Bone Abstracts

The literature has suggested that bone mineral content is modulated by muscle mass and activity. We investigated the relationship between DXA assessed total body bone mineral content, total body lean mass, appendicular lean mass and obesity (a possible marker of inactivity) in a population of 73 boys between the age of 7 and 19 using a Norland XR-46 system.Regression analysis shows that in these growing boys there is a strong positive relationship betwee...

Background: WHO recommends diagnostic criteria of osteoporosis can be based on double energy absorption of x-ray bone densitometry (DXA), but the DXA method has some limitations such as relatively higher cost, relatively longer detection time, inconvenience in equipment transportation and so on. Quantitative Ultrasound (QUS) method has the potential to diagnose osteoporosis and it is convenient to carry around and low-cost. Asian osteoporosis self-assessment tool (OSTA) is an ...

Pain is common in patients with fibrous dysplasia (FD), however the mechanisms and presentation of pain is poorly understood. Retrospective studies have shown that pain in FD presents along a broad spectrum, responds variably to treatment, and does not correlate with FD disease burden. Pain may be generally conceptualized into two categories: nociceptive pain (associated with actual or potentially tissue damaging stimuli) and neuropathic pain (caused by dysfunction of the soma...

Biographical DetailsDr Yasemin Alanay, MD, PhD is a Pediatric Geneticist, Professor of Pediatrics at Acibadem University School of Medicine, Istanbul. She studied medicine at Hacettepe University, Ankara, completed her residency in Pediatrics in 2002. Completed Pediatric Genetics Fellowship at Hacettepe Ihsan Dogramaci Children’s Hospital. In 2006, she was mentored by Prof. D Rimoin ...

Objectives: Spinal muscular atrophy (SMA) results in varying degrees of hypotonic immobility. Prior reports demonstrate an increased risk of fracture and a trend toward low bone mineral density (BMD) in this population. We aim to further characterize bone health in paediatric SMA patients by reporting the prevalence of fractures and low BMD (z-score≤−2.0) by SMA subtype, BMD of the lateral distal femur (LDF; an important fracture location in non-ambulatory...

Osteoclasts resorb bone matrix through a specific adhesion structure called the sealing zone or actin ring, which is based on a belt of podosome. Much remains to be uncovered regarding the molecular mechanisms driving podosome organization into superstructures in particular the osteoclast podosome belt. Proteomic analyses in osteoclasts revealed the adaptor protein tensin 3 as a partner of Dock5, a Rac exchange factor necessary for podosome belt formation and bone resorption. ...

In Canada, and many other countries, a vitamin D supplement of 400 IU/day is recommended for breastfed infants and staple foods are fortified with vitamin D in accordance with public health policy for the primary prevention of rickets. Both vitamin D receptors and 1-alpha hydroxylase enzymes are expressed in human muscle, implicating vitamin D status and metabolism in growth and development. In a randomized dose response study of vitamin D supplementation (400, 800, 1200, or 1...

Objectives: Asfotase alfa (AA), an enzyme replacement therapy, is the only approved treatment for pediatric-onset hypophosphatasia (HPP). We evaluated the safety profile of AA from the clinical trial program spanning pediatric and adult patients.Methods: Safety data were pooled from 4 open-label, multicenter studies in children aged ≤3 years (study 002/003 [NCT00744042/NCT01205152]; n=11) and ≤5 years (study 010-10 [NCT01176266]; <em...

Objectives: Asfotase alfa (AA), an enzyme replacement therapy, is the only approved medical treatment for pediatric-onset hypophosphatasia (HPP), which is caused by deficient tissue-nonspecific alkaline phosphatase activity. We detail the long-term efficacy of AA observed from the pediatric clinical trial program.Methods: Efficacy data collected to study completion were pooled from 3 open-label, multicenter investigations of children who manifested HPP s...

Background: Glucocorticoid immune suppression in kidney transplanted children jeopardizes optimal bone health recovery. So far, there are no studies that evaluate the effect of transplant and corticoid in bone parameters separately.Objective: To determine the effect of early corticosteroid withdrawal in bone parameters.Methods: Randomized, controlled study; two groups: corticosteroid withdrawal (at the 6th day post-transplant, then...