Clinical similarities and differences of patients with X-linked lymphoproliferative syndrome type 1 (XLP-1/SAP deficiency) versus type 2 (XLP-2/XIAP deficiency).
about
Mucosal-associated invariant T cells in autoimmunity, immune-mediated diseases and airways diseaseResponses to Microbial Challenges by SLAMF ReceptorsCytotoxic granule secretion by lymphocytes and its link to immune homeostasisRegulation of Cell Death by IAPs and Their AntagonistsT-regulatory cells in primary immune deficienciesMaking sense of the cytokine storm: a conceptual framework for understanding, diagnosing, and treating hemophagocytic syndromesThe diagnostic approach to monogenic very early onset inflammatory bowel diseaseGenetic variation in IBD: progress, clues to pathogenesis and possible clinical utilityOccurrence of nodular lymphocyte-predominant hodgkin lymphoma in hermansky-pudlak type 2 syndrome is associated to natural killer and natural killer T cell defects.Histiocytic glomerulopathy associated with macrophage activation syndrome.A Nationwide Study of Severe and Protracted Diarrhoea in Patients with Primary Immunodeficiency DiseasesThe role of the innate and adaptive immune system in pediatric inflammatory bowel disease.BID-dependent release of mitochondrial SMAC dampens XIAP-mediated immunity against Shigella.X-Linked Lymphoproliferative Syndrome and Common Variable Immunodeficiency May Not Be Differentiated by SH2D1A and XIAP/BIRC4 Genes Sequence Analysis.Immunologic difference between hypersensitivity to mosquito bite and hemophagocytic lymphohistiocytosis associated with Epstein-Barr virus infectionEvidence for non-neutralizing autoantibodies against IL-10 signalling components in patients with inflammatory bowel diseaseCharacterization of Epstein-Barr virus (EBV)-infected cells in EBV-associated hemophagocytic lymphohistiocytosis in two patients with X-linked lymphoproliferative syndrome type 1 and type 2.Characterization of Ly108 in the thymus: evidence for distinct properties of a novel form of Ly108The diagnostic challenge of very early-onset enterocolitis in an infant with XIAP deficiencyIs Whole Exome Sequencing Clinically Practical in the Management of Pediatric Crohn's Disease?Recent advances in the diagnosis and treatment of hemophagocytic lymphohistiocytosis.Targeted sequencing identifies a novel SH2D1A pathogenic variant in a Chinese family: Carrier screening and prenatal genetic testing.BIRC4 Mutation: An Important Rare Cause of UveitisTargeted high-throughput sequencing for genetic diagnostics of hemophagocytic lymphohistiocytosis.Taking Crohn's disease personallyIntronic SH2D1A mutation with impaired SAP expression and agammaglobulinemia.Treatment of Epstein Barr virus-induced haemophagocytic lymphohistiocytosis with rituximab-containing chemo-immunotherapeutic regimens.Human iNKT and MAIT cells exhibit a PLZF-dependent proapoptotic propensity that is counterbalanced by XIAPSteroid-induced Deficiency of Mucosal-associated Invariant T Cells in the Chronic Obstructive Pulmonary Disease Lung. Implications for Nontypeable Haemophilus influenzae Infection.Disease-causing mutations in the XIAP BIR2 domain impair NOD2-dependent immune signalling.Familial hemophagocytic lymphohistiocytosis: when rare diseases shed light on immune system functioning.Familial hemophagocytic lymphohistiocytosis: a model for understanding the human machinery of cellular cytotoxicity.How does knowledge from translational research impact our clinical care of pediatric inflammatory bowel disease patients?Immunosuppression-related lymphomas and cancers in IBD: how can they be prevented?Interleukin-10 and interleukin-10-receptor defects in inflammatory bowel disease.Diagnostic evaluation of patients with suspected haemophagocytic lymphohistiocytosis.Advances in understanding the pathogenesis of HLH.Hemophagocytic lymphohistiocytosis (HLH): a review of literature.Monogenic diseases associated with intestinal inflammation: implications for the understanding of inflammatory bowel disease.Modern management of primary T-cell immunodeficiencies.
P2860
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P2860
Clinical similarities and differences of patients with X-linked lymphoproliferative syndrome type 1 (XLP-1/SAP deficiency) versus type 2 (XLP-2/XIAP deficiency).
description
2010 nî lūn-bûn
@nan
2010年の論文
@ja
2010年論文
@yue
2010年論文
@zh-hant
2010年論文
@zh-hk
2010年論文
@zh-mo
2010年論文
@zh-tw
2010年论文
@wuu
2010年论文
@zh
2010年论文
@zh-cn
name
Clinical similarities and diff ...... ype 2 (XLP-2/XIAP deficiency).
@en
type
label
Clinical similarities and diff ...... ype 2 (XLP-2/XIAP deficiency).
@en
prefLabel
Clinical similarities and diff ...... ype 2 (XLP-2/XIAP deficiency).
@en
P2093
P50
P1433
P1476
Clinical similarities and diff ...... ype 2 (XLP-2/XIAP deficiency).
@en
P2093
Alain Dabadie
Alain Fischer
Alain Fourmaintraux
Christelle Lenoir
Claire Galambrun
Claudin Schiff
Danielle Canioni
Eduardo Lopez-Granados
Ester Mejstrikova
Filomeen Haerynck
P304
P356
10.1182/BLOOD-2010-07-298372
P407
P50
P577
2010-11-30T00:00:00Z