0000000000877282

AUTHOR

Jonathan Akikusa

showing 2 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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