A5102918400- Autoimmune and Inflammatory Disorders Research
- Systemic Lupus Erythematosus Research
- Adolescent and Pediatric Healthcare
- Chronic Lymphocytic Leukemia Research
- Systemic Sclerosis and Related Diseases
- Immunodeficiency and Autoimmune Disorders
- Autoimmune Bullous Skin Diseases
- Acute Lymphoblastic Leukemia research
- Kawasaki Disease and Coronary Complications
- Rheumatoid Arthritis Research and Therapies
- Lysosomal Storage Disorders Research
- Atherosclerosis and Cardiovascular Diseases
- Elbow and Forearm Trauma Treatment
- Inflammasome and immune disorders
- Research on Leishmaniasis Studies
- Family and Disability Support Research
- Orthopedic Infections and Treatments
- Monoclonal and Polyclonal Antibodies Research
- Bone and Joint Diseases
- Dermatological and Skeletal Disorders
- Parvovirus B19 Infection Studies
- Sepsis Diagnosis and Treatment
- Orthopedic Surgery and Rehabilitation
- Mechanical Circulatory Support Devices
- Cancer-related molecular mechanisms research
Emma Kinderziekenhuis
2013-2025
Amsterdam University Medical Centers
2020-2024
University of Amsterdam
2020-2024
Amsterdam UMC Location University of Amsterdam
2020-2021
Reade
2013
JDM is a rare chronic immune-mediated inflammatory disease with predominant role for type I IFN responses. We aimed to determine the potential of Siglec-1 expression on monocytes as novel IFN-inducible biomarker activity monitoring and prediction treatment response in patients JDM.Siglec-1 was measured by flow cytometry circulating 21 newly diagnosed before start and, 10 these, also during follow-up. The levels five IFN-stimulated genes, MX1, IFI44, IFI44L, LY6E IFIT3, were RT-qPCR signature...
Abstract Objectives For IFN-driven diseases, such as juvenile dermatomyositis (JDM), there is a critical need for targeted therapies. We aimed to develop an in vitro model, using Siglec-1 read-out, evaluate inhibition of IFN-mediated responses with different JAK inhibitors (JAKi). Methods Healthy donor (HD) PBMCs were cultured type I and II IFNs, TLR agonists, plasma or serum from patients (JDM, DM, SLE, COVID-19) HDs. expression on CD14+ monocytes was analyzed flow cytometry. Inhibitory...
Proteasome-associated autoinflammatory syndromes (PRAASs) form a family of recently described rare autosomal recessive disorders disturbed proteasome assembly and proteolytic activity caused by mutations in genes coding for subunits. The treatment options these consist lifelong immunosuppressive drugs or Janus kinase inhibitors, which may have partial efficacy noticeable side effects. Because proteasomes are ubiquitously expressed, it is unknown whether hematopoietic stem cell...
Objectives For selection of high-risk systemic lupus erythematosus (SLE) patients it is necessary to obtain indicators disease severity that predict damage. As in sclerosis, nailfold capillary abnormalities could be such a biomarker SLE. The primary objective this cross-sectional study describe childhood-onset SLE (cSLE) cohort (onset < 18 years) and compare them with matched healthy controls. secondary correlate the observed demographical variables both cohorts disease-specific cSLE...
Objectives To observe if capillary patterns in childhood-onset SLE (cSLE) change over time and find associations between a scleroderma pattern with disease activity, damage or scleroderma-like features. Methods Clinical (yearly) capillaroscopy data from longitudinal cohort of patients cSLE (minimum four Systemic Lupus International Collaborating Clinics (SLICC) criteria, onset <18 years) were analysed. Disease activity was measured by Erythematosus Activity Index (SLEDAI) SLICC Damage...
Abstract Background Anti-tumor necrosis factor (TNF) drugs have improved the prognosis for juvenile idiopathic arthritis (JIA) significantly. However, evidence individual treatment decisions based on serum anti-TNF drug levels and presence of anti-drug antibodies (ADAbs) in children is scarce. We aimed to assess if and/or ADAbs influenced physician’s with JIA. Methods Patients’ records our center were retrospectively screened measurements JIA using etanercept, adalimumab or infliximab....
Abstract Background Double inversion recovery (DIR) MRI has the potential to accentuate synovium without using contrast agents, as it allows simultaneous signal suppression of fluid and fat. The purpose this study was (1) compare DIR conventional contrast-enhanced (CE) for delineation in knee children with juvenile idiopathic arthritis (JIA) (2) assess agreement between CE-MRI regarding maximal synovial thickness measurements. Results In prospective study, 26 JIA who consecutively underwent...
Juvenile idiopathic arthritis (JIA) is a chronic autoimmune disorder that primarily affects the joints in children. Notably, it known to co-occur with uveitis. Adalimumab, monoclonal anti-TNF antibody, effective treating both conditions. A deeper understanding of pharmacokinetics (PK) adalimumab JIA crucial advance more personalized treatment approaches. The objective this study evaluate population PK profile and explain causes for its variability. Adalimumab antidrug antibody concentrations...
In childhood-onset SLE (cSLE), patients have an increased risk of premature atherosclerosis. The pathophysiological mechanisms for this atherosclerosis are not yet completely understood, but besides traditional factors, the endothelium plays a major role. first aim study was to measure levels SLE-associated markers involved in endothelial cell (EC) function and lipids cSLE cohort longitudinally comparison with healthy controls (HC). Next correlate these Systemic Lupus Erythematosus Disease...
