[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"health-studies-list:{\"conditionNormalized\":\"primary-immunodeficiencies\",\"overallStatus\":[\"RECRUITING\",\"AVAILABLE\",\"NOT_YET_RECRUITING\"],\"orderBy\":\"LastUpdateSubmitDate:desc\",\"size\":25,\"offset\":0}":3,"health-study-condition:primary-immunodeficiencies":29},{"pageToken":4,"total":5,"offset":6,"count":5,"results":7},null,2,0,[8,43],{"id":9,"slug":10,"hasResults":11,"nctId":12,"briefTitle":13,"officialTitle":13,"acronym":14,"eligibilityCriteria":15,"healthyVolunteers":11,"sex":16,"minAge":17,"maxAge":18,"enrollmentInfo":19,"targetDuration":4,"studyType":22,"phases":4,"briefSummary":23,"conditions":24,"keywords":4,"overallStatus":30,"whyStopped":4,"lastUpdateSubmitDate":31,"lastUpdatePostDateStruct":32,"startDateStruct":35,"completionDateStruct":37,"leadSponsor":39,"locationsCount":42},"100612253","characterization-of-autoreactive-b-lymphocytes-in-autoimmune-diseases-and-immune-deficiencies-100612253",false,"NCT07251179","Characterization of Autoreactive b Lymphocytes in Autoimmune Diseases and Immune Deficiencies","AutoB-Tetramer","Inclusion Criteria:\n\n* Patients aged between 18 and 70\n* Patients for whom at least one of the following conditions has been confirmed:\n* Systemic lupus erythematosus meeting the 2019 ACR\u002FEULAR classification criteria.\n* Systemic scleroderma meeting the 2013 ACR\u002FEULAR classification criteria. ANCA-associated vasculitis according to the 2022 EULAR\u002FACR classification criteria.\n* Antiphospholipid syndrome according to the 2023 ACR\u002FEULAR criteria.\n* Primary immunodeficiencies according to IUIS criteria.\n* Patients capable of understanding the objectives of the research.\n* Patients affiliated with a social security health insurance scheme (beneficiary or dependant).\n* Patients who have signed and dated the informed consent form for non-identifying genetic testing.\n\nExclusion Criteria:\n\n* Patient refusing to participate in the study\n* Patient in a period of exclusion (determined by a previous or ongoing study) Inability to provide the subject with informed consent (in an emergency or immediate life-threatening situation, difficulties in understanding the subject, etc.)\n* Patient under legal protection\n* Patient under guardianship or conservatorship","ALL","18 Years","70 Years",{"count":20,"type":21},200,"ESTIMATED","OBSERVATIONAL","Autoimmune diseases (AID), whether systemic or organ-specific, affect approximately one in ten people, and their prevalence continues to increase. Many AIDs are linked to the emergence of autoreactive B cells (BCs) directed against components of the self. In healthy individuals, these autoreactive B cells are counter-selected or regulated before reaching the antibody-secreting cell compartment. However, in predisposed individuals, a breakdown in B cell tolerance can occur, leading to the formation of autoantibodies with devastating consequences, such as the emergence of systemic lupus erythematosus (SLE), rheumatoid arthritis, and vasculitis. B-cell depletion is often beneficial in these patients, but paradoxically, therapies targeting B cells are not always effective. Furthermore, B cell depletion via LB-specific antibodies (anti-CD20) or treatment with CD19 chimeric antigen receptor T cells (CAR T) leads to complete and prolonged depression of the entire B compartment without targeting the LB population responsible for the onset of the disease. To date, two pitfalls in studies of human autoreactive LBs often complicate the interpretation of results:\n\ni) the difficulty of identifying autoreactive LBs among all LBs, ii) demonstrating the pathogenicity of autoreactive B lymphocytes when they can be identified individually. We propose to quantify and phenotype these autoreactive\u002Fpathogenic B cells using high-throughput flow cytometry in several clinical situations.",[25,26,27,28,29],"Systemic Lupus Erythematosus","Systemic