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Abstracts tagged "Systemic sclerosis"

  • Abstract Number: 3011 • 2015 ACR/ARHP Annual Meeting

    The Homeoprotein Engrailed-1 Regulates Canonical TGF Beta Signaling in Experimental Systemic Sclerosis

    Tatjana Mallano1, Katrin Palumbo-Zerr1, Christian Beyer1,2, Clara Dees1, Jingang Huang1, Thomas Wohlfahrt1, Oliver Distler3, Georg Schett1 and Jeorg HW Distler1,2, 1Department of Internal Medicine 3 and Institute for Clinical Immunology, University of Erlangen-Nuremberg, Erlangen, Germany, 2Department of Internal Medicine 3, University of Erlangen-Nuremberg, Erlangen, Germany, 3Department of Rheumatology, Center of Experimental Rheumatology and Zurich Center of Integrative Human Physiology, University Hospital Zurich, Zurich, Switzerland

     Background/Purpose: Systemic sclerosis (SSc) is a chronic fibrotic connective disease of unknown etiology that affects the skin and internal organs. Transforming growth factor-β (TGFβ) has…
  • Abstract Number: 3130 • 2015 ACR/ARHP Annual Meeting

    Epstein-Barr Virus Induces Activation of Infammatory Markers Via the TLR8 Transduction Pathway in Infected Scleroderma Monocytes

    Antonella Farina1, Giovanna Peruzzi2,3, Valentina Lacconi4, Edoardo Rosato5, Silvia Quarta6, Stefania Morrone1, Alberto Faggioni1, Maria Trojanowska4 and Giuseppina Alessandra Farina4, 1Department of Experimental Medicine, Sapienza University, Department of Experimental Medicine, Rome, Italy, 2Istituto Italiano di Tecnologia (IIT), CLNS@Sapienza, Rome, Italy, 3Istituto Italiano di Tecnologia (IIT), CLNS@Sapienza, Viale Regina Elena 291, 00161, Rome, Italy, Rome, Italy, 4Arthritis Center, Boston University, Arthritis Center, Boston, MA, 5Department of Clinical Medicine, Clinical Immunology Unit,, Sapienza University,Department of Clinical Medicine, Rome, Italy, 6Department of Clinical Medicine, Clinical Immunology Unit., Sapienza University,Department of Clinical Medicine, Rome, Italy

    Background/Purpose: Monocytes from patients with systemic sclerosis (Scleroderma, SSc), are characterized by the increased expression of IFN-regulatory genes, implicating dysregulation of the innate immune response…
  • Abstract Number: 3135 • 2015 ACR/ARHP Annual Meeting

    Incidences and Predictors of Organ Manifestations in the Early Course of Systemic Sclerosis: A Longitudinal Study

    Veronika K. Jaeger1, Elina G. Wirz2,3, Yannick Allanore4, Gabriela Riemekasten5, Eric Hachulla6, Oliver Distler7, Paolo Airo8, Patricia E. Carreira9, Alexandra Balbir-Gurman10, Mohammed Tikly11, Serena Vettori12, Nemanja Damjanov13, Ulf Müller-Ladner14, Jorg HW. Distler15, Mangtao Li16, Ulrich A. Walker17 and EUSTAR collaborators, 1Department of Rheumatology, University Hospital Basel, Basel, Switzerland, 2Department of Rheumatology, University Hospital Basel, Basel, Switzerland, Basel, Switzerland, 3Department of Dermatology, University Hospital Basel, Basel, Switzerland, 4Department of Rheumatology A, Paris Descartes University, Cochin Hospital, Paris, France, 5Clinic of Rheumatology, University of Luebeck, Lübeck, Germany, 6Service de Médecine Interne, Hôpital Huriez, Université de Lille, Lille, France, 7Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland, 8UO Reumatologia ed Immunologia Clinica, Spedali Civili, Brescia, Italy, 9Servicio de Reumatologia, Hospital Universitario 12 de Octubre, Madrid, Spain, 10B. Shine Rheumatology Unit, Rambam Health Care Campus, Rappaport Faculty of Medicine, Technion - Institute of Technology, Haifa, Israel, 11Division of Rheumatology, Chris Hani Baragwanath Academic Hospital, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa, 12Department of Internal and Experimental Medicine, Rheumatology Unit, Second University of Naples, Naples, Italy, 13Institute of Rheumatology, University of Belgrade Medical School, Belgrade, Serbia, 14Internal Medicine and Rheumatology, Justus-Liebig-University of Giessen, Kerckhoff-Klinik, Bad Nauheim, Germany, 15Department of Internal Medicine 3, University of Erlangen-Nuremberg, Erlangen, Germany, 16Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China, 17Rheumatology, University Hospital, Basel, Basel, Switzerland

