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Publication Index: ScopusSubject: Klinik Nöroloji

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    Citation - WoS: 2
    Citation - Scopus: 2
    The Relationship Between Lung Function, Exercise Capacity, Oxidant and Antioxidant Response in Primary Ciliary Dyskinesia and Cystic Fibrosis
    (Turkish J Pediatrics, 2024) Kartal, Yasemin; Ozel, Cemile Bozdemir; Cakmak, Aslihan; Ulu, Hazal Sonbahar; Ince, Deniz Inal; Tural, Dilber Ademhan; Sabuncuoglu, Suna; Özçelik, Uğur; Inal-ınce, Deniz; Budak, Murat; Arıkan, Hülya
    Background. There is a need to identify the complex interplay between various physiological mechanisms in primary ciliary dyskinesia (PCD) and cystic fibrosis (CF). The study investigated the interaction between respiratory function, exercise capacity, muscle strength, and inflammatory and oxidant/antioxidant responses in patients with PCD and CF. Methods. The study included 30 PCD patients, 30 CF patients, and 29 age and sex-matched healthy subjects. Exercise capacity was assessed using the modified shuttle walk test (MSWT). Handgrip strength (HGS) was used to evaluate general muscle strength. Oxidative stress-inflammatory parameters were also assessed. Pulmonary function test was performed by spirometry. Regarding the forced expiratory volume in 1 second (FEV1) 1 ) z-score, patients with PCD and CF were subdivided into normal, mild, and severe/moderate groups. Results. Forced vital capacity (FVC) z-scores were lower in PCD and CF patients than controls. FEV1, 1 , FEV1/ 1 / FVC, peak expiratory flow (PEF), and forced mid expiratory flow (FEF 25-75% ) z-scores were lower in PCD than in the other groups. HGS was lower in both mild PCD and normal CF patients relative to the controls. MSWT distance was lower in severe/moderate PCD patients than controls. Catalase (CAT), glutathione S-transferase (GST), glutathione peroxidase (GPx), and malondialdehyde (MDA) levels did not differ significantly among the study groups, but superoxide dismutase (SOD) level in severe/moderate PCD, and glutathione (GSH) level in normal CF were higher than in controls. Interleukin-6 (IL-6) level was higher in patients with normal PCD and CF compared to the controls. IL-1 beta level was higher in PCD compared to controls. Additionally, correlations among these parameters were also determined in some patient groups. Conclusion. Homeostasis related to respiratory function, aerobic performance, muscle strength, inflammatory response, and oxidant/antioxidant balance were affected in PCD and CF. Evaluating these mechanisms together may contribute to elucidating the pathophysiology of these rare diseases.
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    Retrospective Evaluation of Childhood Central Nervous System Tumors Followed in a Pediatric Hematology Oncology Center: a Single Center Experience
    (Akad Doktorlar Yayinevi, 2024) Erdem, Arzu Yazal; Emır, Suna; Özyörük, Derya; Genç, Aslı; Yazal Erdem, Arzu
    Central nervous system (CNS) tumors are one of the main causes of cancer-related deaths in childhood. Although approximately 60% of all patients are alive 5 years after diagnosis, a sequela due to the disease and treatments are common. In this study, we aimed to evaluate the demographic, clinical characteristics, and outcomes of the childhood CNS tumors in our center. A total of 141 patients between 0-18 years who were followed up and completed their treatment in our pediatric oncology center were included. The files were reviewed retrospectively. The median age of patients was 7 years (range 1 month-17.6 years). The male/female ratio was 1.1: 1. The most common presenting symptom was headache. The median time from the first symptom to diagnosis was 1.4 months. Medulloblastoma was the most common diagnosis (n= 28, 19.9%), followed by pilocytic astrocytoma (18.4%, n= 26) respectively. Out of 141 patients, a sequela was seen in 55 (39%) patients. The relationship between high-dose radiotherapy and the development of short stature was statistically significant (p= 0.009). The patients with metastatic disease were likely to have lower survival rates than nonmetastatic disease (p= 0.001). The presence of metastasis increased the death status 6.482 times (OR: 6,482, p= 0.001). The overall 5-year survival rate of all patients was found 80%. There was an association between the histopathological subtypes and overall survival rates (p= 0.001). In the multivariate analysis, metastasis was the most important factor in survival. According to Cox regression analysis, the two most important factors affecting overall survival were the histopathological subtype and the presence of metastasis.