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Öğe Dedicator of cytokinesis 8-deficient patients have a breakdown in peripheral B-cell tolerance and defective regulatory T cells(Mosby-Elsevier, 2014) Janssen, Erin; Morbach, Henner; Ullas, Sumana; Bannock, Jason M.; Massad, Christopher; Menard, Laurence; Barlan, IsilBackground: Dedicator of cytokinesis 8 (DOCK8) deficiency is typified by recurrent infections, increased serum IgE levels, eosinophilia, and a high incidence of allergic and autoimmune manifestations. Objective: We sought to determine the role of DOCK8 in the establishment and maintenance of human B-cell tolerance. Methods: Autoantibodies were measured in the plasma of DOCK8-deficient patients. The antibody-coding genes from new emigrant/transitional and mature naive B cells were cloned and assessed for their ability to bind self-antigens. RegulatoryT(Treg) cells in the blood were analyzed by means of flow cytometry, and their function was tested by examining their capacity to inhibit the proliferation of CD4(+)CD25(-) effector T cells. Results: DOCK8-deficient patients had increased levels of autoantibodies in their plasma. We determined that central B-cell tolerance did not require DOCK8, as evidenced by the normally low frequency of polyreactive new emigrant/transitional B cells in DOCK8-deficient patients. In contrast, autoreactive B cells were enriched in the mature naive B-cell compartment, revealing a defective peripheral B-cell tolerance checkpoint. In addition, we found that Treg cells were decreased and exhibited impaired suppressive activity in DOCK8-deficient patients. Conclusions: Our data support a critical role for DOCK8 in Treg cell homeostasis and function and the enforcement of peripheral B-cell tolerance.Öğe The demographic datas of chronic granulomatous disease patients and the comparation of the clinical datas before and after interferon-gamma treatment in our country(Bilimsel Tip Yayinevi, 2013) Filiz, Serkan; Uygun, Dilara Fatma Kocacik; Sanal, Ozden; Camcioglu, Yildiz; Somer, Ayper; Barlan, Isil; Kilic, SebnemObjective: Chronic granulomatous disease (CGD) is a genetically heterogeneous primary immunodeficiency that is characterised by bacteria and fungal infections with defective phagocytosis. Interferon-gamma (INF-gamma) has diverse roles in the innate and adaptive responses. Despite several decades of work on INF-gamma treatment in CGD, contraversy remains about its use. Materials and Methods: Fifty seven patients with CGD from 14 immunology centers were enrolled to our multi-center study. A questionnaire including patients demographic datas and clinical manifestations such as infectious and granulomatous complications up to enrolment was obtained before and after INF-gamma therapy. Results: Fifty seven patients 14 (25%) girls and 34 (75%) boys aged from 2-35 years (mean age: 10.9 +/- 7.4) were enrolled. The mean age of diagnosis were 4.9 +/- 4.8 (0.1-19). 56% of the patient's family had consanguineous marriage and 60% had a primary immunodeficiency (PID) history. Ninety five of the patients were treated with trimethoprim-sulfamethoxazole (TMP-SMX) and 89.5% of them with itraconazol while 60% of them were received INF-gamma treatment. The patients receiving INF-gamma therapy tend to have lower infectious complications like severe infections, pneumonia, soft tissue infections and lymphadenitis. Aspergillus infection, tissue abcesses and granulomatous complications were also lower in this group. The annual infectious complications according to CGD subtypes, were also lower in gp91(phox) with receiving INF-gamma therapy. Conclusion: The demographic and clinical data of CGD patients in our study indicate that INF-gamma prophylaxis treatment decreases the infectious and granulomatous complications in some majority of CGD patients especially in gp91(phox)Öğe Plasmacytoid dendritic cell depletion in DOCK8 deficiency: Rescue of severe herpetic infections with IFN-? 2b therapy(Mosby-Elsevier, 2014) Keles, Sevgi; Jabara, Haifa H.; Reisli, Ismail; McDonald, Douglas R.; Barlan, Isil; Hanna-Wakim, Rima; Dbaibo, Ghassan[Abstract Not Availabe]
