Searchable abstracts of presentations at key conferences on calcified tissues

ba0001pp178 | Cell biology: osteoblasts and bone formation | ECTS2013

Regulation and function of immunosuppressive molecule human leukocyte antigen G5 in human bone tissue

Deschaseaux Frederic , Gaillard Julien , Langonne Alain , Chauveau Christophe , Naji Abderrahim , Bouacida Amina , Rosset Philippe , Heymann Dominique , de Pinieux Gonzague , Rouas-Freiss Nathalie , Sensebe Luc

Bone-marrow mesenchymal stem cells (MSCs) are the origin of bone-forming cells with immunomodulation potential. Among the generated immunosuppressive molecules there is HLA-G5. HLA-G proteins play a crucial role in promoting the acceptance of allografts. However, the mechanisms regulating the expression of HLA-G5 in human MSCs are unknown. We induced differentiation of human MSCs (harvested from iliac crests of healthy volunteers after their informed consent following approved...

ba0001pp451 | Osteoporosis: treatment | ECTS2013

Estimation of vertebral and femoral strength during the first three years of denosumab therapy using an alternative smooth non-linear finite element methodology

Zysset Philippe , Pahr Dieter , Engelke Klaus , Genant Harry , McClung Michael , Kendler David , Recknor Christopher , Kinzl Michael , Schwiedrzik Jakob , Museyko Oleg , Wang Andrea , Libanati Cesar

Denosumab subcutaneous administration every 6 months reduced the incidence of new fractures in postmenopausal women with osteoporosis by 68% at the spine and 40% at the hip over 36 months compared with placebo in the FREEDOM study (Cummings et al., NEJM, 2009:361:756). This efficacy was supported by differential improvements from baseline in vertebral and femoral strength at 36 months (18.2 and 8.6%, respectively) estimated by an established voxel-based finit...

ba0002p70 | (1) | ICCBH2013

Isolated bilateral zeugo-autopodal segments agenesis of the lower limb: unusual malformation case report

Christiaens Antoine , Deprez Pierre M L , Mendola Antonella , Bernard Pierre , Gillerot Yves , Clapuyt Philippe , Lengele Benoit G , Vikkula Miikka , Nyssen-Behets Catherine

: Congenital limb abnormalities represent a prevalence of 0.79/1000 of live births in Massachusetts1. A better understanding of their physiopathology could improve the management of the patients. We report on a 23 weeks female fetus affected by an isolated bilateral terminal transverse defect of the lower limbs with nubbins. Both familial history and chromosomal analyses were irrelevant. We performed a deep morphological examination of the fetus in comparison with a...

ba0004p71 | (1) | ICCBH2015

Muscle and bone impairment in children with Marfan syndrome: correlation with age and FBN1 genotype

Haine Elsa , Tauber Maithe , Van Kien Philippe Khau , Auriol Francoise , Gennero Isabelle , Julia Sophie , Dulac Yves , Salles Jean-Pierre , Edouard Thomas

Background: Marfan syndrome (MFS) is a rare connective tissue disorder caused by mutation in the gene encoding the extracellular matrix protein fibrillin-1 (FBN1), leading to transforming growth factor-beta (TGF-β) signaling dysregulation. Although decreased axial and peripheral bone mineral density (BMD) has been reported in adults with MFS, data about the evolution of bone mass during childhood and adolescence are limited.Objectives: The aim of th...

ba0005cabs.oc2.2 | Oral Communications | ECTS2016

Integrin α5 is an independent prognosis factor and a potential therapeutic target for breast cancer bone metastasis

Pantano Francesco , Croset Martine , Driouch Keltouma , Bonnelye Edith , Bednarz-Knoll Natalia , Hong Saw-See , Iuliani Michele , Fioramonti Marco , Santini Daniele , Tonini Giuseppe , Pantel Klaus , Clezardin Philippe

