Boris Sorin
A5057601907- Vasculitis and related conditions
- Immunodeficiency and Autoimmune Disorders
- SARS-CoV-2 and COVID-19 Research
- interferon and immune responses
- Renal Diseases and Glomerulopathies
- Autoimmune and Inflammatory Disorders
- COVID-19 Clinical Research Studies
- Viral-associated cancers and disorders
- Eosinophilic Disorders and Syndromes
- Kawasaki Disease and Coronary Complications
- Platelet Disorders and Treatments
- Immune Cell Function and Interaction
- NF-κB Signaling Pathways
- Blood groups and transfusion
- Inflammasome and immune disorders
- Systemic Sclerosis and Related Diseases
- Cytomegalovirus and herpesvirus research
- Hepatitis B Virus Studies
- Sarcoidosis and Beryllium Toxicity Research
- Whipple's Disease and Interleukins
- Viral Infections and Immunology Research
- Electric Power Systems and Control
- Industrial Engineering and Technologies
- Immune Response and Inflammation
- Chronic Lymphocytic Leukemia Research
Institut des Maladies Génétiques Imagine
2021-2025
Inserm
1996-2025
Université Paris Cité
2021-2025
Hôpital Cochin
2020-2024
Assistance Publique – Hôpitaux de Paris
2023-2024
Hôpital Saint-Louis
2022-2023
Université Paris-Est Créteil
2023
Hôpitaux Universitaires Henri-Mondor
2023
Sorbonne Université
2015
IKKα, encoded by CHUK, is crucial in the non-canonical NF-κB pathway and part of IKK complex activating canonical alongside IKKβ. The absence IKKα causes fetal encasement syndrome humans, fatal utero, while an impaired IKKα-NIK interaction was reported a single patient combined immunodeficiency. Here, we describe compound heterozygous variants kinase domain female with hypogammaglobulinemia, recurrent lung infections, Hay–Wells syndrome-like features. We showed that both were...
To identify characteristics of granulomatosis with polyangiitis (GPA) associated induction failure, describe salvage therapies and their efficacy.We conducted a nationwide retrospective case-control study GPA failure between 2006 2021. Each patient was randomly paired to three controls matched for age, sex treatment.We included 51 patients (29 men 22 women). At therapy, median age 49 years. Twenty-seven received intravenous cyclophosphamide (ivCYC) 24 rituximab (RTX) as therapy. Patients...
Gain-of-function mutations in stimulator of interferon gene 1 (STING1) result STING-associated vasculopathy with onset infancy (SAVI), a severe autoinflammatory disease. Although elevated type I (IFN) production is thought to be the leading cause symptoms observed patients, STING can induce set pathways, which have roles and severity SAVI remain elucidated. To this end, we performed multi-omics comparative analysis peripheral blood mononuclear cells (PBMCs) plasma from patients healthy...
The aim of this study was to describe the efficacy and safety rituximab MTX (RTX/MTX) combination therapy in ANCA-associated vasculitides (AAV).A retrospective French nationwide conducted patients with AAV who received RTX/MTX for persistently active disease.Seventeen were included. All had granulomatosis polyangiitis (GPA), positive ANCA 76% them, mainly PR3-ANCA specificity. Sixteen (94%) priorly failed achieve remission RTX 11 (65%) CYC. Patients experienced a median 3 (2-4) flares....
IKKα, encoded by
<h3>Background:</h3> Systemic lupus erythematosus is a complex disease with highly variable clinical presentation and immune dysregulation. The study of rare inborn errors immunity developing autoimmunity can help to better understand the pathophysiology more common diseases. In human, haploinsufficiency RELA/p65 has been described in patients dominant inheritance mucocutaneous ulceration systemic inflammation. RELA (p65) major transcription factor canonical NF-kB pathway implicated...
Abstract SARS-CoV-2 infection in children is generally milder than adults, yet a proportion of cases result hyperinflammatory conditions often including myocarditis. To better understand these cases, we applied multi-parametric approach to the study blood cells 56 hospitalized with suspicion infection. The most severe forms MIS-C (multisystem inflammatory syndrome related SARS-CoV-2), that resulted myocarditis, were characterized by elevated levels pro-angiogenesis cytokines and several...
SARS-CoV-2 infection in children is generally milder than adults, yet a proportion of cases result hyperinflammatory conditions often including myocarditis. To better understand these cases, we applied multi-parametric approach to the study blood cells 56 hospitalized with suspicion infection. The most severe forms MIS-C (multisystem inflammatory syndrome related SARS-CoV-2), that resulted myocarditis, were characterized by elevated levels pro-angiogenesis cytokines and several chemokines....
Objective: To improve the methods of calculation series compensation devices (UPK), taking into account make-away bunch trains. Methods: determine maximum losses voltage and currents in catenary circuit probability theory mathematic statistics were used. Results: The paper provides technology for UPK calculation, that allows to capacity between-substation zones, considering real-life traffic schedule allowable at train collectors. Practical importance: Alteration technologies calculations,...
Abstract Gain-of-function mutations in STING1 , which encodes the Stimulator of Interferon Gene (STING), result a severe autoinflammatory disease termed STING-associated vasculopathy with onset infancy (SAVI). Although elevated type I interferon (IFN) production is thought to be leading cause symptoms observed patients, STING can induce set pathways, roles and severity SAVI, remain elucidated. To address this point, we compared single-cell RNA sequencing (scRNA-seq) dataset peripheral blood...