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Since the outbreak of the Syrian civil war in 2011, the population of Arab refugees in Turkey has rapidly increased. While cystic fibrosis (CF) is believed to be rare among Arabs, recent studies suggest it is underdiagnosed. This study aims to present the demographic, clinical, and genetic characteristics of CF patients among Arab refugees in Turkey. Additionally, a comparison is made between the findings in the National CF Registry 2021 in Turkey (NCFRT) and the refugee CF patient group. The study included refugee patients between the ages of 0 and 18 years who were diagnosed with CF and received ongoing care at pediatric pulmonology centers from March 2011 to March 2021. The study examined demographic information, age at diagnosis, age of diagnosis of patients through CF newborn screening (NBS), presenting symptoms, CF transmembrane conductance regulator (CFTR) mutation test results, sputum culture results, weight, height, and body mass index (BMI) z score. Their results were compared with the NCFRT results. The study included 14 pediatric pulmonology centers and 87 patients, consisting of 46 (52.9%) boys and 41 (47.1%) girls. All of the patients were Arab refugees, with 80 (92%) being Syrian. All the patients were diagnosed in Turkey. The median age at diagnosis of patients was 22.33 (interquartile range, 1-258) months. The median age of diagnosis of patients through NBS was 4.2 (interquartile range, 1-12) months. The median age of older patients, who were unable to be included in the NBS program, was 32.3 (interquartile range, 3-258) months. Parental consanguinity was observed in 52 (59.7%) patients. The mutation that was most frequently found was F508del, which accounted for 22.2% of the cases. It was present in 20 patients, constituting 32 out of the total 144 alleles. There was a large number of genetic variations. CFTR genotyping could not be conducted for 12 patients. These patients had high sweat tests, and their genetic mutations could not be determined due to a lack of data. Compared to NCFRT, refugee patients were diagnosed later, and long-term follow-up of refugee CF patients had significantly worse nutritional status and pseudomonas colonization.  Conclusion: Although refugee CF patients have equal access to NBS programs and CF medications as well as Turkish patients, the median age at diagnosis of patients, the median age of diagnosis of patients through NBS, their nutritional status, and Pseudomonas colonization were significantly worse than Turkish patients, which may be related to the difficulties of living in another country and poor living conditions. The high genetic heterogeneity and rare mutations detected in the refugee patient group compared to Turkish patients. Well-programmed NBS programs, thorough genetic studies, and the enhancement of living conditions for refugee patients in the countries they relocate to can have several advantages such as early detection and improved prognosis. What is Known: • Children who have chronic diseases are the group that is most affected by wars. • The outcome gets better with early diagnosis and treatment in patients with Cystic Fibrosis (CF). What is New: • Through the implementation of a newborn screening program, which has never been done in Syria previously, refugee patients, the majority of whom are Syrians were diagnosed with cystic fibrosis within a duration of 4 months. • Despite equal access to the newborn screening program and CF medications for both Turkish patients and refugee patients, the challenges of living in a foreign country have an impact on refugees., (© 2024. The Author(s).)

Introduction: Airway clearance techniques, which include positive expiratory pressure (PEP) devices, are essential in the pulmonary rehabilitation of cystic fibrosis (CF). Bottle-PEP is a low-cost but an effective alternative., Objective: The aim of this study is to document the sustainability and safety of Bottle-PEP therapy as a home rehabilitation aid., Methods: The study has been designed as a prospective case series. Patients with CF at the age of 6-18 years in acute exacerbation period were included in the study. Bottle-PEP training was given by a competent physiotherapist to those patients who did not use any method, and those who currently use another device were followed up with their existing devices. Thus, patients divided into two groups were followed up for 1 year. The patients were evaluated by phone every 2 weeks for exacerbation, regular and proper use of the device, and satisfaction during their follow-up. The patients were evaluated every 3 months with pulmonary function tests, 6-minute walking test (6MWT) and quality of life., Results: Thirty-four patients were included in the study. The acute exacerbation score of the patients was 4.5 in the Bottle-PEP group and 6 in the other group, showing no significant difference ( p = .1). Treatment compliance scores were compared, the median value of the Bottle-PEP group was 24 the other group was 27 and there was no significant difference ( p = .6). During follow-up of, there were no significant differences in FEV1, 6MWT and quality of life data ( p > .05)., Conclusion: Bottle-PEP treatment is not different from other devices in terms of long-term usability and safety in patients diagnosed with cystic fibrosis.

