0000000000288688

AUTHOR

Jordi Anton

showing 10 related works from this author

Proceedings Of The 23Rd Paediatric Rheumatology European Society Congress: Part Two

2017

lcsh:Diseases of the musculoskeletal systemlcsh:RJ1-570lcsh:Pediatricslcsh:RC925-935Meeting Abstracts
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INSAID Variant Classification and Eurofever Criteria Guide Optimal Treatment Strategy in Patients with TRAPS: Data from the Eurofever Registry

2021

Contains fulltext : 231528.pdf (Publisher’s version ) (Closed access) BACKGROUND: TNF receptor-associated periodic syndrome (TRAPS) is a rare autoinflammatory disease caused by dominant mutation of the TNF super family receptor 1A (TNFRSF1A) gene. Data regarding long-term treatment outcomes are lacking. OBJECTIVE: To assess correlations of genotype-phenotypes in patients with TRAPS, as defined by the International Study Group for Systemic Autoinflammatory Diseases (INSAID) classification and Eurofever criteria, with treatment responses. METHODS: Data from 226 patients with variants of the TNFRSF1A gene and enrolled in the Eurofever registry were classified according to the INSAID classifica…

medicine.medical_specialtyAbdominal painAutoinflammatory diseasesGroup AGroup BAA amyloidosis Anakinra Autoinflammatory diseases Colchicine TRAPS Abdominal Pain Colchicine FemaleHumans Mutation Registries Hereditary Autoinflammatory Diseases03 medical and health sciences0302 clinical medicineSettore MED/38 - Pediatria Generale E SpecialisticaAA amyloidosisTNF receptor-associated periodic syndrome (TRAPS) ; TNFRSF1A geneInternal medicinemedicineAA amyloidosisHumansImmunology and AllergyIn patientRegistries030212 general & internal medicineLikely pathogenicAnakinrabusiness.industryHereditary Autoinflammatory DiseasesTRAPSmedicine.diseaseAbdominal PainAnakinra030228 respiratory systemTNF receptor associated periodic syndromeMutationFemalemedicine.symptombusinessColchicineAA amyloidosis; Anakinra; Autoinflammatory diseases; Colchicine; TRAPSInflammatory diseases Radboud Institute for Molecular Life Sciences [Radboudumc 5]medicine.drug
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Proceedings of the 23rd Paediatric Rheumatology European Society Congress: part one

2017

lcsh:Diseases of the musculoskeletal systemlcsh:RJ1-570lcsh:Pediatricslcsh:RC925-935Meeting Abstracts
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Two-year Efficacy and Safety of Etanercept in Pediatric Patients with Extended Oligoarthritis, Enthesitis-related Arthritis, or Psoriatic Arthritis.

2015

Objective.The main objective was to determine the 2-year clinical benefit and safety of etanercept (ETN) in children with the juvenile idiopathic arthritis (JIA) categories of extended oligoarthritis (eoJIA), enthesitis-related arthritis (ERA), or psoriatic arthritis (PsA).Methods.CLIPPER was a 96-week, phase IIIb, open-label, multicenter study. Patients with eoJIA, ERA, or PsA received ETN 0.8 mg/kg once weekly (50 mg max) for up to 96 weeks. The proportions of patients reaching the JIA American College of Rheumatology (ACR) 30/50/70/90/100 and inactive disease responses at Week 96 were calculated. Adverse events (AE) were collected throughout the study (intention-to-treat sample).Results.…

Malemedicine.medical_specialtyAdolescentEnthesitis-related arthritisImmunologyArthritisJuvenilePsoriaticEtanerceptEtanercept03 medical and health sciencesPsoriatic arthritis0302 clinical medicineRheumatologyInternal medicinemedicineImmunology and AllergyHumans030212 general & internal medicinePreschoolChild030203 arthritis & rheumatologyOligoarthritisbusiness.industryArthritisArthritis PsoriaticClinical trial; Enthesitis-related arthritis; Etanercept; Extended oligoarthritis; Juvenile idiopathic arthritis; Psoriatic arthritis; Adolescent; Antirheumatic Agents; Arthritis Juvenile; Arthritis Psoriatic; Child; Child Preschool; Etanercept; Female; Humans; Male; Treatment OutcomeJuvenile idiopathic arthritismedicine.diseaseRheumatologyPharyngitisArthritis Juvenile3. Good healthSurgeryClinical trialUpper respiratory tract infectionTreatment OutcomePsoriatic arthritisAntirheumatic AgentsChild PreschoolExtended oligoarthritisBronchitisFemalemedicine.symptombusinessmedicine.drugThe Journal of rheumatology
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The impact of the Eurofever criteria and the new Infevers MEFV classification in real life: results from a large international FMF cohort

