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排序方式: 共有154条查询结果,搜索用时 31 毫秒
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Susanne Unger Maximilian Seidl Pauline van Schouwenburg Mirzokhid Rakhmanov Alla Bulashevska Natalie Frede Bodo Grimbacher Jens Pfeiffer Klaudia Schrenk Luis Munoz Leif Hanitsch Ina Stumpf Fabian Kaiser Oliver Hausmann Florian Kollert Sigune Goldacker Mirjam van der Burg Baerbel Keller Klaus Warnatz 《The Journal of allergy and clinical immunology》2018,141(2):730-740
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Akbil Bengisu Meyer Tim Stubbemann Paula Thibeault Charlotte Staudacher Olga Niemeyer Daniela Jansen Jenny Mühlemann Barbara Doehn Jan Tabeling Christoph Nusshag Christian Hirzel Cédric Sanchez David Sökler Nieters Alexandra Lother Achim Duerschmied Daniel Schallner Nils Lieberum Jan Nikolaus August Dietrich Rieg Siegbert Falcone Valeria Hengel Hartmut Kölsch Uwe Unterwalder Nadine Hübner Ralf-Harto Jones Terry C. Suttorp Norbert Drosten Christian Warnatz Klaus Spinetti Thibaud Schefold Joerg C. Dörner Thomas Sander Leif Erik Corman Victor M. Merle Uta Kurth Florian von Bernuth Horst Meisel Christian Goffinet Christine 《Journal of clinical immunology》2022,42(6):1111-1129
Journal of Clinical Immunology - Six to 19% of critically ill COVID-19 patients display circulating auto-antibodies against type I interferons (IFN-AABs). Here, we establish a clinically applicable... 相似文献
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Renate Krüger Ulrich Baumann Stephan Borte Uwe Kölsch Myriam Ricarda Lorenz Baerbel Keller Ina Harder Klaus Warnatz Stephan Ehl Klaus Schwarz Volker Wahn Horst von Bernuth 《Scandinavian journal of immunology》2020,91(1):e12811
Hypomorphic mutations in the gene encoding Bruton tyrosine kinase (BTK) may result in milder phenotypes and delayed diagnosis of B-cell related immunodeficiencies due to residual BTK function. Newborn screening for kappa-deleting-recombination-excision circles (KRECs) reliably identifies classical X-linked agammaglobulinaemia (XLA) patients with profound B-cell lymphopenia at birth but has not been evaluated in patients with residual BTK function. We aimed to evaluate clinical findings, BTK function and KREC copy numbers in three patients with BTK mutations presenting with impaired polysaccharide responsiveness without agammaglobulinaemia. One patient had an invasive pneumococcal infection at the age of 4 years. All three patients (two brothers) had visible tonsils, normal to slightly decreased immunoglobulin G levels, undetectable pneumococcal antibodies despite pneumococcal conjugate vaccinations, no antibody response after a diagnostic polysaccharide vaccination as well as profound B-cell lymphopenia with residual B-cell differentiation. BTK mutations were identified by Sanger sequencing. BTK staining and phosphorylation assays were performed on peripheral B cells. KREC copy numbers were determined from dried blood spots obtained within the first week of life as well as once at the age of 8, 6 and 3 years, respectively. BTK staining showed residual protein expression. Also, residual BTK activity could be demonstrated. KREC copy numbers from dried blood spots were above the threshold set for detection of patients with profound B-cell lymphopenia. Male patients with impaired polysaccharide responsiveness should be evaluated for B-cell lymphopenia followed by BTK analyses irrespective of immunoglobulin levels or tonsil size. 相似文献
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Kathrin Pieper Marta Rizzi Matthaios Speletas Cristian R. Smulski Heiko Sic Helene Kraus Ulrich Salzer Gina J. Fiala Wolfgang W. Schamel Vassilios Lougaris Alessandro Plebani Lennart Hammarstrom Mike Recher Anastasios E. Germenis Bodo Grimbacher Klaus Warnatz Antonius G. Rolink Pascal Schneider Luigi D. Notarangelo Hermann Eibel 《The Journal of allergy and clinical immunology》2014
