Bone Abstracts

Objective: To evaluate safety and efficacy after 5 years of treatment with asfotase alfa in adolescents and adults with hypophosphatasia (HPP) in a Phase 2, open-label, randomized, dose-ranging study (NCT01163149).Methods: Treatment with subcutaneous asfotase alfa 0.3 or 0.5 mg/kg per d was compared with no treatment (control) for 6 months in patients aged 13–66 years. After 6 months, all patients (treatment and control groups) received active treat...

Background: Mutations in the PLS3 gene, encoding plastin 3, cause X-linked osteoporosis. Osteoporosis is characterized by low bone mineral density (BMD) and increased susceptibility to fractures. Here we describe a 7-year-old boy with osteoporosis due to a novel PLS3 deletion.Presenting problem: The patient, born to non-consanguineous parents, had a history of one low-energy long-bone fracture, three vertebral fractures (T5, T6 T8) and kyphosis. DXA scan...

Objectives: From late 2015 a new protocol for zoledronic acid was adopted in our centre. This led to many children changing from pamidronate (PAM) to zoledronic acid (ZA) treatment. In a minority of cases the children and/or their families felt strongly that they wanted to change back to PAM. We present the characteristics of that minority and how bone turnover markers (BTMs) and bone mineral densities (BMD) changed whilst on ZA.Method: From Nov 2016 to ...

Background: Primary tumoral calcinosis is an orphan disease. The data about the incidence of this disease, as well as clinical recommendations for treatment are not presented in the literature.Presenting problem: Two patients – 11.5 years old boy and 8 years old girl with primary tumoral calcinosis had multiple foci of the subcutaneous calcification, walking impossibility, wheel-chair condition, fatigue, high fever and equinus deformity of the left ...

It has suggested that BGP is inversely related to body fat. BGP appears to regulate energetic metabolism through the insulin receptor signaling pathway in the osteoblast, which affects bone resorption and BGP activity. Fat tissue is an endocrine organ that secretes different hormones and growth factors; several of them affect bone remodeling and insulin secretion. The present report comparatively studied total BGP levels, glucose homeostasis and body fat mass in spontaneous ob...

Bone remodeling is a crucial process to maintain a healthy bone structure in order to avoid diseases like osteoporosis or osteopetrosis. Osteoclasts contribute to this process by resorbing old and brittle bone allowing osteoblasts to renew the bone substance. During resorption osteoclasts rearrange their actin cytoskeleton by forming an F-actin ring generating a resorptive cavity on the bone surface. Recently, we reported that the F-actin binding protein SWAP-70 regulates oste...

Purpose: To evaluate whether calcium and vitamin D intake is associated with 25-hydroxyvitamin D (25(OH)D) and parathyroid hormone (PTH) serum concentrations or is associated with either the phalangeal dual energy X-ray absorptiometry (pDXA) or the quantitative bone ultrasound (QUS) at the phalanges and the calcaneus in independent elderly men from southwestern Spain.Methods: Serum PTH and 25(OH)D were measured in 199 healthy elderly men (mean age: 73.31...

Introduction: Myostatin (growth differentiation factor 8, GDF-8) has an important role in the regulation of muscle mass, and mice lacking the myostatin gene show a generalized increase in bone density and strength. Type 2 diabetes subjects have an increased risk of fragility fractures despite of higher bone mass. Taking into account the myostatin influence in bone strength a better understanding of myostatin actions in type 2 diabetes is of interest.Obje...

Background: Vitamin D deficiency is highly prevalent and can be associated with adverse health outcomes. Few studies have evaluated the effects of daily consumption of milk fortified with a high dose of vitamin D in a large cohort of healthy postmenopausal women.Objectives: To determine the effect of daily intake of milk enriched with vitamin D (with or without fructooligosaccharides (FOS)) on vitamin D status, bone mass and cardiovascular risk factors.<...

Many pharmacological treatments have been developed in order to prevent or treat postmenopausal osteoporosis. There is not any study in the literature comparing long-term treatments of osteoporosis. The aim of this work was to studythe effects of long-term prevention treatment with zoledronic acid (ZA) and strontium ranelate (SrR) on bone microstructure in ovariectomized rats.Sixty 6-month-old female Wistar rats were used in this study and divided into f...