Kişi: KILIÇ GÜLTEKİN, SARA ŞEBNEM
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KILIÇ GÜLTEKİN
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Yayın Açık Erişim Oxidative stress in common variable immunodeficiency(Sage Publications, 2021-03-01) Başaranoğlu, Sevgen Tanır; Çekiç, Şükrü; Kirhan, Emine; Dirican, Melahat; Kılıç, Sara Şebnem; ÇEKİÇ, ŞÜKRÜ; DİRİCAN, MELEHAT; KILIÇ GÜLTEKİN, SARA ŞEBNEM; Kirhan, Emine; Başaranoğlu, Sevgen Tanır; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Sağlığı ve Hastalıkları Anabilim Dalı.; Bursa Uludağ Üniversitesi/Tıp Fakültesi.; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Tıbbi Biyokimya Anabilim Dalı.; 0000-0002-9574-1842; 0000-0001-8571-2581; 0000-0002-9416-1512; L-1933-2017; AAC-8665-2020; AAH-1658-2021; DBC-8927-2022; ESK-6562-2022Common variable immunodeficiency (CVID) is a heterogenous group of immunologic disorders of unknown etiology. Alterations of the normal cellular balance due to an increase in reactive oxygen species and/or decrease in antioxidant defense may lead to increased oxidative stress. We aimed to evaluate the levels of oxidative stress biomarkers in patients with CVID who had different presentations. We investigated the serum catalase (CAT), erythrocyte superoxide dismutase (SOD), erythrocyte reduced glutathione as antioxidants and serum malondialdehyde levels as lipid peroxidation marker in patients with CVID in Uludag University Hospital Department of Pediatric Allergy and Immunology's outpatient clinics. In the analysis, there were 21 patients and 27 matched healthy controls. The median levels of CAT in patients with CVID was significantly lower than in healthy controls (p = 0.04). Among the patients with CVID, 19% had autoimmune disease, one had Sjogren's syndrome, one had autoimmune alopecia, one had juvenile rheumatoid arthritis, and one had chronic inflammatory demyelinating polyneuropathy. Patients with autoimmune complications had significantly lower CAT levels compared to the ones without autoimmune diseases (p = 0.03). The patients without non-infectious complications (NICs) had lower SOD levels than the patients with NICs (p = 0.05). The analysis of oxidative stress markers in the patients with CVID suggested a series of abnormalities in the anti-oxidant system. The clinical syndrome associations may be a useful tool for future studies to set prediction markers for the prognosis of patients with CVID.Yayın Açık Erişim Clinical and laboratory findings in patients with leukocyte adhesion deficiency type i: A multicenter study in Turkey(Oxford Universitesi, 2021-08-05) Yaz, İsmail; Özbek, Begüm; Bildik, Hacer Neslihan; Tan, Cağman; Halacli, Sevil Oskay; Aytekin, Elif Soyak; Esenboga, Saliha; Keskin, Ozlem; Leeuwen, Karin; Roos, Dirk; Cagdas, Deniz; Tezcan, Ilhan; Çekiç, Şükrü; ÇEKİÇ, ŞÜKRÜ; Kılıç, Sara Sebnem; KILIÇ GÜLTEKİN, SARA ŞEBNEM; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Pediatri Anabilim Dalı.; 0000-0002-9574-1842; 0000-0001-8571-2581; L-1933-2017; AAH-1658-2021Leukocyte adhesion deficiency type I is a rare primary immunodeficiency disorder characterized by mutations in the ITGB2 gene encoding CD18. We present clinical and immunological features of 15 patients with leukocyte adhesion deficiency type 1 (LAD-1). Targeted next-generation sequencing was performed with either a primary immunodeficiency gene panel comprising 266 genes or a small LAD-panel consisting of five genes for genetic analysis. To measure the expression level of integrins on the leukocyte surface, flow cytometry analysis was performed. The median age of the patients at diagnosis was 3 (1-48) months. Eleven (73%) of the 15 patients had a LAD-1 diagnosis in their first 6 months and 14 (93%) patients had consanguineous parents. Delayed separation of the umbilical cord was present in 80% (n = 12) of the patients in our cohort, whereas omphalitis was observed in 53% (n = 