    Background/Purpose: Systemic sclerosis (SSc) is a rare and clinically heterogeneous autoimmune disorder characterised by fibrosis and microvascular obliteration of the skin and internal organs, particularly…
  • Abstract Number: 412 • 2015 ACR/ARHP Annual Meeting

    Update on the Juvenile Systemic Sclerosis Inception Cohort Project. Characteristics of the First 50 Patients at First Assessment. Www.Juvenile-Scleroderma.Com

    Ivan Foeldvari1, Jens Klotsche2, Valda Staņeviča3, Ekaterina Alexeeva4, Maria M. Katsicas5, Maria Teresa Terreri6, Ana Paula Sakamoto7, Rolando Cimaz8, Mikhail Kostik9, Tadey Avcin10, Maria Jose Santos11, Monika Moll12, Dana Nemkova13, Flavio Sztajnbok14, Cristina Battagliotti15, Juergen Brunner16, Despina Eleftheriou17, Alberto Sifuentes Giraldo18, Liora Harel19, Mahesh Janarthanan20, Tilmann Kallinich21, Kirsten Minden22, Susan Mary Nielsen23, Kathryn S. Torok24, Yosef Uziel25 and Nicola Helmus26, 1Hamburger Zentrum für Kinder-und Jugendrheumatologie, Hamburg, Germany, 2Epidemiology unit, German Rheumatism Research Center, Berlin, Germany, 3Pediatric cathedra, Riga Stradiņš University, Riga, Latvia, 4Rheumatology, Scientific Center of Children's Health, Moscow, Russia, 5Immunology & Rheumatology., Hospital de Pediatrìa Garrahan, Buenos Aires, Argentina, 6Pediatrics, Universidade Federal de Sao Paulo, São Paulo, Brazil, 7Assistant doctor, Pediatric Rheumatology Unit, Universidade Federal de São Paulo, São Paulo, Brazil, 8Department of Paediatrics, University of Florence and Anna Meyer Children's Hospital, Florence, Italy, Florence, Italy, 9State Pediatric Medical University, Saint-Petersburg, Russia, 10University Children's Hospital, Ljubljana, Slovenia, 11Rheumatology, Hospital Garcia de Orta, Almada, Portugal, 12Pediatric Rheumatology, University Childrenhospital, Tübingen, Germany, 13Pediatric Rheumatology Unit, Department of Pediatrics and Adolescent Medicine, General University Hospital in Prague, Prague, Czech Republic, 14Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil, 15Hospital de Niños Dr Orlando Alasia, Santa Fé, Argentina, 16Universitätsklinik für Kinder- u. Jugendheilkunde, Innsbruck, Austria, 17Paediatric Rheumatology Department, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom, 18Department of Rheumatology, University Hospital Ramón y Cajal, Madrid, Spain, 19Pediatric Rheumatology Unit, Schneider Children's Medical Center,Tel Aviv University, Petach Tikvah, Israel, 20Pediatric Rheumatology, Chennai, India, 21Charite, University Medicine Berlin, Berlin, Germany, 22Chidlrens´ hospital, Charité University Medicine, Berlin, Germany, 23Rigshospitalet, Copenhagen, Denmark, 24Pediatric Rheumatology, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA, 25Meir Medical Center, Kfar Saba, Israel, 26Hamburg Centre for Pediatric and Adolescent Rheumatology, Hamburg, Germany

