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    Diagnosis of primary immunodeficiency diseases in pediatric patients hospitalized for recurrent, severe, or unusual infections
    (Codon Publications, 2022) Nepesov, Serdar; Fırtına, Sinem; Aygün, Fatma Deniz; Burtenece, Nihan; Çokuğraş, Haluk; Camcıoğlu, Yıldız
    Background: Primary immunodeficiency diseases (PID) usually presents itself with recurrent, severe, and unusual infections, along with autoimmunity and various other malignancies. But, the diversity of PID often makes the diagnosis of patients difficult for physicians other than clinical immunologists. This study aimed to describe the characteristics of patients diagnosed with PIDs during the inpatient treatment for infectious diseases, and to highlight the cases in which a PID diagnosis should be considered.Methods: The clinical, immunological, and molecular features of 81 pediatric patients treated for infectious diseases, who were diagnosed with a PID during hospitalization was retrospec-tively analyzed. The diagnosis was based on the PID criteria of the International Union of Immunological Societies.Results: The five main PID sub-types were identified. Predominantly, antibody deficiencies were the most common (61.7%) group. The average delay in diagnosis was 34.6 months, and the positive family history rate was 24.7%, while the consanguineous marriage rate was 45.7%. Around thirty-five (43%) patients were found to have mutated PID-related genes. While lower respiratory tract infections were the most common symptom, a fever of unknown origin was another remarkable diagnosis. Eight (9.9%) patients underwent allogeneic hematopoietic stem cell transplantation.Conclusions: Clinicians should consider a PID diagnosis, especially in the cases of recurrent, severe, or atypical infections. Increased knowledge of the alarm features of PID can promote early diagnosis
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    Factors associated with pulmonary function decline of patients in the cystic fibrosis registry of Turkey: a retrospective cohort study
    (Wiley, 2024) Emiralioğlu, Nagehan; Çakır, Banu; Sertçelik, Ahmet; Yalçın, Ebru; Kiper, Nural; Şen, Velat; Altıntaş, Derya Ufuk; Serbes, Mahir; Çokuğraş, Haluk; Kılınç, Ayşe Ayzıt; Başkan, Azer Kılıç; Hepkaya, Evrim; Çakır, Erkan
    Background: The decline in pulmonary function is a predictor of disease progression in patients with cystic fibrosis (CF). This study aimed to determine the decline rate of percent predicted forced expiratory volume in 1 s (ppFEV1) based on the data of the CF Registry of Turkey. The secondary aim was to investigate the risk factors related to the decline in ppFEV1. Methods: A retrospective cohort study of CF patients over 6 years old, with pulmonary function data over at least 2 years of follow-up was extracted from the national CF registry for years 2017-2019. Patients were classified according to disease severity and age groups. Multivariate analysis was used to predict the decline in ppFEV1 and to investigate the associated risk factors. Results: A total of 1722 pulmonary function test results were available from 574 patients over the study period. Mean diagnostic age was older and weight for age, height for age, and body mass index z scores were significantly lower in the group of ppFEV1 < 40, while chronic Pseudomonas aeruginosa (p < .001) and mucoid P. aeruginosa colonization (p < .001) were significantly higher in this group (p < .001). Overall mean annual ppFEV1 decline was -0.97% (95% confidence interval [CI] = -0.02 to -1.92%). The mean change of ppFEV1 was significantly higher in the group with ppFEV1 >= 70 compared with the other (ppFEV1 < 40 and ppFEV1: 40-69) two groups (p = .004). Chronic P. aeruginosa colonization (odds ratio [OR] = 1.79 95% CI = 1.26-2.54; p = .01) and initial ppFEV1 >= 70 (OR = 2.98 95% CI = 1.06-8.36), p = .038) were associated with significant ppFEV1 decline in the whole cohort. Conclusions: This data analysis recommends close follow-up of patients with normal initial ppFEV1 levels at baseline; advocates for early interventions for P. aeruginosa; and underlines the importance of nutritional interventions to slow down lung disease progression.
