Searchable abstracts of presentations at key conferences on calcified tissues

ba0007p121 | (1) | ICCBH2019

An Acvr1[R258G] ‘conditional on' mouse model of atypical fibrodysplasia ossificans progressiva (FOP) is Activin A dependent

Huang Lily , Schoenherr Chris , Wang Lili , Wen Xialing , McClain Joyce , Zhang Qian , Nannuru Kalyan , Idone Vincent , Murphy Andrew , Economides Aris , Hatsell Sarah

FOP is an autosomal dominant disorder characterized by early onset, episodic and progressive ossification of skeletal muscle and associated connective tissue. FOP is driven by mutations in the intracellular domain of ACVR1 (ALK2), the most common of which is R206H. However, rare FOP causing mutations exist throughout the GS and the kinase domain of Acvr1. Several of these mutations result what appears to be a more severe FOP phenotype that includes significant developmental ab...

ba0007p44 | (1) | ICCBH2019

Bone mass and vertebral fractures in South African (SA) children on prolonged oral glucocorticoids (GCs) for chronic non-malignant illnesses

Thandrayen Kebashni , Petersen Karen , Kala Udai , Lala Nilesh , Ambaram Priya , Mistry Bhadrish , Hauptfleisch Marc , Hajinicolaou Christina , Okudu Grace , Verwey Charl , Moosa Fatima , Parbhoo Kiran , Pettifor John M

Objectives: To assess lumbar spine (LS) BMD Z-scores and the prevalence of vertebral fractures using DXA lateral vertebral (VFA) assessment in children and adolescents with chronic illnesses on GCs.Methods: All children between the ages of 5 and 17 years with chronic non-malignant illnesses who were on GCs (intravenous or oral) for greater than 3 months duration were evaluated. Study participants were children attending the paediatric sub-specia...

ba0007p147 | (1) | ICCBH2019

Changes in DXA Z-scores during bisphosphonate (BP) therapy in patients with osteogenesis imperfecta (OI) at a tertiary care hospital in South Africa

Siralarala Akhona , Pettifor John Morley , Thandrayen Kebashni

Objectives: The aim of this study was to evaluate and assess the change in bone mineral density in OI children treated with BPs at Chris Hani Baragwanath Academic hospital (CHBAH), South Africa.Methods: Medical files of 104 OI patients who were seen at Metabolic Bone clinic from 01 January 2006 till 31 December 2015 were reviewed. DXA measurements were available on 33 patients that received bisphosphonates from baseline and during therapy at three time p...

ba0001oc5.5 | Treatment of osteoporosis | ECTS2013

Bone anabolic efficacy and safety of ba058, a novel analog of hPTHrP: 12-month extension data from a phase 2 clinical trial in postmenopausal women with osteoporosis

Hattersley Gary , Bilezikian John , Guerriero Jonathan , Kumar Prasanna , Zanchetta Jose , Lyttle C Richard , O'Dea Louis Saint L

A randomized, placebo-controlled phase 2 study evaluated the safety and efficacy of BA058, an analog of hPTHrP(1–34), in postmenopausal women with severe osteoporosis. 221 patients were randomized to received BA058 20, 40, and 80 μg, placebo or teriparatide (TP) 20 μg, by daily s.c. injection. 184 (83%) patients completed an initial 24 weeks of treatment. The mean percent change in lumbar spine BMD at 24 weeks was 1.6% with placebo, 6.7% with BA058 80 μg, a...

ba0001pp450 | Osteoporosis: treatment | ECTS2013

Transdermal delivery of BA058, a novel analog of hPTHrP (1-34), with a short wear time patch in preclinical and clinical studies

Hattersley Gary , Hansen Kris , Determan Amy , Brown Ken , Mckay Kate , Guerriero Jonathan , McCarthy Dan , Lyttle C Richard , St L O'Dea Louis