Scleroderma","ANCA-associated Vasculitis","Antiphospholipid Syndrome","Primary Immunodeficiencies","RECRUITING","2026-08-18",{"date":33,"type":34},"2026-08-20","ACTUAL",{"date":36,"type":34},"2026-01-20",{"date":38,"type":21},"2031-12-31",{"name":40,"class":41},"University Hospital, Strasbourg, France","OTHER",1,{"id":44,"slug":45,"hasResults":11,"nctId":46,"briefTitle":47,"officialTitle":47,"acronym":48,"eligibilityCriteria":49,"healthyVolunteers":11,"sex":16,"minAge":50,"maxAge":51,"enrollmentInfo":52,"targetDuration":4,"studyType":22,"phases":4,"briefSummary":54,"conditions":55,"keywords":57,"overallStatus":63,"whyStopped":4,"lastUpdateSubmitDate":64,"lastUpdatePostDateStruct":65,"startDateStruct":67,"completionDateStruct":69,"leadSponsor":71,"locationsCount":42},"100651773","long-term-psychological-and-cognitive-evaluation-of-children-treated-with-allogeneic-hematopoietic-stem-cell-transplantation-for-immunodeficiency-100651773","NCT07766304","Long-term Psychological and Cognitive Evaluation of Children Treated With Allogeneic Hematopoietic Stem Cell Transplantation for Immunodeficiency","PSY-DIP","Inclusion Criteria:\n\n* Patients who underwent allogeneic transplantation for a primary immunodeficiency at Necker Hospital, regardless of the genetic diagnosis, and at least 2 years post Allogeneic hematopoietic stem cell transplantation.\n* Chronological age at the time of evaluation between 6 years and 8 years 11 months.\n* Holders of parental authority and children or adolescents or adults' patients informed and consenting to participate in the study\n\nExclusion Criteria:\n\n* Child transplanted for a condition other than the primary immunodeficiency or at a different center.\n* Presence of an associated acquired or genetic neurological condition, independent of the PID, likely to significantly impair cognitive development.\n* Severe uncorrected sensory impairment (auditory or visual) rendering the cognitive assessment uninterpretable.\n* Refusal to participate by the child or those with parental authority.\n* Child not fluent in French or not proficient enough in French to complete the cognitive tests and questionnaires.\n* Profound intellectual disability.","6 Years","8 Years",{"count":53,"type":21},30,"Primary immunodeficiencies (PIDs) are a large group of genetic diseases of the immune system with highly variable clinical presentations. Allogeneic hematopoietic stem cell transplantation (HSCT) is one of the treatments offered to some patients with PIDs. It is a curative but particularly demanding treatment, potentially life-threatening, requiring several months of hospitalization, prolonged limitations in social interactions for the patient, and impacting the entire family unit. The short-term complications of HSCT are numerous and well-known. However, few studies describe the long-term psychological and cognitive complications of HSCT, particularly in the context of PIDs. The few published studies in children concern patients transplanted for hematological malignancies, a context very different from that of primary immunodeficiencies.\n\nThis study is a pilot research project focusing on the multidimensional assessment of the neurocognitive, psychological, and psychosocial functioning of children between 6 and 8 years old who have received allogeneic hematopoietic stem cell transplantation for primary immunodeficiency for at least 2 years. These stringent criteria aim to limit biases related to the diversity of ages at which care is provided.",[29,56],"ALLOGENEIC HEMATOPOIETIC STEM CELL TRANSPLANTATION",[58,59,60,61,62],"Primary immunodeficiencies","Allogeneic hematopoietic stem cell transplantation","Neurocognitive functioning","Psychological functioning","Psychosocial functioning","NOT_YET_RECRUITING","2026-08-13",{"date":66,"type":34},"2026-08-14",{"date":68,"type":21},"2026-08",{"date":70,"type":21},"2028-08",{"name":72,"class":41},"Assistance Publique - Hôpitaux de Paris"]