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BackgroundA collaboration between the University of Michigan (UM) Cystic Fibrosis Center (CFC) and Marmara University (MU) CFC was initiated in MU through conducting Quality Improvement projects (QIP). The global aim was to improve nutritional status of children with CF (cwCF), with a specific aim to increase the mean BMI percentile (BMIp) for cwCF by 10 percentile points in 12 months. MethodsBody mass index (BMI) percentiles of cwCF were categorized as: nutritionally adequate (BMIp >= 50%); at risk (BMIp 25%-49%); urgently at risk (BMIp 10%-25%); critically at risk (BMIp < 10%). Appropriate interventions were made according to BMIp category every three months. Forced expiratory volume in one-second percent predicted (FEV1pp), and health-related quality of life (HRQoL) were evaluated. ResultsOne hundred and eight-two cwCF with a mean age of 9.1 +/- 4.3 years were included in the project. Baseline BMIp increased from 25.6 to 37.2 at the 12th month (p < 0.001). In the critically at-risk group BMIp increased from 3.6 to 20.5 (p < 0.001), in the urgently at risk group from 15.9 to 30.8 (p < 0.001), in the at risk group from 37.0 to 44.2 (p < 0.079) and in the nutritionally adequate group the increase was from 66.8 to 69.5 (p < 0.301). FEV1pp also improved significantly, from 81.3 +/- 20.6 to 85.9 +/- 20.8 (p < 0.001). Physical functioning, eating problems, and respiratory symptoms domains of the HRQoL evaluation improved (p < 0.05). ConclusionThis project has led to significant improvements in BMIp, FEV1pp and HRQoL of cwCF; similar projects could easily be implemented by centers in other developing countries.

Background and Aim Pulmonary embolism (PE) is a potentially life-threatening disease in children. The objective of the study is to evaluate the utility of adult-based pulmonary embolism rule-out criteria (PERC), Pediatric PE Model, and D-dimer in the diagnosis of PE in children. Material and Methods The study consisted of patients under 18 years of age who were consulted to the Pediatric Pulmonology Clinic for the evaluation of PE. Patients were divided into two groups based on the confirmation of PE. The group with the presence of PE (n = 20) consisted of children who were diagnosed with PE. The group with the absence of PE (n = 28) consisted of children with clinically suspected PE but negative diagnostic imaging. Adult validated clinical decision PERC rule and Pediatric PE Model were retrospectively applied to the patients. Results In the study, PERC demonstrated a sensitivity of 60% and a specificity of 46% for the diagnosis of PE in children. When PE Model was evaluated for the children, it was found a 50% sensitivity and 75% specificity. Combining PE Model and PERC rule with D-dimer did not increase the specificity and sensitivity. Smoking was found to be relevant for PE in the childhood. Twenty-five percent of the patients had a genetic tendency for PE. All of the patients had an underlying disease as well. Conclusion None of the current risk assessment tools (PE Model, PERC, D-dimer) were found to be accurate in predicting PE. Further larger population studies are still required to develop a better diagnostic approach.

Introduction: Polygraphy (PG) can be used as an alternative test for the diagnosis of obstructive sleep apnea syndrome (OSAS) in children. Night‐to‐night variability of PG in children is not known. Our aim was to determine whether a single night PG was reliable for OSAS diagnosis in children with symptoms of sleep‐disordered breathing (SDB). Materials and Methods: Otherwise healthy children who had been evaluated for symptoms of SDB were included. Two nocturnal PGs were performed 2–7 days apart. Demographic and clinical characteristics, Pediatric Sleep Questionnaire, and modified Epworth Sleepiness Scale were recorded. OSAS was diagnosed if obstructive apnea–hypopnea index was (oAHI) ⩾ 1/h and classified as mild (oAHI: 1–4.9/h), moderate (oAHI: 5–9.9/h), and severe (oAHI ⩾ 10/h). Results: Forty‐eight patients were included (37.5% female, age 10.8 ± 3.9 years) to the study. There were no significant differences in oAHI values and other respiratory parameters between the two PGs (p > 0.05). Thirty‐nine children were diagnosed with OSAS if the highest oAHI over any single night was used for diagnosis. Thirty‐ three of the 39 children (84.6%) were diagnosed with OSAS with the first PG while 35 of 39 (89.7%) children were diagnosed with OSAS with the second PG. There was an agreement for identifying OSAS and its severity between the two PGs in our study even though there were few individual intra‐subject differences in oAHI. Conclusion: There was no significant first‐night effect for PG in this study which suggests that a single night PG is adequate for diagnosis of OSAS in children with SDB‐ related symptoms.

