Éric Jeziorski
A5085174982- Immunodeficiency and Autoimmune Disorders
- Blood disorders and treatments
- Histiocytic Disorders and Treatments
- Blood groups and transfusion
- Parvovirus B19 Infection Studies
- Autoimmune and Inflammatory Disorders Research
- Platelet Disorders and Treatments
- Streptococcal Infections and Treatments
- Viral-associated cancers and disorders
- Respiratory viral infections research
- Inflammasome and immune disorders
- Viral gastroenteritis research and epidemiology
- Cytomegalovirus and herpesvirus research
- Pneumonia and Respiratory Infections
- Orthopedic Infections and Treatments
- Vascular Malformations and Hemangiomas
- Venous Thromboembolism Diagnosis and Management
- Blood Coagulation and Thrombosis Mechanisms
- Adolescent and Pediatric Healthcare
- Infective Endocarditis Diagnosis and Management
- Antimicrobial Resistance in Staphylococcus
- Pneumocystis jirovecii pneumonia detection and treatment
- Mycobacterium research and diagnosis
- Diabetes and associated disorders
- Neutrophil, Myeloperoxidase and Oxidative Mechanisms
Inserm
2008-2025
Université de Montpellier
2016-2025
Centre Hospitalier Universitaire de Montpellier
2015-2025
Hôpital Arnaud de Villeneuve
2015-2024
Société Française de Cardiologie
2024
Centre de Référence des Maladies Autoinflammatoires et des Amyloses
2019-2024
Établissement Français du Sang
2024
Hôpital Lapeyronie
2024
Pathogenesis and Control of Chronic and Emerging Infections
2022-2024
Sorbonne Université
2023
Langerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasia with a broad spectrum of clinical manifestations and outcomes in children. The somatic BRAF(V600E) mutation occurs frequently, but significance remains to be determined.BRAF(V600E) was investigated French LCH cohort. We analyzed associations between status presentation, extent disease, reactivation rate, response therapy, long-term permanent sequelae.Among 315 patients successfully determined BRAF status, 173 (54.6%)...
Inflammasomes are multiprotein complexes that sense pathogens and trigger biological mechanisms to control infection. Nucleotide-binding oligomerisation domain-like receptor (NLR) containing a PYRIN domain 1 (NLRP1), NLRP3 NLRC4 plays key role in this innate immune system by directly assembling inflammasomes regulating inflammation. Mutations linked hereditary autoinflammatory diseases, whereas polymorphisms NLRP1 associated with autoimmune disorders such as vitiligo rheumatoid arthritis....
Background Langerhans cell histiocytosis (LCH) is a rare clonal granulomatous disease that affects mainly children. LCH can involve various tissues such as bone, skin, lung, bone marrow, lymph nodes, and the central nervous system, frequently responsible for functional sequelae. The pathophysiology of unclear, but uncontrolled proliferation cells (LCs) believed to be primary event in formation granulomas. present study was designed further investigate nature proliferating immune mechanisms...
Summary The French national cohort of children with Langerhans cell histiocytosis ( LCH ) has included 1478 patients since it was established in 1983. therapeutic strategies substantially changed 1998, so we have divided the into two 15‐year periods. Starting therapy duration increased from 6 to 12 months, repeated induction performed cases showing a poor response first vinblastine and steroids, refractory disease risk organ RO +) treated cladribine cytarabine. A total 483 (33%) were...
Abstract Neurodegenerative ( ND ) complications in Langerhans cell histiocytosis LCH are a late‐onset but dramatic sequelae for which incidence and risk factors not well defined. Based on national prospective registry of paediatric patients, we determined the rate clinical cND ‐ analysed factors, taking into account disease extent molecular characteristics. Among 1897 36 (1·9%) were diagnosed with . The 10‐year cumulative was 4·1%. typically affected patients previously treated multisystem,...
Life-threatening “breakthrough” cases of critical COVID-19 are attributed to poor or waning antibody (Ab) response SARS-CoV-2 vaccines in individuals already at risk. Preexisting auto-Abs neutralizing type I IFNs underlie least 15% pneumonia unvaccinated individuals; their contribution hypoxemic breakthrough vaccinated people is unknown. We studied a cohort 48 (aged 20 86 years) who received two doses messenger RNA (mRNA) vaccine and developed infection with 2 weeks 4 months later. Ab levels...
Abstract The spectrum of somatic mutations in pediatric histiocytoses and their clinical implications are not fully characterized, especially for non‐Langerhans cell histiocytosis (‐LCH) subtypes. A cohort 415 children with from the French registry was reviewed analyzed BRAF V600E . Most WT samples were by next‐generation sequencing (NGS) a custom panel genes myeloid neoplasia. Of case samples, there 366 LCH, 1 Erdheim‐Chester disease, 21 Rosai‐Dorfman disease (RDD), juvenile xanthogranuloma...
An expanding number of monogenic defects have been identified as causative severe forms very early-onset inflammatory bowel diseases [VEO-IBD]. The present study aimed at defining how next-generation sequencing [NGS] methods can be used to improve identification known molecular diagnosis and adapt treatment.A total 207 children were recruited in 45 paediatric centres through an international collaborative network [ESPGHAN GENIUS working group] with a clinical presentation VEO-IBD [n = 185]...
Background. About 10% of pediatric patients with invasive pneumococcal disease (IPD) die from the disease. Some primary immunodeficiencies (PIDs) are known to confer predisposition IPD. However, a systematic search for these PIDs has never been carried out in children presenting Methods. We prospectively identified cases IPD requiring hospitalization between 2005 and 2011 28 wards throughout France. was defined as positive culture, polymerase chain reaction result, and/or soluble antigen...
The BRAFV600E mutation is reported in half of patients with Langerhans cell histiocytosis (LCH). This study investigated the detection allele circulating cell-free (ccf) DNA a paediatric LCH cohort. Children -mutated were to detect ccf at diagnosis (n = 48) and during follow-up 17) using picolitre-droplet digital PCR assay. At diagnosis, was positive 15/15 (100%) risk-organ multisystem (RO+ MS) LCH, 5/12 (42%) RO- MS 3/21 (14%) single-system (SS) (P < 0·001, Fisher's exact test). load higher...
Evans syndrome (ES) is a rare autoimmune disorder whose long-term follow-up characteristics are unknown. Patients under 18 at the time of diagnosis first cytopenia have been included since 2004 in national prospective observational cohort. In 2014, 156 children diagnosed between 1981 and 2014 with ES, were analyzed. The median age initial was 5.4 (0.2-17.2) years old. For 85 sequential cases, delay episodes AIHA ITP 2.4 (0.1–16.3). ES 6.5 (0.1-28.8). revealed underlying diseases 10%...
The purpose of this study was to describe the natural history severe congenital neutropenia (SCN) in 14 patients with G6PC3 mutations and enrolled French SCN registry. Among 605 included registry, we identified 8 pedigrees that autosomal recessive mutations. Median age at last visit 22.4 years. All had developed various comordibities, including prominent veins (n = 12), cardiac malformations intellectual disability 7), myopathic syndrome recurrent painful cramps 1). Three Crohn's disease,...
Langerhans cell histiocytosis (LCH) is a rare disease that affects mainly young children, and which features granulomas containing Langerhans-type dendritic cells. The role of several human herpesviruses (HHV) in the pathogenesis LCH was suggested by numerous reports but remains debated. Epstein-barr virus (EBV, HHV-4), & Cytomegalovirus (CMV, HHV-5) can infect cells, EBV, CMV HHV-6 have been proposed to be associated with based on detection these viruses clinical samples.We investigated...