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 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 Evaluation of the cases with non-cystic fibrosis bronchiectasis(Galenos Yayıncılık, 2018-01-01) Korkmaz, Serpil; Canıtez, Yakup; Çekiç, Şükrü; Efe, Hülya Poyraz; Ocakoğlu, Gökhan; Sapan, Nihat; Korkmaz, Serpil; CANITEZ, YAKUP; ÇEKİÇ, ŞÜKRÜ; Efe, Hülya Poyraz; OCAKOĞLU, GÖKHAN; SAPAN, NİHAT; Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Alerji Anabilim Dalı.; Uludağ Üniversitesi/Tıp Fakültesi/Biyoistatistik Anabilim Dalı.; 0000-0002-9574-1842; 0000-0002-1114-6051; HLG-6346-2023; L-1933-2017; CJQ-2060-2022; CPN-6371-2022; FFE-8839-2022; FUI-8766-2022INTRODUCTION: Bronchiectasis is a chronic inflammatory disease characterized by a permanent dilation of the bronchial wall. We aimed to evaluate pediatric cases diagnosed with non-cystic fibrosis bronchiectasis.METHODS: A total of 98 cases who were diagnosed with non-cystic fibrosis bronchiectasis and were being followed-up between January 2010 and January 2015 at our clinic were included. Clinical, laboratory and radiological evaluations were recorded from patients electronic files.RESULTS: Female to male ratio of the cases was 1.3 (56/42). The median age of the cases was 12.2 years (1-18), the median age of diagnosis was 6 years (1-17 years), and the median duration of follow-up was 38 months (3-140 months).The most common symptom of the cases was cough (n: 91, 93%) followed by sputum expectoration (n: 56, 57%). In the pulmonary function tests performed during the initial phase of diagnosis; restrictive pattern was seen in 29.1% (n = 21) of patients and obstructive pattern was seen in 23.2% (n = 17). Furthermore, in last control visit, restrictive pattern was seen in 17.3% (n=9) of them and obstructive pattern was seen in 44.2% (n = 23). The most frequent etiological cause was infectious diseases (n: 41, 42%). Bronchiectasis was seen most commonly in the left lower lobe of the lung (n: 52, 53%), followed by the right lower lobe (n=32, 33.7%).DISCUSSION and CONCLUSION: Bronchiectasis is still a major health problem in developing countries and causes irreversible damage to the lung when untreated. Postinfectious lung diseases are one of the most important causes of bronchiectasis in children. Early diagnosis improves treatment success.Publication Evaluation of school age asthmatic cases with childhood asthma control test(Galenos Yayincilik, 2017-12-01) ÇEKİÇ, ŞÜKRÜ; SAPAN, NİHAT; CANITEZ, YAKUP; Demirbaş, Başak Ceylan; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Çocuk sağlığı ve Hastalıkları Anabilim Dalı.; 0000-0002-9574-1842; L-1933-2017Introduction: Asthma is the most common chronic disease in children. It is known that seasonal changes affect the number of asthma attacks and the condition of the disease under control as well. We evaluated children aged 6-11 years of age who had been diagnosed with asthma using the childhood asthma control test (C-ACT) to determine the factors affecting the control of asthma.Methods: One hundred and three follow-up cases diagnosed with asthma between the ages of 6-11 were included. All cases were asked to fill in the C-ACT and the asthmatic questionnaire that we had prepared to evaluate the patients.Results: It was determined that the 47.5% of the cases (n=49) were under control according to the results of the C-ACT. The number of annual attacks in the group under control (median=2/year, minimum=0/year, maximum=10/year) was significantly less than for the uncontrolled cases (median=3.5/year, minimum=0/year, maximum=10/year) (p=0.005). We determined that 14.3% (n=7) of the families of the group under control and 38.9% (n=21) of the families of the uncontrolled cases had a history of eczema (p=0.005). The hospital admission rates of the group under control were found to be 30.6% (n=15) in September, 32.7% (n=16) in October, 30.6% (n=15) in November, and 6.10% (n=3) in December, and the rates for the uncontrolled cases were 5.6% (n=3) in September, 25.9% (n=14) in October, 33.3% (n=18) in November, and 35.2% (n=19) in December. We detected that the uncontrolled cases were admitted more frequently than the ones under control in December (p=0.001).Discussion and Conclusion: C-ACT is a practical application that helps physicians with the follow-up of patients. We have determined in our study that a significant percentage of the asthmatics are uncontrolled cases. We estimated that the increase of complaints in December in patients with asthma may be related to regional climatic changes as well as environmental conditions.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 The use of cyclosporine a in the treatment of toxic epidermal necrolysis; case report and review of literature(Galenos Yayincilik, 2014-08-01) Canıtez, Yakup; CANITEZ, YAKUP; Çekiç, Şükrü; ÇEKİÇ, ŞÜKRÜ; Sapan, Nihat; SAPAN, NİHAT; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Sağlığı ve Hastalıkları Anabilim Dalı.; 0000-0002-9574-1842; L-1933-2017Toxic epidermal necrolysis (TEN) is a serious and life-threatening acute mucocutaneous disease that is often triggered by drugs, can affect skin, eyes, mucous membranes and multiple organs.While an eleven-year-old female patient with epilepsy had been using valproic acid for three years, lamotrigine was added to the treatment one month ago. The patient was admitted complaints such as fever, malaise, sores in the mouth, eye redness, watering of eyes, rashes and many bullous lesions in the skin that had started just 2 days