Advances in technology in recent years increased the long-term survival rate of patients with chronic respiratory failure. As a result, the number of patients receiving long-term home mechanical ventilation (LTHV) support is increasing worldwide. The most important step in the management of patients with home mechanical ventilation (HMV) is to ensure adequate oxygenation and ventilation. Successful care at home requires the presence of trained caregivers and accessibility to medical devices and a care team. The aims of this study were, to describe the characteristics of children on LTHV in Istanbul, to compare the patients receiving non-invasive ventilation (NIV) and invasive ventilation, and to evaluate the frequency and risk factors of hospital admission in this population.

Background: A collaboration between the University of Michigan (U of M) Cystic Fibrosis Center (CFC) and Marmara University (MU) CFC was initiated to improve the health status of people with cystic fibrosis (pwCF) at MU through implementing Quality Improvement (QI) initiatives. The main aim was to improve lung function in children with FEV1pp

BackgroundThe aim of this study was to evaluate the prevalence of anxiety, depression, sleep, and associated factors in caregivers of children with spinal muscular atrophy (SMA). Materials and MethodsBeck Depression Inventory (BDI), the State-Trait Anxiety Inventory-State (STAI-S), the State-Trait Anxiety Inventory-Trait (STAI-T), and Pittsburgh Sleep Quality Index (PSQI) were used to assess the anxiety, depression, and sleep quality of the caregivers of children with SMA. Higher scores indicated worse outcome for all three questionnaires. ResultsFifty-six caregivers of children with SMA were included in the study. Median age of children was 6 (3.2-10) years and mean age of the caregivers was 37.0 +/- 6.5 years. Median scores of the BDI, STAI-S, STAI-T, and PSQI were 12 (7.2-17), 35.5 (31-44), 40.5 (35-48), and 7.0 (5.0-10.0), respectively. There was a positive correlation between BDI and PSQI scores (p < 0.05). There was a negative correlation between the age of the caregivers and PSQI, BDI, STAI-T scores (p = 0.01, r = -0.341; p = 0.006, r = -0.364; p = 0.003, r = -0.395, respectively). There was a negative correlation between the age of the patients and the PSQI scores of the caregivers (p = 0.01, r = -0.33). There was a negative correlation between BDI scores and household income (p = 0.01, r = -0.34). ConclusionCaregivers of children with SMA had elevated depression and anxiety levels and they also had decreased sleep quality. Economic and social support resources are needed to help caregivers of those children.

BACKGROUND: Cystic fibrosis (CF) is a chronic disease causing recurrent respiratory tract infections. Viral respiratory tract infections are more severe in CF. The first case of COVID-19 was seen in our country on March 11, 2020 and nationwide school closure and lockdown were implemented. School closure and home confinement might have adverse effects on children's physical and mental health. In this study, we aimed to compare the effect of COVID-19 pandemic on psychological reactions of CF patients and healthy controls. METHODS: This is a controlled cross-sectional study including 7-18 year-old children with CF. The survey included questions regarding family environment and peer relations, self care and psychological reactions to COVID-19 pandemic. The questionnaire was applied to children via telephone call under parental supervision. RESULTS: We evaluated 132 CF patients and 135 their healthy peers. Mean age was 11.5±2.9 years in CF group and 11.8±3.2 years in control group (p=0.98). There were 55 girls (41.7%) in CF group and 81 girls (60%) in control group (p=0.027). The socioeconomic status of families was similar. CF patients were found to be less anxious for family members having the risk of COVID-19, less upset for school closure, less anxious about the COVID-19 pandemic (p