Abstract Background Knowledge of the synovial and tenosynovial appearance clinically non-arthritic symptomatic juvenile wrist using contrast-enhanced magnetic resonance imaging (MRI) is sparse. Objectives To analyze MRI findings non-inflamed pediatric wrist, focusing on enhancing membrane. evaluate coexistent presence (teno)synovial enhancement, joint fluid, bony depressions medullary changes suggestive bone marrow edema. Materials methods We included 20 children (15 girls; age range:...
Abstract Objective This study investigates the relationship of child, caregiver, and caring context measurements with care-related quality life (CRQoL) health-related (HRQoL) caregivers children juvenile idiopathic arthritis (JIA). Methods We performed a cross-sectional analysis baseline data on JIA from Canada Netherlands collected for “Canada-Netherlands Personalized Medicine Network in Childhood Arthritis Rheumatic Diseases” June 2019 to September 2021. used CRQoL questionnaire...
Abstract Systemic juvenile idiopathic arthritis (sJIA, also called Still's disease) is a rare childhood auto‐inflammatory disease with significant morbidity. This case report illustrates the clinical course and highlights diagnostic challenges. FDG‐PET/CT imaging may be beneficial in process for some cases, order to achieve rapid diagnosis early treatment.
The aim of this study was to compare pain-scores in three targeted treatment-strategies JIA-patients and identify characteristics predicting persistent pain. In the BeSt-for-Kids-study 92 DMARD-naïve were randomized 3 treatment-strategies: 1) initial sequential DMARD-monotherapy 2) methotrexate (MTX)/prednisolone-bridging or 3) MTX/etanercept. Potential differences VAS pain scores (0-100 mm) over time between compared using linear mixed models with visits clustered within patients. A...
Galactosialidosis (GS) is a rare inherited lysosomal storage disorder (LSD) which characterized by defect in the glycoprotein catabolism. We report, for first time, case of child affected GS presenting with recurrent episodes extensive joint inflammation both knee joints. The aim this case-report to describe clinical presentation as well laboratory, radiologic and microscopic features unique GS. Furthermore, we explore inflammatory mechanisms potentially responsible origination arthritic...
To directly compare and describe the differences between juvenile idiopathic arthritis (JIA) patients pediatric controls regarding features of synovial tenosynovial membrane on contrast-enhanced magnetic resonance imaging (MRI) wrist. T1-weighted MRI scans 25 JIA with clinically active wrist children without a history joint complaints nor any clinical signs inflammation were evaluated by two readers blinded to data. The synovium was scored at five anatomical sites based thickening (0-3...
Multisystem Inflammatory Syndrome in Children (MIS-C) is a severe inflammatory disease children related to SARS-CoV-2 with multisystem involvement including marked cardiac dysfunction and clinical symptoms that can resemble Kawasaki Disease (KD). We hypothesized MIS-C KD might have commonalities as well unique responses studied these both diseases. In total, fourteen (n=8) (n=6) were included the period of March-June 2020. Clinical routine blood parameters, follow-up, SARS-CoV-2-specific...
The recommended infliximab (IFX) dose in (pediatric) rheumatology practice is 3-6 mg/kg every 4-8 weeks. Higher dosage regimens (>10 mg/kg) of IFX are effective and safe. To optimize treatment patients with juvenile idiopathic arthritis (JIA), therapeutic drug monitoring might be beneficial. support routine regimen optimization for JIA, in-depth knowledge the pharmacokinetic (PK) variability needed. As soon as optimal ranges known, PK model-based simulation can used to individualize dosing...
Background: Juvenile Idiopathic Arthritis (JIA) is the most common auto-immune disease in children and pain a symptom. Despite treatment advances recent years, often persists when inactive. Treating JIA to target has been widely recommended 1 , but effectiveness reduction not proven yet 2 . Objectives: To compare scores over two years three arms patients that were treated identify baseline characteristics predicting unfavorable outcomes. Methods: DMARD naïve who participated BeSt for kids...
Background: Systemic lupus erythematosus (SLE) is a severe, lifelong autoimmune disease known for its multisystem organ involvement. SLE patients are to be at risk premature atherosclerosis relatively young age (1). Endothelial dysregulation one of the pathophysiologic mechanisms that can lead higher cardiovascular in (2). Multiple endothelial markers with have been described so far, which some associated activity. Objectives: To report systematic literature review regarding dysregulated and...
Background: Clinical phenotyping and predicting treatment responses in Systemic Lupus Erythematosus (SLE) patients is challenging. Extensive blood transcriptional profiling has identified various gene modules that are promising for stratification of SLE patients. We aimed to translate existing transcriptomic data into simpler signatures suitable daily clinical practice. Methods: RT-PCR multiple genes from the Interferon M1·2, M5·12, neutrophil (NPh) plasma cell (PLC) followed by a principle...
<h3>Background</h3> MRI plays an increasingly important role in the assessment and monitoring of disease activity juvenile idiopathic arthritis (JIA). Awareness incidence distribution pattern abnormalities JIA is a valuable tool daily practice reading radiologist treating clinician. Preferred locations for pathology within target joints are facilitated by knowledge on common patterns abnormalities, enabling rapid differentiation between normal variants. <h3>Objectives</h3> To determine (1)...