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190 results on '"Bénédicte, Neven"'

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1. Lack of association between classical HLA genes and asymptomatic SARS-CoV-2 infection

2. Part 5: Allogeneic HSCT in refractory SJIA with lung disease; recent cases from centers in North America & Europe

3. NBEAL2 deficiency in humans leads to low CTLA-4 expression in activated conventional T cells

4. On behalf of the SFGM‐TC: Real‐life use of third‐party virus‐specific T‐cell transfer in immunocompromised transplanted patients

5. Sustained remission after haploidentical bone marrow transplantation in a child with refractory systemic juvenile idiopathic arthritis

6. Early-onset autoimmunity associated with SOCS1 haploinsufficiency

7. Bayesian Modeling Immune Reconstitution Apply to CD34+ Selected Stem Cell Transplantation for Severe Combined Immunodeficiency

8. Physicochemical Stability Study of Oral Suspension Containing Ruxolitinib in Children with Steroid-Refractory Acute Graft-Versus-Host Disease

9. Emerging Place of JAK Inhibitors in the Treatment of Inborn Errors of Immunity

10. Impact of age at diagnosis, sex, and immunopathological manifestations in 886 patients with pediatric chronic immune thrombocytopenia

11. Long term follow-up of pediatric-onset Evans syndrome: broad immunopathological manifestations and high treatment burden

12. Different Clinical Presentations and Outcomes of Disseminated Varicella in Children With Primary and Acquired Immunodeficiencies

13. Safety and efficacy of brentuximab vedotin as a treatment for lymphoproliferative disorders in primary immunodeficiencies

14. STING Gain-of-Function Disrupts Lymph Node Organogenesis and Innate Lymphoid Cell Development in Mice

15. Long term follow-up after haematopoietic stem cell transplantation for mucopolysaccharidosis type I-H: a retrospective study of 51 patients

16. Type I interferon-mediated autoinflammation due to DNase II deficiency

17. A web-based collection of genotype-phenotype associations in hereditary recurrent fevers from the Eurofever registry

18. Lymphadenopathy driven by TCR-Vγ8Vδ1 T-cell expansion in FAS-related autoimmune lymphoproliferative syndrome

19. Proceedings of the 23rd Paediatric Rheumatology European Society Congress: part one

20. Hematopoietic stem cell transplantation for adolescents and adults with inborn errors of immunity: an EBMT IEWP study

21. Chronic Intestinal Pseudo-Obstruction and Lymphoproliferative Syndrome as a Novel Phenotype Associated With Tetratricopeptide Repeat Domain 7A Deficiency

22. JAK inhibition in Aicardi-Goutières syndrome: a monocentric multidisciplinary real-world approach study

23. HLA-Haploidentical Stem Cell Transplantation in Children with Inherited Bone Marrow Failure Syndromes: A Retrospective Analysis on Behalf of EBMT Severe Aplastic Anemia Working Party

24. Practice harmonization workshops of EBMT: an expert-based approach to generate practical and contemporary guidelines within the arena of hematopoietic cell transplantation and cellular therapy

25. DOCK11 deficiency in patients with X-linked actinopathy and autoimmunity

26. Current Understanding and Future Research Priorities in Malignancy Associated With Inborn Errors of Immunity and DNA Repair Disorders: The Perspective of an Interdisciplinary Working Group

27. Human type I IFN deficiency does not impair B cell response to SARS-CoV-2 mRNA vaccination

28. Hematopoietic cell transplantation and cellular therapies in Europe 2021. The second year of the SARS-CoV-2 pandemic. A Report from the EBMT Activity Survey

29. Impact of graft function on health status and quality of life in 112 very long-term survivors who received an HSCT for Inborn Errors of Immunity, a prospective study of the CEREDIH

30. Inherited human ITK deficiency impairs IFN-γ immunity and underlies tuberculosis

31. Copy number variations and founder effect underlying complete IL-10Rβ deficiency in Portuguese kindreds.

32. Mutations in the adaptor-binding domain and associated linker region of p110δ cause Activated PI3K-δ Syndrome 1 (APDS1)

33. Hydroxyurea does not affect the spermatogonial pool in prepubertal patients with sickle cell disease

34. Two Novel Homozygous Mutations in Phosphoglucomutase 3 Leading to Severe Combined Immunodeficiency, Skeletal Dysplasia, and Malformations

35. Human T-bet governs the generation of a distinct subset of CD11c high CD21 low B cells

36. Allogeneic stem cell transplantation compared to conservative management in adults with inborn errors of immunity

37. Inherited TNFSF9 deficiency causes broad Epstein–Barr virus infection with EBV+ smooth muscle tumors

38. Expanding the Nude SCID/CID Phenotype Associated with FOXN1 Homozygous, Compound Heterozygous, or Heterozygous Mutations

39. Bone Marrow Transplantation in Congenital Erythropoietic Porphyria: Sustained Efficacy but Unexpected Liver Dysfunction

40. The 2021 Eurpean Alliance of Associations for Rheumatology/American College of Rheumatology points to consider for diagnosis and management of autoinflammatory type i interferonopathies: CANDLE/PRAAS, SAVI and AGS

41. International retrospective study of allogeneic hematopoietic cell transplantation for activated PI3K-delta syndrome

42. Monoclonal antibody-mediated neutralization of SARS-CoV-2 in an IRF9-deficient child

43. Actin dynamics regulation by TTC7A/PI4KIIIα axis limits DNA damage and cell death during leukocyte migration

44. Inherited human c-Rel deficiency disrupts myeloid and lymphoid immunity to multiple infectious agents

45. Pediatric-Onset Evans Syndrome Is Associated with Broad Immunopathological Manifestations, High Treatment Burden and Mortality in Long-Term Follow-up

46. Use of ruxolitinib in COPA syndrome manifesting as life-threatening alveolar haemorrhage

47. Outcomes of Salvage Haploidentical Transplant with Post-Transplant Cyclophosphamide for Rescuing Graft Failure Patients: a Report on Behalf of the Francophone Society of Bone Marrow Transplantation and Cellular Therapy

48. Improved Outcome in Young Children Compared to Adolescents and Adults After Allogeneic Hematopoietic Stem Cell Transplant for Acute Myeloid Leukemia: a Retrospective Study From Francophone Society of Bone Marrow Transplantation and Cell Therapy (SFGM-TC) (SFGM-TC)

49. Alternative pathways for the development of lymphoid structures in humans

50. EBMT/ESID inborn errors working party guidelines for hematopoietic stem cell transplantation for inborn errors of immunity

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