Bone Abstracts

see PP291....

Objective: Children with hypophosphatasia (HPP) treated with asfotase alfa in a Phase 2 study (NCT00952484) and its open-label extension (NCT01203826) experienced significant improvements in skeletal mineralization and physical function that were sustained through 5 years of treatment (1). Herein, we report data from these studies with a maximum of 7 years of treatment.Methods: Children with HPP aged 6–12 years at baseline received asfotase alfa (3 ...

see PP245....

Osteocytes differentiate from osteoblasts, are embedded in mineralised matrix and are critical regulators of bone remodelling. In vitro osteocyte models are limited to cell lines in monolayer, which do not represent their 3D environment in vivo. We have shown that osteoblasts in 3D gels differentiate along the osteocytic pathway. Since IGF1 regulates osteoblasts, and is involved in osteocyte response to mechanical loading, we hypothesised that IGF1 modulates ...

Purpose: Systemic sclerosis (SSc) is a rare inflammatory rheumatic disease that has been associated with an increased risk of low bone mineral density (BMD). However, data on risk factors associated with bone loss in SSc are scarce. The objective of this study was to investigate the prevalence of and the risk factors for low BMD in patients with SSc.Methods: Cross-sectional data of 61 patients with SSc were collected. BMD in the lumbar spine, total hip a...

Background: It is known that one of the features of rheumatoid arthritis (RA) is a generalized bone loss, therefore important tasks for the physician are timely evaluating of the risk of fractures and approving osteoporosis treatment in patients with RA.Objectives: Through the Programme of the Russian Association of Rheumatology for the diagnosis, prevention and treatment osteoporosis (OP) in patients with RA we evaluated the 10 year probability of major...

It is well-established that in vitro differentiation of embryonic stem cells (ESCs) and induced pluripotent stem cells (iPSCs) can recapitulate embryonic development through germ layer induction, enrichment and expansion of specific lineages. We have used PSC technology and developed a novel, mESC differentiation system for investigating the mechanisms of chondrocyte and osteoblast lineage commitment and differentiation. This step-wise, serum-free protocol uses specif...

The main cause of the bone destruction in rheumatoid arthritis (RA) is a destructive act of aggressively growing pannus, growth and angiogenesis of which are caused by the proliferation of fibroblasts in the synovium (S) due to the activation of fibroblast growth factor (FGF).Objectives: To reveal relationships of FGF with ultrasonic and arthroscopic and proliferative-destructive histological figures in RA.Methods: Complete compreh...

Objectives: There are conflicting data on the skeletal health of patients with perinatal HIV. We aimed to evaluate the bone profile of a paediatric population followed in a reference centre for perinatal HIV.Methods: The following data were recorded: dietary calcium intake, extra-curricular exercise, fracture history, medications and comorbidities. All patients were assessed for growth and skeletal deformities. They underwent laboratory tests:CD4 count, ...

Geroderma osteodysplasticum (GO) is a rare autosomal recessive connective tissue disorder characterised by progeria like facies, wrinkled lax skin, joint hypermobility, congenital dislocation of hips and propensity to fragility fractures. In the past 25 years, five patients (three females and two males) diagnosed with GO were referred to our Paediatric metabolic bone service for assessment and management of secondary bone problems. All five children were born to consanguineous...