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Jan Rohr Karin Beutel Andrea Maul-Pavicic Thomas Vraetz Jens Thiel Klaus Warnatz Ilka Bondzio Ute Gross-Wieltsch Michael Sch��ndeln Barbara Sch��tz Wilhelm Woessmann Andreas H. Groll Brigitte Strahm Julia Pagel Carsten Speckmann Gritta Janka Gillian Griffiths Klaus Schwarz Udo zur Stadt Stephan Ehl 《Haematologica》2010,95(12):2080-2087
Background
Familial hemophagocytic lymphohistiocytosis is a genetic disorder of lymphocyte cytotoxicity that usually presents in the first two years of life and has a poor prognosis unless treated by hematopoietic stem cell transplantation. Atypical courses with later onset and prolonged survival have been described, but no detailed analysis of immunological parameters associated with typical versus atypical forms of familial hemophagocytic lymphohistiocytosis has been performed.Design and Methods
We analyzed disease manifestations, NK-cell and T-cell cytotoxicity and degranulation, markers of T-cell activation and B-cell differentiation as well as Natural Killer T cells in 8 patients with atypical familial hemophagocytic lymphohistiocytosis due to mutations in UNC13D and STXBP2.Results
All but one patient with atypical familial hemophagocytic lymphohistiocytosis carried at least one splice-site mutation in UNC13D or STXBP2. In most patients episodes of hemophagocytic lymphohistiocytosis were preceded or followed by clinical features typically associated with immunodeficiency, such as chronic active Epstein Barr virus infection, increased susceptibility to bacterial infections, granulomatous lung or liver disease, encephalitis or lymphoma. Five of 8 patients had hypogammaglobulinemia and reduced memory B cells. Most patients had a predominance of activated CD8+ T cells and low numbers of Natural Killer T cells. When compared to patients with typical familial hemophagocytic lymphohistiocytosis, NK-cell cytotoxicity and NK-cell and CTL degranulation were impaired to a similar extent. However, in patients with an atypical course NK-cell degranulation could be partially reconstituted by interleukin-2 and cytotoxic T-cell cytotoxicity in vitro was normal.Conclusions
Clinical and immunological features of atypical familial hemophagocytic lymphohistiocytosis show an important overlap to primary immunodeficiency diseases (particularly common variable immunodeficiency and X-linked lymphoproliferative syndrome) and must, therefore, be considered in a variety of clinical presentations. We show that degranulation assays are helpful screening tests for the identification of such patients. 相似文献9.
Ormsby T Schlecker E Ferdin J Tessarz AS Angelisová P Köprülü AD Borte M Warnatz K Schulze I Ellmeier W Horejsí V Cerwenka A 《Blood》2011,118(4):936-945
The triggering receptor expressed on myeloid cells 1 (TREM-1) has been implicated in the production of proinflammatory cytokines and chemokines during bacterial infection and sepsis. For downstream signal transduction, TREM-1 is coupled to the ITAM-containing adaptor DAP12. Here, we demonstrate that Bruton tyrosine kinase (Btk), a member of the Tec kinases, becomes phosphorylated upon TREM-1 triggering. In U937-derived cell lines, in which expression of Btk was diminished by shRNA-mediated knockdown, phosphorylation of Erk1/2 and PLCγ1 and Ca2? mobilization were reduced after TREM-1 stimulation. Importantly, TREM-1-induced production of the pro-inflammatory cytokines, TNF-α and IL-8, and up-regulation of activation/differentiation cell surface markers were impaired in Btk knockdown cells. Similar results were obtained upon TREM-1 stimulation of BMDCs of Btk(-/-) mice. The analysis of cells containing Btk mutants revealed that intact membrane localization and a functional kinase domain were required for TREM-1-mediated signaling. Finally, after TREM-1 engagement, TNF-α production by PBMCs was reduced in the majority of patients suffering from X-linked agammaglobulinemia (XLA), a rare hereditary disease caused by mutations in the BTK gene. In conclusion, our data identify Btk as a positive regulator in the ITAM-mediated TREM-1/DAP12 pathway and suggest its implication in inflammatory processes. 相似文献
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