دورية أكاديمية
Thrombotic manifestations in pediatric Behcet syndrome: A multicenter comparative study from the EUROFEVER registry.
| العنوان: | Thrombotic manifestations in pediatric Behcet syndrome: A multicenter comparative study from the EUROFEVER registry. |
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| المؤلفون: | Mastrolia MV; Rheumatology Unit, ERN ReCONNET center, Meyer Children's Hospital IRCCS, Firenze, Italy; NEUROFARBA Department, University of Florence, Firenze, Italy., Matucci-Cerinic C; UOC Reumatologia e Malattie Autoinfiammatorie, IRCCS Istituto G. Gaslini, [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Genova, Italy., Ozen S; Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Türkiye., Kasapcopur O; Department of Pediatric Rheumatology, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Instanbul, Türkiye., Gaggiano C; Department of Medical Sciences, Surgery and Neurosciences, Rheumatology Unit, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Siena, Italy., Koné-Paut I; Department of Pediatric Rheumatology, Reference Centre for Autoinflammatory Disorders and inflammatory amyloidosis, CEREMAIA, Bicêtre Hospital, University of Paris Saclay, ERN RITA member, Paris, France., Cantarini L; Department of Medical Sciences, Surgery and Neurosciences, Rheumatology Unit, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Siena, Italy., Dusser P; Department of Pediatric Rheumatology, Reference Centre for Autoinflammatory Disorders and inflammatory amyloidosis, CEREMAIA, Bicêtre Hospital, University of Paris Saclay, ERN RITA member, Paris, France., Kaya-Akça Ü; Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Türkiye., Yildiz M; Department of Pediatric Rheumatology, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Instanbul, Türkiye., Brunner J; Department of Pediatrics, Innsbruck Medical University, Faculty of Medicine and Dentistry, Danube Private University, Innsbruck, Austria., Filocamo G; Pediatric Immunorheumatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy., Gallizzi R; Department of Medical of Health Sciences, Magna Graecia University, Catanzaro, Italy., Insalaco A; Division of Rheumatology, [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy., Pastore S; Institute for Maternal and Child Health, IRCCS 'Burlo Garofolo', Trieste, Italy., Rigante D; Department of Life Sciences and Public Health, Fondazione Policlinico Universitario 'A. Gemelli' IRCCS, Università Cattolica Sacro Cuore Rome, Italy., Sanchez-Manubens J; Pediatric Rheumatology, Department of Pediatrics, Parc Taulí Sabadell University Hospital, Institute for Research and Innovation I3PT, Sant Joan de Deu Hospital, Autonomous University of Barcelona, Barcelona, Spain., Tsitsami E; Pediatric Rheumatology Unit, 1st Department of Pediatrics, Children's Hospital 'Aghia Sophia', University of Athens, Athens, Greece., Ruperto N; UOC Reumatologia e Malattie Autoinfiammatorie, IRCCS Istituto G. Gaslini, [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Genova, Italy., Gattorno M; UOC Reumatologia e Malattie Autoinfiammatorie, IRCCS Istituto G. Gaslini, [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Genova, Italy. Electronic address: marcogattorno@gaslini.or., Simonini G; Rheumatology Unit, ERN ReCONNET center, Meyer Children's Hospital IRCCS, Firenze, Italy; NEUROFARBA Department, University of Florence, Firenze, Italy. |
| مؤلفون مشاركون: | EUROFEVER REGISTRY and Pediatric Rheumatology International trial organization (PRINTO) |
| المصدر: | Seminars in arthritis and rheumatism [Semin Arthritis Rheum] 2024 Jun; Vol. 66, pp. 152454. Date of Electronic Publication: 2024 Apr 23. |
| نوع المنشور: | Journal Article; Multicenter Study; Comparative Study |
| اللغة: | English |
| بيانات الدورية: | Publisher: W.B. Saunders Country of Publication: United States NLM ID: 1306053 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1532-866X (Electronic) Linking ISSN: 00490172 NLM ISO Abbreviation: Semin Arthritis Rheum Subsets: MEDLINE |
| أسماء مطبوعة: | Publication: Philadelphia Pa : W.B. Saunders Original Publication: New York, Stratton. |
| مواضيع طبية MeSH: | Behcet Syndrome*/complications , Behcet Syndrome*/epidemiology , Registries* , Thrombosis*/etiology , Thrombosis*/epidemiology, Humans ; Male ; Female ; Child ; Adolescent ; Retrospective Studies ; Europe/epidemiology ; Prevalence |
| مستخلص: | Introduction: Vascular events account for a considerable burden of morbidity and mortality in Behçet syndrome (BS). Thrombosis occurs in 1.8-21 % pediatric BS patients, even if the real prevalence is still largely unknown. Objectives: To report clinical features and outcomes of pediatric BS patients with thrombosis and to compare the demographic and clinical characteristics of BS patients with and without thrombosis. Methods: Retrospective data collection of BS patients with thrombosis (T+) included in the EUROFEVER registry. BS patients without thrombosis (T-), belonging to the same rheumatology units, were matched in a 2:1 ratio. Results: 37 T+ were compared to 74 T- patients. At onset, ICBD criteria fulfillment was higher in the T- group (p = 0.015). Caucasian patients were more often T-, Turkish patients were more frequent in T+ group (p = 0.002). At onset, pustulosis was most frequently observed in the T- (p < 0.001) as well as gastrointestinal symptoms (p < 0.001) and ocular involvement (p = 0.022). Neurological symptoms were more often described in T+ (p = 0.034). As for T+, thrombosis was reported at BS presentation in 8/37 (21.6 %). For the T + e patients who developed thrombosis later, oral aphthosis (p = 0.003), genital aphthosis (p = 0.014) were more frequently observed at BS onset, while pustulosis (p = 0.005) and fever (p = 0.043) coexisted with thrombosis. Thrombosis was mainly venous (26/37,70.3 %), involving the cerebral sinuses (21/37, 56.8 %). After thrombosis, 35/37 (94.6 %) T+ patients received an immunomodulatory treatment compared with 16/29 (55.2 %) pre-thrombosis. A recurrence was reported in 6/31(19.4 %). Conclusion: Thrombosis was reported at BS presentation in one fifth of cases. Pustolosis and fever were more frequently concomitant to thrombosis. Sinus veins were the most frequent site. Competing Interests: Declaration of competing interest All authors declare no conflicts of interest. (Copyright © 2024 Elsevier Inc. All rights reserved.) |
| فهرسة مساهمة: | Keywords: Behçet syndrome; Child; Thrombosis |
| تواريخ الأحداث: | Date Created: 20240428 Date Completed: 20240512 Latest Revision: 20240711 |
| رمز التحديث: | 20240712 |
| DOI: | 10.1016/j.semarthrit.2024.152454 |
| PMID: | 38678817 |
| قاعدة البيانات: | MEDLINE |
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Full Text Finder | تدمد: | 1532-866X |
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| DOI: | 10.1016/j.semarthrit.2024.152454 |