2022

INTRODUCTION: New Eurofever/PRINTO classification criteria (EPCC) for Familial Mediterranean Fever (FMF) and other recurrent fevers have been recently developed, together with the classification of the pathogenicity of MEFV variants. OBJECTIVES: To evaluate the impact in real life of both the EPCC and INSAID pathogenicity classification of MEFV variants in the large international Eurofever FMF cohort. METHODS: Baseline demographic, genetic and clinical data of FMF patients included in the Eurofever registry were evaluated. The EPCC and the 2018 INSAID classification for MEFV variants were applied in all eligible FMF patients. RESULTS: Since November 2009, clinical information was available …

Male*Genetic analysis*Autoinflammatory diseasesPyrinFamilial Mediterranean fever*Classification criteriaCohort StudiesAnesthesiology and Pain MedicineRheumatologySettore MED/38 - PEDIATRIA GENERALE E SPECIALISTICAMutation*Familial mediterranean feverHumansFemale*RegistryRegistriesAutoinflammatory diseases Classification criteria Familial mediterranean fever Genetic analysis Recurrent fevers Registry Cohort Studies Colchicine Female Humans Male Mutation Pyrin Registries Familial Mediterranean FeverColchicine*Recurrent fevers
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Phenotypic variability and disparities in treatment and outcomes of childhood arthritis throughout the world: an observational cohort study

2019

Made available in DSpace on 2019-10-05T16:54:20Z (GMT). No. of bitstreams: 0 Previous issue date: 2019-04-01 IRCCS Istituto Giannina Gaslini Background To our knowledge, the characteristics and burden of childhood arthritis have never been studied on a worldwide basis. We aimed to investigate, with a cross-sectional study, the prevalence of disease categories, treatment methods, and disease status in patients from across different geographical areas and from countries with diverse wealth status. Methods In this multinational, cross-sectional, observational cohort study, we asked international paediatric rheumatologists from specialised centres to enrol children with a diagnosis of juvenile …

Malemedicine.medical_specialtyChildhood arthritisCross-sectional studyPopulationGlobal HealthPediatrics03 medical and health sciences0302 clinical medicine030225 pediatricsEpidemiologymedicineDevelopmental and Educational PsychologyJournal ArticleHumansPediatrics Perinatology and Child Health; Developmental and Educational Psychology030212 general & internal medicineHealthcare DisparitiesChildeducationDisease burdenPain MeasurementRetrospective Studieseducation.field_of_studyOligoarthritisbusiness.industryPerinatology and Child HealthJuvenile idiopathic arthritismedicine.diseaseJUVENILE IDIOPATHIC ARTHRITIS; OF-RHEUMATOLOGY RECOMMENDATIONS; DISEASE-ACTIVITY SCORE; DEFINING CRITERIA; CLASSIFICATION; CHILDREN; EPIDEMIOLOGY; VALIDATION; COUNTRIES; VALIDITYArthritis Juvenilechildhood arthritisphenotypic variabilityobservational cohort studyCross-Sectional StudiesBiological Variation PopulationSettore MED/38 - PEDIATRIA GENERALE E SPECIALISTICAAntirheumatic AgentsChild PreschoolPediatrics Perinatology and Child HealthQuality of LifeFemalePolyarthritisJuvenile idiopatic arthritis of-rheumatology recommentadions disease-activity score defining criteria classification children epidemiology validation countries validitybusinessDemographyCohort study
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Canakinumab for the Treatment of Autoinflammatory Recurrent Fever Syndromes.

