Bone Abstracts

Objectives: Haemophilia A (FVIII deficiency) is an X-linked disorder of haemostasis with bleeding tendency, mainly in joints and muscles. Recurrent haemarthroses, subsequent immobilization and avoidance of contact sports, may affect these patients’ skeletal health.Methods: Evaluation of bone health was performed in 51 children with Haemophilia A (severe: 41, all on prophylaxis treatment), mean age: 11.7±3.6 years. Dual-energy X-Ray absorptiomet...

Although EGF has been known to inhibit osteoblast differentiation, its molecular mechanism has not been clearly elucidated. Smurf1 acts as a negative regulator of BMP signaling by inducing ubiquitination and proteasomal degradation of BMP type I receptor and R-Smads. In this study, we investigated the effect of EGF on the expression of Smurf1 and the role of Smurf1 in EGF-induced inhibition of BMP2-induced osteogenesis. EGF increased Smurf1 expression which was blocked by trea...

Pseudovitamin D-deficiency rickets (PDDR) is a rare autosomal recessive disorder resulting from a defect in renal 25-hydroxyvitamin D 1α-hydroxylase, which is encoded by the CYP27B1 gene. To our best knowledge, 48 mutations of the CYP27B1 gene have been identified so far. In the present study, we investigated CYP27B1 mutations in seven individuals from six separate families and identified nine different mutations: two novel missense mutations (G194R, R259L), three novel a...

In clinical research protocols, HR-pQCT images (XtremCT Scanco, voxel size: 82 (μm3) are carried out to evaluate trabecular and cortical bone changes induced by osteoporosis and treatments. Micro-computed tomography (μ-CT) has become a standard tool for examination of trabecular and cortical bone in 3D.The purpose of this study is to evaluate the accuracy of cortical and trabecular measurements derived from HR-pQCT images with morpho...

Spinal cord injury (SCI) has been associated with a marked increase in bone loss. This study analysed the effect of Wnt signalling antagonists (sclerostin and DKK-1) and their relationship with bone turnover markers and BMD evolution in patients with a recent SCI.Methods: Patients with a recent complete motor SCI (AIS A or B); (<6 months) were prospectively included. Bone turnover markers (bone formation: P1NP, bone AP; bone resorption: sCTx), Wnt an...

Objective: The incidence of eating disorders has increased in 15–19 years old1. There is growing concern as to its impact on bone health during adolescence where peak bone mass acquisition is of paramount importance. This paper describes bone size and size adjusted bone mineral content in an adolescent/young adult eating disorder population.Methods: A total of 68 patients (63 F/5 M, 90% anorexia nervosa and 10% atypical eating disorder), ...

Children bone knowledge is relatively sparse. This is especially true for infant and for bone microarchitecture data. We have investigated, in this study, the age-related modifications of spine microarchitectural texture, as assessed by TBS, on male and female infants during their two first years of life.The study group was composed of 143 and 109 healthy female and male infants aged between 0 and 2 years. Height and weight Z-scores were signifi...

Low protein diet (LPD) impairs body growth, decreases serum IGF1 and increases serum FGF21, two hepatokines influencing bone growth. Low calcium-phosphate diet (LCaPiD) increases serum calcitriol. We hypothesized that LPD effects on bone growth may differ according to calcium and phosphate intakes through the hormonal modulation of calcitriol, IGF1 and FGF21. One-month old rats were fed isocaloric diets containing 10 or 5% casein (10Prot, 5Prot), with 1.0% calcium and 0.8% pho...

Background: Patients with differentiated thyroid cancer (DTC) are treated with L-thyroxine (L-T4) in relatively hight doses to suppress endogenous TSH levels. Very long-term effect of thyroid hormones supplementation on bone are controversial.Objetive: To study the possible negative effects on bone mineral density (BMD) and bone markers in DTC patients followed in our center.Methods: A 46 po...

BMD alone is not sufficient to predict the fracture risk for an individual. Others parameters, such as microarchitecture play a key role in bone fragility. Several cross-sectional studies have shown the ability of TBS to discriminate fractured from healthy subjects in European populations. The aim of our study is to assess the ability of TBS, evaluated at the lumbar spine, to discriminate subjects with and without fracture in a large white US population....