Bone Abstracts

Background: Rett Syndrome (RS) is a disabling condition due to mutations in MECP2. Girls affected with RS are at risk of developing osteoporosis and fractures at a young age because of their lack of mobility and through a direct effect of MECP2 on bone mineralization. In these girls, bone fragility inflicts pain and may seriously impair the quality of life.Objective: To retrospectively assess the effect of IV bisphosphonates on fracture, bone mineral den...

Objectives: Endocrine late effects, including osteopathologies, following diagnosis and treatment of childhood malignancies are studied in adult survivors with alarming results. However, in pediatric patients with brain tumors the risk to develop endocrine late effects is high even during childhood and adolescence.Aim and design: To investigate osteopathologies and endocrine function in juvenile survivors of pediatric brain tumors we conducted a cross-se...

Objectives: FGF23 is expressed in clusters of osteocytes at the trabecular periphery suggesting that FGF23-expressing osteocytes are confined to specific basic multicellular units (BMUs) at the trabecular surface. Higher numbers of FGF23-expressing osteocytes are found in chronic kidney disease (CKD) patients with preserved skeletal mineralization indices. We thus combined immunohistochemistry and quantitative backscattered electron imaging (qBEI) to explore the hypothesis tha...

Background: Sheffield Children’s NHS Foundation Trust offers a highly specialised metabolic bone service for children and young people living with Osteogenesis Imperfecta (OI). OI is a chronic health condition causing bone fragility, bone pain, bone deformity, frequent fractures and variable physical limitations with wide reaching consequences on both children and families; the potential to experience elevated psychological distress is common.Presen...

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="...

No treatment for osteoporosis abolishes the risk of fracture. Even under the ‘ideal’ conditions of adherence and monitoring, in the controlled pivotal trials, a significant proportion of individuals receiving the active drug still suffer new fractures. In everyday practice the situation is even more challenging. Adherence to medication is poor, patients are often older than in trials, or suffer from a number of comorbidities that could have excluded them from the ori...

There has been much published about the incidence, detection and prosecution of publication fraud, less about fraud and misconduct in clinical research. We should be equally concerned about research fraud. It is clear that all misconduct is not fraud, and sloppiness would not be so labelled, but the protection of patients must be uppermost of all concerns.Whichever definition is used, the fact that patients have been exploited remains. This exploitation ...

Introduction: Bone mineral density decreases before menopause and is held to be due to trabecular, not cortical, bone loss. Yet neither a negative bone balance, nor accelerated remodelling occurs before 45 years of age. We hypothesized that bone loss will first appear after 45 years and will be cortical (as 80% of bone is cortical).Methods/design: Images of distal tibia acquired using high-resolution peripheral quantitative computed tomography (Scanco Me...

Metastases to the bone are the incurable final outcomes of cancer, reducing both length and quality of life in an aggressive way. Despite the discoveries of many factors involved, no cure has been found. Metastatic outgrow starts in interaction with the bone micro-environment. The first attachment in bone is with the osteoblasts lining the endosteal surface. Our aim is to study the role of the osteoblasts in metastatic tumor spread and growth.We used an ...

Osteoclast (OC)-mediated bone destruction is a key feature of rheumatoid arthritis (RA). In RA synovial tissue a reduced density of catecholaminergic nerve fibres has been observed. Studies on sweat gland innervation proved that catecholaminergic fibres have the ability to undergo a phenotypic transition to cholinergic nerves. The sympathetic neurotransmitters norepinephrine (NE), acetylcholine (ACh), and vasoactive intestinal peptide (VIP) affect osteoclastogenesis oppositely...