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Hemophagocytic Lymphohistiocytosis (HLH) Genetic Variants in Childhood-onset...
Background/Purpose: Hemophagocytic lymphohistocytosis (HLH) is a life-threatening hyper-inflammatory syndrome characterized by excessive activation and proliferation of T-lymphocytes and macrophages....
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Prevalence of Paradoxical Psoriasis After Exposure to Tumor Necrosis Factor...
Background/Purpose: Paradoxical psoriasis after exposure to tumor necrosis factor inhibitors (TNFi) has been increasingly reported in the adult population. Systematic studies in pediatric population...
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Characteristics of the New Childhood Arthritis and Rheumatology Research...
Background/Purpose: The New CARRA Registry of Juvenile Myositis (JM) was developed in 2017 to collect 10-year longitudinal data to increase knowledge of the course of JM and facilitate clinical and...
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Patients Perspectives on Living with a Systemic Autoinflammatory Disease:...
Background/Purpose: Systemic autoinflammatory diseases (SAIDs) encompass multiple clinical entities in which spontaneous inflammation occurs due to dysregulation of the innate immune response. The...
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Sun Protection Use in Patients Followed in a Pediatric Rheumatology Clinic in...
Background/Purpose: Exposure to sunlight has been implicated as a major contributing factor in the pathogenesis of systemic lupus erythematosus (SLE) and juvenile dermatomyositis (JDM). Despite this...
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Allogenic Hematopoietic Stem Cell Transplantation for Refractory Childhood...
Background/Purpose: Patients with refractory rheumatic diseases face poor quality of life, long-term sequelae and life-threatening complications. With advances in allogenic hematopoietic stem cell...
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Non-criteria Antiphospholipid Antibodies Associated with Pediatric Rheumatic...
Background/Purpose: Non-criteria antiphospholipid antibodies (NC-aPL) bind molecules such as phosphatidylethanolamine (PE), phosphatidylserine (PS), and prothrombin (Pt), but not the targets of routine...
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Patient-Proxy Agreement on Mental Health and Neuropsychological Symptoms...
Background/Purpose: Adolescents with juvenile fibromyalgia syndrome (JFMS) present with a myriad of mental health and neuropsychological symptoms, including dyscognition (“brain fog”); however,...
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Abatacept Treatment Reduces Cutaneous and Joint Activity in Juvenile...
Background/Purpose: Juvenile localized scleroderma (jLS) is an autoimmune disease commonly associated with damage. Damage includes dyspigmentation, tissue atrophy, arthropathy, hemiatrophy, vision...
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Incidence and Risk Factors of Hypogammaglobulinemia and Infectious...
Background/Purpose: B-cell depletion therapy has increasingly been used for the treatment of childhood-onset rheumatic diseases. Previous studies investigating whether rituximab results in increased...
View ArticleCD3 Downregulation on T-cells Is Concomitant with Arginase Upregulation on...
Background/Purpose: Rationale: Juvenile idiopathic arthritis (JIA) is an inflammatory autoimmune disorder driven by dysfunction of the joint tissue and abnormal immune responses. As deep phenotyping of...
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Failure of Methotrexate Monotherapy and Subsequent Response to Tumor Necrosis...
Background/Purpose: Pediatric non-infectious uveitis is often treated with systemic immunomodulators in a step-wise fashion, with methotrexate (MTX) followed by tumor necrosis factor alpha inhibitors...
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Predictors of Moderate/High Disease Activity in Childhood-Onset Systemic...
Background/Purpose: Substantial risk of early morbidity and mortality exists for patients with childhood-onset systemic lupus erythematosus (cSLE), but there are limited studies identifying predictors...
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Medication Adherence in Adolescents and Young Adults with Childhood Onset SLE
Background/Purpose: Compared to adults with SLE, young patients with SLE face particular challenges, including more severe disease, more renal involvement and medications side effects like growth...
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Therapeutic Interferon Gamma Neutralization with Emapalumab in Patients with...
Background/Purpose: Interferon gamma (IFNγ) is a pivotal mediator of HLH. Emapalumab (a fully human anti-IFNγ monoclonal antibody) is efficacious in patients with primary HLH. In autoinflammatory...
View ArticleThe First 18 Months of a Pediatric Rheumatology Service at a Tertiary...
Background/Purpose: Pediatric rheumatic diseases impart a significant disease burden upon children and their families with the potential to cause diminished quality of life and significant direct and...
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Novel Algorithm to Increase Sensitivity of Detecting Active Arthritis in...
Background/Purpose: Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in children. The use of infrared thermal imaging for screening JIA has not utilized standardized image...
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The Impact of Psychiatric Diagnosis and Treatment on Medication Adherence in...
Background/Purpose: Youth with systemic lupus erythematosus (SLE) experience significant health care burden and high rates of psychiatric disorders. Optimizing medication adherence is critical for...
View ArticleGenetic Susceptibility Loci for Periodic Fever, Aphthous Stomatitis,...
Background/Purpose: Periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis (PFAPA) syndrome is the most common periodic fever syndrome of childhood. Although familial clustering of the...
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