Searchable abstracts of presentations at key conferences on calcified tissues

ba0001pp208 | Cell biology: osteoblasts and bone formation | ECTS2013

The positional origins of human osteoblasts dictate growth and differentiation potential and capacity for paracrine vascular cell cross-talk via VEGF

Shah Mittal , Gburcik Valentina , Sankey Andrew , Reilly Peter , Emery Roger , Clarkin Claire , Pitsillides Andrew

Successful long-term, cementless fixation of human shoulder components in osteoporotic (OP) and osteoarthritic (OA) patients poses major challenges. The possibility that enhanced osseointegration may rely on both the region of bone targeted and its relationship with the vasculature remains unexplored. We hypothesise that bone cells derived from subchondral (SC), cortical (C) and trabecular (Trb) bone regions exhibit differing osteogenic potential, which will be diminished in b...

ba0003ht3 | (1) | ECTS2014

Consistent, marked and rapid increases in hip and spine BMD with the PTHrP1-34 analog, abaloparatide (BA058), compared to placebo and teriparatide

Yates John , Alexandersen Peter , Krogsaa Annesofie , Nedergaard Bettina , Clarkin Marcie , Hattersley Gary , Karsdal Morten , Christiansen Claus

Background: Treatments that result in greater increases in bone mass of normal quality by increasing bone formation rather than decreasing resorption are needed. Abaloparatide is a synthetic analog of PTHrP1-34 that has shown strong efficacy to increase bone mass and bone strength in animals. We conducted two phase 2 placebo-controlled studies both of which included abaloparatide 80 μg sc daily (ABL) in postmenopausal women with osteoporosis. Study 1 also inclu...

ba0003pp351 | Osteoporosis: treatment | ECTS2014

A transdermal patch delivering the PTHrP1–34 analog, abaloparatide (BA058), dose-dependently increases spine and hip bmd compared to placebo

Yates John , Alexandersen Peter , Krogsaa Annesofie , Nedergaard Bettina , Clarkin Marcie , Hattersley Gary , Hansen Kris , Karsdal Morten , Christiansen Claus

Abaloparatide (BA058) is a synthetic analog of PTHrP1–34 which greatly increases bone mass and bone strength with preservation of normal bone quality in animal models of osteoporosis. Daily s.c. abaloparatide (ABLSC) at doses of up to 80 μg daily in postmenopausal women with osteoporosis for up to 48 weeks were associated with increases in spine and femoral neck BMD of up to 12.9 and 4.1% respectively and good safety and tolerability. The increa...

ba0006p192 | (1) | ICCBH2017

Development of an osteogenesis imperfecta specific quality of life measure

Hill Claire , Baird Wendy , Walters Stephen

Objectives: Osteogenesis Imperfecta (OI) is a hereditary disorder effecting approximately 1 in 20 000 births. Symptoms include; low bone mass, recurrent fractures, varying degrees of short stature and deformity. There is currently no disease specific quality of life (QoL) measure for children with OI. This study used a mixed methods approach to develop a QoL measure for the paediatric OI population. Patient reported outcome measure development is an iterative process, moving b...

ba0004p13 | (1) | ICCBH2015

Galactosyltransferase-1 deficiency: a novel cause of bone fragility due to impaired proteoglycan synthesis

Moon Rebecca , Salter Claire , Foulds Nicola , Davies Justin

Proteoglycans is a component of extracellular matrix, forming a mesh around structural proteins including collagen and elastin, and providing elasticity to tissue structure. Proteoglycans is formed by the attachment of glycosaminoglycans to a core protein, a process that requires three enzymes: galactosyltransferase-1, galactosyltransferase-2 and glucoronosyltransferase-1, encoded by B4GALT7, B3GALT6 and B3GAT3 respectively. B3GALT6 mutation...

ba0004p88 | (1) | ICCBH2015

Vitamin D and bone health: A practical clinical guideline for management in children and young people in the UK

Thurston Anne , Bowring Claire , Shaw Nick , Arundel Paul

Background: There is currently considerable clinical and academic interest in vitamin D in children and young people. This partly relates to recognition of a resurgence of symptomatic vitamin D deficiency with reports of children presenting with rickets or hypocalcaemic symptoms. An additional development has been the recognition that vitamin D may have a physiological extraskeletal role beyond its traditional function as a key regulator of calcium and bone metabolism.<p c...

ba0006p177 | (1) | ICCBH2017

The elbow in type V osteogenesis imperfecta: is early functional loss related to radiographic findings?

Hill Claire , Offiah Amaka , Bishop Nick , Arundel Paul

Objectives: Type V osteogenesis imperfecta (OI) results in abnormal modelling of the ulna, dislocation of the radial head and interosseous membrane calcification (IOM). Individuals develop reduced functional ability as a consequence of reduced range of movement (ROM) including elbow flexion and/or supination, which may be intrinsic or secondary to the radiographic findings. We describe the evolution of radiographic and functional parameters in a cohort seen in our centre.<...

ba0007p196 | (1) | ICCBH2019

Sleep related problems in children with osteogenesis imperfecta

Murphy Kieran , Hill Claire , Barker Nicki , Kingshott Ruth

Osteogenesis Imperfecta (OI) is a genetic disorder affecting 1 in 10,000 births with a wide variability in phenotypes. Clinical Manifestations include; recurrent fractures, bone pain, varying degrees of short stature and deformity, scoliosis, kyphosis, and respiratory failure in the severest types. Sleep disorders are under-acknowledged and are often more problematic in children with chronic illnesses. Moldosky1 identified the link between pain affecting quality of ...

ba0007p208 | (1) | ICCBH2019

Motor developmental outcomes in 2 babies with very severe osteogenesis imperfecta (type II)

Sweeney Claire , O'Sullivan Lizzie , Sahota Jaskiran , Saraff Vrinda , Shaw Nick

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...