Searchable abstracts of presentations at key conferences on calcified tissues

ba0006p011 | (1) | ICCBH2017

Neonatal hypocalcemia – transient neonatal pseudohypoparathyroidism

Kutilek Stepan , Vracovska Martina , Pikner Richard , Fejfarkova Zlatka

Background: Neonatal hypocalcemia is defined as S-Ca <2 mmol/l in fullterm newborns and <1.75 mmol/l in preterm newborns. Neonatal hypocalcemia is either early onset (<3 days of age) or late onset (>3 days of age). Newborns with hypocalcemia are often asymptomatic, but mays present with hypotonia, apnea, poor feeding, jitteriness, seizures, cardiac failure. Signs of hypocalcemia rarely occur unless S-Ca drops below 1.75 mmol/l.Case presen...

ba0006p117 | (1) | ICCBH2017

Uptake of influenza vaccine in UK patients with fibrodysplasia ossificans progressiva

Gak Nataliya , Vinton Jacqueline , Jacobs Benjamin , Keen Richard

Fibrodysplasia ossificans progressiva (FOP; OMIM #135100) begins in childhood and leads to irreversible restriction of movement, functional impairment, and shortened life-span. Individuals with FOP develop progressive limitations in chest expansion, resulting in restrictive lung disease. Current management guidelines published in 2011 (1) highlight that Influenza may be a causative factor for FOP flare-ups, and can also cause potentially deadly cardiopulmonary complications, e...

ba0003pp223 | Osteoporosis: evaluation and imaging | ECTS2014

Bone mineral density and bone turnover markers in patients with thyroid cancer and L-T4 suppressive therapy after 25 years of follow-up

de Mingo Dominguez Maria Luisa , Iglesias Sonsoles Guadalix , Alvarez Maria Begona Lopez , Diaz-Guerra Guillermo Martinez , Carranza Federico Hawkins

Background: Patients with differentiated thyroid cancer (DTC) are treated with L-thyroxine (L-T4) in relatively hight doses to suppress endogenous TSH levels. Very long-term effect of thyroid hormones supplementation on bone are controversial.Objetive: To study the possible negative effects on bone mineral density (BMD) and bone markers in DTC patients followed in our center.Methods: A 46 po...

ba0007lb1 | (1) | ICCBH2019

Genetic inactivation of osteocalcin in Col1a1Jrt/+ mice, a model of dominant osteogenesis imperfecta, restores glucose metabolism to wild-type levels

Tauer Josephine T , Komarova Svetlana V

Objective: Osteocalcin, an osteoblast-derived hormone, is among the most abundant proteins in bone and is involved in the regulation of whole-body metabolism, muscle adaptation, and reproduction. High bone turnover and low bone mass are clinical hallmarks of Osteogenesis Imperfecta (OI), a bone disease mainly caused by mutations in the collagen-I gene. Recently, we have shown that growing mice with a severe dominant form of OI, Col1a1Jrt/+ mice, displayed significantly elevate...

ba0003oc6.3 | Osteoporosis treatment and the effects of physical activity | ECTS2014

Vitamin D is low in obesity, and this is due to greater volume of distribution

Walsh Jennifer S , Evans Amy L , Bowles Simon , Naylor Kim E , Gossiel Fatma , Jacques Richard , Schoenmakers Inez , Jones Kerry S , Eastell Richard

Low circulating levels of total 25-hydroxyvitamin D (25OHD) have been reported in obese people of different ethnicities in several countries. Low total 25OHD in obesity could be due to lower binding proteins (with normal free 25OHD), lower dietary intake or sunlight exposure, greater volume of distribution (pool size) or more rapid metabolic clearance.The aims of this study were to determine if free 25OHD and 1,25-dihydroxyvitamin D (1,25(OH)2...

ba0007p226 | (1) | ICCBH2019

The experience of canakinumab in 2 patients with primary tumor (tumoral) calcinosis

Kostik M , Petukhova V , Pigareva T , Likhacheva T , Idrisova R , Snegireva L , Krasnogorskaya O , Suspisin E

Background: Primary tumoral calcinosis is an orphan disease. The data about the incidence of this disease, as well as clinical recommendations for treatment are not presented in the literature.Presenting problem: Two patients – 11.5 years old boy and 8 years old girl with primary tumoral calcinosis had multiple foci of the subcutaneous calcification, walking impossibility, wheel-chair condition, fatigue, high fever and equinus deformity of the left ...

ba0001pp41 | Bone biomechanics and quality | ECTS2013

Mechanical contrasts between osteons and interstitial bone measured by scanning acoustic microscopy

Fix Dmitri , Puchegger Stephan , Pilz-Allen Christine , Roschger Paul , Fratzl Peter , Weinkamer Richard

For a reliable assessment of bone’s material quality in a clinical environment, a fast way to measure the mechanical properties of bone is needed. The investigation of material heterogeneity and anisotropy resulting from bone remodeling and mineralization requires an imaging technique with micrometer resolution. Scanning acoustic microscopy (SAM) using high-frequency lenses allows measuring the stiffness of bone under wet conditions in a non-destructive way with this spat...

ba0001pp85 | Bone development/growth and fracture repair | ECTS2013

The retention and bioactivity of rhBMP-2 released from a bisphosphonate-linked hyaluronan-based hydrogel

Hulsart-Billstrom Gry , Yuen Pik-Kwan , Marsell Richard , Hilborn Jons , Larsson Sune , Ossipov Dmitri

Introduction: There are several disadvantages with the present carriers used in rhBMP-2 products including risk for immunological response, inefficient release and poor handling properties. Our aim was to examine the release of rhBMP-2 from a bisphosphonate-linked hyaluronan hydrogel with the hypothesis that it would cause a slower release.Methods: Triplicates of hydrogels with rhBMP-2 and with (HA-BP) or without (HA) bisphosphonate were prepared, after ...

ba0003oc6.5 | Osteoporosis treatment and the effects of physical activity | ECTS2014

The effect of bisphosphonate treatment on osteoclast precursor cells in postmenopausal women with rsteoporosis: The TRIO study

Gossiel Fatma , Hoyle Christopher , McCloskey Eugene , Walsh Jennifer , Peel Nicola , Eastell Richard

Bisphosphonates are used to treat bone disease characterised by increased bone resorption by inhibiting the activity of mature osteoclasts, resulting in decreased bone turnover. Bisphosphonates may reduce the population of osteoclast precursor cells (OPCs). Our aims were to investigate the effect of bisphosphonates on i) OPCs and ii) bone turnover in postmenopausal women with osteoporosis compared with healthy premenopausal women. Participants were 62 postmenopausal women (mea...

ba0003pp125 | Cell biology: osteoblasts and bone formation | ECTS2014

The significance of Bcl-2-associated athanogene-1 (BAG-1)-mediated protein interactions in osteoblast development

Greenhough Joanna , Papadakis Emmanouil , Cutress Ramsey , Townsend Paul , Oreffo Richard , Tare Rahul

BAG-1 interacts with diverse proteins including heat shock proteins (HSC70/ HSP70) and nuclear hormone receptors to modulate cell proliferation, differentiation and apoptosis. We have shown that bone marrow stromal cells (BMSCs) of Bag-1+/− female mice display reduced BMP-2-directed osteogenic differentiation potential. Estradiol binds to estrogen receptors (ERs) and is functionally involved in BMP-directed osteogenic differentiation of osteoprogenito...