Bone Abstracts

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 ...

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Patients with osteoporosis require treatment with therapies that reduce the risk of fracture which at the spine is significantly influenced by bone microarchitecture. Quantitative computed tomography (QCT) and dual X-ray absorptiometry (DXA) allow measurement of bone mineral density (BMD), a known indicator of fracture risk. Trabecular bone score (TBS) is a novel gray-level measurement derived from spine DXA image texture that is related to microarchitecture and associates wit...

Objectives: Bisphosphonates are frequently used in children with skeletal disorders, however optimal dosing and regimens are unknown. Early treatment focused on pamidronate (PAM), a second-generation formulation, however use of zoledronate (ZOL), a more potent third-generation bisphosphonate, has recently increased due to shorter and less frequent infusions. The objective of this study is to compare short-term safety of ZOL and PAM in a pediatric population.<p class="abste...

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-),...

Objective: Total body less head (TBLH) and spine are the recommended DXA sites for bone health assessment in children and adolescents. However, inter-machine differences will affect use and interpretation of results in clinical care and research applications. We examined BMC and areal-BMD (aBMD) at 4 skeletal sites among healthy children to identify the magnitude of inter-machine differences in Z-scores.Methods: BMDCS evaluated healthy participa...

Background and Objectives: The relations among cortical volumetric bone mineral density (CortBMD) and comprehensive measures of mineral metabolism have not been addressed in chronic kidney disease (CKD). The aim was to identify determinants of CortBMD in childhood CKD. A secondary objective was to assess if CortBMD was associated with subsequent fracture.Design/participants: This prospective cohort study in children, adolescents and young adults (ages 5&...

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...

Background: X-linked hypophosphatemic rickets (XLHR) represents the most common form of hypophosphatemia.Aim: The aim of this prospective study was to describe and analyze the incidence of cranial and cervico-occipital junction (COJ) anomalies in a series of children with XLHR.Patients and methods: Seventeen children (13 girls, 4 boys, mean age 7.3 years) followed for XLHR at the French national reference center for rare diseases o...