Searchable abstracts of presentations at key conferences on calcified tissues

ba0003pp113 | Cell biology: osteoblasts and bone formation | ECTS2014

Bone-anabolic effects of sulforaphane, a naturally occurring isothiocyanate on bone metabolism

Thaler Roman , Dudakovic Amel , Rucci Nadia , Maurizi Antonio , Sturmlechner Ines , Spitzer Silvia , Rumpler Monika , Van Wijnen Andre J , Teti Anna , Klaushofer Klaus , Varga Franz

Few drugs generate bone-stimulatory effects via epigenetic mechanisms. Modulation of CpG-residues hydroxymethylation in gene-promoters of key osteoblast-related factors (e.g., DLX5) induces their expression and increases osteoblast differentiation in vitro. The chemical properties of sulforaphane (SFN), a natural compound abundantly present in cruciferous vegetables like broccoli, suggest that it may have similar molecular and biological effects. Previous stu...

ba0005ni9 | Abstract Presentations | ECTS2016

Inflammatory conditions induces a new subset of osteoclasts that prime TNFα-producing CD4+ T cells

Ibanez Lidia , Abou-Ezzi Grazia , Ciucci Thomas , Amiot Vanessa , Mansour Anna , Roleau Matthieu , Wakkach Abdelilah , Blin-Wakkach Claudine

Chronic inflammatory diseases are characterized by a bone destruction mediated by an increased osteoclast (OCL) activity. OCLs are phagocytic cells arising from the myeloid lineage. Indeed, OCLs derive from monocytes (MN-OCLs) and, in an inflammatory context, they also derive from dendritic cells (DC-OCLs). Despite this origin, their role in the immune responses is still unclear. OCLs in steady state have been reported to act as antigen-presenting cells that activate CD8+...

ba0005p186 | Cell biology: osteoclasts and bone resorption | ECTS2016

Inflammatory conditions induces a new subset of osteoclasts that prime TNFα-producing CD4+T cells

Ibanez Lidia , Abou-Ezzi Grazia , Ciucci Thomas , Amiot Vanessa , Mansour Anna , Roleau Matthieu , Wakkach Abdelilah , Blin-Wakkach Claudine

Chronic inflammatory diseases are characterized by a bone destruction mediated by an increased osteoclast (OCL) activity. OCLs are phagocytic cells arising from the myeloid lineage. Indeed, OCLs derive from monocytes (MN-OCLs) and, in an inflammatory context, they also derive from dendritic cells (DC-OCLs). Despite this origin, their role in the immune responses is still unclear. OCLs in steady state have been reported to act as antigen-presenting cells that activate CD8+ regu...

ba0005p407 | Osteoporosis: treatment | ECTS2016

Impact of 3-year vitamin D and calcium supplementation on mineral and organic matrix formation of trabecular bone in postmenopausal osteoporosis

Paschalis E P , Gamsjaeger S , Hassler N , Fahrleitner-Pammer A , Dobnig H , Stepan J J , Eriksen E F , Klaushofer K

Clinical trials involving drug therapies for postmenopausal osteoporosis typically compare effects of the active drug combined with vitamin D (vit D) and calcium (Ca) vs vit D and Ca supplementation on its own. Bone strength is estimated based on the amount of bone, frequently expressed as bone mineral density determined by dual X-ray absorptiometry, and quality of bone, hardly measured in clinical practice.The purpose of the present study was to compare...

ba0005p409 | Osteoporosis: treatment | ECTS2016

Atypical femur fracture in an adolescent with X-linked osteoporosis based on PLS3 mutation

van de Laarschot Denise M , Zillikens M Carola

Background: Long-term use of bisphosphonates has raised concerns about the association with Atypical Femur Fractures (AFFs) that have been reported mainly in postmenopausal women.Clinical case: An 18-year-old patient with juvenile osteoporosis based on X-linked osteoporosis due to a PLS3 mutation developed a low trauma femoral fracture after seven years of intravenous and two years of oral bisphosphonate use, fulfilling the revised ASBMR diagnostic crite...

