Bone Abstracts

Background: Hyperphosphatemic familial tumoral calcinosis (HFTC) is characterized by hyperphosphatemia and the formation of tumor-like extra-osseous calcifications. Tumors often necessitate surgical management although medical treatment may limit the need for surgical intervention. We present two cases of HFTC successfully managed with combination acetazolamide (ACTZ) and sevelamer carbonate.Case Report #1: A 5-year-old African boy with a loss-of-functio...

Duchenne muscular dystrophy (DMD) causes progressive muscle weakness and loss of ambulation. While glucocorticoid (GC) therapy improves motor function, many boys sustain fractures due to osteoporosis. Recently, i.v. bisphosphonate (i.v.BP) therapy has shown promise in the treatment of DMD-related osteoporosis. At the same time, bone histomorphometry revealed lowered bone volume and significant reductions in bone formation pre-i.v.BP treatment, and a further drop after 2 years&...

Objectives: Previously we have shown in healthy pre-pubertal boys that short periods of whole body vibration (WBV) increased the bone formation marker PINP by 25.1% and resorption marker CTX by 10.9%. The aim of this study was to see if otherwise healthy boys with a history of fracture would respond to WBV in the same way.Methods: In addition to 11 pre-pubertal boys measured previously in the same way, 20 pre-pubertal boys aged 7-13 years, who were at le...

Aim: To determine the prevalence of vitamin D deficiency among adolescent Muslim girls attending a school in the UK, which adheres to a conservative dress code.Methods: Fifty-six (31%) out of 180 girls attending a Muslim High School for Girls (median age 13.2years, (IQR 12.5–13.8 years)) took part in this cross-sectional study. Seventy-nine percent (n=45) were of South Asian origin, 3.5% were Black African origin (n=2), 1.8% was Mi...

Objectives: Osteoporosis is a frequent cause of morbidity in boys with glucocorticoid (GC)-treated Duchenne Muscular Dystrophy (DMD). We sought to determine the frequency and characteristics of the two most debilitating types of fractures, vertebral and long bone (VF and LBF), in pediatric DMD.Methods: This was a prospective, bi-centre, single-visit observational study in boys 4 to 17 years of age with genetically-confirmed DMD. The bone health assessmen...

In rural Gambian prepubertal children with low calcium intakes (mean 300 mg/day) we reported positive effects, sustained for at least a year (y), on bone mineral content (BMC) after 12 months supplementation with calcium carbonate to international levels1,2. The group was followed up regularly until the end of height growth, and supplementation did not affect height growth in the girls3. Our aim was to determine whether the supplementation altered the tim...

Assessment of metaphyseal bone growth in children by peripheral quantitative computed tomography (pQCT) is limited by use of a thin (2.3 mm) 2D slice to represent the ~20 mm metaphyseal region within which there is substantial local variation in bone properties (Marjanovic et al., 2009). In addition, scan positioning is performed by manual visual growth plate identification from which a region located proximal to the growth plate at 4% of tibia leng...

Background: Severe vitamin D deficiency (VDD), is a common disorder, which has complications including rickets, hypocalcaemia, hypotonia, delayed development and cardiomyopathy. Although nutritional rickets associated craniosynostosis has been reported, there is little awareness of this or knowledge about its clinical course or severity. We present five cases of late onset craniosynostosis in association with nutritional rickets.Clinical presentation: Th...

After trauma or carious lesion dental pulp healing is difficult to predict. In addition systemic diseases like diabetes mellitus can impair the regenerative capacity. New regenerative strategies target prolyl hydroxylase (PHD) by pharmacological inhibitors to stimulate hard and soft tissue healing. PHD inhibitors such as L-mimosine (L-MIM) induce vascular endothelial growth factor (VEGF) production by promoting angiogenesis. However, it is unclear if L-MIM is a feasible tool t...

Osteoblasts have a high rate of energy consumption during bone formation and bone protein synthesis. Therefore, it is likely that decreased energy production that accompanies aging, could contribute to reduced osteoblast activity, a critical feature of senile osteoporosis, and that this reduction might be counteracted by favoring energy availability. Cells of the osteoblastic lineage generate 40–80% of the requested energy through fatty acid degradation, thus the modulati...