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Publisher: Turkish J Pediatrics

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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
    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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    Citation - WoS: 6
    Citation - Scopus: 8
    Endonasal Choanal Atresia Repair; Evaluating the Surgical Results of 58 Cases
    (Turkish J Pediatrics, 2021) Bajin, Munir Demir; Onay, Ovsen; Gunaydin, Riza Onder; Unal, Omer Faruk; Yucel, Omer Taskin; Akyol, Umut; Aydin, Canset
    Background. Choanal atresia is the most common congenital nasal anomaly, with an incidence of 1:5000-1:8000 live births. Atresia can be seen as membraneous, bony or mixed type. When it is bilateral, it is accepted life-threatening, therefore bilateral atresia necessitates immediate intervention. Diagnosis is confirmed by endoscopic examination and computed tomography. The absolute treatment is surgical, and different approaches have been proposed. Methods. Herein, we describe our 15-year experience in the treatment of 58 patients of congenital choanal atresia with transnasal endoscopic approach, and we compare the efficacy of placement of an intranasal stent and applying mitomycin while endoscopic microsurgical repair. Results. The study included 41 female patients (71%) and 17 male patients (29%) with congenital CA. The mean age was 3 years ranging from 10 days to 16 years. The atretic plate was bilateral in 24 patients (41%) and unilateral in 34 (59%). The most common atresia type was the mixed type with 29 patients (50%). A total of 17 patients (29%) required postoperative revision(s). Postoperative revisions were more frequent among patients with bilateral CA (50%), and with mixed CA (31%). Stenting was used additionally by surgical correction for 10 patients. After stenting, fibrosis and restenosis was seen in 7 patients (79%). Mitomycin C was applied peroperatively in 8 patients. Restenosis after mitomycin application was seen in 4 patients (50%). Conclusions. By our experience, endoscopic microsurgical repair of atresia proved to be an effective and safe procedure, results compared with adjuvant treatment modalities like stent or mitomycin C use, was not better. Restenosis was the major problem seen after surgical correction.