Person: ÇEKİÇ, ŞÜKRÜ
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ÇEKİÇ
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ŞÜKRÜ
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Publication Neurological involvement in primary immunodeficiencies(Springer/plenum Publishers, 2022-04-01) KÖSE, HÜLYA; KARALI, ZUHAL; Çekiç, Şükrü; ÇEKİÇ, ŞÜKRÜ; KILIÇ GÜLTEKİN, SARA ŞEBNEM; Tıp Fakültesi; Çocuk Sağlığı ve Hastalıkları Ana Bilim Dalı; 0000-0002-9574-1842; 0000-0001-8571-2581; L-1933-2017; AAH-1658-2021Publication 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; Tıp Fakültesi; Çocuk Sağlığı ve Hastalıkları Ana Bilim 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 T-cell/histiocyte-rich large B-cell lymphoma in a patient with a novel frameshift MSH6 mutation(Wiley, 2022-09-24) Çekiç, Şükrü; Aydın, Firdevs; Karalı, Yasin; Sevinir, Betül Berrin; Canöz, Özlem; Boztuğ, Kaan; Ünal, Ekrem; Kılıç, Sara Şebnem; ÇEKİÇ, ŞÜKRÜ; KARALI, YASİN; SEVİNİR, BETÜL BERRİN; KILIÇ GÜLTEKİN, SARA ŞEBNEM; Tıp Fakültesi; Pediatrik İmmünoloji Ana Bilim Dalı; 0000-0002-9574-1842; 0000-0002-3232-7652; 0000-0002-2691-4826; 0000-0001-8571-2581; ISC-9139-2023; L-1933-2017; AAH-1658-2021; AAH-1570-2021Publication 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; Tıp Fakültesi; Çocuk Alerji Ana Bilim 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 Omalizumab treatment in childhood chronic urticaria(Wiley, 2018-08-01) Çekiç, Şükrü; Karali, Yasin; Canıtez, Yakup; Sapan, Nihat; ÇEKİÇ, ŞÜKRÜ; KARALI, YASİN; CANITEZ, YAKUP; SAPAN, NİHAT; Tıp Fakültesi; Pediatri ve Alerji Bölümü; 0000-0002-9574-1842; L-1933-2017; FFS-1974-2022; CJQ-2060-2022; FUI-8766-2022Publication Atypical localization of eczema discriminates dock8 or stat3 deficiencies from atopic dermatitis(Springer/plenum Publishers, 2023-07-29) Kasap, Nurhan; Kara, Altan; Celik, Velat; Eltan, Sevgi Bilgic; Haci, Idil Akay; Kose, Hulya; Aygun, Ayse; Akkelle, Emre; Yakici, Nalan; Guner, Sukru Nail; Reisli, Ismail; Keles, Sevgi; Karaca, Neslihan Edeer; Gulez, Nesrin; Genel, Ferah; Ozen, Ahmet; Yucelten, Ayse Deniz; Karakoc-Aydiner, Elif; Schmitz-Abe, Klaus; Baris, Safa; KILIÇ GÜLTEKİN, SARA ŞEBNEM; ÇEKİÇ, ŞÜKRÜ; Çekiç, Şükrü; 0000-0002-8893-9420; 0000-0003-0561-3343; 0000-0003-0738-4679; 0000-0002-9574-1842; 0000-0001-8571-2581; 0000-0002-3343-6949; 0000-0003-4150-5200; 0000-0002-7242-8861; 0000-0002-4730-9422; ACY-0399-2022; AAH-1658-2021; R-6749-2017; HKN-1599-2023; LBH-2414-2024; IUN-0044-2023; ABF-5609-2020; ABF-6314-2020; L-1933-2017; JWO-2146-2024PurposeAutosomal recessive dedicator of cytokinesis 8 (DOCK8(-/-)) and autosomal dominant signal transducer and activator of transcription 3 (STAT3(-/+)) deficiencies are inborn errors of immunity (IEI) disorders present with the classic features of eczema and create a dilemma during differentiation from atopic dermatitis (AD). Therefore, an appropriate approach is required for eczema to diagnose DOCK8(-/-) and STAT3(-/+) early. Here, we described a set of clinical and immunological variables, including atypical AD localizations and lymphocyte subsets, to differentiate DOCK8(-/-) or STAT3(-/+) from AD.MethodsThis multicenter study involved 100 patients with DOCK8(-/-) and STAT3(-/+) and moderate/severe AD. We recruited disease manifestations, including detailed localizations of eczema, infections, and allergy. Principle component analysis (PCA) was used to discriminate DOCK8(-/-) or STAT3(-/+) from AD.ResultsThere were 43 patients with DOCK8(-/-), 23 with STAT3(-/+), and 34 with AD. Pneumonia, severe infections, mucocutaneous candidiasis, and skin abscesses were commonly observed in DOCK8 and STAT3 deficiencies. Atypical skin involvement with neonatal rash, retro auricular, axillary, sacral, and genital eczema discriminate DOCK8(-/-) and STAT3(-/+) from AD with high specificity ranges between 73.5 and 94.1% and positive predictive index ranges between 55 and 93.1%. Together with using absolute numbers of CD3(+), CD4(+), and CD8(+) T cells, the combined clinical and laboratory features showed perfect differentiation between DOCK8(-/-) or STAT3(-/+) and AD via PCA.ConclusionsThe described features can be easily implemented by physicians providing early diagnosis of DOCK8 and STAT3 deficiencies.Publication 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; Tıp Fakültesi; Çocuk Alerjisi Ana Bilim 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 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; Tıp Fakültesi; Pediatrik İmmünoloji Ana Bilim 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 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; Tıp Fakültesi; Pediatri İmmünoloji ve Romatoloji Bölümü; CZC-9168-2022; CIO-5200-2022; HXT-7953-2023; FEK-8430-2022Publication Immunological evaluation of the patients with CAPS(Wiley, 2020-08-01) Çekiç, Şükrü; Kılıç, Sara Şebnem; ÇEKİÇ, ŞÜKRÜ; KILIÇ GÜLTEKİN, SARA ŞEBNEM; Tıp Fakültesi; Çocuk Alerji ve Klinik İmmünoloji Ana Bilim Dalı; 0000-0002-9574-1842; 0000-0001-8571-2581; AAH-1658-2021; L-1933-2017