Bone Abstracts

A clinical and densitometric study was performed in the group of 62 adults with cerebral palsy (CP) under institutionalized care in the district of Przemyśl (Southern Poland), 12 of whom had the history of 15 radiologically confirmed non-phalangeal limb fractures. The study assessed correlation between the prevalence of fractures and age, body mass, locomotor abilities determined by Gross Motor Function Classification System (GMFCS), presence of epilepsy, antiepileptic dr...

STRUCTURE was a phase 3, open-label study evaluating the effect of romosozumab or TPTD for 12 months in women with postmenopausal osteoporosis transitioning from bisphosphonate therapy (NCT01796301). This study enrolled women with postmenopausal osteoporosis who had taken an oral bisphosphonate for ≥3 years prior to screening and alendronate in the year prior to screening; had a BMD T-score ≤−2.5 at the total hip (TH), lumbar spine (LS), or femoral neck (FN);...

Osteoporosis in Duchenne Muscular Dystrophy (DMD) is arguably one of the most severe bone fragility conditions among children with chronic illnesses. This is hardly surprising, given the deleterious effects of the myopathy and glucocorticoid (GC) therapy on bone strength. The severity of the osteoporotic phenotype is highlighted by observations that 60% of boys will sustain long bone fractures during childhood and a third will present with back pain due to vertebral fractures ...

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...

Fibrous dysplasia is a mosaic disease resulting from post-zygotic activating mutations of the GNAS locus which codes for the α subunit of the Gs G-coupled protein receptor. In bone, impaired signalling results in impaired differentiation and proliferation of bone marrow stromal cells which are replaced by fibrous tissue resulting in bone fragility and dysplasia. All children diagnosed with Fibrous Dysplasia at The Royal National Orthopaedic Hospital since 2009 (103 childr...

Hypophosphatasia is a recessively inherited disorder with a wide phenotypic manifestation ranging from lethality in neonates to asymptomatic in adults. The severity of the phenotype is largely determined by the nature of the ALPL mutations. We describe a previously asymptomatic adult whose phenotype dramatically changed after he developed renal failure. A 50-year-old man was diagnosed with IgA nephropathy. At age 52 (eGFR 50 ml/min) he suffered his first metatarsal fr...

We have previously demonstrated the presence of circulating mesenchymal stem cells (MSCs) in fracture patients. However, the exact time for their appearance in circulation remains unknown. Nine patients (five females, four males, age 70±12 years, range 55–89 years) with a fresh femoral neck fracture were recruited in the study, which was approved by the local ethical committee. Bone marrow (BM) sample drawn from the iliac crest of all patients served as the individua...

Calcification in the vasculature is one of the leading causes of cardiovascular diseases and mortality outcomes. Therefore, the aim of our study was to investigate changes in the gene expression of calcification regulators (CR) in arterial vessels during different stages of atherosclerosis and to document potential corresponding changes in the bone. OPG, RANKL, OPN, MGP, BSP-II and RUNX2 were candidate genes for our study in bone, aorta and arteria ilica externa tissue samples...

Tumour stromal macrophages differentiate into tumour associated macrophages (TAMs), with characteristics resembling the immunosuppressive M2 polarization instead of the pro-inflammatory M1. TAMs have a central role in promoting tumour vascularization, cancer cell dissemination and suppression of anti-cancer immune response. Cancer cell dissemination leads to metastasis formation which, e.g. in breast cancer often happens in bone marrow. We have studied the in vitro mo...

Calcium represents one of the most versatile and universal signalling particles regulating many different cellular processes. Changes in [Ca2+]i give rise to a vast diversity of modulatory events, amongst others, influencing activities of kinases and ion channels.It was demonstrated that nuclear magnetic resonance therapy (NMRT) treatment in osteoarthritis led to reduced pain and improved function followed by increase in quality of ...