    Background/Purpose: Juvenile systemic sclerosis (jSSc) is an orphan autoimmune disease. Currently just retrospective data exist regarding evolvement of organ involvement. In the previous retrospective studies…
  • Abstract Number: 413 • 2015 ACR/ARHP Annual Meeting

    Is There a Difference in the Presentation of Diffuse and Limited Subtype of Juvenile Systemic Sclerosis in Childhood? Results from the Juvenile Scleroderma Inception Cohort Www.Juvenile-Scleroderma.Com

    Ivan Foeldvari1, Jens Klotsche2, Valda Stanevicha3, Maria M. Katsicas4, Maria Teresa Terreri5, Ana Paula Sakamoto6, Rolando Cimaz7, Mikhail Kostik8, Tadey Avcin9, Maria Jose Santos10, Monika Moll11, Dana Nemkova12, Flavio Sztajnbok13, Cristina Battagliotti14, Juergen Brunner15, Despina Eleftheriou16, Alberto Sifuentes Giraldo17, Liora Harel18, Mahesh Janarthanan19, Tilmann Kallinich20, Kirsten Minden21, Susan Mary Nielsen22, Kathryn S. Torok23, Yosef Uziel24 and Nicola Helmus25, 1Hamburger Zentrum für Kinder-und Jugendrheumatologie, Hamburg, Germany, 2Epidemiology unit, German Rheumatism Research Center, Berlin, Germany, 3Pediatric cathedra, University Childrens Hospital, Riga, Latvia, 4Service of Immunology & Rheumatology. Hospital de Pediatrìa Prof Dr.Juan.P. Garrahan, MD, Buenos Aires, Argentina, 5Pediatrics, Universidade Federal de Sao Paulo, São Paulo, Brazil, 6Assistant doctor, Pediatric Rheumatology Unit, Universidade Federal de São Paulo, São Paulo, Brazil, 7Department of Paediatrics, University of Florence and Anna Meyer Children's Hospital, Florence, Italy, Florence, Italy, 8State Pediatric Medical University, Saint-Petersburg, Russia, 9University Children's Hospital, Ljubljana, Slovenia, 10Rheumatology, Hospital Garcia de Orta, Almada, Portugal, 11Pediatric Rheumatology, University Childrenhospital, Tübingen, Germany, 12Pediatric Rheumatology Unit, Department of Pediatrics and Adolescent Medicine, General University Hospital in Prague, Prague, Czech Republic, 13Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil, 14Hospital de Niños Dr Orlando Alasia, Santa Fé, Argentina, 15Universitätsklinik für Kinder- u. Jugendheilkunde, Innsbruck, Austria, 16Paediatric Rheumatology Department, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom, 17Department of Rheumatology, University Hospital Ramón y Cajal, Madrid, Spain, 18Pediatric Rheumatology Unit, Schneider Children's Medical Center,Tel Aviv University, Petach Tikvah, Israel, 19Pediatric Rheumatology, Chennai, India, 20Charite, University Medicine Berlin, Berlin, Germany, 21Chidlrens´ hospital, Charité University Medicine, Berlin, Germany, 22Rigshospitalet, Copenhagen, Denmark, 23Pediatric Rheumatology, Scleroderma Center of Pittsburgh, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA, 24Meir Medical Center, Kfar Saba, Israel, 25Hamburg Centre for Pediatric and Adolescent Rheumatology, Hamburg, Germany

    Background/Purpose: Juvenile systemic sclerosis (jSSc) is an orphan autoimmune disease. Several publications in adults looked at the differences between limited and diffuse subtypes. There is…
  • Abstract Number: 414 • 2015 ACR/ARHP Annual Meeting

    Performance of the Adult Systemic Sclerosis Classification in Juvenile Systemic Sclerosis Patients. Results from the Juvenile Systemic Sclerosis Inception Cohorte Www.Juvenile-Scleroderma.Com