2018

BACKGROUND: Familial Mediterranean fever, mevalonate kinase deficiency (also known as the hyperimmunoglobulinemia D syndrome), and the tumor necrosis factor receptor-associated periodic syndrome (TRAPS) are monogenic autoinflammatory diseases characterized by recurrent fever flares. METHODS: We randomly assigned patients with genetically confirmed colchicine-resistant familial Mediterranean fever, mevalonate kinase deficiency, or TRAPS at the time of a flare to receive 150 mg of canakinumab subcutaneously or placebo every 4 weeks. Patients who did not have a resolution of their flare received an add-on injection of 150 mg of canakinumab. The primary outcome was complete response (resolution…

Male0301 basic medicineInterleukin-1betaFamilial Mediterranean fever0302 clinical medicineMonoclonalChildMedicine(all)Mevalonate kinase deficiencySubcutaneousMedicine (all)Interleukin-1betaAntibodies MonoclonalGeneral MedicineFamilial Mediterranean FeverRecurrent feverChild PreschoolFemaleTumor necrosis factor alphaDrugInflammatory diseases Radboud Institute for Molecular Life Sciences [Radboudumc 5]medicine.drugAdultAdolescentFeverInjections SubcutaneousHereditary Autoinflammatory DiseasesAntibodies Monoclonal HumanizedAdolescent; Adult; Antibodies Monoclonal/administration & dosage; Antibodies Monoclonal/adverse effects; Antibodies Monoclonal/therapeutic use; Child; Child Preschool; Dose-Response Relationship Drug; Double-Blind Method; Familial Mediterranean Fever/drug therapy; Female; Fever/drug therapy; Hereditary Autoinflammatory Diseases/drug therapy; Humans; Injections Subcutaneous; Interleukin-1beta/antagonists & inhibitors; Male; Mevalonate Kinase Deficiency/drug therapy; Young AdultAntibodiesInjectionsDose-Response RelationshipYoung Adult03 medical and health sciencesAll institutes and research themes of the Radboud University Medical CenterDouble-Blind MethodGeneral & Internal MedicinemedicineHumansPreschoolAdolescent; Adult; Antibodies Monoclonal; Child; Child Preschool; Dose-Response Relationship Drug; Double-Blind Method; Familial Mediterranean Fever; Female; Fever; Hereditary Autoinflammatory Diseases; Humans; Injections Subcutaneous; Interleukin-1beta; Male; Mevalonate Kinase Deficiency; Young Adult; Medicine (all)030203 arthritis & rheumatologyDose-Response Relationship Drugbusiness.industryHereditary Autoinflammatory DiseasesHyperimmunoglobulinemia Dmedicine.diseaseCanakinumab030104 developmental biologyImmunologyMevalonate Kinase Deficiencybusiness
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Development and initial validation of the macrophage activation syndrome/primary hemophagocytic lymphohistiocytosis score, a diagnostic tool that dif…

2017

OBJECTIVE: To develop and validate a diagnostic score that assists in discriminating primary hemophagocytic lymphohistiocytosis (pHLH) from macrophage activation syndrome (MAS) related to systemic juvenile idiopathic arthritis. STUDY DESIGN: The clinical, laboratory, and histopathologic features of 362 patients with MAS and 258 patients with pHLH were collected in a multinational collaborative study. Eighty percent of the population was assessed to develop the score and the remaining 20% constituted the validation sample. Variables that entered the best fitted model of logistic regression were assigned a score, based on their statistical weight. The MAS/HLH (MH) score was made up with the i…

Male0301 basic medicineHemophagocyticLogistic regressionPediatricshemophagocytic syndrome0302 clinical medicine*diagnostic scoreDiagnosisMedicineCutoffChildprimary hemophagocytic lymphohistiocytosiLymphohistiocytosiseducation.field_of_studyprimary hemophagocytic lymphohistiocytosisPerinatology and Child Healthdiagnostic scoreQuartileSettore MED/38 - PEDIATRIA GENERALE E SPECIALISTICAMacrophage activation syndromeChild Preschool*macrophage activation syndromeAbsolute neutrophil countFemale*primary hemophagocytic lymphohistiocytosisHumanmedicine.medical_specialtyAdolescentPopulationLymphohistiocytosis HemophagocyticDiagnosis Differential03 medical and health sciencesInternal medicineHumansPreschooleducation030203 arthritis & rheumatologyReceiver operating characteristicbusiness.industryInfantReproducibility of Resultsmedicine.diseaseSurgery030104 developmental biologydiagnostic score; hemophagocytic syndrome; macrophage activation syndrome; primary hemophagocytic lymphohistiocytosis; Adolescent; Child; Child Preschool; Diagnosis Differential; Female; Humans; Infant; Lymphohistiocytosis Hemophagocytic; Macrophage Activation Syndrome; Male; Reproducibility of Results; Pediatrics Perinatology and Child HealthMacrophage activation syndromeDifferentialPediatrics Perinatology and Child Health*hemophagocytic syndromeDifferential diagnosisbusiness
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THE USE OF INTERLEUKIN 1 RECEPTOR ANTAGONIST (ANAKINRA) IN KAWASAKI DISEASE: A RETROSPECTIVE CASES SERIES