8) of the patients. Leukocytosis with neutrophil predominance was observed in 73% (n = 11) patients. Nine distinct variants in the ITGB2 gene in 13 of the 15 patients with LAD-1 were characterized, two of which (c.305_306delAA and c.779_786dup) are novel homozygous mutations of ITGB2. Four unrelated patients from Syria had a novel c.305_306delAA mutation that might be a founder effect for patients of Syrian origin. Four (27%) patients underwent hematopoietic stem cell transplantation. Two patients died because of HSCT complications and the other two are alive and well. Early differential diagnosis of the patients is critical in the management of the disease and genetic evaluation provides a basis for family studies and genetic counseling.Yayın Açık Erişim Genetic analysis of a cohort of 275 patients with hyper-ige syndromes and/or chronic mucocutaneous candidiasis(Springer, 2021-08-14) Frede, Natalie; Rojas-Restrepo, Jessica; Caballero Garcia de Oteyza, Andres; Buchta, Mary; Huebscher, Katrin; Gamez-Diaz, Laura; Proietti, Michele; Saghafi, Shiva; Chavoshzadeh, Zahra; Soler-Palacin, Pere; Galal, Nermeen; Adeli, Mehdi; Aldave-Becerra, Juan Carlos; Al-Ddafari, Moudjahed Saleh; Ardenyz, Omur; Atkinson, T. Prescott; Kut, Fulya Bektas; Celmeli, Fatih; Rees, Helen; Kirovski, Ilija; Klein, Christoph; Kobbe, Robin; Korganow, Anne-Sophie; Lilic, Desa; Lunt, Peter; Makwana, Niten; Metin, Ayse; Ozgur, Tuba Turul; Karakaş, Ayse Akman; Seneviratne, Suranjith; Sherkat, Roya; Sousa, Ana Berta; Unal, Ekrem; Patiroglu, Turkan; Wahn, Volker; von Bernuth, Horst; Whiteford, Margo; Doffinger, Rainer; Jouhadi, Zineb; Grimbacher, Bodo; Kılıç, Sara S.; KILIÇ GÜLTEKİN, SARA ŞEBNEM; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Sağlığı ve Hastalıkları Anabilim Dalı.; 0000-0001-8571-2581.; AAH-1658-2021.Hyper-IgE syndromes and chronic mucocutaneous candidiasis constitute rare primary immunodeficiency syndromes with an overlapping clinical phenotype. In recent years, a growing number of underlying genetic defects have been identified. To characterize the underlying genetic defects in a large international cohort of 275 patients, of whom 211 had been clinically diagnosed with hyper-IgE syndrome and 64 with chronic mucocutaneous candidiasis, targeted panel sequencing was performed, relying on Agilent HaloPlex and Illumina MiSeq technologies. The targeted panel sequencing approach allowed us to identify 87 (32 novel and 55 previously described) mutations in 78 patients, which generated a diagnostic success rate of 28.4%. Specifically, mutations in DOCK8 (26 patients), STAT3 (21), STAT1 (15), CARD9 (6), AIRE (3), IL17RA (2), SPINK5 (3), ZNF341 (2), CARMIL2/RLTPR (1), IL12RB1 (1), and WAS (1) have been detected. The most common clinical findings in this cohort were elevated IgE (81.5%), eczema (71.7%), and eosinophilia (62.9%). Regarding infections, 54.7% of patients had a history of radiologically proven pneumonia, and 28.3% have had other serious infections. History of fungal infection was noted in 53% of cases and skin abscesses in 52.9%. Skeletal or dental abnormalities were observed in 46.2% of patients with a characteristic face being the most commonly reported feature (23.1%), followed by retained primary teeth in 18.9% of patients. Targeted panel sequencing provides a cost-effective first-line genetic screening method which allows for the identification of mutations also in patients with atypical clinical presentations and should be routinely implemented in referral centers.Yayın Sadece Metadata Anti-tumor necrosis factor treatment in the management of pediatric noninfectious uveitis: Infliximab versus adalimumab(Mary Ann Liebert, Inc, 2021-01-28) Uçan Gündüz, Gamze; Yalçınbayır, Özgür; Çekiç, Şükrü; Yiıdız, Meral; Kılıç, Sara Şebnem; UÇAN GÜNDÜZ, GAMZE; YALÇINBAYIR, ÖZGÜR; ÇEKİÇ, ŞÜKRÜ; YILDIZ, MERAL; KILIÇ GÜLTEKİN, SARA ŞEBNEM; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Göz