    Ivan Foeldvari1, Jens Klotsche2, Valda Stanevicha3, Ekaterina Alexeeva4, Maria M. Katsicas5, Maria Teresa Terreri6, Ana Paula Sakamoto7, Rolando Cimaz8, Mikhail Kostik9, Tadey Avcin10, Maria Jose Santos11, Monika Moll12, Dana Nemkova13, Flavio Sztajnbok14, Cristina Battagliotti15, Juergen Brunner16, Despina Eleftheriou17, Alberto Sifuentes Giraldo18, Liora Harel19, Mahesh Janarthanan20, Tilmann Kallinich21, Kirsten Minden22, Susan Mary Nielsen23, Kathryn S. Torok24, Yosef Uziel25 and Nicola Helmus26, 1Hamburger Zentrum für Kinder-und Jugendrheumatologie, Hamburg, Germany, 2Epidemiology unit, German Rheumatism Research Center, Berlin, Germany, 3University Childrens Hospital, Riga, Latvia, 4Rheumatology, Scientific Center of Children's Health, Moscow, Russia, 5Hospital de Pediatria, Buenos Aires, Argentina, 6Pediatrics, Universidade Federal de Sao Paulo, São Paulo, Brazil, 7Assistant doctor, Pediatric Rheumatology Unit, Universidade Federal de São Paulo, São Paulo, Brazil, 8Pediatric Rheumatology, Anna Meyer Children's Hospital, Florence, Italy, 9State Pediatric Medical University, Saint-Petersburg, Russia, 10University Children's Hospital, Ljubljana, Slovenia, 11Rheumatology, Hospital Garcia de Orta, Almada, Portugal, 12Pediatric Rheumatology, University Childrenhospital, Tübingen, Germany, 13Pediatric Rheumatology Unit, Department of Pediatrics and Adolescent Medicine, General University Hospital in Prague, Prague, Czech Republic, 14Pediatric Rheumatology Division, Adolescent Health Care Unit, Universida de do Estado do Rio de Janeiro., Rio de Janeiro, Brazil, 15Hospital de Niños Dr Orlando Alasia, Santa Fé, Argentina, 16Universitätsklinik für Kinder- u. Jugendheilkunde, Innsbruck, Austria, 17Paediatric Rheumatology Department, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom, 18Department of Rheumatology, University Hospital Ramón y Cajal, Madrid, Spain, 19Pediatric Rheumatology Unit, Schneider Children's Medical Center,Tel Aviv University, Petach Tikvah, Israel, 20Pediatric Rheumatology, Chennai, India, 21Charite, University Medicine Berlin, Berlin, Germany, 22Epidemiology, Charite, DRFZ, Berlin, Germany, 23Rigshospitalet, Copenhagen, Denmark, 24Pediatric Rheumatology, Scleroderma Center of Pittsburgh, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA, 25Meir Medical Center, Kfar Saba, Israel, 26Hamburg Centre for Pediatric and Adolescent Rheumatology, Hamburg, Germany

    Background/Purpose: The new adult classifiction criteria (1) for Systemic Sclerosis (SSc) were recently published and the pediatric criteria were published in 2007 (2). None of…
  • Abstract Number: 824 • 2015 ACR/ARHP Annual Meeting

    Familial Risk of Systemic Sclerosis and Co-Aggregation of Autoimmune Diseases in Affected Families: A Nationwide Population Study

    Chang-Fu Kuo1, Matthew J. Grainge2, Ana Valdes3, Shue-Fen Luo4, Lai-Chu See5, Kuang-Hui Yu6, Weiya Zhang7 and Michael Doherty8, 1Division of Rheumatoplogy, Allergy and Immunology, Chang Gung Memorial Hospital, Tayuan, Taiwan, 2Division of Epidemiology and Public Health, School of Community Health Sciences, University of Nottingham, Nottingham, United Kingdom, 3Division of Rheumatology, Orthopaedics and Dermatology, University of Nottingham, Nottingham, United Kingdom, 4Rheumatology, Chang Gung Memorial Hosp, Taipei, Taiwan, 5Biostatistics Core Laboratory, Molecular Medicine Research Center, Chang Gung University, Taoyuan, Taiwan, 6Division of Rheumatoplogy, Allergy and Immunology, Chang Gung Memoria Hospital, Tayuan, Taiwan, 7Academic Rheumatology, School of Clinical Sciences, University of Nottingham, Nottingham, United Kingdom, 8Academic Rheumatology, City Hospital, Nottingham, United Kingdom

