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Page 1
X-linked lymphoproliferative syndrome in mainland China: review of clinical, genetic, and immunological characteristic.
Xu T, Zhao Q, Li W, Chen X, Xue X, Chen Z, Du X, Bai X, Zhao Q, Zhou L, Tang X, Yang X, Kanegane H, Zhao X. Xu T, et al. Eur J Pediatr. 2020 Feb;179(2):327-338. doi: 10.1007/s00431-019-03512-7. Epub 2019 Nov 21. Eur J Pediatr. 2020. PMID: 31754776 Free PMC article.
X-linked lymphoproliferative syndrome (XLP) is a rare primary immunodeficiency disease that can be divided into two types: SAP deficiency (XLP1) and XIAP deficiency (XLP2), caused by mutations in the SH2D1A and XIAP genes, respectively. ...Significantl …
X-linked lymphoproliferative syndrome (XLP) is a rare primary immunodeficiency disease that can be divided into …
Single-Cell Transcriptomic Analysis of Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis.
Suzuki T, Sato Y, Okuno Y, Torii Y, Fukuda Y, Haruta K, Yamaguchi M, Kawamura Y, Hama A, Narita A, Muramatsu H, Yoshikawa T, Takahashi Y, Kimura H, Ito Y, Kawada JI. Suzuki T, et al. J Clin Immunol. 2024 Apr 20;44(4):103. doi: 10.1007/s10875-024-01701-0. J Clin Immunol. 2024. PMID: 38642164
Three pediatric patients with EBV-HLH with different backgrounds, one with X-linked lymphoproliferative syndrome type 1 (XLP1), two with chronic active EBV disease (CAEBV), and two patients with EBV-IM were enrolled. ...This proliferative cluster was s …
Three pediatric patients with EBV-HLH with different backgrounds, one with X-linked lymphoproliferative syndrome …
X chromosome linked immunodeficiency.
Schwaber J, Rosen FS. Schwaber J, et al. Immunodefic Rev. 1990;2(3):233-51. Immunodefic Rev. 1990. PMID: 1981831 Review.
X-linked chronic granulomatous disease and Wiskott-Aldrich syndrome map to loci on the short arm of the X chromosome; X-linked severe combined immunodeficiency, X-linked agammaglobulinemia, X-linked immunodeficiency with hyper-IgM, and X-linked lymphoproliferativ …
X-linked chronic granulomatous disease and Wiskott-Aldrich syndrome map to loci on the short arm of the X chromosome; X-linked severe combin …
Applications of flow cytometry for the study of primary immune deficiencies.
Oliveira JB, Notarangelo LD, Fleisher TA. Oliveira JB, et al. Curr Opin Allergy Clin Immunol. 2008 Dec;8(6):499-509. doi: 10.1097/ACI.0b013e328312c790. Curr Opin Allergy Clin Immunol. 2008. PMID: 18978463 Review.
RECENT FINDINGS: The immunophenotypic evaluation of selected PIDs provides diagnostic clues as well as information useful to classify patients and predict clinical outcome. In addition, the evaluation of intracellular proteins associated with selected PIDs has evolved as a …
RECENT FINDINGS: The immunophenotypic evaluation of selected PIDs provides diagnostic clues as well as information useful to classify patien …
XIAP restrains TNF-driven intestinal inflammation and dysbiosis by promoting innate immune responses of Paneth and dendritic cells.
Wahida A, Müller M, Hiergeist A, Popper B, Steiger K, Branca C, Tschurtschenthaler M, Engleitner T, Donakonda S, De Coninck J, Öllinger R, Pfautsch MK, Müller N, Silva M, Usluer S, Thiele Orberg E, Böttcher JP, Pfarr N, Anton M, Slotta-Huspenina JB, Nerlich AG, Madl T, Basic M, Bleich A, Berx G, Ruland J, Knolle PA, Rad R, Adolph TE, Vandenabeele P, Kanegane H, Gessner A, Jost PJ, Yabal M. Wahida A, et al. Sci Immunol. 2021 Nov 5;6(65):eabf7235. doi: 10.1126/sciimmunol.abf7235. Epub 2021 Nov 5. Sci Immunol. 2021. PMID: 34739338
Deficiency in X-linked inhibitor of apoptosis protein (XIAP) is the cause for X-linked lymphoproliferative syndrome 2 (XLP2). About one-third of these patients suffer from severe and therapy-refractory inflammatory bowel disease (IBD), but the exact ca …
Deficiency in X-linked inhibitor of apoptosis protein (XIAP) is the cause for X-linked lymphoproliferative syndrome …
XIAP deficiency in humans causes an X-linked lymphoproliferative syndrome.