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., (© 2021 Wiley Periodicals LLC.)

Introduction: Caregiver burden impacts both the social and economic framework of society. Cystic fibrosis (CF) causes significant caregiver burden, but the current data is scarce. In the case of primary ciliary dyskinesia (PCD), even less is known. This study aims to compare the caregiver burden of the parents of patients with CF and PCD., Methods: Patients with CF and PCD between the ages of 6 to 13 and their parents were included. Patients' clinical information and parents' demographics were recorded. Caregiver burden was measured with Zarit Caregiver Burden Scale (ZCB), while the quality of life (QOL) was measured with CFQOL-revised (CFQOL-R) and PCD QOL questionnaire as the patients' age and diagnosis indicated., Results: A total of 63 patients, 44 with CF (69%) and 85 caregivers (35 mothers, 6 fathers, and 22 mother-father dyads) participated in the study. Caregiver burden was significantly higher in mothers of the CF group with a mean ZCB of 30.5 ± 10.7 when compared to the PCD group with a mean ZCB of 21.93 ± 8.26 (P = .006). This was similar in fathers with mean ZCB of 27.5 ± 9.21 in the CF group and 20.36 ± 7.43 in the PCD group (P = .03). In correlation analyses, mothers' caregiver burden moderately and inversely correlated with CFQOL-R subscales in the CF population., Conclusion: Caregiver burden is significantly higher in the CF population when compared to PCD. It is correlated with pulmonary functions and QOL in patients with CF., (© 2019 Wiley Periodicals, Inc.)

Objective: Cystic fibrosis (CF) patients may develop hypoxemia during sleep. Limited information is available on nocturnal oxygen saturation in CF children with less severe lung disease. The aim of this study was to investigate the degree of nocturnal oxygen desaturation and factors that correlate with nocturnal oxygenation in CF children with normal pulmonary function tests (PFTs) or mild to moderate lung disease., Method: Awake resting and post-exercise SpO2 were measured by pulse oximetry. Each patient had overnight oximetry monitorization at home. Six minutes walk test (6MWT), Shwachman-Kulczycki (S-K), Brasfield and computed tomography (CT) scores, blood gas analysis and nutritional status of patients were evaluated., Results: Twenty-four patients with a median age of 9.5 years were included. Nocturnal mean SpO2 did not differ according to the severity of lung disease based on PFT. However, lowest SpO2 obtained was lower in children with both mild and moderate lung disease compared to normals (87.4% vs. 91.7%, respectively, p = 0.009). 95.8% of CF children with normal PFT or mild to moderate lung disease had desaturation events during sleep. Nocturnal mean SpO2 correlated with S-K (Spearman's rho = 0.64, p < 0.0001), Brasfield (Spearman's rho = 0.31, p = 0.007) and CT scores (Spearman's rho = -0.67, p < 0.0001) as well as PaO2 (Spearman's rho = 0.28, p = 0.021), SaO2 (Spearman's rho = 0.28, p = 0.023), z-score of weight (Spearman's rho = 0.23, p = 0.20) and height (Spearman's rho = 0.20, p = 0.30), there was no correlation with 6MWT., Conclusions: In CF children with normal PFT or mild-to-moderate lung disease, nocturnal oxygenation may correlate with S-K, Brasfield and CT scores as well as PaO2, SaO2, z-score of weight and height., (2007 Wiley-Liss, Inc.)