    Background/Purpose: To investigate familial aggregation of systemic sclerosis and the relative risks (RRs) of other autoimmune disease in relatives of affected patients. Methods: We identified…
  • Abstract Number: 2708 • 2014 ACR/ARHP Annual Meeting

    Post-Occlusive Reactive Hyperemia (PORH) Test Mirrors Vascular Changes in Systemic Sclerosis (SSc):a Laser Speckle Contrast (LASCA) Study

    Alessandra Della Rossa1, Anna d'Ascanio2, Massimo Cagnoni1, Chiara Stagnaro3, Alice Parma4, Marta Mosca5 and Stefano Bombardieri6, 1malattie muscoloscheletriche e cutanee, Rheumatology Unit, Pisa, Italy, 2Malattie muscolo-scheletriche e cutanee, Rheumatology Unit, Pisa, Italy, 3Malattie scheletriche e muscolocutanee, Rheumatology Unit, Pisa, Italy, 4University of Pisa, Rheumatology Unit, Pisa, Italy, 5Rheumatology Unit, University of Pisa, Pisa, Italy, 6Rheumatology Unit, Pisa, Italy

    Background/Purpose Vascular involvement is a key feature of Systemic sclerosis (SSc) and involves both the micro and macrovasculature. Vascular changes are central in the disease’s…
  • Abstract Number: 2706 • 2014 ACR/ARHP Annual Meeting

    Ulnar and Radial Stenosis in Systemic Sclerosis

    María Eugenia Lara1, Mariano Rivero1, Julia Romero1, Guadalupe Palacios2, Ignacio Carrillo3, Claudia L. Giraldo4, Amalia Schiel5, Hugo Armando Laborde1, Marina Khoury6, Saez Diego7, Gustavo Citera8, Oscar L. Rillo9 and Juan C. Barreira1, 1Rheumatology, British Hospital, Buenos Aires, Argentina, 2British Hospital, Buenos Aires, Argentina, 3Rheumatology section, Instituto de Rehabilitación Psicofísica, Buenos Aires, Argentina, 4Rheumatology, Hospital Gral. de Agudos Dr. E. Tornú, Buenos Aires, Argentina, 5Central Laboratory, British Hospital, Buenos Aires, Argentina, 6Teaching and Research, British Hospital, Buenos Aires, Argentina, 7Imaging, British Hospital, Buenos Aires, Argentina, 8Instituto de Rehabilitación Psicofísica, Buenos Aires, Argentina, 9Rheumatology Unit, Hospital General de Agudos Dr. E. Tornú, Buenos Aires, Argentina

    Background/Purpose: Systemic sclerosis (SSc) is a chronic, autoimmune disease. Endothelial damage has been recognized as the initial pathogenic factor. The involvement of the microvasculature is…
  • Abstract Number: 1724 • 2014 ACR/ARHP Annual Meeting

    Loss of IRF5 Ameliorates Tissue Fibrosis in a Murine Model of Systemic Sclerosis

    Ryosuke Saigusa1, Yoshihide Asano1, Takashi Taniguchi1, Yohei Ichimura1, Takehiro Takahashi1, Tetsuo Toyama1, Ayumi Yoshizaki1, Tadatsugu Taniguchi2 and Shinichi Sato1, 1Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan, 2Molecular Immunology, Institute of Industrial Science, University of Tokyo, Tokyo, Japan

    Background/Purpose Systemic sclerosis (SSc) is a multisystem autoimmune disorder with clinical manifestations that result from fibrosis development, immune activation and vascular injuries. A genome-wide association…
  • Abstract Number: 1715 • 2014 ACR/ARHP Annual Meeting

    Serum Levels of CD163/Tweak Predict Risk of Digital Ulcers in Patients with Systemic Sclerosis