Rigaud S, Fondanèche MC, Lambert N, Pasquier B, Mateo V, Soulas P, Galicier L, Le Deist F, Rieux-Laucat F, Revy P, Fischer A, de Saint Basile G, Latour S. Rigaud S, et al. Nature. 2006 Nov 2;444(7115):110-4. doi: 10.1038/nature05257. Nature. 2006. PMID: 17080092
In humans, defects in immune homeostasis result in lymphoproliferation disorders including autoimmunity, haemophagocytic lymphohystiocytosis and lymphomas. The X-linked lymphoproliferative syndrome (XLP) is a rare, inherited immunodeficiency that is ch …
In humans, defects in immune homeostasis result in lymphoproliferation disorders including autoimmunity, haemophagocytic lymphohystiocytosis …
Mapping the X-linked lymphoproliferative syndrome.
Skare JC, Milunsky A, Byron KS, Sullivan JL. Skare JC, et al. Proc Natl Acad Sci U S A. 1987 Apr;84(7):2015-8. doi: 10.1073/pnas.84.7.2015. Proc Natl Acad Sci U S A. 1987. PMID: 2882515 Free PMC article.
The X-linked lymphoproliferative syndrome is triggered by Epstein-Barr virus infection and results in fatal mononucleosis, immunodeficiency, and lymphoproliferative disorders. ...It is now possible to predict which members of a family with X …
The X-linked lymphoproliferative syndrome is triggered by Epstein-Barr virus infection and results in fatal mono …
Malignant lymphoma in the X-linked lymphoproliferative syndrome.
Harrington DS, Weisenburger DD, Purtilo DT. Harrington DS, et al. Cancer. 1987 Apr 15;59(8):1419-29. doi: 10.1002/1097-0142(19870415)59:8<1419::aid-cncr2820590807>3.0.co;2-p. Cancer. 1987. PMID: 3815312
Male patients with the X-linked lymphoproliferative syndrome (XLP) have an inherited immune deficiency to Epstein-Barr virus (EBV) infection that results in fatal infectious mononucleosis (IM), acquired hypogammaglobulinemia- or agammaglobulinemia, vir …
Male patients with the X-linked lymphoproliferative syndrome (XLP) have an inherited immune deficiency to Epstei …
Clinical similarities and differences of patients with X-linked lymphoproliferative syndrome type 1 (XLP-1/SAP deficiency) versus type 2 (XLP-2/XIAP deficiency).
Pachlopnik Schmid J, Canioni D, Moshous D, Touzot F, Mahlaoui N, Hauck F, Kanegane H, Lopez-Granados E, Mejstrikova E, Pellier I, Galicier L, Galambrun C, Barlogis V, Bordigoni P, Fourmaintraux A, Hamidou M, Dabadie A, Le Deist F, Haerynck F, Ouachée-Chardin M, Rohrlich P, Stephan JL, Lenoir C, Rigaud S, Lambert N, Milili M, Schiff C, Chapel H, Picard C, de Saint Basile G, Blanche S, Fischer A, Latour S. Pachlopnik Schmid J, et al. Blood. 2011 Feb 3;117(5):1522-9. doi: 10.1182/blood-2010-07-298372. Epub 2010 Nov 30. Blood. 2011. PMID: 21119115 Free article.
Recurrent splenomegaly often associated with cytopenia and fever was preferentially observed in XLP-2 (XLP-1, 7%; XLP-2, 87%) and probably represents minimal forms of HLH as documented by histopathology. ...
Recurrent splenomegaly often associated with cytopenia and fever was preferentially observed in XLP-2 (XLP-1, 7%; XLP-2, 87%) and pro …
Clinical aspects on Epstein-Barr virus infection.
Andersson JP. Andersson JP. Scand J Infect Dis Suppl. 1991;80:94-104. Scand J Infect Dis Suppl. 1991. PMID: 1666450 Review.
An inhibition of oropharyngeal EBV replication was verified but minimal effects on clinical symptoms was observed. A combination of intravenous acyclovir and prednisolone treatment for 10 days was therefore tried in 15 patients with fulminant mononucleosis in a pilot study …
An inhibition of oropharyngeal EBV replication was verified but minimal effects on clinical symptoms was observed. A combination of i …
44 results