Bone Abstracts

Introduction: Although previously babies with genetic type II Osteogenesis Imperfecta (OI) would not have expected to survive, they are now surviving beyond the neonatal period. We describe two such children who have survived beyond infancy.Aim & methods: To identify differences in motor developmental progress between a typical severe (type III) OI child vs two Type II OI children and suggest possible causes. Medical, nursing and therapy (physiothera...

Differences in fracture rates and bone mass in families and individuals of different ethnic origins may be due to differing lifestyles and/or genetic backgrounds. This study aimed to assess the associations of bone mass and fracture prevalence in adolescents with maternal bone mass and fracture history, and sibling fracture history.Data from 1389 adolescent-biological mother pairs from the Birth to Twenty (Bt20) longitudinal study were obtained. Question...

CCN family member 2(CCN2)/Connective tissue growth factor (CTGF) is a multi-functional factor in proliferation, differentiation and migration of mesenchymal cells such as chondrocytes, osteoblasts and vascular endothelial cells. Recently, we also reported that CCN2/CTGF promotes osteoclastgenesis via induction of and interaction with DC-STAMP. We and other researchers reported that CCN2 binds to cytokines and receptors and modified their signaling. Therefore, we compr...

Chronic tophaceous gout is the natural evolution of untreated gouty arthritis and is characterized by the deposition of solid monosodium urate crystal aggregated in a variety of tissue including joints, bursae and tendons. Tophaceous gout is well-known to cause bone erosions and is characterized by enhanced osteoclasts development. Peripheral blood mononuclear cells (PBMCs) from patients with severe erosive gout showed the preferential ability to transform into osteoclast-like...

Rooibos (Aspalathus linearis) tea is a refreshing and caffeine-free tea indigenous to the Western Cape regions of South Africa. This tea, which is rich in polyphenols and antioxidants, has shown many beneficial effects in health, however, its anti-osteoclastogenic potential remains unexplored. In this study, the in vitro effects of an aqueous extract of fermented rooibos tea were examined on osteoclast formation and bone resorption in RAW264.7 murine macropha...

LIGHT (TNFSF14), expressed by different cells of the immune system, binds two trans-membrane receptors: HVEM and LTβR. It is over-expressed in erosive rheumatoid arthritis and lytic myeloma-bone disease and controversial data have been published on its role osteoclast (OC) formation in vitro. Here, we investigated the role of LIGHT on in vitro murine osteoclastogenesis model and bone phenotype in LIGHT−/− mice. Firstly, we showed that murin...

PHP1B -iPPSD3 per the new proposed classification- is a rare disorder characterized in most patients by proximal tubular resistance to PTH resulting in hypocalcemia, hyperphosphatemia and elevated PTH. Loss-of-methylation (LOM) at the Differentially Methylated Region (DMR) at GNAS exon A/B occurs in all PHP1B patients, but methylation changes at other DMRs within GNAS occur in some familial and most sporadic PHP1B cases. All patients with autosomal dominant PHP1B (AD-...

Background: Pycnodysostosis (PDO) is a rare autosomal recessive high bone mass disorder caused by absence of active cathepsin K, which is a lysosomal cysteine protease that plays an important role in degrading the organic matrix of bones. In spite of open fontanelles, raised intracranial pressure has been reported in children with PDO.Presenting problem: We describe a 13-year-old boy with PDO who developed raised intracranial pressure (ICP) which led to ...

Introduction: The availability of bone turnover markers (BTMs) that are kidney independent has facilitated the monitoring of bone turnover in renal patients with hyperparathyroid bone disease. The effects of Cinacalcet on BTMs and on the relationship between parathyroid hormone (PTH) and BTMs were studied.Methods: The formation marker procollagen type 1 N propeptide (P1NP) and resorption marker tartrate resistant acid phosphatase 5b (TRACP5b) were measur...

We studied changes in intracellular Ca2+ concentration in bone cell cultures obtained from human subjects with osteoporosis and osteoarthritis, to evaluate differences between these patients and healthy subjects. We enrolled 36 patients: 12 undergoing primary total hip arthroplasty for osteoporotic femoral fractures (group A, mean age range 57–80), 12 for hip osteoarthritis (group B, mean age range 57–80), and 12 healthy subjects who suffered a high-energy...