Bone Abstracts

Objective: To investigate whether patients with secondary osteoporosis or other metabolic bone disease (SECOB) have a higher re-fracture or mortality risk.Method: Patients with a recent non-vertebral fracture who visited the Fracture Liaison Service (FLS) of a hospital were prospectively followed for 2 years. Pearson Chi-square, Fisher’s Exact test, independent samples T-test, and Cox regression models were used.Results: In to...

We have shown previously that TSG–6 acts as an autocrine regulator of osteoclast activity in vitro, capable of inhibiting RANKL-mediated osteoclastic bone resorption with a similar potency to OPG1,2. Thus, the TSG–6 protein has the potential to be developed as a novel treatment for osteoporosis, which is associated with excessive bone loss3.The aim of this study was to determine the therapeutic potential of the is...

Severe generalised recessive dystrophic epidermolysis bullosa (RDEB) is a rare disorder resulting from loss of function mutations in the type VII collagen gene (COL7A1). Although RDEB is characterised by severe skin blistering and erosions following minor mechanical trauma, it is a multisystem disorder with pubertal delay and low bone mass as part of the many complications.Children with RDEB have been described as having inadequate gains in bone...

Improvements on the overall cure rates for childhood acute lymphoblastic leukemia (ALL) have allowed the cure of over 85% of treated patients. At least 70% of survivors of childhood ALL cancers have substantial morbidities as a result of their treatment. Long term musculoskeletal complications in this population are currently receiving increasing attention because of their negative impact on the quality of life and ability to perform appropriate activities. Our objective was t...

Overall cure rates for childhood acute lymphoblastic leukemia (ALL) have improved allowing the cure of over 85% of patients. At least 70% of survivors of childhood ALL cancers have substantial morbidities as a result of their treatment. There is evidence that survivors of ALL have increased rates of long-term skeletal muscle dysfunction and weakness. Our objective was to determine the percentage of patients with muscle dysfunctions (muscle force, power, endurance, and % of fit...

Background: Dilated cardiomyopathy is the leading cardiac cause of death in children. Treatment options include heart transplantation. Reversible causes are rare but hypocalcaemia secondary to vitamin D deficiency is a recognised cause and presents during infancy in at risk populations. Risk factors include babies who were breast fed, dark skinned and from mothers with vitamin D deficiency. We present an unusual case of vitamin D replete, hypocalcaemic cardiomyopathy secondary...

Vascular calcification (VC) involves hydroxyapatite deposition in the arteries and cardiac muscle. VC is thought to share some outward similarities to skeletal mineralisation and has been associated with the transdifferentiation of vascular smooth muscle cells (VSMCs) to an osteoblast-like phenotype. We have previously shown that ATP, UTP and synthetic ATP-analogues (α, β-meATP, β, γ-meATP, Bz-ATP) (≥1 μM) act to potently inhibit both bone minera...

Background: Atypical Femur Fracture (AFF) has become widely reported as a complication of bisphosphonate therapy in adults since the first case report in 2005. A trend towards a similar pattern of fractures has been reported in children in Sheffield in 2012. A 13 year old boy was reported in 2014 with an ‘AFF’ of the tibia but that fracture did not meet the standard diagnostic criteria of AFF. Last year a 16-year-old girl treated with pamidronate for idiopathic juven...

Background: ‘Play Plans’ are now being routinely used at the NHS England Sheffield Severe, Complex and Atypical Osteogenesis Imperfecta Service (SCAOI) as an intervention designed to enhance early development. These plans are MDT formulated and encompass carefully targeted activities designed to meet the child’s needs at their exact stages of development across five domains; Cognition, Social & Emotional Development, Motor, Adaptive Skills and Communication ...

Background: A minority of children with Osteogenesis Imperfecta (OI) seen within the Sheffield National Severe, Complex and Atypical Service (SCAOI) were also identified as showing symptoms consistent with an Autism Spectrum Disorder (ASD) (Balasubramanian et al. 2018). Diagnosis of ASD in conjunction with OI may be delayed due to presenting problems being inappropriately attributed to OI resulting in specialised ASD input not being received by children.<p class="...