg, South Africa). Whole body (WB) BMC was assessed by dual-energy X-ray...

Objectives: Maternal vitamin D status in pregnancy is associated with neonatal bone mass, and altered DNA methylation. Mice exposed to early life vitamin D deficiency have lower bone mass and reduced bone accrual in response to mechanical loading. Using tibias from these mice we assessed DNA methylation of promoters of genetic loci important for bone growth and development.Methods: C57/BL6 mice received a vitamin D replete or deplete diet for 6 weeks per...

Objectives: Achondroplasia, the most common form of short limb dwarfism, is a rare genetic disorder caused by a gain-of-function mutation of the FGFR3 receptor (FGFR3-G380R) and there are currently no effective treatments available. We have developed TA-46, a recombinant human soluble FGFR3(sFGFR3) form of the FGFR3 cell surface receptor containing an extra-cellular ligand-binding domain, including three IgG domains, which confer binding specificity. TA-46 is designed to block...