Searchable abstracts of presentations at key conferences on calcified tissues

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...

ba0005p89 | Calciotropic and phosphotropic hormones and mineral metabolism | ECTS2016

Endogenous expression and biological functionality of secreted Klotho in human mesenchymal stem cells

Mentrup Birgit , Pauslus Sofia , Krug Melanie , Hafen Bettina , Jakob Franz

The biological relevance of Klotho in aging processes was impressively demonstrated in transgene and knock out mouse experiments and its impact on calcium, vitamin D and phosphate metabolism suggests multifunctional modes of action.The human alpha Klotho gene encodes two transcripts, one for a protein of 130 kDa with a short intracellular and a transmembrane region and the two extracellular domains KL1 and KL2. A second transcript exists due to alternati...

ba0007p13 | (1) | ICCBH2019

Clinical implications of modeling the maturational spurt

Boeyer Melanie , Leary Emily , Sherwood Richard , Duren Dana

Objective: The treatment of many skeletal growth and/or developmental disorders often relies on a child’s biological maturity status, frequently determined by a skeletal maturity assessment. Rapid changes in the rate of skeletal maturation (i.e., the maturational spurt) during adolescence can significantly influence biological maturity status, affecting treatment type and timing as well as clinical outcomes. However, the chronological age at which peak maturational veloci...

ba0003pp46 | Bone development/growth and fracture repair | ECTS2014

Midkine deficiency significantly delayed chondrogenesis during fracture healing in mice

Haffner-Luntzer Melanie , Heilmann Aline , Rapp Anna , Schinke Thorsten , Amling Michael , Ignatius Anita , Liedert Astrid

One growth factor that potentially plays a role in fracture healing is midkine (Mdk). Mdk is expressed in chondrocytes during bone repair (Ohta et al. 1999) and has been shown to influence bone mass and mechanotransduction (Neunaber et al. 2010, Liedert et al. 2011). The aim of the present study was to evaluate the effects of Mdk-deficiency on bone repair in a standardized mouse femur osteotomy model.Mdk-deficient and wildtype ...

ba0005p54 | Bone development/growth and fracture repair | ECTS2016

Role of interleukin-6 in the early and late fracture healing phase

Prystaz Katja , Kovtun Anna , Kaiser Kathrin , Heidler Verena , Kroner Jochen , Haffner-Luntzer Melanie , Ignatius Anita

Interleukin-6 (IL-6) plays an important role in bone metabolism and regulates fracture healing in a presently unknown process. In the fracture callus IL-6 expression is biphasic; it peaks during the inflammatory phase and again during intramembranous and endochondral ossification (Ai-Aql et al. 2008). Few studies using IL-6 knockout mice indicate that IL-6 might be crucial for bone healing (Yang et al. 2007). However, a generalized IL-6 knockout induces multi...

ba0005p55 | Bone development/growth and fracture repair | ECTS2016

Calcium and vitamin-D supplementation post-trauma improves bone healing and decreases posttraumatic bone resorption in an osteoporotic mouse model

Heidler Verena , Haffner-Luntzer Melanie , Prystaz Katja , Kroner Jochen , Schinke Thorsten , Amling Michael , Ignatius Anita

Chronic calcium- and vitamin-D-deficiencies are crucial risk factors for osteoporosis. However, their significance for fracture healing is still poorly investigated, despite the clinical evidence that osteoporotic bone healing is disturbed. This study addressed the important question, whether chronic deprivation of calcium and vitamin D compromises bone repair and if this could be rescued by a supplementation post-trauma. Because clinical hints suggest that a fracture induces ...

ba0007p183 | (1) | ICCBH2019

Functional outcomes of an adult with Osteogenesis Imperfecta after rehabilitation post-bilateral Girdlestone procedure: a case report

Supnet Isabella , Abiera Joycie Eulah , Alcausin Maria Melanie Liberty , Javier Juanito , Sumpaico Carlo Emmanuel

Osteogenesis imperfecta is a disorder characterized by bone fragility. Current management includes the usage of bisphosphonates to improve bone stock and manage pain. Outcomes in adults have mainly been reported in terms of presentation compared to genotype, most probably due to the heterogeneity of the disease. The head and neck resection of the femur, or Girdlestone procedure, was a common procedure to treat infections of the hip but it has fallen out of favor due to the adv...

ba0005p59 | Bone development/growth and fracture repair | ECTS2016

Midkine is involved in the pathogenesis of delayed osteoporotic fracture healing after ovariectomy in mice

Haffner-Luntzer Melanie , Kemmler Julia , Heidler Verena , Prystaz Katja , Schinke Thorsten , Amling Michael , Ignatius Anita , Liedert Astrid

Previous studies demonstrated a negative influence of the growth- and differentiation factor midkine (MDK) on bone formation during bone remodeling and fracture healing. Additionally, MDK-deficiency protected mice from a loss of trabecular bone mass after ovariectomy (OVX). Therefore, we hypothesized that MDK may also be involved in the pathogenesis of delayed, osteoporotic fracture healing after OVX in mice. Thus, we analyzed the expression of MDK during bone regeneration and...

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 ...