Respiratory failure is the leading cause of mortality in spinal muscular atrophy type 1 (SMA1) children. The current study aims to evaluate the effect of nusinersen treatment on respiratory outcome of the patients with SMA1.In this retrospective, single-center study, 52 SMA1 patients treated with nusinersen were included in the analysis. Patients were divided into two groups based on their age at the time of their first nusinersen treatment (Group 1: ≤6 months, Group 2:6 months). Respiratory outcome on the 180th day of treatment is defined as the type of ventilation support (spontaneous breathing, noninvasive ventilation (NIV), and tracheostomized or intubated on invasive mechanical ventilation). Demographic data, respiratory outcome, and Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders scores were obtained from medical records.On the 180th day of treatment, 46 of the 52 (88.4%) children were alive. Prevalence of the mortality was similar in both groups (P = 0.65). The comparison of respiratory outcome in patients between group 1 and group 2 was as follows: spontaneous breathing, 7 (43.7%) versus 4 (13.3%) (P = 0.03); NIV16 h/day, 3 (18.7%) versus 4 (13.3%) (P = 0.68); invasive mechanical ventilation, 6 (37.5%) versus 22 (73.3%) (P = 0.01). There were no patients using NIV ≥16 h/day. There were significant improvements in Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders scores of the patients at day 180 in comparison with the baseline (P 0.001).Early initiation of nusinersen treatment in SMA1 patients may alter the disease's natural course.

Background Due to advances in technology, home ventilation in children has increased in recent years. The provision of proper care for a home-ventilated (HV) child can have a strong impact on the lifestyle of caregivers. The aim of this study was to evaluate the depression and anxiety levels of the mothers of home-ventilated children during the current COVID-19 pandemic and compare them to those of mothers of healthy peers. Methods This cross-sectional study was conducted on HV children (n=21) and a control group of healthy peers (n=32) by means of a questionnaire completed by the mothers of the children of both groups. Psychometric scales, such as the Beck Depression Inventory (BDI), the State-Trait Anxiety Inventory-State (STAI-S), and the State-Trait Anxiety Inventory-Trait (STAI-T), were used to assess participants. Results During the pandemic signs of depression were present in eight (38.1 %) of the case group and eight (25%) of the healthy control (HC) group. Comparison of the BDI scores from before and during the pandemic showed no difference between mothers of the HV children (p=0.09). Scores for BDI and STAI-T were higher in the case group than in the control group, whereas there was no significant difference in STAI-S scores. Conclusion Depression and anxiety levels of mothers of HV children were found to be higher during the COVID-19 pandemic. Economic, medical and social support resources are needed in order to reduce levels of depression and anxiety and help mothers of those children dependent on technology. This article is protected by copyright. All rights reserved.

Introduction: Empyema is an exudative type pleural effusion secondary to lung infections. Pulmonary function tests (PFTs) are used in measuring the function and volume of the lungs, diagnosing and grading diseases, and evaluating the response to treatment. Our aim was to evaluate pulmonary function in patients previously treated with empyema and compare it with age-matched healthy controls. Materials and methods: This is a controlled cross-sectional study evaluating the PFTs in twenty-one children previously managed for empyema and 40 controls. Spirometry and lung clearence index (LCI) tests were used to compare both groups. Demographic data, history of previous lung disease, physical examination findings, diagnostic method and treatment regimens were recorded. Results: Of the 21 patients, 7 (33.3%) were female and 14 (66.6%) were male. The mean age was 9.4 ±4.5 years. The most common presentation findings were high fever (95.2%), tachypnea (85.7%) and cough (85.7%). Empyema was on bilateral lungs in 9 (42.9%) patients, on the right in 8 (38.1%) patients and on the left in 4 (19%) patients. In spirometric examination, the mean forced expiratory volume (FEV1) % predicted was 93.76 ±11.78%, the mean FEV1 z score was -0.24 ±0.79 and no significant spirometric disorder was observed. The mean LCI value of the patients was 7.50 ±0.72, which was significantly higher than the control group (6.97 ±0.75) (p: 0.003). Conclusion: In the long-term follow-up of patients with empyema, airway disease that could not be detected by spirometry developed and LCI method was found to be superior to spirometry in detecting airway diseases.