    Otylia M. Kowal-Bielecka1, Marek Bielecki2, Beata Trzcinska-Butkiewicz3, Malgorzata Michalska-Jakubus4, Marek Brzosko3, Dorota Krasowska4 and Krzysztof Kowal5, 1Department of Rheumatology and Internal Medicine, Medical University in Bialystok, Bialystok, Poland, 2Department of Orthopedics and Traumatology, Medical University of Bialystok, Bialystok, Poland, 3Department of Rheumatology and Internal Diseases, Pomeranian Medical University, Szczecin, Poland, 4Department of Dermatology, Venereology and Pediatric Dermatology, Medical University of Lublin, Lublin, Poland, 5Department of Allergology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland

    Background/Purpose: TNF-like weak inducer of apoptosis (TWEAK) regulates inflammation, angiogenesis and tissue remodeling. CD163 molecule, a scavenger receptor and marker of alternatively activated macrophages, modulates…
  • Abstract Number: 766 • 2014 ACR/ARHP Annual Meeting

    Endothelial Fli1 Deficiency Delays Wound Healing Due to Impaired Anastomosis of Newly Formed Vessels – a Possible Mechanism of Refractory Skin Ulcers in Systemic Sclerosis

    Yoshihide Asano1 and Maria Trojanowska2, 1Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan, 2Arthritis Center, Boston University, Boston, MA

    Background/Purpose Systemic sclerosis (SSc) is a multisystem inflammatory and vascular disease resulting in fibrosis of the skin and certain internal organs. Although the pathogenesis of…
  • Abstract Number: 740 • 2014 ACR/ARHP Annual Meeting

    Right Ventricular Diastolic Impairment Is Common in Systemic Sclerosis and Is a Marker of Several Organ-Target Damage of the Disease

    Christophe Meune1, Dinesh Khanna2, Jamil Aboulhosn3, Jerome Avouac4, Andre Kahan5, Daniel E. Furst6 and Yannick Allanore4, 1Paris 13 University, University Hospital of Paris-Seine-Saint-Denis, Cardiology Department, Bobigny, France, 2University of Michigan Health System, Ann Arbor, MI, 3Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA, 4Paris Descartes University, Rheumatology A Department and INSERM U1016, Cochin Hospital, Paris, France, 5Paris Descartes University, Rheumatology A department, Cochin Hospital, Paris, France, 6University of California, Los Angeles, Department of Medicine, Los Angeles, CA

    Background/Purpose Heart failure and cardiac dysfunctions both of intrinsic or secondary origin and targeting LV (left ventricule) and/or RV (right ventricule) are critical complications promoting…
  • Abstract Number: 748 • 2014 ACR/ARHP Annual Meeting

    Increased Expression of Chemerin in Endothelial Cells Due to Fli1 Deficiency May Contribute to the Development of Digital Ulcers in Systemic Sclerosis

    Kaname Akamata, Yoshihide Asano, Takashi Taniguchi, Hayakazu Sumida, Naohiko Aozasa, Shinji Noda, Tetsuo Toyama, Takehiro Takahashi, Yohei Ichimura, Ayumi Yoshizaki and Shinichi Sato, Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan

    Background/Purpose: Systemic sclerosis (SSc) is a multisystem autoimmune disorder characterized by vascular injuries and fibrosis development. In SSc lesional skin, transcription factor Friend leukemia virus…
  • Abstract Number: 721 • 2014 ACR/ARHP Annual Meeting

    Muscle Disease in Systemic Sclerosis Is Associated with an Increased Risk for Cardiac Involvement

    Jison Hong1, Antonia Valenzuela2, David Fiorentino3 and Lorinda Chung4, 1Medicine- Immunology & Rheumatology, Stanford University School of Medicine, Palo Alto, CA, 2Division of Immunology and Rheumatology, Stanford University School of Medicine, Palo Alto, CA, 3Dermatology, Stanford University, Redwood City, CA, 4Immunology and Rheumatology, Stanford University School of Medicine, Palo Alto, CA

    Background/Purpose Patients with systemic sclerosis (SSc) and muscle involvement (myopathy/myositis) have more severe disease and worse outcomes. We sought to determine the prevalence of muscle…
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