Yakup Canıtez

Background. Respiratory colonization with Pseudomonas aeruginosa is associated with increased morbidity and mortality in cystic fibrosis (CF) patients. This study aims to assess the clinical characteristics and associated factors of CF infants under two years of age with P. aeruginosa colonization in Türkiye. Method. Of the 1637 patients registered in the Cystic Fibrosis Registry of Türkiye in 2019, 284 patients under two years of age were included in this retrospective cross-sectional study. Patients were classified into two groups: those with P. aeruginosa colonization (Group 1) and those without (Group 2). Cystic fibrosis transmembrane conductance regulator (CFTR) gene functions were categorized according to CFTR mutation functional class. Results. Twenty-three patients (8.1%) were categorized as Group 1 and 262 participants (91.9%) were classified as Group 2. Infants with P. aeruginosa colonization (Group 1) were more likely to have minimal CFTR function compared with those without colonization (87% vs. 39.8%, p = 0.017). In addition, both Staphylococcus aureus colonization (47.8% vs. 7.3%, p < 0.001) and methicillin-resistant S. aureus positivity (17.4% vs. 6.1%, p = 0.042) were observed more commonly in Group 1. There were no statistical differences between the groups in terms of age at diagnosis, gender, mean z-scores of weight and height, newborn screening test positivity, sweat chloride test results, and pancreatic insufficiency (p > 0.05). Univariate logistic regression analysis did not identify significant associated factors for P. aeruginosa colonization. Conclusions. Our findings suggest that minimal CFTR function and S. aureus colonization are associated with P. aeruginosa colonization in CF patients under two years of age. Further studies are needed to investigate associated factors for early P. aeruginosa colonization, eradication treatment effectiveness, and longitudinal outcomes of in CF patients under two years of age.

Objective: Asthma is the most common chronic disease of childhood. The goal of asthma treatment is to achieve complete control of the damage. This study aimed to investigate the control status of children diagnosed with asthma aged 4-18 years, using asthma control tests, and the factors affecting this control.Methods: 500 asthmatic children aged 4-18 years were included in the study. Patients were assessed using the C-ACT in the 4-11 age group and the ACT in the 12-18 age group. Patients with asthma aged 4-11 years were classified as under-controlled based on the C-ACT results between 20 and 27 points, and as uncontrolled if they scored 19 or below. Patients with asthma aged 12-18 years were classified as well-controlled based on the ACT results between 20 and 25 points and poorly controlled if they scored 19 or below. To compare the vitamin D levels of the patients, the vitamin D levels of 100 children aged 4-18 years without any chronic disease were recorded retrospectively. Results: Of the cases, 55.6% (n=278) were male and 44.4% (n=222) were female. When evaluated in general, the median age at diagnosis was 7.2 years (minimum=1, maximum=16). According to the results of C-ACT, 51.7% (n=151) of the cases with asthma in the 4-11 age group were under control, and according to the results of ACT, 58.7% (n=122) of the cases between the ages of 12-18 were well controlled. Number of emergency visits; median=2 in the 4-11 age group (minimum=1, maximum=30), median=2 in the 12-18 age group (minimum=1, maximum=20) (p>0.05). Use of antibiotics at least once in the past year was significantly higher in the 4-11 age group (n=259, 88.70%) than in the 12-18 age group (n=156, 75%) (p<0.001). Asthma-related school absences (median=3, min=0, max=30) were significantly higher in the 12-18 age group than in the 4-11 age group (median, min=0, max=40) (p = 0.007). There was no significant difference in serum 25-OH-vitamin D levels between asthma and controls in either age group.Conclusion: In our study, a significant portion of the cases were uncontrolled despite regular follow-up and treatment. The low rate of influenza vaccination in asthmatic children and the higher-than-expected rate of smoking at home suggest a lack of education on this topic. No significant difference was found between asthmatic patients’ and controls’ vitamin D levels.

Objectives: Cystic fibrosis-related diabetes (CFRD) is the most common comorbidity in patients with cystic fibrosis (CF). This study aimed to determine the prevalence and risk factors of CFRD using data from the CF Registry of Türkiye (CFRT). Methods: All patients with CF who underwent diabetes screening in 2022 or earlier were included in the study. The clinical and demographic characteristics of patients with CFRD, pre-diabetes (impaired glucose tolerance, impaired fasting glucose, indeterminate glucose tolerance), and those with normal screening results (NSR) were compared. Results: Of the 1,702 patients registered in the data system in 2022, 916 patients from 21 centers who underwent diabetes screening were included in the study. CFRD was diagnosed in 102 patients (11.2 %), there were 56 patients (6.1 %) with pre-diabetes, and NSR were found in 757 patients (82.7 %). The youngest patient diagnosed with diabetes was 6 years old. In patients with CFRD, the median age of patients was found to be higher (pPseudomonas aeruginosa (P. aeruginosa) colonization (p<0.001), severe mutations (60.8 %, p<0.001), and pancreatic insufficiency (56.1 %, p<0.001) were more frequent; median FEV1% and FVC% values were significantly lower (p<0.001 and p<0.002, respectively) compared to non-diabetic patients. Conclusions: CFRD is associated with impaired pulmonary function, poor nutritional status, and increased chronic bacterial colonization of the airways. Advanced age, decreased respiratory function, severe genotype, and pancreatic insufficiency are risk factors for CFRD.