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 postintervention assessments were compared through written and practical tests conducted on the same day. Results A significant improvement was observed in the written test score after the training. The median number of correct answers of the written test including 23 questions increased 26%, from 12 to 18 (p < .001). The median number of correct steps in tracheostomy tube change (from 9 to 16 correct steps out of 16 steps, 44% increase) and suctioning the tracheostomy tube (from 9 to 17 correct steps out of 18 steps, 44% increase) also improved significantly after the training (p < .001, for both). Conclusion Theoretical courses and practical hands-on-training (HOT) courses are highly effective in improving the practices in TC. A standardized training program including HOT should be implemented before discharge from the hospital. Still there is a need to assess the impact of the program on tracheostomy-related complications, morbidity, and mortality in the long term.

Background Chronic pulmonary infection is the leading cause of mortality and morbidity in patients with cystic fibrosis (CF). The most common pathogens isolated in CF are Staphylococcus aureus (SA) and Pseudomonas aeruginosa (PA). Chronic infection of PA and methicillin-resistant S. aureus (MRSA) are associated with worse survival and antibiotic eradication treatment is recommended for both. This study compared the outcomes between intravenous (IV) and non-IV antibiotics in eradication of PA and MRSA. Methods This was a single-center retrospective study. All respiratory specimen cultures of 309 CF patients and eradication regimens between 2015 and 2019 were reviewed. Patients received eradication treatment in case of first ever isolation or new isolation after being infection-free >= 1 year. The primary analysis was the comparison of the percentage of successful eradication after receiving IV and non-IV eradication regimens. Demographic and clinical risk factors for eradication failure were also analyzed. Results One hundred and two patients with PA isolations and 48 patients with MRSA were analyzed. At 1 year, 21.6% in PA group and 35.4% in MRSA group were successfully eradicated. There was not any statistically significant difference between IV versus non-IV antibiotic regimens on eradication in either group. Additionally, none of the clinical risk factors was significantly associated with eradication failure in PA and MRSA groups. Conclusion In the eradication of PA and MRSA, IV and non-IV treatment regimens did not show any superiority to one another. Non-parenteral eradication could be a better option considering the cost-effectiveness and the treatment burden of IV treatments due to hospitalization and the need for IV access.

BACKGROUND Nebulizers can be contaminated with microorganisms and may be a source of infection in the lower airways in patients with cystic fibrosis (CF). OBJECTIVE Primary aim of this study was to determine the level of knowledge regarding nebulizer hygiene and adherence to CF foundation infection prevention and control (IPC) measures of CF patients in our center. We also evaluated the effect of a standardized training program on nebulizer cleaning and disinfection practises with pre and posttest. METHODS Caregivers of 173 CF patients followed at Marmara University CF Center filled a questionnaire (pretest) regarding nebulizer hygiene and received didactic education including pictures and videos based on the cystic fibrosis foundation (CFF) IPC guidelines, patients were also provided educational materials. Posttest was performed 1-3 months after the education session. RESULTS Following standardized training, usage of appropriate methods according to CFF IPC guidelines improved significantly. Frequency of nebulizer cleaning after each use increased from 58.4% to 78% (p

Introduction: The pediatric tracheostomy related mortality mostly occurs due to accidental decannulation and cannula obstruction. The caregivers may be discharged with inadequate knowledge. Aims and objectives: Simulation training can highlight skills regarding tracheostomy care. Our aim is to investigate if a standardized hands-on-training improves knowledge and skills among caregivers. Methods: Before the traning, we applied a pretest to caregivers in order to assess their knowledge and current practises. Skills were evaluated by two simulation scenarios in a patient model: cannula change and aspiration. After one hour theoretical and practical hands-on training, the same post-test was applied, skills were reevaluated regarding cannula change and aspiration with practical tests. Results: The multicenter study included 65 caregivers of patients with tracheostomy. The median score of the theoretical test including 23 questions improved from 12 to 18 (p