Hastalıkları Anabilim Dalı; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Pediatrik Alerji ve Klinik İmmünoloji; 0000-0002-5458-1686; 0000-0002-7311-5277; 0000-0002-9574-1842; 0000-0001-8571-2581; AAH-1885-2021; AAH-6625-2021; AAH-6661-2021; IYJ-9408-2023; AAH-1658-2021; JAC-9459-2023; L-1933-2017Purpose: Management of uveitis displays a particular challenge in childhood. This study aims to compare the efficacy and safety of infliximab (IFX) and adalimumab (ADA) in pediatric noninfectious uveitis that were refractory to conventional immunosuppresives.Methods: This retrospective single-center study included 33 patients who were treated with anti-tumor necrosis factor (TNF) agents (16 with IFX and 17 with ADA). Patients had diverse etiologies, including juvenile idiopathic arthritis, idiopathic uveitis, and Behcet's disease. Demographic characteristics, systemic diagnosis, findings of the ophthalmological examination, control of ocular inflammation, response to treatment, and the rate of clinical remission were studied.Results: Fourteen (87.5%) patients receiving IFX and 10 (58.8%) patients receiving ADA achieved response to treatment during the follow-up (P = 0.118). The agents were discontinued with complete clinical remission in 6 (37.5%) patients receiving IFX and in 2 (11.8%) patients receiving ADA (P = 0.118). Baseline visual acuities and parameters of inflammation improved significantly in both groups after anti-TNF therapy.Conclusion: Both IFX and ADA are safe and effective for pediatric noninfectious uveitis.Yayın Sadece Metadata Infliximab therapy in an infant with Netherton Syndrome(Wiley, 2021-04-22) Çiçek, Fatih; Çekiç, Şükrü; Kılıç, Sara Şebnem; ÇİÇEK, FATİH; ÇEKİÇ, ŞÜKRÜ; KILIÇ GÜLTEKİN, SARA ŞEBNEM; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Pediatrik İmmünoloji Anabilim Dalı.; 0000-0002-9574-1842; 0000-0001-8571-2581; 0000-0001-7348-7081; AAH-1658-2021; JKI-5906-2023; L-1933-2017We present a patient with Netherton syndrome and severe skin manifestations treated with infliximab. By 6 months of age, the child had intractable pruritus, scaling, dry skin, and generalized eczematous lesions resistant to conventional therapies for atopic dermatitis. Clinical improvement was observed following the third infusion of infliximab, and by 12 months of age, the skin lesions completely resolved. Infliximab is a promising option for the management of skin inflammation in Netherton syndrome, even in infants.Yayın Sadece Metadata Rituximab therapy for recalcitrant idiopathic sclerosing orbital inflammation(Lippincott Williams & Wilkins, 2021-05-01) Yazıcı, Bülent; Çekiç, Şükrü; Yalçınkaya, Ulviye; Kılıç, S. Şebnem; ÇEKİÇ, ŞÜKRÜ; YALÇINKAYA, ÜLVİYE; KILIÇ GÜLTEKİN, SARA ŞEBNEM; Yazıcı, Bülent; Uludağ Üniversitesi/Tıp Fakültesi; 0000-0001-8889-1933; 0000-0002-9574-1842; 0000-0001-8571-2581; AAA-5384-2020; L-1933-2017; AAH-1658-2021; AAH-8924-2021Three patients (3 female patients; aged 7, 35, and 61 years) who had recalcitrant idiopathic sclerosing orbital inflammation were treated with rituximab. The disease was bilateral in 1 patient (4 orbits in total): diffuse in 2 and localized in 2 orbits. It caused optic neuropathy in 1 orbit of each patient. Conventional immunotherapy and tumor debulking surgery were unsuccessful in controlling the disease. After rituximab infusions (375 mg/m(2)/week for 4 weeks), all patients improved symptomatically. Radiologically, the local lesions resolved completely and diffuse lesions partially. Two patients with recurrent inflammation during follow up (78, 58, and 51 months) responded well to immediate, short-term steroid treatments. Short-term rituximab therapy can induce effective remissions in patients with refractory idiopathic sclerosing orbital inflammation. Early and local lesions may respond better to treatment than diffuse lesions. Nevertheless, inflammatory exacerbations