Person: ÇEKİÇ, ŞÜKRÜ
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ÇEKİÇ
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ŞÜKRÜ
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Publication 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-8408-2023Publication 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.Publication 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-1599-2023Publication Immunological evaluation of the patients with CAPS(Wiley, 2020-08-01) Çekiç, Şükrü; Kılıç, Sara Şebnem; ÇEKİÇ, ŞÜKRÜ; KILIÇ GÜLTEKİN, SARA ŞEBNEM; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Alerji ve Klinik İmmünoloji Anabilim Dalı.; 0000-0002-9574-1842; 0000-0001-8571-2581; AAH-1658-2021; L-1933-2017Publication Cftr mutations unidentified in cftr2 database and their phenotypic characteristics: Data from cystic fibrosis registry of Turkey(European Respiratory Soc Journals Ltd, 2020-09-07) Cinel, Güzin; Dogru, Deniz; Çakir, Erkan; Eyüboğlu, Tuğba Şişmanlar; Çobanoğlu, Nazan; Pekcan, Sevgi; Yalçın, Ebru; Kiper, Nural; Sen, Velat; Sen, Hadice Selimoğlu; Ercan, Ömür; Keskin, Özlem; Eltan, Sevgi Bilgiç; Al Shadfan, Lina Muhammed; Yazan, Hakan; Altıntaş, Derya Ufuk; Sasihüseyinoğlu, Şenay; Cokugras, Haluk; Atabek, Ayşe Ayzıt; Gürsoy, Tuğba Ramaşlı; Aslan, Ayşe Tana; Bingol, Aysen; Başaran, Abdurrahman Erdem; Özdemir, Ali; Köse, Mehmet; Hangül, Melih; Emiralioğlu, Nagehan; Tuğcu, Gökçen; Yüksel, Hasan; Yılmaz, Özge; Orhan, Fazıl; Aydın, Zeynep Gokçe Gayretli; Topal, Erdem; Tamay, Zeynep; Can, Demet; Bal, Cem Murat; Çaltepe, Gönül; Özçelik, Uğur; Sapan, Nihat; SAPAN, NİHAT; Çekiç, Sükrü; ÇEKİÇ, ŞÜKRÜ; Bursa Uludağ Üniversitesi/Tıp Fakültesi; 0000-0002-9574-1842; L-1933-2017; FUI-8766-2022Publication Evaluation of a patient with ikaros gene deficiency(Wiley, 2020-08-01) Çekiç, Şükrü; Kılıç, Sara Şebnem; ÇEKİÇ, ŞÜKRÜ; KILIÇ GÜLTEKİN, SARA ŞEBNEM; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Alerji ve Klinik İmmünoloji Anabilim Dalı.; 0000-0002-9574-1842; 0000-0001-8571-2581; L-1933-2017; AAH-1658-2021Publication 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.Publication Clinical features of children with chronic non-bacterial osteomyelitis: A multicenter retrospective case series from Turkey(Turkish League Against Rheumatism, 2021-09-01) Acari, Ceyhun; Comak, Elif; Turkucar, Serkan; Dundar, Hatice Adiguzel; Akman, Sema; Makay, Balahan; Unsal, Sevket Erbil; Cekic, Sukru; ÇEKİÇ, ŞÜKRÜ; Kilic, Sara Sebnem; KILIÇ GÜLTEKİN, SARA ŞEBNEM; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Pediatri Anabilim Dalı.; 0000-0002-9574-1842; 0000-0001-8571-2581; 0000-0001-7131-8911; ACH-9790-2022; L-1933-2017; C-1860-2016; JZU-0721-2024; AAH-1658-2021; HQZ-1577-2023; C-1642-2016Objectives: This study aims to evaluate demographic, clinical, and radiological characteristics of Turkish children with chronic non-bacterial osteomyelitis.Patients and methods: Between January 2008 and December 2018, a total of 28 patients (10 males, 18 females; median age: 12.5 years; range, 4.5 to 21 years) who were diagnosed with chronic non-bacterial osteomyelitis in three pediatric rheumatology centers were retrospectively analyzed. The demographic, clinical, and laboratory findings of the patients were recorded.Results: The median age at the time of diagnosis was 10.2 years. The median time from symptom onset to diagnosis was 6.5 months. The median follow-up was 18.5 months. The most frequent symptom at onset was arthralgia (75.0%). The most frequently involved bone was the femur (67.9%). Eight (63.6%) of 12 patients had at least one Mediterranean fever gene (MEFV) mutation, indicating a significantly higher prevalence than the Turkish population (14.8%). Five of these patients fulfilled the diagnostic criteria for familial Mediterranean fever (FMF). All patients received non-steroidal anti-inflammatory drugs. Other drugs were methotrexate (46.4%), sulfasalazine (39%), corticosteroids (25%), anti-tumor necrosis factor (anti-TNF) agents (32%), pamidronate (25%), and colchicine (21.4%). Six of eight patients with MEFV mutations were administered with colchicine, and all of them responded to treatment.Conclusion: Clinical evolution and imaging investigations should be carefully performed to prevent any delay in the diagnosis of patients with chronic non-bacterial osteomyelitis. Based on our study results, FMF coexistence is worth investigating in patients with chronic non-bacterial osteomyelitis, particularly in the Turkish population.Publication 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.Publication 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-5744-2023