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Öğe Abatacept as a Long-Term Targeted Therapy for LRBA Deficiency(Elsevier, 2019) Kiykim, Ayca; Ogulur, Ismail; Dursun, Esra; Charbonnier, Louis Marie; Nain, Ercan; Cekic, Sukru; Dogruel, DilekBACKGROUND: LPS-responsive beige-like anchor (LRBA) deficiency presents with susceptibility to infections, autoimmunity, and lymphoproliferation. The long-term efficacy of cytotoxic T-lymphocyte-associated antigen 4-immunoglobulin (abatacept) as targeted therapy for its immune dysregulatory features remains to be established. OBJECTIVE: To determine the clinical and immunologic features of LRBA deficiency and long-term efficacy of abatacept treatment in controlling the different disease manifestations. METHODS: Twenty-two LRBA-deficient patients were recruited from different immunology centers and followed prospectively. Eighteen patients on abatacept were evaluated every 3 months for long-term clinical and immunologic responses. LRBA expression, lymphocyte subpopulations, and circulating T follicular helper cells were determined by flow cytometry. RESULTS: The mean age of the patients was 13.4 +/- 7.9 years, and the follow-up period was 3.4 +/- 2.3 years. Recurrent infections (n = 19 [86.4%]), immune dysregulation (n = 18 [81.8%]), and lymphoproliferation (n = 16 [72.7%]) were common clinical features. The long-term benefits of abatacept in 16 patients were demonstrated by complete control of lymphoproliferation and chronic diarrhea followed by immune dysregulation, most notably autoimmune cytopenias. Weekly or every other week administration of abatacept gave better disease control compared with every 4 weeks. There were no serious side effects related to the abatacept therapy. Circulating T follicular helper cell frequencies were found to be a reliable biomarker of disease activity, which decreased on abatacept therapy in most subjects. However, high circulating T follicular helper cell frequencies persisted in 2 patients who had a more severe disease phenotype that was relatively resistant to abatacept therapy. CONCLUSIONS: Long-term abatacept therapy is effective in most patients with LRBA deficiency. (C) 2019 American Academy of Allergy, Asthma & ImmunologyÖğe DO CUT-OFF VALUES OF IgE AND EOSINOPHIL LEVELS HELP DISCRIMINATE HYPERIGE SYNDROME FROM ATOPIC DISEASES?(Springer/Plenum Publishers, 2016) Karakoc-Aydiner, Elif; Kiykim, Ayca; Yuce, Ezgi G.; Baris, Ezgi; Nain, Ercan; Keles, Sevgi; Akturk, Hacer[Abstract Not Availabe]Öğe ILC3 deficiency and generalized ILC abnormalities in DOCK8-deficient patients(Wiley, 2020) Eken, Ahmet; Cansever, Murat; Okus, Fatma Zehra; Erdem, Serife; Nain, Ercan; Azizoglu, Zehra Busra; Haliloglu, YesimBackgroundDedicator of cytokinesis 8 (DOCK8) deficiency is the main cause of the autosomal recessive hyper-IgE syndrome (HIES). We previously reported the selective loss of group 3 innate lymphoid cell (ILC) number and function in a Dock8-deficient mouse model. In this study, we sought to test whether DOCK8 is required for the function and maintenance of ILC subsets in humans. MethodsPeripheral blood ILC1-3 subsets of 16 DOCK8-deficient patients recruited at the pretransplant stage, and seven patients with autosomal dominant (AD) HIES due to STAT3 mutations, were compared with those of healthy controls or post-transplant DOCK8-deficient patients (n = 12) by flow cytometry and real-time qPCR. Sorted total ILCs from DOCK8- or STAT3-mutant patients and healthy controls were assayed for survival, apoptosis, proliferation, and activation by IL-7, IL-23, and IL-12 by cell culture, flow cytometry, and phospho-flow assays. ResultsDOCK8-deficient but not STAT3-mutant patients exhibited a profound depletion of ILC3s, and to a lesser extent ILC2s, in their peripheral blood. DOCK8-deficient ILC1-3 subsets had defective proliferation, expressed lower levels of IL-7R, responded less to IL-7, IL-12, or IL-23 cytokines, and were more prone to apoptosis compared with those of healthy controls. ConclusionDOCK8 regulates human ILC3 expansion and survival, and more globally ILC cytokine signaling and proliferation. DOCK8 deficiency leads to loss of ILC3 from peripheral blood. ILC3 deficiency may contribute to the susceptibility of DOCK8-deficient patients to infections.Öğe Serum IgE Düzeyi ve Eozinofil Sayısı ile Atopik Hastalıklar Hiper IgE Sendromu'ndan Ayırt Edilebilir mi?(2017) Kıykım, Ayça; Yüce, Ezgi Gizem; Barış, Ezgi; Nain, Ercan; Keleş, Sevgi; Aktürk, HacerGiriş: Yüksek serum IgE ve eozinofil değerleri ağır atopik dermatit, persistan gıda allerjileri ve tedavisi zor allerjik astımın olduğu kadar Hiper IgE sendromu başta olmak üzere bazı primer immün yetmezliklerin de laboratuvar bulgularıdır. Çalışmamızda, IgE yüksekliği ile seyreden kronik granülomatöz hastalık ve HİES tanılı hastalar ile atopik dermatit, IgE aracılı gıda allerjisi, allerjik astım ve/ veya rinitli çocukların IgE ve eozinofil değerlerini, duyarlanmalarını karşılaştırarak atopik hastalıklar ile immün yetmezlik hastalıklarını ayırt etmede bir kesim değeri bulmayı hedefledik. Gereç ve Yöntem: Toplam 315 hasta çalışmaya alındı. Hastaların serum IgE ve eozinofil değerleri, duyarlanmaları ve yaşları değerlendirildi. ROC analizi ile IgE ve eozinofil seviyeleri için ideal kesim değeri hesaplandı. Bulgular: Primer immün yetmezlik grubunda IgE ortanca değeri 2542, eozinofil ortanca değeri 1000, atopi grubunda ise IgE ortanca değeri 265, eozinofil ortanca değeri ise 400 hesaplandı. Gruplar karşılaştırıldığında IgE ve eozinofil değerleri primer immün yetmezlik grubunda atopiklere göre anlamlı derecede yüksekti. Allerjenlerin etkisi karşılaştırıldığında gıda allerjenlerine duyarlanmış hastalarda sadece ev tozu akarı ve/veya ot poleni duyarlılara göre IgE anlamlı yüksekti. HİES tanısı için kesim değeri IgE2000 IU/l alındığında duyarlılık 74, özgünlük 96, IgE5000 IU/l için duyarlılık 56, özgünlük 99, eozinofil1500/mm3 alındığında duyarlılık 51, özgünlük 96, eozinofil2500/mm3 için duyarlılık 41, özgünlük 98 olarak belirlendi. Sonuç: ROC analizinde ideal kesim değeri IgE için 2500 IU/l veya eozinofil için 1500/mm3 belirlendiğinde atopi ile HİES'i ayırmak mümkündür. Bu değerlerin altında atopik hastalıklar ön planda düşünülebilir. Ancak IgE 5000IU/l veya eozinofil 2500/mm3 olan hastalar aksi ispat edilene kadar HİES kabul edilmelidir.
