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    Eligibility of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) modulator therapies: cohort of cystic fibrosis registry of Türkiye
    (Turkish J Pediatrics, 2025) Erdal, Meltem Akgul; Buyuksahin, Halime Nayir; Sen, Velat; Kilinc, Ayse Ayzit; Cokugras, Haluk; Dogan, Guzide; Yilmaz, Asli Imran
    Background. Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) variants are essential for determining eligibility for CFTR modulator drugs (CFTRms). In contrast to Europe and the USA, the treatment eligibility profile of cystic fibrosis (CF) patients in T & uuml;rkiye is not known. In this study we aimed to determine the eligibility of CF patients in T & uuml;rkiye for the CFTRms. Methods. The Cystic Fibrosis Registry of T & uuml;rkiye (CFrT) data was used to determine the age of patients in the year 2021 and the genetic variants they were carrying. Age- and CFTR-variant appropriate modulator therapies were determined using the Vertex (R) algorithm. Results. Among a total of 1930 registered patients, CTFR gene analysis was performed on a total of 1841 (95.4%) patients. Mutations were detected in one allele in 10.7% (198 patients), and in both alleles in 79% (1455 patients) of patients. A total of 855 patients (51.7% for whom at least 1 mutation was detected) were eligible for the drugs. The most appropriate drug among genotyped patients was found to be elexacaftor/tezacaftor/ivacaftor for 486 patients (26.4%), followed by ivacaftor for 327 patients (17.7%) and lumacaftor/ivacaftor for 42 patients (2%). Conclusions. Only half of patients registered in CFrT were eligible for CFTRms, which is a significant difference from the CFTR variant profile seen in USA and Europe. However, access to treatment is hampered for some patients whose genes are not analysed. Further studies in CF populations, where rare mutations are relatively more common, will contribute to the field of CFTR modulator treatments for such rare mutations.
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    Importance of flexible bronchoscopy in the diagnosis of childhood respiratory diseases
    (2020) Ayzit Kilinc, Ayse; Cokugras, Haluk
    Aim: One of the methods used for the diagnosis of childhood respiratory diseases is flexible fiber optic bronchoscopy (FFB). This diagnostic method allows examination of the nose, pharynx, larynx, and tracheobronchial tree. In this study, we present 2 years of FFB experience and highlight its diagnostic advantages in pediatric medicine. Material and Methods: We retrospectively evaluated 213 FFB procedures that were performed between March 2017 and April 2019. Age, sex, FFB indication, bacterial growth in bronchoalveolar lavage (BAL) analysis, the presence of pathological bronchoscopy findings and complications after bronchoscopy were assessed.Results: Of the 213 patients included in the study, 116 (54%) were female and 97 (46%) were male. The mean age was 4.7 ± 6.32 months. The most common indications for bronchoscopy were abnormal radiological findings (30.5%), stridor (13.6%), and chronic cough (11.3%). In 152 patients (71%), diagnostic and therapeutic findings were obtained after bronchoscopy. Transbronchial biopsy was performed in eight patients for whom the etiology could not be determined; a diagnosis was obtained in five of these patients. Bacterial growth in BAL was detected in 68 patients (32%). Temporary complications developed in 17 patients (7.9%) with the most common complication being coughing during the procedure in 8 patients (3.8%).Conclusion: FFB is an important method that allows visualization of the upper and lower airways. The widespread use of pediatric FFB will enable early and non-invasive diagnosis of many respiratory diseases.
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    Isolated Pulmonary Langerhans-Cell Histiocytosis Mimicking Miliary Tuberculosis
    (Aves Yayincilik, Ibrahim Kara, 2009) Ozen, Metehan; Akcakaya, Necla; Camcioglu, Yildiz; Cokugras, Haluk; Oz, Buge
    Isolated involvement of lungs in the course of Langerhans'-cell histiocytosis (LCH) is very rare in childhood. Spontaneous pneumothorax (PTX) may occur during the course which necessitates rapid intervention. We present a 17 months-old girl who was sent with the pre-diagnosis of miliary tuberculosis. Despite antituberculosis therapy, her pulmonary function deteriorated. The chest tomography showed a different lung pattern which is suggestive of LCH. The biopsy findings and immunohistochemistry staining supported the diagnosis. She experienced recurrent pneumothorax incidences which were managed by drainage. She responded well to chemotherapy. Although rare, isolated pulmonary LCH should be remembered in differential diagnosis of cystic lung disease in childhood. On time diagnosis, appropriate therapy and rapid intervention for pneumothorax have important impacts on patient prognosis.
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    Non-cystic fibrosis bronchiectasis: Etiologic approach and effects of long-term azithromycin in children
    (2021) Ayzit Kilinc, Ayse; Cokugras, Haluk
    Aim: Non-cystic fibrosis bronchiectasis (NCFB) is a chronic pulmonary problem that includes a group of heterogeneous diseases. Macrolide antibiotics are increasingly prescribed for patients with NCFB, but there are very few studies on their use in children. This study aimed to search the clinical features of children with NCFB and the effect of long-term use of azithromycin on the frequency of aggravation, microbiological reproduction, and pulmonary function tests.Materials and Methods: A total of 79 cases diagnosed with NCFB were recorded. Clinical, laboratory, and radiological evaluations were also recorded. Exacerbation frequency, sputum cultures, and pulmonary function tests of 27 children who received azithromycin before and during prophylaxis were also analyzed.Results: The median age of children was 7.6 years old (1 to 16 years), when the children were diagnosed with NCFB. Bronchiectasis etiology was detected in 62 patients. Primary ciliary dyskinesia (PCD) was the most common cause of bronchiectasis, which 24 (30%) children were diagnosed with PCD. Azithromycin treatment was given to 27 bronchiectasis patients for six months. A statistically significant decrease was detected in pulmonary aggravation frequency and sputum microbiology during azithromycin treatment (p 0.05)Conclusion: The use of azithromycin in children with NCFB improves aggravation frequency but has no significant effect on the pulmonary function test.