0000000000877397

AUTHOR

Isabel Bolt

showing 3 related works from this author

American College of Rheumatology Provisional Criteria for Clinically Relevant Improvement in Children and Adolescents With Childhood-Onset Systemic L…

2019

OBJECTIVE: To develop a Childhood Lupus Improvement Index (CHILI) as a tool to measure response to therapy in childhood-onset systemic lupus erythematosus (cSLE), with a focus on clinically relevant improvement (CRIc SLE ). METHODS: Pediatric nephrology and rheumatology subspecialists (n = 213) experienced in cSLE management were invited to define CRIc SLE and rate a total of 433 unique patient profiles for the presence/absence of CRIc SLE . Patient profiles included the following cSLE core response variables (CRVs): global assessment of patient well-being (patient-global), physician assessment of cSLE activity (MD-global), disease activity index score (here, we used the Systemic Lupus Eryt…

medicine.medical_specialtyOutcome AssessmentHealth Care/methodsAdolescentDelphi TechniqueAntirheumatic Agents/therapeutic useSeverity of Illness IndexChild healthArticle03 medical and health sciences0302 clinical medicineRheumatologyInternal medicineSeverity of illnessOutcome Assessment Health CaremedicinePediatric nephrologyHumansLupus Erythematosus Systemicskin and connective tissue diseasesChild030203 arthritis & rheumatologySystemic lupus erythematosusLupus erythematosusLupus ErythematosusReceiver operating characteristicbusiness.industryConsensus conferencechildhood-onset systemic lupus erythematosusOutcome Assessment Health Care/methodsmedicine.diseaseRheumatologyRheumatologySystemic lupus erythematosusautoimmune inflammatory diseaseAntirheumatic AgentsLupus Erythematosus Systemic/drug therapySystemic/drug therapybusinessAlgorithmsArthritis Care and Research
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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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Dissecting the Heterogeneity of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis

2015

Objective.To seek insights into the heterogeneity of macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (sJIA) through the analysis of a large patient sample collected in a multinational survey.Methods.International pediatric rheumatologists and hemato-oncologists entered their patient data, collected retrospectively, in a Web-based database. The demographic, clinical, laboratory, histopathologic, therapeutic, and outcome data were analyzed in relation to (1) geographic location of caring hospital, (2) subspecialty of attending physician, (3) demonstration of hemophagocytosis, and (4) severity of clinical course.Results.A total of 362 patients were incl…

MaleHEMOPHAGOCYTIC SYNDROMESInternationalityDatabases FactualHepatosplenomegalyJuvenileComorbidityHEMOPHAGOCYTIC LYMPHOHISTIOCYTOSISSeverity of Illness IndexHEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS; HEMOPHAGOCYTIC SYNDROMES; MACROPHAGE ACTIVATION SYNDROME; SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS; Adolescent; Age Distribution; Arthritis Juvenile; Child; Child Preschool; Cohort Studies; Comorbidity; Databases Factual; Female; Humans; Internationality; Macrophage Activation Syndrome; Male; Multivariate Analysis; Prevalence; Prognosis; Retrospective Studies; Severity of Illness Index; Sex Distribution; Survival Analysis; Immunology and Allergy; Rheumatology; ImmunologyCohort StudiesPrevalenceImmunology and AllergyChildMacrophage Activation SyndromePrognosisChild PreschoolFemaleHemophagocytosismedicine.symptommedicine.medical_specialtyAdolescentSYSTEMIC JUVENILE IDIOPATHIC ARTHRITISImmunologyHemophagocytic Lymphohistiocytosis Hemophagocytic Syndromes Macrophage activation syndromes Systemic Juvenile Idiopathic Arthritis Adolescent Age Distribution Arthritis Juvenile Child Child Preschool Cohort Studies Comorbidity Databases Factual Female Humans Internationality Macrophage Activation Syndrome Male Multivariate Analysis Prevalence Prognosis Retrospective Studies Severity of Illness Index Sex Distribution Survival Analysis Immunology and Allergy Rheumatology ImmunologyDatabasesAge DistributionRheumatologyInternal medicineSeverity of illnessmedicineHumansSex DistributionPreschoolFactualRetrospective StudiesHemophagocytic lymphohistiocytosisbusiness.industryArthritisRetrospective cohort studymedicine.diseaseSurvival AnalysisComorbidityArthritis JuvenileRheumatologyMacrophage activation syndromeMultivariate AnalysisImmunologyMacrophage activation syndromesbusinessThe Journal of Rheumatology
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