BACKGROUND Depression and anxiety symptoms in patients with cystic fibrosis (CF) and their caregivers are 2-3 times higher than in the normal population. This study aims to evaluate the frequency and severity of depression and anxiety symptoms and to determine possible risk factors in CF patients and their mother and/or fathers at Marmara University CF center. METHODS The study included 132 CF patients who were followed up at our CF center. Patient Health Questionnaire (PHQ-9) and the Generalized Anxiety Disorder Questionnaire (GAD-7) were used to screen depression and anxiety. The questionnaires were completed by 50 CF patients (aged 12-17 years) and 132 parents of patients (aged 0-17 years). RESULTS While moderate to severe depressive symptoms were seen in 26% of patients, 33.7% of mothers and 14.6% of fathers; moderate to severe anxiety were present in 18%, 21.8% and 8.5%, respectively. None of the demographic characteristics was identified as a predictor of depression or anxiety. GAD-7 scores have shown a higher prevalence of anxiety in mothers of patients with chronic methicillin-resistant Staphylococcus aureus (p = .034). Additionally, hospitalization in the last 12 months was significantly correlated with higher PHQ-9 scores in fathers (p = .043). Analysis of patients' adherence to medical treatment and airway clearance showed higher depression and anxiety in mothers of the nonadherent group (p = .002). CONCLUSION Depression and anxiety were common in CF patients and their parents. These results illustrate the importance of depression/anxiety screening and psychosocial support for the CF patient and their parents.

Background Cystic fibrosis (CF) is a chronic disease causing recurrent respiratory tract infections. Viral respiratory tract infections are more severe in CF. The first case of COVID-19 was seen in Turkey on March 11, 2020 and nationwide school closure and lockdown were implemented. School closure and home confinement might have adverse effects on children’s physical and mental health. In this study, we aimed to compare the effect of COVID-19 pandemic on psychological reactions of CF patients and healthy controls. Methods This is a controlled cross-sectional study including 7-18 year-old children with CF. The survey included questions regarding family environment and peer relations, self care and psychological reactions to COVID-19 pandemic. The questionnaire was applied to children via telephone call under parental supervision. Results We evaluated 132 CF patients and 135 their healthy peers. Mean age was 11.5±2.9 years in CF group and 11.8±3.2 years in control group (p=0.98). There were 55 girls (41.7%) in CF group and 81 girls (60%) in control group (p=0.027). The socioeconomic status of families was similar. CF patients was feeling less anxious about the COVID-19 pandemic compared to controls (p=0.01). CF patients were found to be less anxious for family members having the risk of COVID-19, less upset for the school closure, less anxious about the COVID-19 pandemic (p

Background: Depression and anxiety symptoms in patients with cystic fibrosis (CF) and their caregivers are 2-3 times higher than in the normal population. This study aims to evaluate the frequency and severity of depression and anxiety symptoms and to determine possible risk factors in CF patients and their mother and/or fathers at Marmara University CF center. Methods: The study included 132 CF patients who were followed up at our CF center. Patient Health Questionnaire (PHQ-9) and the Generalized Anxiety Disorder Questionnaire (GAD-7) were used to screen depression and anxiety. The questionnaires were completed by 50 CF patients (aged 12 - 17 years) and 132 parents of patients (aged 0-17 years). Results: While moderate to severe depression were seen in 25.5% of patients, 33.7% of mothers and 14.6% of fathers; moderate to severe anxiety were present in 17.6%, 21.8% and 8.5%, respectively. None of the demographic characteristics was identified as a predictor of depression or anxiety. GAD-7 scores have shown a higher incidence of anxiety in mothers of patients with chronic methicillin-resistant Staphylococcus Aureus (p = 0.034). Additionally, hospitalization in the last 12 months was statistically significantly higher in PHQ-9 scores of fathers (p = 0.043). Analysis of patients’ adherence to medical treatment and airway clearance showed higher depression and anxiety in mothers of the non-adherent group (p = 0.002). Conclusion: Depression and anxiety were common in CF patients and their parents. These results illustrate the importance of depression/anxiety screening and psychosocial support for the CF patient and their parents.