BA058 is being developed as an anabolic therapy for the treatment of osteoporosis. Daily BA058 SC injection has produced promising safety and efficacy results in early clinical studies, and is currently enrolling in a Phase 3 fracture prevention study. There is, however, a significant need for an alternative to injection that improves patient convenience and compliance. We have investigated the use of a solid Microstructured Transdermal System (3M) for transdermal (TD) deliver...

ba0002p180 | (1) | ICCBH2013

Beyond brittle bones: a preliminary report from the osteogenesis imperfecta adult natural history initiative

Tosi Laura , McKiernan Fergus , Oetgen Matthew , Rak Melanie , Tucker Carole , Mulroy Kyle , Simmonds Barbara , Mancuso Angela , Kennelly Ann , Greco Lauren , Blankenship Winslow , Floor Marianne , Huber Mary Beth , Hart Tracy

Background: Osteogenesis imperfecta (OI) is a heterogeneous, rare disorder most commonly affecting type I collagen. The OI Adult Natural History Initiative (OI ANHI) was established following a 2010 Ostegenesis Imperfecta Foundation (OIF) national meeting at which patient participants noted that i) there is little information about the natural history and progression of OI beyond childhood, and ii) most of the data available are focused on musculoskeletal issues. Adults with O...

ba0001pp186 | Cell biology: osteoblasts and bone formation | ECTS2013

Involvement of Runx2 in the differentiation process of osteoblastic precursor cells after radiation exposure

Hu Yueyuan , Lau Patrick , Hellweg Christine , Baumstark-Khan Christa , Reitz Guenther

Astronauts on exploratory space missions will experience a complex environment that includes microgravity and radiation. While the deleterious effects of unloading on bone are well established, fewer studies have focused on the effects of radiation. Space radiation produces distinct biological damages which, up to now, little is known about the correlation between radiation exposure and bone tissue. In our study we used osteoblastic precursor cells to investigate the radiation...

ba0003w4.2 | Endocrine regulation of bone | ECTS2014

Bone sparing glucocorticoids

Cooper Mark

Therapeutic glucocorticoids are still widely used for their anti-inflammatory effects. There are however significant side effects associated with their use. These include effects on bone leading to osteoporosis and fracture but also on muscle (increasing risk of falls) and systemic fuel metabolism (leading to diabetes and increased cardiovascular risk). Research over many decades has tried to develop agents which retain the anti-inflammatory effects of glucocorticoids but have...

ba0003pp336 | Osteoporosis: treatment | ECTS2014

The effect of alendronate oral jelly in the treatment of osteoporosis in clinical settings

Okimoto Nobukazu , Arita Shinobu , Akahoshi Syojiro , Yoshioka Toru , Fuse Yoshifumi

Alendronate is one of the most popular anti-resorptive drugs and widely used to treat osteoporotic patients in the world. In Japan, alendronate oral jelly (ALN-J) as a new formulation was launched in 2013. Dissolution test and bioequivalence study were performed between ALN-J and alendronate oral weekly tablet (ALN-T), and bioequivalence of both drugs was approved. Dosage and administration of ALN-J is the same as ALN-T. Moreover, ALN-J has special features of easy swallowing ...

ba0005p114 | Cancer and bone: basic, translational and clinical | ECTS2016

Increased zinc accumulation in mineralized osteosarcoma tissue

Rauwolf Mirjam , Pemmer Bernhard , Roschger Andreas , Turyanskaya Anna , Simon Rolf , Lang S. , Puchner S E , Windhager Reinhard , Klaushofer Klaus , Wobrauschek Peter , Hofstaetter Jochen G , Roschger Paul , Streli Christina

Abnormal tissue levels of certain trace elements such as Zinc (Zn) were reported in various types of cancer. Little is known about the role of Zn in osteosarcoma.Using confocal synchrotron radiation micro X-ray fluorescence analysis, we characterized the spatial distribution of Zn in high-grade (G3) sclerosing osteosarcoma of nine patients (4 f/5 m; 7 knee/1 humerus/1 femur) following chemotherapy and wide surgical resection. The study was done in accord...