ba0001pp436 | Osteoporosis: treatment | ECTS2013

Bone mineral density changes in patients with prior fracture suboptimally treated with a bisphosphonate: results from denosumab (DMAb)/ibandronate and DMAb/risedronate trials

Recknor Christopher , Roux Christian , Ho Pei-Ran , Hall Jesse , Bone Henry , Bonnick Sydney , van den Bergh Joop , Ferreira Irene , Wagman Rachel , Brown Jacques P

: In osteoporosis, poor adherence to bisphosphonate (BP) therapy is common, and is associated with poor outcomes and increased treatment costs (Siris 2006; Recker 2005). Although compliance is improved with monthly vs weekly dosing (Reginster 2008), no evidence suggests cycling through BP agents offers therapeutic benefit, assessed by bone mineral density (BMD). In two randomized, open-label studies in postmenopausal women aged ≥55 years previously treated with, but subo...

ba0006p181 | (1) | ICCBH2017

Generalized arterial calcinosis of infancy: a case of a new mutation with central nervous system involvement and good response to bisphosphonates

Doulgeraki Artemis , Nika A. , Vakaki M. , Grigoriadou G. , Servos G. , Athanasopoulou H. , Katsieri K. , Kapetanakis I.

Background: Mutations in the ENPP1 gene have been identified in individuals with generalized arterial calcification of infancy (GACI), a life-threatening disorder characterized by calcification in the blood vessels, because of reduced availability of pyrophosphate. We describe a case of GACI due to a novel ENPP1 mutation.Presenting problem: The patient, born at term to non-consanguineous parents, was referred to us at birth with weak femoral pulses for e...

ba0004p101 | (1) | ICCBH2015

X-linked spinal muscular atrophy caused by de novo c.1731C>T substitution in the UBA1 gene

Jedrzejowska Maria , Kostera-Pruszczyk Anna , Jakubowska-Pietkiewicz Elzbieta

Infantile spinal muscular atrophy X-linked 2 (SMAX2) is a rare form of spinal muscular atrophy manifesting in severe hypotonia, areflexia, arthrogryposis, facial weakness and cryptorchidism, as frequently accompanied by bone fractures.We present a boy patient with SMAX2 who presented typical symptoms from birth, as preceded by reduced fetal movements in the second and third trimester of pregnancy. In the first days of life the patient was found to have s...

ba0001pp433 | Osteoporosis: treatment | ECTS2013

Denosumab is associated with progressive improvements in hip cortical mass and thickness

Poole K , Treece G , Gee A , Brown J P , McClung M R , Wang A , Libanati C

Denosumab (DMAb) significantly improves bone strength at the hip, estimated by FEA from QCT scans, from baseline (B/L) and vs placebo (Pbo) (Keaveny ASBMR 2010). We determined the extent and distribution of mass and thickness changes at the proximal femur, a key skeletal site for fracture risk, using a novel cortical bone mapping technique on the same serial QCT scans. A FREEDOM substudy included 80 women who underwent hip QCT scanning at B/L and months 12, 24 and 36 during DM...

ba0003pp331 | Osteoporosis: treatment | ECTS2014

Prevalence of osteoporosis treatment depending on the risk evaluation of the osteoporotic fractures in patients with rheumatoid arthritis in Russia

Dydykina Irina , Vetkova Elizaveta , Podvorotova Marina , Taskina E , Smirnov A , Sinenko A , Ruskina T , Peshekhonov D , Myasoedova S , Zavodovski B , Dydykina P , Petrova E , Zhigulin V , Alekseeva L , Nasonov E

Background: It is known that one of the features of rheumatoid arthritis (RA) is a generalized bone loss, therefore important tasks for the physician are timely evaluating of the risk of fractures and approving osteoporosis treatment in patients with RA.Objectives: Through the Programme of the Russian Association of Rheumatology for the diagnosis, prevention and treatment osteoporosis (OP) in patients with RA we evaluated the 10 year probability of major...