Background: We aimed to increase the knowledge of cystic fibrosis(CF) patients’ caregivers by a training program for nebulizer cleaning and disinfection.We performed pre - posttest, took swab cultures from the nebulizers. Methods: A questionnaire about nebulizer cleaning was applied to caregivers of the 103 CF patients. Training sheets were given, one-hour practical training was performed, swab samples were taken from the nebulizers. Questionnaire was applied after 1-3 months again. Results: The study included 103 patients, mean age was 9.8±6 years.The person completing the questionnaire was 82.5% mother.The cleaning rate of the nebulizer after each use was %54.4 while disinfection rate was %29. After the education, this ratio was %77.7 and %69, respectively.While the most frequently used cleaning method was placing the nebulizer in vinegar water(30%) in the pretests;it was changed to warm water with liquid dish soap (39.8%, n=41) after the education. 46.6%(n=48) of the patients were not disinfecting their nebulizers. After the education, this ratio was decreased to 8.7 %. Swab samples were taken from 67 patients’ nebulizers. Organisms such as coagulase negative staphylococcus,Candida parapsilosis,S. maltophilia were identified in 1-3% of the cultures. Microbiological colonisation rate in the group of the ones who disinfects nebulizers after each use(n=21) was 38.1 %(n=8) while it was 60%(n=15) in the group of the ones who never disinfects(n=15), (p=0.23). Conclusions: After the education, the rate of disinfection and cleaning was increased. Cleaning of nebulizer parts should be taught via education programs.

Introduction: Polysomnography (PSG) is the gold standard for diagnosing obstructive sleep apnea (OSA) in children. Polgraphy (PG) is used as an alternative test when PSG is not available. PSG may underestimate the severity of OSA in the first night. Night-to-night variability of PG in children is not know. Our aim was to determine whether a single PG was reliable for OSA diagnosis in children with symptoms of sleep-disordered breathing (SDB). Methods: Children without any chronic underlying disease and who were evaluated for SDB in Pediatric Respirology clinic and consented to the study were included. Two nocturnal PGs were performed in the sleep laboratory 1 to 7 days apart. Demographic data and Pediatric Sleep Questionnaire (PSQ) were recorded. OSA was diagnosed if obstructive apnea-hypopnea index (oAHI) was >1/hour. OSA was classified as mild (oAHI:1-5), moderate (oAHI:5-10) and severe (oAHI>10). Results: 30 children were included to the study. Mean age was 10.8±4 years; 53.3% were male. Presenting symptoms were snoring (66.7%), mouth breathing (60%), apnea (36.7%), daytime sleepiness (23.3%) and hyperactivity (20%). PSQ was positive in 63.3%. There was no difference in oAHI between the two nights. oAHI1 and oAHI2 positively correlated (Spearman’s rho:0.825,p Conclusion: There is no first night effect in polygraphy performed in the sleep laboratory for OSA diagnosis in otherwise healthy children.

Background: Obstructive sleep apnea syndrome (OSAS) is an uncommon cause of pulmonary hypertension (PHT). Our aim is to evaluate the frequency of OSAS in children with PHT. Methods: The study included 11 patients who were referred to identify the underlying etiology in the last one year. Demographic and clinical features of the patients, sleep-related symptoms, PSG results and the etiology of PHT were evaluated. Results: Mean age of the patients was 7,2±6,1 years (54.5 % female). Median age at diagnosis was 4.1 years and onset of semptoms was 44 months. Snoring, open mouth sleeping and witnessed apnea was present in 45.4%, 45.4% and 36.3 % of the patients, respectively. Hypercarbia was present in 3 patients. Primary PHT was detected in 8 patients (72.7%), while secondary PHT was detected in 3 patients (27.3%). One of the patients with secondary PHT had arteriovenous malformation in the lung, one had cardiac anomaly and one had diaphragm hernia and right lung hypoplasia. Adenotonsillectomy was performed in the follow-up of two patients. Conclusions: OSAS should be kept in mind in the situation that the etiology of PHT can not be explained. Patients with PHT should be questioned about sleep-related symptoms. Sleep studies are methods without alternative to determine etiology.

