Searchable abstracts of presentations at key conferences on calcified tissues

ba0007oc16 | (1) | ICCBH2019

A natural history study of generalized arterial calcification of infancy (GACI) and autosomal recessive hypophosphatemic rickets (ARHR2) due to ENPP1 or ABCC6 deficiency: interim analysis

Nitschke Yvonne , Kintzinger Kristina , Hackbarth Mary , Botschen Ulrike , Wang Sisi , Gafni Rachel I , Mueller Kerstin , Ahmed Ruhi , Yuen Eric , Gahl William A , Ferreira Carlos R , Rutsch Frank

Introduction: ENPP1 Deficiency manifests as GACI type 1 in infants, a disorder characterized by extensive arterial calcifications and stenoses, often fatal in utero or in early infancy. Beyond six months, the mortality rate significantly decreases among survivors, who may later develop ARHR2, characterized clinically by short stature, bone deformities and pain. ABCC6 Deficiency also manifests as GACI type 2 in infants and is clinically indistinguishable from GACI type 1. Anima...

ba0003pp263 | Osteoporosis: pathophysiology and epidemiology | ECTS2014

Interval changes in bone mineral density in exercising young women with apparent athlete triad syndrome

To William , Wong Margaret

Objective: To evaluate the bone mineral density (BMD) of the axial and appendicular skeleton in a group of collegiate dance students undergoing intensive training between a 24 months interval and to correlate these changes to the presence or absence of apparent athlete triad syndrome (ATS).Methods: Forty full time collegiate dance students were recruited from a tertiary Performing Arts Institute. All subjects had basic anthropometric measurements, a full...

ba0003pp269 | Osteoporosis: pathophysiology and epidemiology | ECTS2014

Comparison of peripheral volumetric bone mineral density, quantitative ultrasound and standard radiological methods for monitoring bone mineral density changes 2 years postpartum

To William , Wong Margaret

Objective: To evaluate the BMD changes around 2 years after delivery using standard dual energy X-ray absorptiometry (DXA) of the lumbar spine and hip, peripheral quantitative computerized tomography (pQCT) of the distal tibia and radius, and quantitative ultrasound (QUS) of the oscalcis.Methods: Consecutive patients with uncomplicated singleton pregnancies were recruited from a general obstetric clinic. Standard BMD measurements of the lumbar spine and ...

ba0007p136 | (1) | ICCBH2019

Intravenous bisphosphonate treatment in severe infantile hypercalcemia associated with Williams Syndrome

Guarneri Alissa M , Patel Nisha , Bowden Sasigarn A

Background: Infantile hypercalcemia occurs in ~15% of patients with Williams Syndrome (WS) and is typically mild. Severe hypercalcemia has been reported in infants/toddlers with WS, requiring treatment with intravenous (IV) saline hydration, furosemide, calcitonin, calcium and vitamin D restriction, and in some cases IV bisphosphonates.Presenting problem: Three cases of infants with WS age 9–13 months presented with severe hypercalcemia, failure to ...

ba0001pp320 | Osteoporosis: evaluation and imaging | ECTS2013

Quantitative ultrasound of os calcis BMD vs conventional DXA and peripheral QCT in interval assessment of BMD changes in adolescent females

To William W K , Wong Margaret W N

Objective: To compare whether interval BMD changes in adolescent females that can be detected using conventional dual energy X-ray absorptiometry (DXA) can also be detected using quantitative peripheral quantitative computerized tomography scans (pQCT) and quantitative ultrasound (QUS)of the os calcis.Methods: Two groups of adolescent females were recruited for assessment of BMD changes over an interval of 22–24 months. These included full time coll...

ba0001pp321 | Osteoporosis: evaluation and imaging | ECTS2013

Use of os calcis quantitative ultrasound for bone mineral density screening in adolescents with menstrual dysfunction

To William W K , Wong Margaret W N

Background: Prolonged hypothalamic amenorrhoea with anovulation has been associated with hypo-oestrogenism in adolescents and has been shown to be associated with lower bone mineral density (BMD) values.Objective: To determine whether differences in BMD between oligo/amenorrhoeic adolescents at risk of low BMD and normal eumenorrhoeic controls can be detectable by quantitative ultrasound (QUS) of the os calcis.Methods: Adolescents ...

ba0003pp146 | Cell biology: osteoblasts and bone formation | ECTS2014

Strontium ranelate increases osteoblast cell proliferation and differentiation and the formation of bone-like mineralized matrix on different titanium substrates

Querido William , Farina Marcos , Anselme Karine

The integration of biomaterials into the calcified bone tissue is essential for the clinical success of bone implants. A particular strategy to improve such integration is the use of specific molecules to increase osteoblast cell adhesion, proliferation, and/or differentiation on the surface of the implants, aiming to enhance the interaction of cells with biomaterials. The goal of this study was to better understand the potential of the anti-osteoporotic drug strontium ranelat...

ba0005p176 | Cell biology: osteoclasts and bone resorption | ECTS2016

Impaired c-kit signaling couples bone resorption to bone formation through wnt10b in kitw-Sh/W-Sh mice

Lotinun Sutada , Krishnamra Nateetip , Horne William

Kit ligand/c-Kit receptor tyrosine kinase complex has been implicated as a target for bone remodeling process. Loss of function mutation in c-Kit causes low bone mass in KitW/W-v (W/Wv) mice. However, these mice are sterile and it is unclear whether the observed skeletal phenotype is secondary to sex hormone deficiency. To address this question, the skeletal phenotype of KitW...

ba0001pp495 | Other diseases of bone and mineral metabolism | ECTS2013

Long-term effects of symptomatic vs intensive bisphosphonate therapy for Paget's disease of bone: the PRISM-EZ study

Goodman Kirsteen , MacLennan Graeme , Fraser William , Selby Peter , Ralston Stuart

Paget’s disease of bone (PDB) is a common metabolic bone disease characterised by increased and disorganised bone remodelling affecting one or more skeletal sites. Bisphosphonates are highly effective at suppressing bone turnover in PDB but it remains unclear whether greater suppression of bone turnover improves clinical outcome. In the PRISM study, we previously reported that PDB patients randomised to ‘intensive’ treatment aimed at normalising alkaline phospha...