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...

Background: Recently, the prevalence of osteoporosis and sarcopenia in the elderly has dramatically increased. However the relationship between theses disease is not clear.Object: We aimed to determine the independent relations of muscle mass to osteoporosis (femur neck) in relation to body weight, fat mass, and other confounders.Design: We analyzed body composition and BMD data of 570 males and 734 females who are older than 65 ye...

In children with spinal muscular atrophy (SMA): i) describe bone mineral density (BMD) measured by dual-energy x-ray absorptiometry (DXA); ii) examine bone health factors; iii) examine change in BMD over time and pamidronate effect.DXA results, medications, ambulation, 25-OH-Vit D levels, and fracture history were retrospectively reviewed in 14 children (five girls) with SMA. Sites scanned included whole body (WB), lumbar spine (LS), and lateral distal f...

Objective: To examine the bone mineral density (BMD), fracture occurrence and history in a cross-section of subjects with Pelizaeus Merzbacher Disease (PMD).To describe the effect of pamidronate therapy on BMD over time in treated PMD patients.Methods: We examined the medical history, medications, level of ambulation, fracture history and DXA results in 15 boys with PMD. Body sites scanned included whole body (WB) when possible, lu...

Objectives: Examine the effects of a ramped dosage schedule of pamidronate on BMD, fracture rate and location compared to a uniform 5-course regimen. The ramping regimen is intended to alter the tendency for post-treatment fractures to occur at the juncture of pamidronate bands where stress-riser related fractures have been described.Methods: Ten non-ambulatory children (seven females) with neuromuscular disabilities who received IV pamidronate with a ta...

The endocannabinoid system has important effects on bone mass and bone turnover. Mice with targeted inactivation of type 1 (CB1) and type 2 (CB2) cannabinoid receptors develop osteoporosis with increasing age raising the possibility that cannabinoid receptor agonists might protect against age-related bone loss. Since cannabis is the most widely used illegal drug and its main psychotropic component -Δ9-tetrahydrocannabinol (THC)- is an agonist at CB1 and CB2 receptors, we ...

Odanacatib (ODN), a selective and reversible cathepsin K inhibitor was shown to histomorphometrically reduce trabecular (Tb) and intracortical (Ic) bone remodeling while preserving endocortical (Ec) and stimulating periosteal (Ps) bone formation (BF) in monkeys. Here, we investigate the bone site specific mechanism of ODN on bone modeling (Mo) versus remodeling (Re)-based osteons. Rhesus monkeys (13–19 yrs, n=8–11/group) were ovariectomized and treated with ...

Objectives: To establish the correlations between the body composition, somatotypes and average bone mineral density (BMD) and bone mineral content (BMC) in young (17–18 y.o.) male living in Donbass region (Ukraine).Materials and methods: Anthropometric and skinfold measurements were carried out. Estimations of the calcaneal BMD (g/cm2) and BMC, (r), estimated on ALOKA-5.0 DXA machine among 156 male were done. Total body fat perc...

Objective: In girls with Rett syndromeDescribe bone mineral density (BMD) and contributing factors in a cross-section of subjects.Examine serial DXA measures.Examine effect of pamidronate on BMD over time.Methods: We reviewed the clinical course, medications, level of ambulation, 25-OH-Vit D levels, fracture history and DXA results in 13 girls with Rett syndrome. Eight subjects had more...

Objectives: i) Describe bone mineral density (BMD) of children with MPS IV (Morquio disease), a rare genetic disorder which produces skeletal deformity, small stature and results in physical limitations such as the ability to walk.ii) Examine fracture history and factors affecting bone health in Morquio.iii) Describe technical issues encountered in assessing BMD by DXA in Morquio.Methods: In this prospective ...