2018

Introduction: Persistent fever and inflammation after infusion of 2g/kg of IVIG, the standard treatment of KD represents a high-risk situation for coronary aneurysms in Kawasaki disease. Identifying patients at risk for IVIG resistance is difficult outside the Asian population, and there remains a critical unmet need to identify an anti-inflammatory treatment that is efficacious in all KD patients. Recent evidence from studies in animals and humans suggest a critical role for interleukin-1 (IL-1) α and β in the pathogenesis of KD. Objectives: To identify the clinical characteristics, reasons for use and response to treatment with anakinra in a retrospective series of patients with Kawasaki …

Male0301 basic medicineBLOCKADEPlacebo-controlled studyCHILDRENSUSCEPTIBILITYPLACEBO-CONTROLLED TRIALPediatricsDOUBLE-BLIND0302 clinical medicineSettore MED/38 - Pediatria Generale E SpecialisticaRetrospective StudieINTERLEUKIN 1 RECEPTOR ANTAGONIST ANAKINRA KAWASAKI DISEASEImmunology and AllergyJUVENILE IDIOPATHIC ARTHRITISChildPediatricAnakinra coronary artery aneurysmPrognosis1107 ImmunologyChild PreschoolDisease ProgressionFemaleVasculitisLife Sciences & BiomedicineHumanmedicine.drugVasculitismusculoskeletal diseasesAutoinflammatory diseaseVasculitimedicine.medical_specialtyMyocarditisPrognosiImmunologyMucocutaneous Lymph Node SyndromeAnakinra coronary artery aneurysms03 medical and health sciencesInternal medicineINFLIXIMABMANAGEMENTmedicineINTRAVENOUS IMMUNOGLOBULINHumansRetrospective Studies030203 arthritis & rheumatologyAnakinraScience & TechnologyKawasaki diseasebusiness.industryInfantReceptors Interleukin-1Retrospective cohort studymedicine.diseaseInfliximabInterleukin 1 Receptor Antagonist Protein030104 developmental biologyInterleukin 1 receptor antagonistKawasaki diseasebusinessInterleukin-1
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The European network for care of children with paediatric rheumatic diseases : Care across borders

2019

Objectives. To provide an overview of the paediatric rheumatology (PR) services in Europe, describe current delivery of care and training, set standards for care, identify unmet needs and inform future specialist service provision. Methods. An online survey was developed and presented to national coordinating centres of the Paediatric Rheumatology International Trials Organisation (PRINTO) (country survey) and to individual PR centres (centre and disease surveys) as a part of the European Union (EU) Single Hub and Access point for paediatric Rheumatology in Europe project. The survey contained components covering the organization of PR care, composition of teams, education, health care and …

Transition to Adult Caremedicine.medical_specialtyBiomedical Researchstandards of careChild Health ServicesPopulationDiseaseHealth Services AccessibilityTransition management (governance)paediatric rheumatology03 medical and health sciences0302 clinical medicineRheumatologyRheumatic DiseasesHealth caremedicineJournal ArticleHumansmedia_common.cataloged_instancePharmacology (medical)030212 general & internal medicineEuropean unionChildeducationIntersectoral Collaborationmedia_common030203 arthritis & rheumatologyService (business)Biological ProductsHealth Services Needs and Demandeducation.field_of_studyEducation Medicalbusiness.industryStandard of CareDrug Utilizationpaediatric rheumatology service provision standards of careEuropeEastern europeanClinical trialHealth Care SurveysFamily medicineservice provisionHealth Services ResearchDrug MonitoringbusinessDelivery of Health CareRheumatology (United Kingdom)
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