earlier. On the physical examination, whose overall situation was bad, that were common maculopapular rash, purpuric macules on the whole body, atypical target-like skin lesions, and many bullae that affected more than 30% of the body in different stages and whose largest one was 6-10 cm in diameter were detected. There were ulcerated lesions in the oral mucosa and keratitis, blepharitis and conjunctival hyperemia in both eyes. The patient was diagnosed with TEN through the existing findings. The lamotrigine treatment was discontinued; the supportive treatment, methylprednisolone, intravenous immunoglobulin, necessary care for skin and eye lesions and the necessary topical treatment were applied. As no improvement was observed in clinical picture and skin lesions in the follow-up period, cyclosporine A was added to the treatment. Significant improvement was observed in the clinical picture and skin lesions of the patient who responded well to the treatment of cyclosporine A.The basis of the treatment of TEN is composed of discontinuation of the suspicious drug and the supportive therapy. The immunosuppressive agents such as systemic corticosteroids and IVIG therapy may be used in the treatment of TEN. Cyclosporine can positively contribute to the treatment in cases who are resistant to treatment and with a severe TEN condition. There is a need for in-depth studies to investigate the place and impact of cyclosporine in the treatment of TEN.Publication Neurological involvement in primary immunodeficiencies(Springer/plenum Publishers, 2022-04-01) KÖSE, HÜLYA; KARALI, ZUHAL; Çekiç, Şükrü; ÇEKİÇ, ŞÜKRÜ; KILIÇ GÜLTEKİN, SARA ŞEBNEM; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Sağlığı ve Hastalıkları Anabilim Dalı.; 0000-0002-9574-1842; 0000-0001-8571-2581; L-1933-2017; AAH-1658-2021Publication A comprehensive assessment of long-term complications in patients with stevens-johnson syndrome and toxic epidermal necrolysis(Karger, 2023-07-26) Çekiç, Sükrü; Canıtez, Yakup; Yüksel, Hale; Gündüz, Gamze Ucan; Karalı, Zühal; Yalçınbayır, Özgür; Vural, Pınar; Sapan, Nihat; ÇEKİÇ, ŞÜKRÜ; CANITEZ, YAKUP; Yüksel, Hale; UÇAN GÜNDÜZ, GAMZE; KARALI, ZUHAL; VURAL, AYŞE PINAR; YALÇINBAYIR, ÖZGÜR; SAPAN, NİHAT; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Alerjisi Anabilim Dalı.; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Göz Hastalıkları Anabilim Dalı.; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Çocuk ve Ergen Psikiyatrisi Anabilim Dalı.; 0000-0002-9574-1842; 0009-0002-4004-449X; 0000-0002-5458-1686; 0000-0002-7311-5277; 0000-0001-8929-679X; L-1933-2017; AAH-1789-2021; IZZ-9492-2023; AAH-6661-2021; CZC-9168-2022; IYJ-9408-2023; GIK-1690-2022; FUI-8766-2022Introduction: Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and SJS/TEN overlap syndrome are rare severe hypersensitivity reactions that lead to epithelial sloughing. Studies investigating the chronic multisystem effects of these syndromes and assessing patients in terms of quality of life (QOL), depression, and anxiety in the pediatric population are limited. In this study, we aimed to investigate the long-term effects of these diseases from a multisystem perspective. Method: Sixteen pediatric patients diagnosed with SJS, TEN, and SJS/TEN overlap syndrome were evaluated between September 2020 and March 2021. Physical and eye examinations were performed. To evaluate QOL and psychological status, Children's Dermatology Life Quality Index (CDLQI), Screen for Child Anxiety-Related Emotional Disorders (SCARED), and Children's Depression Inventory (CDI) were conducted. The patients' general characteristics, symptoms, and examination findings at their first admission were retrospectively obtained from the hospital's electronic records. Results: Nineteen percent of the patients were female (n = 3). There were 7 patients (44%) with the diagnosis of SJS, 5 patients (31%) with TEN, and 4 patients (25%) with SJS/TEN overlap. The median follow-up time of the subjects was 6.5 years. The most common sequelae in the chronic period were skin changes (n = 13, 81%). Hyperpigmentation was the most common skin change (n = 9, 56%). In the last evaluation, 9 cases had eye involvement. In two cases, eye examination was normal in the acute phase, while ocular involvement was present in the chronic period. In 4 (50%) patients, there was height and/or weight percentile loss. Three patients' SCARED scores and 2 patients' CDI scores were high. According to the CDLQI survey, SJS, TEN, or SJS/TEN overlap syndrome had a small to moderate effect on the QOL in the 43% (n = 6) of the patients. The ANA values of 3 patients (60%) were positive at the follow-up and negative at the first admission. Conclusion: SJS, TEN, and SJS/TEN overlap syndrome may cause sequelae even after a long time of the onset of the disease. Patients' QOL and psychological status can be affected negatively. Ocular symptoms may develop in the follow-up, even without involvement in the acute period. Patients with SJS, TEN, and SJS/TEN overlap syndrome should be followed up in the chronic period and approached multidisciplinary.Publication Evaluation of patients with pediatric behcet's disease: A tertiary center experience(BMJ Publishing Group, 2022-06-01) Karalı, Zuhal; Çekiç, Şükrü; Çakır, İ.; Kılıç, Sabriye Senem; KARALI, ZUHAL; ÇEKİÇ, ŞÜKRÜ; Çakır, İ.; Kılıç, Sabriye Senem; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Pediatri İmmünoloji ve Romatoloji Bölümü; CZC-9168-2022; CIO-5200-2022; HXT-7953-2023; FEK-8430-2022