Person: KARALI, ZUHAL
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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 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 Risk factors and clinical outcomes of infections caused by acinetobacter spp. in children: Results of a 5 year study(Aves Yayincilik, Ibrahim Kara, 2010-03-01) Çelebi, Solmaz; ÇELEBİ, SOLMAZ; Hacımustafaoğlu, Mustafa; HACIMUSTAFAOĞLU, MUSTAFA KEMAL; Yüce, Necla; Karalı, Zuhal; KARALI, ZUHAL; Gül, Yahya; Çakır, Deniz; Gedikoğlu, Suna; Tıp Fakültesi; Çocuk Sağlığı ve Hastalıkları Ana Bilim Dalı; 0000-0003-4646-660X; 0000-0002-7056-0615; GQP-2135-2022Objective: The number of infections caused by microorganisms of the genus Acinetobacter has increased in recent years. The aim of this study was to evaluate the risk factors and clinical outcomes associated with multidrug resistant (MDR) Acinetobacter infections in children and to define the predisposing factors associated with Acinetobacter spp. infection related mortality.Material and Method: We conducted a case-control study between January 1, 2004 and December 31, 2008 at the Uludag University Pediatric Clinic. Multidrug resistance was defined as resistance to all antibiotics apart from colistin. All patients with MDR Acinetobacter spp. infections were compared to patients with non-MDR Acinetobacter spp. infections. Risk factors analyzed included prior antibiotic use, underlying diseases, invasive medical devices, and other demographic characteristics.Results: Acinetobacter spp. infections were diagnosed in 95 of the 8879 patients hospitalized in our center between January 1, 2004 and December 31, 2008 (overall incidence, 10.6 per 1,000 admissions). The mean age of patients was 62.1+ 61.2 months (15 days-18 years) and 56% were male. The prevalence of MDR isolates among Acinetobacter spp. was found to be 33.6%. In this study, risk factors for MDR Acinetobacter spp. infections included prolonged hospitalization and prolonged exposure to broad-spectrum antibiotics (p< 0.05). The mortality rate of Acinetobacter spp. infection was found to be 26.3%. Predisposing factors associated with mortality were pediatric intensive care unit stay, male gender, ventilator associated pneumonia, presence of immunodeficiency or renal disease, presence of mechanical ventilation and MDR Acinetobacter spp. infections (p< 0.05).Conclusion: In this study, the prevalence of multidrugresistant isolates among Acinetobacter spp. was 33.6 %. The mortality (50%) for patients in the MDR group was significantly higher than the mortality for patients in the non-MDR group (11%) (p< 0.05).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 Neurocognitive functions and immunological findings in digeorge syndrome(Elsevier, 2023-05-23) Karali, Zuhal; Karali, Yasin; Kılıç, Sara; KARALI, ZUHAL; KARALI, YASİN; KILIÇ GÜLTEKİN, SARA ŞEBNEM; Tıp Fakültesi; Pediatrik İmmünoloji; 0000-0002-6132-2236; CZC-9168-2022; ISC-9139-2023; IDK-5744-2023Publication Immunological and neurocognitive functions in digeorge syndrome(Wiley, 2023-12-01) Karalı, Z.; Çekiç, S.; Karalı, Y.; Kılıç, S. S.; KARALI, ZUHAL; ÇEKİÇ, ŞÜKRÜ; KARALI, YASİN; KILIÇ GÜLTEKİN, SARA ŞEBNEM; Tıp Fakültesi; 0000-0002-9574-1842; CZC-9168-2022; GBO-8694-2022; IMT-6140-2023; ISC-9139-2023Publication Monitoring of immunoglobulin treatment compliance of patients with an inborn error of immunity during the pandemic(Wiley, 2023-10-01) KARALI, YASİN; KARALI, ZUHAL; Karalı, Zuhal; Çekiç, Şükrü; ÇEKİÇ, ŞÜKRÜ; Kılıç, Sara Şebnem; KILIÇ GÜLTEKİN, SARA ŞEBNEM; Tıp Fakültesi; Pediatri Ana Bilim Dalı; 0000-0002-9574-1842; 0000-0001-8571-2581; AAH-1658-2021Publication Recombinase activating gene defects, phenotypic diversity(Wiley, 2023-12-01) Yorgun, M.; Aydıner, E. Karakoç; Özen, A.; Barıs, S.; Oğuzkaya, Y. Şadırvan; ŞADIRVAN OĞUZKAYA, YAĞMUR HAZAL; Çekiç, Şükrü; ÇEKİÇ, ŞÜKRÜ; Karalı, Zuhal; KARALI, ZUHAL; Kılıç, Sara Şebnem; KILIÇ GÜLTEKİN, SARA ŞEBNEM; Tıp Fakültesi; 0000-0002-9574-1842; 0000-0002-4730-9422Publication Therapeutic modalities and clinical outcomes in a large cohort with lrba deficiency and ctla4 insufficiency(Mosby-elsevier, 2023-12-05) Taghizade, Nigar; Babayeva, Royala; Kara, Altan; Karakuş, Ibrahim Serhat; Çatak, Mehmet Cihangir; Bulutoğlu, Alper; Haskoloğlu, Zehra Şule; Hacı, Idil Akay; Dalgıç, Ceyda Tunakan; Karabiber, Esra; Eltan, Sevgi