Background:We aimed to evaluate anxiety among children with cystic fibrosis (CF) and their mothers related to the COVID-19 pandemic.Methods: A total of 45 patients with CF and their mothers were enrolled in the study together with 90 age-matched healthy children and their mothers as a control group.The State and Trait Anxiety Inventory (STAI) was administered by teleconference with children aged 13-18 years old and their mothers. The STAI for children was administered with children aged 9-12 years. Results were compared with age-matched healthy children and their mothers. The relationship between anxiety scores of children with CF and their mothers was evaluated by comparing with clinical data of children with CF. At the conclusion of the teleconference, mothers were asked whether their anxiety had changed as a result of the interview.Results: It was found that healthy children aged 13-18 years had higher state anxiety scores than age-matched children with CF. Mothers of children with CF had higher trait anxiety scores, especially those of children aged 0-12 years, than mothers of healthy children (p<0.05). For mothers of children with CF, state anxiety scores were higher among those whose children had chronic Pseudomonas infection (p<0.05).Most mothers of children with CF stated that their anxiety decreased following the interview.

Patients with interstitial lung disease due to surfactant protein C (SFTPC) mutations are rare and not well characterised.We report on all subjects collected over a 15-year period in the kids-lung register with interstitial lung disease and a proven SFTPC mutation. We analysed clinical courses, interventions and outcomes, as well as histopathological and radiological interrelations.17 patients (seven male) were followed over a median of 3 years (range 0.3-19). All patients were heterozygous carriers of autosomal dominant SFTPC mutations. Three mutations ( p.L101P, p.E191 K and p.E191*) have not been described before in the context of surfactant protein C deficiency. Patients with alterations in the BRICHOS domain of the protein (amino acids 94-197) presented earlier. At follow-up, one patient was healthy (2 years), six patients were "sick-better" (2.8 years, range 0.8-19), seven patients were "sick-same" (6.5 years, 1.3-15.8) and three patients were "sick-worse" (0.3 years, 0.3-16.9). Radiological findings changed from ground-glass to increasing signs of fibrosis and cyst formation with increasing age. Empiric treatments had variable effects, also in patients with the same genotype.Prospective studies with randomised interventions are urgently needed and can best be performed in the framework of international registers. @ERSpublications Genotype alone does not predict the clinical course of surfactant protein C deficiency in children and young adults http://ow.ly/GRhCc

CF should be considered in the differential diagnosis of metabolic alkalosis in young children. Vomiting and loss of appetite are important warning signs of possible PB in CF patients, particularly before 4 years of age. To prevent serious complications, it is crucial that parents and physicians recognise PB as early as possible.

Bronchiectasis is still common among some developing countries like Turkey. The aim of this study was to document the number of children with non-cystic fibrosis (CF) bronchiectasis, to evaluate the risk factors and to emphasize early diagnosis and treatment. All children, except those diagnosed with CF, with bronchiectasis established by chest radiogram, bronchography and/or computed tomography or biopsy material, were retrospectively reviewed. They were tested for serum total eosinophil count, nasal smear, serum levels of immunoglobulins A, G, M, E, and serum alpha-1 antitrypsin level. Pulmonary function tests, rigid bronchoscopy, nasal biopsy, lung scintigraphy, and echocardiogram were also performed. There were 204 patients whose most common presenting symptoms were cough, sputum expectoration, and dyspnea. Bronchiectasis was present mostly in the left lower lobe. The cause could not be determined in 49 per cent of patients. Among the identified causes, infection was present in most patients, followed by asthma, primary ciliary dyskinesia, congenital immune deficiency, and foreign body aspiration. It is possible to prevent bronchiectasis in children with vaccinations and improved nutrition in developing countries. Early diagnosis and treatment will increase the quality of life and survival of patients with bronchiectasis, which has irreversible and progressive complications if untreated.

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.