Searchable abstracts of presentations at key conferences on calcified tissues

ba0004p154 | (1) | ICCBH2015

A longitudinal, prospective, long-term registry of patients with hypophosphatasia

Kishnani Priya , Langman Craig , Linglart Agnes , Mornet Etienne , Ozono Keiichi , Rockman-Greenberg Cheryl , Seefried Lothar , Bedrosian Camille , Fujita Kenji , Cole Alex , Hogler Wolfgang

Objective: Hypophosphatasia (HPP) is a rare, inherited metabolic disease characterized by bone mineralization defects and osteomalacia, as well as systemic manifestations, including seizures, respiratory insufficiency, muscle weakness, nephrocalcinosis, and pain. The biochemical hallmark of HPP is low serum alkaline phosphatase, resulting from loss-of-function mutations in the gene encoding tissue non-specific alkaline phosphatase. HPP presents a broad spectrum of disease seve...

ba0007oc8 | (1) | ICCBH2019

Bisphosphonate improves hip range of motion and pain but not femoral head sphericity: A multicentre, randomized clinical trial of children with Perthes disease

Jamil Kamal , Zacharin Margaret , Foster Bruce , Donald Geoffrey , Hassall Timothy , Siafarikas Aris , Johnson Michael , Tham Elanie , Whitehead Colin , Gebski Val , Barnes Liz , Cowell Chris , Little David , Munns Craig

Introduction: Perthes disease (PD), idiopathic femoral head avascular necrosis, often results in deformity. The underlying cause is unclear and long-term function is directly related to the roundness of femoral head. Current treatment include mechanical treatments and various surgical procedures, which are therapeutic but can’t prevent collapse. A multicentre, prospective, randomised controlled trial of 12 months zoledronic acid (ZA) in children with PD was conducted. We ...

ba0003pp69 | Bone development/growth and fracture repair | ECTS2014

Enhancement of fracture repair by upregulation of the innate immune response

Santo Ana Isabel Espirito , Chan James K , Glass Graeme E , Ersek Adel , Freidin Andrew , Williams Garry A , Gowers Kate , Jeffery Rosemary , Otto William R , Poulsom Richard , Feldmann Marc , Rankin Sara M , Horwood Nicole J , Nanchahal Jagdeep

Osteoporotic fractures are very common and represent an enormous unmet medical need. Our group has previously reported that addition of rTNF to the fracture site promotes fracture healing in C57/BL6 mice (Glass et al. PNAS 2011). Using a murine fracture model of endochondral healing, we observed that local addition of rTNF only accelerates fracture repair if administered within the first 24 h following injury. The optimal therapeutic dose is 1 ng. TNF is firs...

ba0007oc9 | (1) | ICCBH2019

Efficacy and safety of intravenous zoledronic acid for the treatment of pediatric glucocorticoid-induced osteoporosis: An international, randomized placebo-controlled trial

Ward Leanne M , Alos Nathalie , Cabral David A , Rodd Celia , Sbrocchi Anne Marie , Padidela Raja , Shaw Nick , Kostik Mikhail , Alexeeva Ekaterina , Thandrayen Kebashni , Aftring Paul , Choudhury Anup , Sunkara Gangadhar , Sayyed Sarfaraz , Munns Craig F.

Objectives: We evaluated the efficacy and safety of intravenous zoledronic acid (IV ZA) in children with glucocorticoid-induced osteoporosis (GIOP) through a randomized, placebo (PBO)-controlled trial.Methods: In this multi-national Phase 3 trial (NCT00799266), children 5–17 years of age with GIOP and low-trauma vertebral fractures (VF) were randomized 1:1 to IV ZA 0.05 mg/kg or IV PBO every six months for one year. Changes in lumbar spine areal bon...

ba0007oc14 | (1) | ICCBH2019

Burosumab resulted in greater improvement in clinical outcomes than continuation with conventional therapy in younger (1-4 years-old) and older (5-12 years-old) children with X-linked hypophosphatemia

Ward Leanne , Imel Erik , Whyte Michael , Munns Craig , Portale Anthony , Hogler Wolfgang , Simmons Jill , Padidela Raja , Namba Noriyuki , Cheong Hae , Nilsson Ola , Mao Meng , Skrinar Alison , Chen Chao-Yin , Martin Javier San , Glorieux Francis

Objective: We compared the efficacy and safety of burosumab, a monoclonal antibody against FGF23, to conventional therapy [oral phosphate and active vitamin D (Pi/D)] in children with X-linked hypophosphatemia (XLH).Methods: In this Phase 3 trial (NCT02915705), 61 children with XLH (1-12 years-old) were randomized 1:1 after a 7-day Pi/D washout to receive burosumab starting at 0.8 mg/kg SC Q2W or reinitiate Pi/D optimally titrated by investigators. Eligi...

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 ...

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 ...