Background: Depression and anxiety symptoms in patients with cystic fibrosis (CF) and their caregivers are 2-3 times higher than normal. The aim of this study is to evaluate the frequency and severity of depression and anxiety disorders and determine possible risk factors in CF patients and their parents at Marmara University CF center. Methods: The study included 132 CF patients who were followed up at our CF center. Patient Health Questionnaire (PHQ-9) and Generalised Anxiety Disorder Questionnaire (GAD-7) were used to screen depression and generalized anxiety disorders. The questionnaires were completed by 51 CF patients (aged >12 years) and 130 parents of patients (aged 0-17 years). Results: While moderate to severe depression were seen in 25.5% of patients, 33.7% of mothers and 14.6% of fathers; moderate to severe anxiety were present in 17.6%, 21.8% and 8.5%, respectively. None of the demograpich characteristics were identified as a predictor of depression or anxiety. GAD-7 scores have shown higher incidence of anxiety in mothers of patients with chronic Methicillin-resistant Staphylococcus aureus(p:0.034). Additionally, hospitalisation in the last 12 months was statistically significant in PHQ-9 scores of fathers(p:0.043). Conclusions: Depression and anxiety were common in CF patients and their parents. This results show the importance of depression/anxiety screening tests and psychosocial support in patient follow-up.

BACKGROUND Congenital lung malformations (CLM) are rare disorders and surgical intervention is the definitive treatment. Our aim is to evaluate the long-term lung function of patients with CLM after surgery compared to healthy children. METHODS Sixteen children with CLM (M/F: 9/7) and 30 age-matched, healthy controls (M/F: 13/17) were included in the study. Demographic data were recorded and both groups were compared by spirometry and the nitrogen-based Lung Clearance Index (LCI). RESULTS Mean ± SD age of the patients was 12.0 ± 5.4 years. The mean forced expiratory volume in 1 s (FEV1 ), forced vital capacity (FVC), FEV1 /FVC, and forced expiratory flow between 25% and 75% of force expiration (FEF25-75 ) predicted was, 86.68 ± 16.65, 88.00 ± 14.58, 97.44 ± 9.89, and 79.00 ± 26.41, respectively in the patient group. Patients with CLM had significantly lower values in FEV1 , FVC, FEF25-75 than healthy controls (P = 0.002, P 0.007, P 0.045). While the mean LCI2,5% value in patients' group was 8.33 ± 1.52, it was 7.28 ± 0.80 in healthy controls (P = 0.023). Strong inverse correlation between LCI and FEV1 , FEV1 /FVC was detected in the patient group (P = 0.023; r: -0.581, P 0.017; r: -0.606 respectively). CONCLUSION This study revealed that, in long-term follow-up, patients who had surgery because of CLM have impairment in the pulmonary function compared to healthy children and LCI may be more accurate in detecting airway diseases early than spirometry.

Background: Chest physiotherapy (CP) is a recommended treatment modality in primary ciliary dyskinesia (PCD). Objective: Primary aim was to compare the efficacy and safety of the conventional chest physiotherapy (CCP) and oscillatory positive expiratory pressure therapy (OPEPT). Secondary aims were to compare the exacerbation rate, time until the first exacerbation, patient compliance and comfort between the two CP methods. Methods: This is a 6 month randomized, controlled crossover trial. Patients >6 years of age with PCD were randomized into two groups, first group was assigned to OPEPT (Acapella®) for 3 months while second group was assigned to CCP. Groups were crossed over to the other modality after a 15-day washout period. Pulmonary function tests (PFTs) and compliance were monitored by monthly clinic visits. Results: There was a significant increase in FEV1 , FEF25-75 , and PEF values (p = .018, p = .020, and p = .016, respectively) in the OPEPT group and in FVC values (p = .007) in CCP group compared to baseline. However PFT increase at 3rd month was not superior to each other with both physiotherapy methods. Median acute pulmonary exacerbation rate and time period until the first exacerbation were similar in both groups (p = .821, p = .092, respectively). Comfort and effectiveness of OPEPT was higher than CCP according to patients (p = .029 and p = .042, respectively). There were no adverse effects with either therapy. Conclusions: OPEPT was as effective as CCP in PCD patients. OPEPT was more comfortable and effective than CCP according to patients. OPEPT might be an efficient alternative method for airway cleareance in PCD patients.