can occur during late follow up.Yayın Sadece Metadata Superb microvascular imaging in assessment of synovitis and tenosynovitis in juvenile idiopathic arthritis(Lippincott Williams & Wilkins, 2021-03-01) Kandemirli, Sedat Giray; Çicek, Fatih; Erdemli Gürsel, Başak; Bilgin, Cem; Kiliç, Sara Sebnem; Yazıcı, Zeynep; Çicek, Fatih; ÇİÇEK, FATİH; Erdemli Gürsel, Başak; ERDEMLİ GÜRSEL, BAŞAK; Bilgin, Cem; BİLGİN, CEM; Kiliç, Sara Sebnem; KILIÇ GÜLTEKİN, SARA ŞEBNEM; Yazıcı, Zeynep; YAZICI, ZEYNEP; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Radyoloji Anabilim Dalı.; 0000-0001-7348-7081; 0000-0001-8571-2581; AAH-1658-2021; HHS-7433-2022; JKI-5906-2023; AAH-6568-2021The aim of this study is to evaluate the diagnostic utility of superb microvascular imaging (SMI) in assessment of synovitis/tenosynovitis in juvenile idiopathic arthritis in comparison to power Doppler ultrasound. Thirty juvenile idiopathic arthritis cases with active clinical findings and ultrasound features of effusion and/or tenosynovitis were further imaged with power Doppler and SMI. For classification of synovial inflammation, a semiquantitative scale (4 points) adopted by Outcome Measures in Rheumatology was used.A total of 35 knee, 2 hip, 2 ankle, 2 wrist, 2 elbow joints, and 6 flexor hallucis longus/tibialis posterior tenosynovitis were assessed. In knee joint, power Doppler and SMI scales were the same for 23 (65.7%) joints, SMI upgraded scale from 0 to 2 in single joint (2.9%); 1 to 2 (14.3%) in 5 joints; and 2 to 3 (17.1%) in 6 joints. For other joints, power Doppler and SMI scales were the same for 5 (62.5%) joints. Superb microvascular imaging upgraded scale from 1 to 2 (25%) in 2 joints and 1 to 3 (12.5%) in a single joint. For flexor hallucis longus/tibialis posterior tenosynovitis, power Doppler and SMI scales were the same for two cases (33.3%). Superb microvascular imaging upgraded scale from 0 to 2 in two cases (33.3%); and 2 to 3 (33.3%) in 2 cases. There was no case of SMI scale downgraded compared with power Doppler scale.Superb microvascular imaging is a feasible technique in the assessment of synovial inflammation and tenosynovitis in juvenile idiopathic arthritis. Superb microvascular imaging has higher sensitivity compared with power Doppler ultrasound in depiction of increased vascularity.Yayın Sadece Metadata The evaluation of radiosensitivity in patients with STAT3 deficiency(Springer, 2021-04-01) Çekiç, Şükrü; Hüriyet, Hüzeyfe; Hortoğlu, Melika; Barış, Safa; Metin, Ayşe; Özen, Ahmet; Aydıner, Elif Karakoç; Abakay, Candan; Çavaş, Tolga; Kılıç, Sara; ÇEKİÇ, ŞÜKRÜ; Hüriyet, Hüzeyfe; BEKTAŞ HORTOĞLU, MELİKA; DEMİRÖZ ABAKAY, CANDAN; ÇAVAŞ, TOLGA; KILIÇ GÜLTEKİN, SARA ŞEBNEM; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Sağlığı ve Hastalıkları Anabilim Dalı/Çocuk İmmunoloji Bilim Dalı.; 0000-0002-9574-1842; 0000-0001-8494-601X; 0000-0003-4150-5200; AAA-4154-2022; JBJ-7521-2023; R-6749-2017; L-1933-2017; HKN-1599-2023Yayın Sadece Metadata The assessment of brain evoked potentials in patients with caps(Springer, 2021-04-01) Çekiç, Şükrü; Demir, Aylin Bican; Kılıç, Sara; ÇEKİÇ, ŞÜKRÜ; BİCAN DEMİR, AYLİN; KILIÇ GÜLTEKİN, SARA ŞEBNEM; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Uludağ Üniversitesi Çocuk Alerji ve İmmünoloji bilim Dalı; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Nöroloji Anabilim Dalı; 0000-0002-9574-1842; 0000-0001-6739-8605; L-1933-2017; KHB-9765-2024; IDK-5744-2023Yayın Açık Erişim The impact of the SARS-CoV-2 pandemic in PID patients receiving ig replacement therapy(Springer, 2021-01-15) Çekiç, Şükrü; Çiçek, Fatih; Kılıç, Sara Şebnem; ÇEKİÇ, ŞÜKRÜ; ÇİÇEK, FATİH; KILIÇ GÜLTEKİN, SARA ŞEBNEM; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Sağlığı ve Hastalıkları Anabilim Dalı/İmmünoloji ve Romatoloji Anabilim Dalı; 0000-0002-9574-1842; 0000-0001-7348-7081; 0000-0001-8571-2581; L-1933-2017; AAH-1658-2021; JMD-8408-2023