Aims: Using an extensive bioinformatic approach we identified integrin α5 subunit as a novel potential target to treat bone dissemination from breast cancer. Aim of this study is to confirm the value of this target.Methods: Integrin α5 mRNA expression levels were quantified by qRT-PCR, using radically resected primary tumors of 427 breast cancer patients. α5 expression at protein level by IHC on primary tumor was correlated, in an addition...

ba0005p131 | Cancer and bone: basic, translational and clinical | ECTS2016

MicroRNA-30 family regulates bone tropism and osteomimicry in human breast cancer cells

Martine Croset , Francesco Pantano , Casina Kan , Edith Bonnelye , Catherine Alix-Panabieres , Charles Lecellier , Saw-See Hong , Kai Bartkowiak , Klaus Pantel , Philippe Clezardin

Breast cancer cells that escape primary tumors disseminate to bone with high affinity at late-stage disease. Circulating breast tumor cells that invade the bone marrow (BM) express sets of deregulated genes and/or microRNAs (miRNAs) that facilitate their bone tropism and enhance engraftment of disseminated tumor cell (DTC) in BM which may subsequently induce osteolytic lesions. MiRNA transcriptomic profiling of the human breast cancer DTC cell line, BC-M1, and of the osteotrop...

ba0001pp138 | Cancer and bone: basic, translational and clinical | ECTS2013

New chondrosarcoma cell lines and mouse models to study the link between chondrogenesis and chemoresistance

Monderer David , Luseau Alexandrine , Bellec Amelie , David Emmanuelle , Ponsolle Stephanie , Saiagh Soraya , Bercegeay Sylvain , Piloquet Philippe , Denis Marc , Lode Laurence , Redini Francoise , Biger Marine , Heymann Dominique , Heymann Marie-Francoise , Bot Ronan Le , Gouin Francois , Blanchard Frederic

Chondrosarcomas are cartilage-forming, poorly vascularized tumors. With an estimated annual incidence of 1 in 200 000, they represent the second malignant primary bone tumor of adults after osteosarcoma. These tumors are resistant to chemotherapy and radiotherapy, surgical excision remaining the only therapeutic option. However, very few cell lines and animal models are available, and the mechanisms behind their chemoresistance remain largely unknown. Our goal was to establish...

ba0005cabs.oc1.2 | Oral Communications | ECTS2016

Bone and metabolic parameters are associated with overall survival in patients with bone metastases from adenocarcinoma lung cancer: the POUMOS study

Confavreux Cyrille B. , Chambard Lauriane , Ollier Edouard , Brevet Marie , Duboeuf Francois , Rousseau Jean-Charles , Pialat Jean-Baptiste , Wegrzyn Julien , Tescaru Agnes , Szulc Pawel , Carlier Marie-Christine , Girard Nicolas , Clezardin Philippe

Rational: Mortality due to non-small cell lung cancers is the first cause of cancer death in men around the world. Lung adenocarcinoma regularly induces bone metastases responsible for high morbidity and impaired life quality. Overall survival of these patients is poor. Thus we aimed to identify if some bone and metabolic parameters were associated with overall survival.Patients and Methods: POUMOS is a prospective cohort of patients suffering from adeno...

ba0007p51 | (1) | ICCBH2019

Higher dose of burosumab is needed for treatment of children with severe forms of X-linked hypophosphatemia

Zhukouskaya Volha V , Audrain Christelle , Lambert Anne-Sophie , Colao Annamaria , Kamenicky Peter , Adamsbaum Catherine , Nevoux Jerome , Chaussain Catherine , Wicart Philippe , Briot Karine , Di Rocco Federico , Trabado Severine , Prie Dominique , Rothenbuhler Anya , Linglart Agnes

Background and aim: Burosumab is a monoclonal antibody against anti-FGF23, which has been recently approved for treatment of X-linked hypophosphatemia (XLH). Beyond clinical trials, little is known about its efficacy/safety in clinical practice which is the aim of study.Patients/Methods: 39 children with XLH were switched from conventional therapy to burosumab (starting dose 0.4 mg/kg), because of following indications: non-responder to conventional ther...