Background The prevalence of non-cystic fibrosis (CF) bronchiectasis is increasing in both developed and developing countries in recent years. Although the main features remain similar, etiologies seem to change. Our aim was to evaluate the clinical and laboratory characteristics of our recent non-CF bronchiectasis patients and to compare these with our historical cohort in 2001. Methods One hundred four children with non-CF bronchiectasis followed between 2002 and 2019 were enrolled. Age of diagnosis, underlying etiology and microorganisms in sputum culture were recorded. Clinical outcomes were evaluated in terms of lung function tests and annual pulmonary exacerbation rates at presentation and within the last 12 months. Results Mean FEV1 and FVC %predicted at presentation improved compared to historical cohort (76.6 ± 17.1 vs. 63.3 ± 22.1 and 76.6 ± 15.1 vs. 67.3 ± 23.1, respectively; p p p = 0.001), decrease in idiopathic cases (19.2% vs. 37.8%; p = 0.03) with no change in postinfectious and immunodeficiencies as underlying etiology. Sputum cultures were positive in 77.9% of patients which was 46.9% in the historical cohort (p = 0.001). Conclusion Baseline pulmonary function tests were better and distribution of underlying etiology had changed with a remarkable increase in diagnosis of PCD in the recent cohort.

Introduction and Aim: We aimed to evaluate respiratory support need in patients treated with Nusinersen and to report their respiratory follow-up during the treatment period. Material and Methods: The study included patients treated with 4 and more doses of Nusinersen. Patients were evaluated regarding respiratory findings before the initiation of Nusinersen treatment and after the 4.th doses in pediatric pulmonology department. Results: Twenty-seven patients who were followed up from our clinic were included in the study. 44% of the patients were female (n = 12); The mean age was 47.81 ± 6.27 months (7-120 months). The mean treatment duration of the patients was 36.1 ± 7.57 months (2-111 months). Sixteen patients were on home ventilation via tracheostomy, 6 patients were in ICU and mechanically ventilated (5 of them were entubated and 1 of them was tracheostomised). One patient was on non-invasive ventilation and needed O2 support. Four patients breathing spontaniosly in room air.56.2% (n = 9) of home ventilated patients and 50% (n = 3) of patients staying in ICU had clinical improvement. One of the entubated patients was tracheostomized and home ventilation was initiated. Four patients atients breathing spontaniously did not require any respiratory support. The o2 need of the non invasive ventilated patient was ended after the 4.th doses of Nusinersen. Home ventilation therapy was stopped by a family without the knowledge of the clinician after the third doses of Nusinersen theraphy but unfortunately the patient was passed away. Discussion: Long term studies including more patients treated with Nusinersen and specified respiratory scoring systems are needed to evalute the respiratory follow up.

Introduction and Aim: Since January 1, 2015, newborn screening program has been implemented in our country. The aim of this study is to report the clinical and genotype characteristics of patients who were diagnosed as CF after false negative CFNBS results since January 2015. Material and Methods: 1. The IRT value is greater than 90 ng/ml and 2. IRT value greater than 70 were considered suspicious and referred to a sweat test. The sweat test value above 60 mmol/L was considered positive. Results: 63 patients were diagnosed as CF in our clinic between 2015-2018. While 84.2% (n = 53) of the patients were diagnosed by positive CFNBS result, 15.8% of the patients (n = 10) had false negative results of IRT values. Of the 10 patients included in the study, 50% were girls, with a mean age of 30.7 ± 8.8 months (12-39 months); The mean age of diagnosis was 152 ± 147 days (28-480 days). Genetic mutation was detected in all patients. The first IRT level was above 90 µg/l in 40% (n = 4) of the patients. 40% of the patients had pancreatic insufficiency and Pseudobartter was detected in 70% of patients. Pseudomonas aeroginosa growth in sputum culture was detected in 60% of patients whereas MRSA growth was 10%. 30% of the patients had anemia. Conclusion: Regardless of the results of the newborn screening tests, patients with symptoms supporting CF should be further evaluated. For the patients with intermediate sweat test results, genetic analysis should be performed for CF. As seen in our study, it should be kept in mind that IRT negativity may also occur in mutations that cause severe CF.