Bone Abstracts

We studied changes in intracellular Ca2+ concentration in bone cell cultures obtained from human subjects with osteoporosis and osteoarthritis, to evaluate differences between these patients and healthy subjects. We enrolled 36 patients: 12 undergoing primary total hip arthroplasty for osteoporotic femoral fractures (group A, mean age range 57–80), 12 for hip osteoarthritis (group B, mean age range 57–80), and 12 healthy subjects who suffered a high-energy...

Introduction: Liver transplant candidates have bone and mineral metabolism disorders that may influence the development of fractures after liver transplantation (LT).Objective: To analyze the levels of 25-hydroxyvitamin D (25-OH-D) in patient candidates for LT and the factors associated with vitamin D deficiency.Methods: Between January 2010 and May 2012, 116 liver transplant candidates (85 male and 31 female patients) were include...

Background: Surgical and conservative treatments are both crucial method to deal with osteoporotic fracture, anti-osteoporotic therapy is the most important among conservative treatments and diphosphonate medicine is the first-line choice. The pharmacological action of diphosphonate medicine is to inhibit the function of osteoclast, so there is no consensus if it will inhibit porosis and union of fracture.Purpose: To find out the influence of diphosphona...

Objectives: In XLH, FGF23-mediated hypophosphatemia leads to defective bone mineralization and rickets. Investigational product KRN23 binds FGF23 and inhibits its activity. The objective of this Phase 2 study was to evaluate the safety and efficacy of KRN23 in 52 children with XLH (ages 5–12 years, ≤Tanner 2).Methods: Patients were randomized to receive KRN23 biweekly (Q2W) or monthly (Q4W) by SC injection. KRN23 dose was titrated (maximum 2 m...

Objectives: This study analysed prospective associations between two distinct developmental trajectories of objectively-measured physical activity and late adolescent bone parameters (age 17 yr) by exploring the mediating effects of lean soft tissue (LST), a surrogate of muscle mass.Methods: In approximately 349 participants (191 girls) of the Iowa Bone Development Study, physical activity was measured by accelerometry starting at age 5 and continuing at...

Biographical DetailsLaurence Legeai-Mallet is currently Director of Research at Imagine Institute-Paris Descartes University. She received her PhD in genetic from University of Paris V, she is a member of International skeletal dysplasia Society, European Skeletal Dysplasia Network and the French reference center of bone dysplasias. She has been involved in the field of skeletal disease s...

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 ...

Objective: We compared the efficacy and safety of burosumab, a monoclonal antibody against FGF23, to conventional therapy [oral phosphate and active vitamin D (Pi/D)] in children with X-linked hypophosphatemia (XLH).Methods: In this Phase 3 trial (NCT02915705), 61 children with XLH (1-12 years-old) were randomized 1:1 after a 7-day Pi/D washout to receive burosumab starting at 0.8 mg/kg SC Q2W or reinitiate Pi/D optimally titrated by investigators. Eligi...

Introduction: Hypophosphatasia (HPP) characterized by reduced mineralization occurs from mutations in the tissue non-specific alkaline phosphatase (ALPL) gene. Individuals harbouring bi-allelic mutations are generally reported to be severely affected. We report the findings of in vitro functional studies following site-directed mutagenesis in bi-allelic mutations causing extreme clinical phenotypes; severe perinatal and asymptomatic HPP.Objectiv...

Introduction: Intracellular fat within muscle and visceral tissue has been suggested to adversely influence bone development.Design: We aimed to compare associations between intramuscular and subcutaneous fat and cortical bone outcomes in young adults, in cross-sectional analyses based on the Avon Longitudinal Study of Parents and Children.Method: Data were collected from a research clinic conducted at 17 years of age. Intramuscula...