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2. Severe adult hemophagocytic lymphohistiocytosis (HLHa) correlates with HLH-related gene variants

3. Childhood Langerhans cell histiocytosis hematological involvement: severity associated with BRAFV600E loads

4. Actin dynamics regulation by TTC7A/PI4KIIIα limits DNA damage and cell death under confinement

8. Human RIPK1 deficiency causes combined immunodeficiency and inflammatory bowel diseases

10. The European Society for Immunodeficiencies (ESID) Registry Working Definitions for the Clinical Diagnosis of Inborn Errors of Immunity

12. Contributors

13. Pediatric-onset Evans syndrome: Heterogeneous presentation and high frequency of monogenic disorders including LRBA and CTLA4 mutations

15. Severe adult hemophagocytic lymphohistiocytosis (HLHa) correlates with HLH-related gene variants

18. Autoimmune and inflammatory manifestations occur frequently in patients with primary immunodeficiencies

22. Germline HAVCR2 mutations altering TIM-3 characterize subcutaneous panniculitis-like T cell lymphomas with hemophagocytic lymphohistiocytic syndrome

23. X-linked primary immunodeficiency associated with hemizygous mutations in the moesin (MSN) gene

28. Immune deficiency–related enteropathy-lymphocytopenia-alopecia syndrome results from tetratricopeptide repeat domain 7A deficiency

31. Innenrücktitelbild: Small Molecule Inhibitors of Interferon‐Induced JAK‐STAT Signalling (Angew. Chem. 32/2022)

32. Inside Back Cover: Small Molecule Inhibitors of Interferon‐Induced JAK‐STAT Signalling (Angew. Chem. Int. Ed. 32/2022)

33. The risk of hemophagocytic lymphohistiocytosis in Hermansky-Pudlak syndrome type 2

39. Author Correction: Germline HAVCR2 mutations altering TIM-3 characterize subcutaneous panniculitis-like T cell lymphomas with hemophagocytic lymphohistiocytic syndrome

40. Small Molecule Inhibitors of Interferon‐Induced JAK‐STAT Signalling

44. Clinical similarities and differences of patients with X-linked lymphoproliferative syndrome type 1 (XLP-1/SAP deficiency) versus type 2 (XLP-2/XIAP deficiency)

46. X-linked lymphoproliferative disease due to SAP/SH2D1A deficiency: a multicenter study on the manifestations, management and outcome of the disease

47. Autoimmune and inflammatory manifestations occur frequently in patients with primary immunodeficiencies

48. Improving the diagnostic efficiency of primary immunodeficiencies with targeted next-generation sequencing

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