Bilgiç; Altunbaş, Melek Yorgun; Sefer, Asena Pınar; Sezer, Ahmet; Karadağ, Sefika Ilknur Kökcü; Arık, Elif; Kont, Aylin Özhan; Tuzer, Can; Karaman, Sait; Mersin, Selver Seda; Kasap, Nurhan; Çelik, Enes; Uygun, Dilara Fatma Kocacık; Aydemir, Sezin; Kıykım, Ayca; Aydoğmus, Ciğdem; Yücel, Esra Özek; Çelmeli, Fatih; Karatay, Emrah; Bozkurtlar, Emine; Demir, Semra; Metin, Ayse; Karaca, Neslihan Edeer; Kütükçüler, Necil; Aksu, Güzide; Güner, Şükrü Nail; Keleş, Sevgi; Reisli, Ismail; Demirkol, Yasemin Kendir; Arikoğlu, Tuğba; Gulez, Nesrin; Genel, Ferah; Aytekin, Caner; Keskin, Özlem; Yıldıran, Alişan; Özcan, Dilek; Altıntaş, Derya Ufuk; Ardeniz, Fatma Ömür; Dogu, Esin Figen; Ikincioğulları, Kamile Aydan; Karakoç-Aydıner, Elif; Özen, Ahmet; Barış, Safa; Karali, Zuhal; KARALI, ZUHAL; Kilic, Sara Sebnem; KILIÇ GÜLTEKİN, SARA ŞEBNEM; Tıp Fakültesi; İmmunoloji ve Romatoloji Ana Bilim Dalı; CZC-9168-2022; AAH-1658-2021Background: LPS-responsive beige-like anchor (LRBA) deficiency (LRBA-/-) and cytotoxic T-lymphocyte-associated antigen-4 (CTLA4) insufficiency (CTLA41/-) are mechanistically overlapped diseases presenting with recurrent infections and autoimmunity. The effectiveness of different treatment regimens remains unknown. Objective: Our aim was to determine the comparative efficacy and long-term outcome of therapy with immunosuppressants, CTLA4-immunoglobulin (abatacept), and hematopoietic stem cell transplantation (HSCT) in a single-country multicenter cohort of 98 patients with a 5-year median follow-up.Methods: The 98 patients (63 LRBA-/- and 35 CTLA41/-) were followed and evaluated at baseline and every 6 months for clinical manifestations and response to the respective therapies.Results: The LRBA-/- patients exhibited a more severe disease course than did the CTLA41/- patients, requiring more immunosuppressants, abatacept, and HSCT to control their symptoms. Among the 58 patients who received abatacept as either a primary or rescue therapy, sustained complete control was achieved in 46 (79.3%) without severe side effects. In contrast, most patients who received immunosuppressants as primary therapy (n = 61) showed either partial or no disease control (72.1%), necessitating additional immunosuppressants, abatacept, or transplantation. Patients with partial or no response to abatacept (n = 12) had longer disease activity before abatacept therapy, with higher organ involvement and poorer disease outcomes than those with a complete response. HSCT was performed in 14 LRBA-/- patients; 9 patients (64.2%)showed complete remission , 3 (21.3%) continued to receive immunosuppressants after transplantation. HSCT , abatacept therapy gave rise to similar probabilities of survival. Conclusions: Abatacept is superior to immunosuppressants in controlling disease manifestations over the long term, especially when started early, and it may provide a safe and effective therapeutic alternative to transplantation.Publication The evaluation of local and systemic reactions to subcutaneous house dust mite allergen immunotherapy(Galenos Publ House, 2022-06-15) Canitez, Yakup; CANITEZ, YAKUP; Karali, Zuhal; KARALI, ZUHAL; ÇEKİÇ, ŞÜKRÜ; SAPAN, NİHAT; Şadırvan, Yağmur Hazal; ŞADIRVAN OĞUZKAYA, YAĞMUR HAZAL; Tıp Fakültesi; Pediatri Ana Bilim Dalı; L-1933-2017Objective: Allergen -specific immunotherapy is an effective treatment method that enables the development of immunotolerance against allergens in allergic rhinitis, asthma, and venom allergy. This study investigated the local and systemic reactions during subcutaneous house dust mite allergen immunotherapy. Methods: Injection -related local and systemic reactions of 45 patients who received subcutaneous mite immunotherapy were evaluated retrospectively. Results: Forty-five children, 15 (33.3%) females and 30 (66.4%) male were included in the study. A total of 582 injections were administered. A local reaction was observed in 23 (3.94%) of all injections and the systemic reaction was observed in only 1 (0.17%) injection. Sixteen (37.7%) of the children had local reactions during the immunotherapy process and 1 (2.2%) had a systemic reaction. Conclusion: Although subcutaneous mite immunotherapy is a safe treatment, it should only be applied in centers with appropriate emergency equipment and trained healthcare professionals due to possible systemic reactions.