Searchable abstracts of presentations at key conferences on calcified tissues

ba0007p38 | (1) | ICCBH2019

Clinical features and approach to treatment in pediatric patients with McCune-Albright syndrome: monocentric experience

Makazan Nadezhda , Orlova Elizaveta , Kareva Maria , Kolodkina Anna , Kalinchenko Natalia , Petrov Michael , Zubkova Natalia , Peterkova Valentina

McCune-Albright-Syndrome (MAS) is a rare multisystem disorder presenting with polyostotic fibrous dysplasia accompanied by a list of extraskeletal features including café-aulait spots and various endocrine hyperfunctioning. There is no effective treatment for FD in MAS nowadays. Patients with MAS (n=60, 49 girls(G) and 11 boys (B) have been diagnosed and followed up during 20 years in the Institute of Pediatric Endocrinology. First clinical manifestations were pe...

ba0007p77 | (1) | ICCBH2019

Long-term efficacy profile of asfotase alfa in the treatment of patients with hypophosphatasia: a pooled analysis

Hogler Wolfgang , Rockman-Greenberg Cheryl , Petryk Anna , Zhou Shanggen , Whyte Michael P , Bishop Nick

Objectives: Asfotase alfa (AA), an enzyme replacement therapy, is the only approved medical treatment for pediatric-onset hypophosphatasia (HPP), which is caused by deficient tissue-nonspecific alkaline phosphatase activity. We detail the long-term efficacy of AA observed from the pediatric clinical trial program.Methods: Efficacy data collected to study completion were pooled from 3 open-label, multicenter investigations of children who manifested HPP s...

ba0007p180 | (1) | ICCBH2019

A retrospective review of modern spine surgery in the skeletal dysplasia population

Lovecchio Francis , Shafi Karim , Steinhaus Michael , Samuel Andre , Farmer James , Sava Maria , Carter Erin , Raggio Cathleen

Objective: Existing case series examining outcomes following spine surgery for patients with skeletal dysplasias demonstrate high rates of complications. Yet, these earlier studies are from a single institution, contain surgeries performed almost 50 years ago, and only include patients with a diagnosis of achondroplasia. The objective is to determine contemporary outcomes after spinal surgery in patients with skeletal dypslasias, focusing on complications and revisions. We pre...

ba0007lb2 | (1) | ICCBH2019

Lumbar spine quantitative computed tomography (QCT) is a better predictor of vertebral fracture in boys with Duchenne muscular dystrophy (DMD) than either DXA or peripheral QCT

Crabtree Nicola , Machin Michael , Padidela Raja , Kariki Eleni , Hughes Imelda , Shaw Nick , Mughal Zulf

Objectives: Vertebral fractures are common in boys with DMD taking daily corticosteroids. Treatment is usually initiated when vertebral fractures have been identified. However, prophylactic treatment may be possible if reliable risk factors for vertebral fracture can be identified. The aim of this work was to compare the diagnostic accuracy of three different bone strength assessment techniques in a cohort of DMD boys.Methods: Thirty-three boys with DMD ...

ba0004p95 | (1) | ICCBH2015

Osteomyelitis and septic arthritis in children: first data from the EUCLIDS network

Zenz Werner , Trobisch Andreas , Klobassa Daniela , Binder Alexander , Sperl Matthias , Martinon-Torres Federico , Rivero Irene , Carrol Enitan D , Paulus Stephane , Kalifa Bojang , Anderson Suzanne , Schlapbach Luregn , Emonts Marieke , van der Flier Michiel , de Groot Ronald , Boeddha Navin , Herberg Jethro , Agapow Paul , Levin Michael , -on behalf of EUCLIDS consortium

Background and aims: Bone and joint infections like osteomyelitis and septic arthritis occur in ~3–12/100.000 children per year in high-income countries with predominance in males. The most common causative pathogen is Staphylococcus aureus, however, only in 50% pathogen detection succeeds. The aim of this study is to describe clinical characteristics of osteomyelitis and septic arthritis in children recruited within the EUCLIDS network (<a href="http://www.eucli...