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    Humoral and cellular immunity to SARS-COV-2 vaccination with BNT162B2 MRNA vaccine in pediatric kidney transplant and dialysis patients
    (Springer, 2022) Gülmez, Rüveyda; Özbey, Doğukan; Ağbaş, Ayşe; Aksu, Bağdagül; Yıldız, Nurdan; Üçkardeş, Diana; Saygılı, Seha; Yıldırım, Zeynep Yürük; Taşdemir, Mehmet; Kıykım, Ayça; Çokuğraş, Haluk; Canpolat, Nur; Nayır, Ahmet; Kocazeybek, Bekir S.; Çalışkan, Salim
    Humoral And Cellular Immunity To Sars-Cov-2 Vaccination With Bnt162b2 Mrna Vaccine In Pediatric Kidney Transplant And Dialysis Patients
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    Long-term Home Mechanical Ventilation of Children in İstanbul
    (AVES, 2025) Yanaz, Mürüvvet; Ünal, Füsun; Hepkaya, Evrim; Yazan, Hakan; Can Oksay, Sinem; Köstereli, Ebru; Yılmaz Yeğit, Cansu; Başkan, Azer Kılıç; Onay, Zeynep Reyhan; Gulieva, Aynur; Soyyiğit, Aslınur; Kalyoncu, Mine; Küçük, Hanife Büşra; Ayhan, Yetkin; Ergenekon, Almala Pınar; Atağ, Emine; Uzuner, Selçuk; İkizoğlu, Nilay Baş; Kılınç, Ayşe Ayzıt; Ay, Pınar; Eralp, Ela Erdem; Gökdemir, Yasemin; Öktem, Sedat; Çakır, Erkan; Girit, Saniye; Uyan, Zeynep Seda; Çokuğraş, Haluk; Ersu, Refika; Karadağ, Bülent; Karakoç, Fazilet
    OBJECTIVE: The aims of this multi-center study were to describe the characteristics of children receiving long-term home mechanical ventilation (HMV) in İstanbul and to compare the patients receiving non-invasive and invasive ventilation. MATERIAL AND METHODS: This cross-sectional multicenter study included all children receiving long-term HMV followed by admission to six tertiary hospitals. The data were collected between May 2020 and May 2021. Demographic data and data regarding HMV were collected from the patient charts. RESULTS: The study included 416 participants. The most common diagnoses were neuromuscular (35.1%) and neurological diseases (25.7%). Among the patients, 49.5% (n = 206) received non-invasive ventilation (NIV), whereas 50.5% (n = 210) received invasive ventilation. The median age at initiation was significantly younger in the invasive ventilation group than in the NIV group (10 vs. 41 months, P < 0.001). Most subjects in the NIV group (81.1%) received ventilation support only during sleep, whereas most subjects in the invasive ventilation group (55.7%) received continuous ventilator support (P < 0.001). In addition to ventilation support, 41.9% of the subjects in the invasive ventilation group and 28.6% in the NIV group received oxygen supplementation (P = 0.002). Within the last year, 59.1% (n = 246) of the subjects were hospitalized. The risk factors for hospitalization were invasive ventilation, continuous ventilatory support, oxygen supplementation, tube feeding, and swallowing dysfunction (P = 0.002, 0.009, <0.001, <0.001 and <0.001 respectively). CONCLUSION: Despite the increasing use of NIV in most studies, half of the study population received invasive ventilation. Patients receiving invasive ventilation were more likely to require continuous ventilator support and oxygen supplementation and were at increased risk of hospitalization. © 2025 The Author.
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    The Relationship between Genotype and Phenotype in Primary Ciliary Dyskinesia Patients
    (Ali Cangül, 2021) Kilinc, Ayse Ayzit; Cebi, Memnune Nur; Ocak, Zeynep İpek; Çokuğraş, Haluk
    Objectives: Primary ciliary dyskinesia (PCD) is a chronic genetic disease that affects the respiratory tract, characterized by different clinical and laboratory features. It has a very difficult diagnosis, and high morbidity. In recent years, with the advances in genetics, the rate of diagnosis has increased considerably. In this study, it was aimed to evaluate the relationship between PCD patients' clinical, radiological and laboratory features and genetic analysis. Methods: The study included 14 children who were diagnosed with PCD between 2015-2019 and underwent exome analysis. Diagnostic ages, body mass indexes (BMI)- Z score, clinical and radiological findings, pulmonary function tests, sputum culture reproduction and gene analysis were evaluated and compared. Results: Six of the patients (43%) were girls and 8 (57%) were boys, and the median age at the time of diagnosis was 9 (min-max: 3-16) years. Genetic analysis revealed pathogenic mutations in DNAH5 (n=4, 29%), DNAH11 (n=2, 14%), RSPH4A (n=2, 14%), CCDC40 (n=2, 14%), DNAH9 (n=1, 7%), HYDIN (n=1, 7%), DNAH1 (n=1, 7%), and ARMC4 (n=1, 7%). Although not statistically significant, it was found that the diagnosis age was lower and the BMI Z-score was lower in CCDC40 mutations. Growth parametres were normal in DNAH5, DNAH11, RSPH4A and ARMC4 pathogenic variants. No significant correlation was found between genetic analysis and clinical features, culture reproduction and pulmonary function tests of the patients. Conclusion: It is thought that more detailed information about the possible clinical features and prognosis of the disease can be obtained by genetic examinations of PCD. However, clinical trials with higher patient numbers are still needed