A5080992616- Autoimmune and Inflammatory Disorders Research
- Inflammasome and immune disorders
- Ocular Diseases and Behçet’s Syndrome
- Adolescent and Pediatric Healthcare
- Renal Diseases and Glomerulopathies
- Diabetes and associated disorders
- Systemic Lupus Erythematosus Research
- Immunodeficiency and Autoimmune Disorders
- Drug-Induced Adverse Reactions
- Kawasaki Disease and Coronary Complications
- Coagulation, Bradykinin, Polyphosphates, and Angioedema
- Ion Transport and Channel Regulation
- Otitis Media and Relapsing Polychondritis
- Sarcoidosis and Beryllium Toxicity Research
- Adrenal Hormones and Disorders
- Asthma and respiratory diseases
- Protein Tyrosine Phosphatases
- Streptococcal Infections and Treatments
- Gout, Hyperuricemia, Uric Acid
- Flavonoids in Medical Research
- Vasculitis and related conditions
- Natural product bioactivities and synthesis
- Acute Lymphoblastic Leukemia research
- Neurogenetic and Muscular Disorders Research
- Amyotrophic Lateral Sclerosis Research
Johns Hopkins University
2021-2023
Johns Hopkins All Children's Hospital
2021-2023
National Institutes of Health
2018-2021
National Institute of Arthritis and Musculoskeletal and Skin Diseases
2018-2021
Institut thématique Génétique, génomique et bioinformatique
2021
Children's National
2016-2019
Moffitt Cancer Center
2008
University of South Florida
2008
Molecular Oncology (United States)
2008
Objective To develop treatment recommendations for children with juvenile idiopathic arthritis manifesting as non‐systemic polyarthritis, sacroiliitis, or enthesitis. Methods The Patient/Population, Intervention, Comparison, and Outcomes ( PICO ) questions were developed refined by members of the guideline development teams. A systematic review was conducted to compile evidence benefits harms associated treatments these conditions. GRADE (Grading Recommendations Assessment, Development...
To develop recommendations for the screening, monitoring, and treatment of uveitis in children with juvenile idiopathic arthritis (JIA).Pediatric rheumatologists, ophthalmologists expertise uveitis, patient representatives, methodologists generated key clinical questions to be addressed by this guideline. This was followed a systematic literature review rating available evidence according GRADE (Grading Recommendations Assessment, Development Evaluation) methodology. A group consensus...
Drug reaction with eosinophilia and systemic symptoms (DRESS) is a severe, delayed hypersensitivity (DHR). We observed DRESS to inhibitors of interleukin 1 (IL-1) or IL-6 in small group patients Still's disease atypical lung disease. sought characterise features compared drug-tolerant controls. analysed human leucocyte antigen (HLA) alleles for association inhibitor-related DHR, including Kawasaki (KD) cohort.
Screening of the NCI diversity set compounds has led to identification 5 (NSC-117199), which inhibits protein tyrosine phosphatase (PTP) Shp2 with an IC50 47 μM. A focused library incorporating isatin scaffold was designed and evaluated for inhibition Shp1 PTP activities. Several were identified that selectively inhibit over PTP1B low submicromolar activity. model binding active is proposed.
To determine whether systemic juvenile idiopathic arthritis (JIA) susceptibility loci that were identified by candidate gene studies demonstrate association with JIA in the largest study population assembled to date.
Abstract Objectives Elevation of serum IL-18 in adult-onset Still's disease (AOSD) and systemic JIA (sJIA) suggests the role inflammasome these diseases. Gasdermin D is a pore-forming protein playing central roles inflammasome-mediated inflammation, but its rheumatic unknown. We aimed to elucidate auto-inflammatory mechanisms AOSD sJIA. Methods Patients with AOSD, sJIA, hemophagocytic lymphohistiocytosis (HLH) Behçet's followed at Yokohama City University (YCU), or US National Institutes...
Abstract MA is a rare, autosomal recessive disorder characterized by episodes of inflammation and periodic fevers. In its most severe form, it can result in facial dysmorphism, growth inhibition, ataxia, liver dysfunction, intellectual disability, at times be fatal. A number case reports exist stating that SCT curative these patients. We present the patient diagnosed with birth, who underwent age 14 months intent to cure. She achieved complete engraftment urine mevalonate became...
Congenital malaria is rare in the United States, but an important diagnosis to consider when evaluating febrile infants. Herein, we describe a case of congenital Plasmodium falciparum 2-week-old infant born States mother who had emigrated from Nigeria 3 months before delivery.
ABSTRACT In Saper et al (2019), we described systemic JIA patients who developed a high-fatality diffuse lung disease (DLD) while on IL-1 or IL-6 inhibitors. We observed severe delayed drug hypersensitivity reactions (DHR) in significant subset. Because alleles of the human leukocyte antigen (HLA) loci can mediate DHR, investigated HLA genotype association with these DHR. typed subjects treated inhibitors: 34 sJIA/DHR/DLD, 11 sJIA/DHR without DLD, 18 drug-tolerant sJIA, and 19 Kawasaki (KD)...