A5055510400- Renal Diseases and Glomerulopathies
- Autoimmune Bullous Skin Diseases
- Chronic Lymphocytic Leukemia Research
- Vasculitis and related conditions
- Systemic Lupus Erythematosus Research
- Immunodeficiency and Autoimmune Disorders
- Platelet Disorders and Treatments
- Coagulation, Bradykinin, Polyphosphates, and Angioedema
- T-cell and B-cell Immunology
- Systemic Sclerosis and Related Diseases
- Pregnancy and Medication Impact
- Reproductive System and Pregnancy
- Immune Cell Function and Interaction
- Immunotherapy and Immune Responses
- Renal Transplantation Outcomes and Treatments
- Urticaria and Related Conditions
- Amyloidosis: Diagnosis, Treatment, Outcomes
- Chronic Kidney Disease and Diabetes
- Adolescent and Pediatric Healthcare
- Biomedical Research and Pathophysiology
- Autoimmune and Inflammatory Disorders Research
- Hereditary Neurological Disorders
- Galectins and Cancer Biology
- Viral Infections and Outbreaks Research
- Lymphatic System and Diseases
Istituti di Ricovero e Cura a Carattere Scientifico
2012-2025
Bambino Gesù Children's Hospital
2016-2025
University Medical Center Hamburg-Eppendorf
2024
Johns Hopkins University
2024
University Medical Center
2024
Sorbonne Université
2024
Inserm
2024
Aarhus University
2024
Heidelberg University
2024
University Hospital Heidelberg
2024
The pathogenesis of nephrotic syndrome is unclear. However, the efficacy rituximab, a B cell–depleting antibody, in suggests pathogenic role cells. In this retrospective study, we determined by flow cytometry levels and T cell subpopulations before after rituximab infusion 28 pediatric patients with frequently relapsing or steroid–dependent syndrome. At baseline, had lower median percentages transitional mature cells than age–matched healthy controls ( P <0.001). Rituximab induced full...
Minimal change disease and primary focal segmental glomerulosclerosis in adults, along with idiopathic nephrotic syndrome children, are immune-mediated podocytopathies that lead to syndrome. Autoantibodies targeting nephrin have been found patients minimal disease, but their clinical pathophysiological roles unclear.
Significance Statement Children with frequently relapsing, steroid-dependent nephrotic syndrome (FRSDNS) often require multiple courses of rituximab. However, long-term effects from repeated treatments remain unknown. In this international, multicenter study 346 children receiving 1149 rituximab, the risk relapse decreased and relapse-free survival significantly improved treatments. Important side effects, including hypogammaglobulinemia, neutropenia, infections, were mostly mild, but...
Significance Statement In patients with steroid-dependent and calcineurin inhibitor–depe ndent nephrotic syndrome, rituximab, a chimeric monoclonal anti body directed against CD20+ B cells, helps maintain remission, but relapse within year is common. This randomized trial investigated wheth er ofatumumab, fully human anti-CD20 antibody, superior to rituximab in maintaining oral drug–free remission this condition. The findings show ofatumumab not achieving at 1 of follow-up, had similar...
Anti-CD20 therapy is effective in idiopathic nephrotic syndrome (INS). However, transient or sustained hypogammaglobulinemia predisposing to an increased risk of infectious diseases can follow treatment some patients. We analyzed the long-term effects anti-CD20 on immunological memory 27 frequently-relapsing/steroid-dependent INS pediatric patients after more than 4 years from first and at least 2 last infusion. Twenty-one children, never treated with under intense oral immunosuppression...
Introduction The clinical evolution of steroid-sensitive forms pediatric idiopathic nephrotic syndrome (INS) is highly heterogeneous following the standard treatment with prednisone. To date, no prognostic marker has been identified to predict severity disease course starting from first episode. Methods In this monocentric prospective cohort study we set up a reproducible and standardized flow cytometry panel using two sample tubes (one for B-cell one T-cell subsets) extensively...
Human serum IgM Abs are composed of heavily glycosylated polymers with five glycosylation sites on the μ (heavy) chain and one site J chain. In contrast to IgG glycans, which vital for a number biological functions, virtually nothing is known about structure-function relationships glycans. Natural earliest Ig produced recognizes multiple Ags low affinity, whereas immune induced by Ag exposure characterized higher specificity. anti-lymphocyte present in healthy individuals increases...
B cell depleting therapies permit immunosuppressive drug withdrawal and maintain remission in patients with frequently relapsing nephrotic syndrome (FRNS) or steroid–dependent (SDNS), but lack of biomarkers for treatment failure. Post-depletion immune reconstitution may identify patients, previous characterizations suffered from methodological limitations flow cytometry. Time-of-flight mass cytometry (CyTOF) is a comprehensive analytic modality that simultaneously quantifies over 40 cellular...
Cholera toxin B subunit (CTB) is an efficient mucosal carrier molecule for the generation of immune responses to linked antigens. There also good evidence that CTB acts as immunosuppressant, it able down-modulate human monocyte/macrophage cell line activation and suppress Th1-type responses. In present study, we examined possibility recombinant (rCTB) may affect dendritic (DC) functions in response LPS stimulation induce DC with capacity generate CD4(+) regulatory T cells (Tregs). Our...