ba0005p72 | Bone development/growth and fracture repair | ECTS2016

Sulfated hyaluronan improves bone defect healing in type 2 diabetic rats by increasing osteoblast function

Picke Ann-Kristin , Salbach-Hirsch Juliane , Hintze Vera , Rauner Martina , Moller Stephanie , Kascholke Christian , Hacker Michael C , Schulz-Siegmund Michaela , Schnabelrauch Matthias , Scharnweber Dieter , Hofbauer Lorenz C , Hofbauer Christine

Bone fractures of patients suffering from type 2 diabetes mellitus (T2DM) represent an emerging socioeconomic problem. Underling mechanisms are poorly understood and therapies are limited. Our previous studies have shown that sulfated hyaluronan (sHA3) suppresses osteoclast activity while supporting osteoblast function in vitro. Hence, we now investigated if sHA3 can improve the delayed fracture healing in rats with T2DM.Porous, cross-linked lactide-base...

ba0005p464 | Other diseases of bone and mineral metabolism | ECTS2016

The course and management of craniofacial fibrous dysplasia: a case series

Majoor Bas , Appelman-Dijkstra Natasha , Bruggemann Jens , van de Sande Michiel , Dijkstra Sander , Hamdy Neveen

Introduction: Cranialfacial fibrous dysplasia (CFD) presents with pain, facial asymmetry and/or neurological complications. It has been suggested that patients with CFD respond favourably to treatment with bisphosphonates, by a decrease in pain and arrest of progression. Therefore, we performed a retrospective study of 56 patients with CFD in our center.Methods: We assessed clinical characteristics and disease course. Furthermore, clinical and biochemica...

ba0003pp318 | Osteoporosis: treatment | ECTS2014

Preferences of patients and health care professionals for osteoporosis drug treatment: a discrete choice experiment

Hiligsmann Mickael , Dirksen Carmen , Dellaert Benedict , van der Weijden Trudy , Goemaere Stefan , Reginster Jean-Yves , Watson Verity , Boonen Annelies

Objectives: This study aims to evaluate and compare the preferences of patients and health care professionals for osteoporotic drug treatment.Materials and methods: A discrete choice experiment was conducted among patients and health care professionals (general physicians, rheumatologists, and geriatricians) in Belgium. Participants were asked to choose between two hypothetical unlabelled drug treatments (and an opt-out option) that vary in several attri...

ba0005oc1.1 | Clinical trials and osteoporosis treatment | ECTS2016

Efficacy of odanacatib in postmenopausal women with osteoporosis: subgroup analyses of data from the phase 3 long-term odanacatib fracture trial (LOFT)

Saag Kenneth G , Alexandersen Peter , Benhamou Claude-Laurent , Gilchrist Nigel , Halse Johan , Michael Lewiecki E. , Lippuner Kurt , McClung Michael , Shiraki Masataka , DaSilva Carolyn A , Verbruggen Nadia , Scott Boyd B , Lombardi Antonio

Odanacatib (ODN), a selective oral inhibitor of cathepsin K, is in development for the treatment of osteoporosis. In the primary efficacy analysis of the Phase 3, Long-Term ODN Fracture Trial (LOFT; NCT00529373), ODN significantly reduced fracture risk compared with placebo in postmenopausal women with osteoporosis. Pre-specified subgroup analyses evaluated the efficacy of ODN in patient subgroups.Women aged ≥65 years, without baseline radiographic...

ba0001pp21 | Arthritis and other joint diseases: translational and clinical | ECTS2013

Monosodium urate crystals inhibit tenocyte viability and function: implications for periarticular involvement in chronic gout

Chhana Ashika , Callon Karen , Dray Michael , Pool Bregina , Naot Dorit , Gamble Greg , Coleman Brendan , McQueen Fiona , Cornish Jillian , Dalbeth Nicola

Background: In patients with gout, urate deposition has been observed both adjacent to and within tendons, suggesting that monosodium urate monohydrate (MSU) crystals are likely to be in direct contact with tenocytes, the stromal cells of tendons. The aim of this study was to determine the effects of MSU crystals on tenocyte viability and function.Methods: Cultures of primary rat tenocytes were prepared from Wistar rat tails. Primary human tenocytes were...