Background Cystic fibrosis (CF) care has been implemented in Turkey for a long time; however, there had been no patient registry. For this purpose, the Turkish National CF Registry was established. We present the first results of registry using data collected in 2017. Methods The data were collected using a data‐entry software system, which was accessed from the internet. Demographic and annually recorded data consisted of 15 and 79 variables, respectively. Results There were 1170 patients registered from 23 centers; the estimated coverage rate was 30%. The median age at diagnosis was 1.7 years (median current age: 7.3 years); 51 (4.6%) patients were aged over 18 years. Among 293 patients who were under 3 years of age, 240 patients (81.9%) were diagnosed through newborn screening. Meconium ileus was detected in 65 (5.5%) patients. Genotyping was performed in 978 (87.4%) patients and 246 (25.2%) patients' mutations were unidentified. The most common mutation was deltaF508 with an allelic frequency of 28%, followed by N1303K (4.9%). The median FEV1% predicted was 86. Chronic colonization with Pseudomonas aeruginosa was seen in 245 patients. The most common complication was pseudo‐Bartter syndrome in 120 patients. The median age of death was 13.5 years in a total of 15 patients. Conclusions Low coverage rate, lack of genotyping, unidentified mutations, and missing data of lung functions are some of our greatest challenges. Including data of all centers and reducing missing data will provide more accurate data and help to improve the CF care in Turkey in the future.

BackgroundPseudo‐Bartter syndrome (PBS) is a rare complication of cystic fibrosis (CF) and there are limited data in the literature about it. We aimed to compare clinical features and accompanying findings of patients with PBS in a large patient population.MethodsThe data were collected from the Cystic Fibrosis Registry of Turkey where 1170 CF patients were recorded in 2017. Clinical features, diagnostic test results, colonization status, complications, and genetic test results were compared in patients with and without PBS.ResultsTotally 1170 patients were recorded into the registry in 2017 and 120 (10%) of them had PBS. The mean age of diagnosis and current age of patients were significantly younger and newborn screening positivity was lower in patients with PBS (P < .001). There were no differences between the groups in terms of colonization status, mean z‐scores of weight, height, BMI, and mean FEV1 percentage. Types of genetic mutations did not differ between the two groups. Accompanying complications were more frequent in patients without PBS.ConclusionPBS was detected as the most common complication in the registry. It could be due to warm weather conditions of our country. It is usually seen in younger ages regardless of mutation phenotype and it could be a clue for early diagnosis of CF.

Background Subglottic stenosis (SGS) is a complication that develops after intubation and is characterized by respiratory distress. The aim was to evaluate patients with post‐intubation SGS and to discover the factors contributing to its development. Methods A total of 112 patients who had a history of intubation were included. The case group consisted of 50 patients with post‐extubation persistent respiratory symptoms for which flexible bronchoscopy (FOB) was conducted and showed SGS. The control group consisted of 62 patient with no post‐extubation persistent respiratory symptoms, for whom FOB was not done (n = 54), and who had post‐extubation persistent respiratory symptoms and underwent FOB, which did not show subglottic stenosis (n = 8). Results No significant differences were detected related to age, gender, and gestational age. The median number of recurrent intubations was 2.5 and 3 in the case group and in control group, respectively (P = 0.14). The median duration of intubation was 20.5 days in the case group, and 6 days in the control group (P < 0.001). The Myer–Cotton classification indicated a degree of obstruction of grade 1 (mild) in 30% (n = 15), grade 2 in 16% (n = 8), grade 3 in 48% (n = 24), and grade 4 in 6% (n = 3) of the case group. Conclusion The duration of intubation was found to be a significant risk factor for SGS development. Age at intubation, gender, gestational age, indication of intubation, and the number of recurrent intubations were found to have no significant association. Patients with post‐extubation persistent respiratory problems, especially those with prolonged intubations, should be evaluated for SGS.

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Background: Tracheostomy-related morbidity and mortality mainly occur due to decannulation, misplacement, or obstruction of the tube. A standardized training can improve the skills and confidence of the caregivers in tracheostomy care (TC).Objective: Our primary aim was to evaluate the efficiency of standardized training program on the knowledge and skills (changing-suctioning the tracheostomy tube) of the participants regarding TC.Materials and Methods: Sixty-five caregivers of children with tracheostomy were included. First, participants were evaluated with written test about TC and participated in the practical tests. Then, they were asked to participate in a standardized training session, including theoretical and practical parts. Baseline and