Obstructive Sleep Apnoea Syndrome (OSAS) is increasingly recognised in the paediatric population. It is important to consider that certain pulmonary and endocrinological disorders may be influenced by OSAS or exhibit a more severe clinical course due to the presence of OSAS. This study aims to highlight the application of polysomnography in the paediatric population, particularly in the diagnosis and treatment of sleep disorders associated with chronic comorbidities—most notably endocrinological and respiratory conditions—and to emphasise the importance of improving quality of life measures to support healthy development in affected children. Polysomnography (PSG) recordings obtained between January 2020 and April 2025 at the Sleep Disorders Unit of the Department of Neurology, Faculty of Medicine, Bursa Uludag University, were retrospectively reviewed. Among these PSG records, patients referred from the Departments of Paediatric Endocrinology and Metabolism, Paediatric Pulmonology, and Paediatric Immunology and Allergy were identified. Demographic data, medical history, and PSG findings of the included patients were analysed. The results of polysomnography (PSG) performed in our center showed that the incidence of obstructive sleep apnea syndrome (OSAS) in children with endocrinologic or respiratory diseases was higher (25.7% vs. 3%) and the mean age was older (7 years vs. 4 years) compared to the normal pediatric population. The prevalence of sleep disorders was similar in these patients and normal children (54.4% vs. >50%), as was the prevalence of central apnea (2.9%). There were no gender differences among patients with sleep disorders.

Investigation of Cat and Dog Allergen Sensitivities in Childhood Asthma - The Journal of Current Pediatrics

Food allergies are more prevalent in childhood and can result in life-threatening reactions. Although prevalence rates vary among populations, studies have shown that the global prevalence of food allergies ranges from 1% to 10%. The most common food allergens include milk, eggs, wheat, peanuts, tree nuts, fish, and shellfish. This study aimed to evaluate the allergen profile of children diagnosed with IgE-mediated food allergies who were admitted to the Pediatric Allergy Outpatient Clinic at Bursa Uludağ University Faculty of Medicine Hospital between June 2015 and June 2020. Among the patients, 63.6% were male, and the mean age at the time of presentation was 3.06 years. The majority (80.4%) presented with dermatological symptoms, and 10.2% experienced food-induced anaphylaxis. Atopy-related conditions were observed in 39.8% of the patients, with atopic dermatitis being the most common coexisting allergic disease frequently associated with IgE-mediated food allergies. Skin prick tests and serum-specific IgE analyses revealed that eggs and milk were the most prevalent allergens, respectively. Other commonly identified allergens included hazelnuts, peanuts, and walnuts. Management strategies involved a combination of dietary elimination and pharmacological treatment tailored to the individual patient's condition. This study underscores the importance of early identification of food allergies and the implementation of appropriate treatment strategies. Accurate diagnosis, avoidance of allergenic foods, and timely therapeutic interventions are essential for safeguarding patient health and preventing adverse allergic reactions.

The Cystic Fibrosis Registry of Türkiye (CFRT) was established by the Turkish Pediatric Respiratory Diseases and Cystic Fibrosis Society and has provided detailed information on demographic, clinical, genetic, and treatment-related aspects of cystic fibrosis (CF) patients since 2017. We aimed to describe the current status of CF in Türkiye using CFRT's 2023 annual data. Demographic, clinical, and treatment data were taken from CFRT's 2023 record. In 2023, 2,258 patients from 34 centers were recorded. The median age of patients was 9.1 years, and 46.9% were female, with a median age at diagnosis of 0.3 years. Only 14.9% of the patients were older than 18 years. Genetic analyses were completed in 97.3% of patients. The most common variant, F508del, had a total variant frequency of 22.1%. The median percent predicted FEV1 and FVC were 88.0 and 94.0 in those aged 6-17 years 71.0 and 84.0 in those aged ≥18 years, respectively. The median values of body mass index z-scores were -0.5, and -0.5 for patients 2-18 and older than 18 years, respectively. Chronic colonization with Pseudomonas aeruginosa was present in 17.2% of the patients. Most patients used inhaled recombinant human DNase (87.1%) and oral pancreatic enzyme replacement treatment (83.0%). CF transmembrane conductance regulator (CFTR) modulators were used by 15.9% of patients. Over the year, 24 patients died, with a median age at death of 13.3 years. The CFRT report provides a valuable resource showing the clinical and laboratory data of patients with CF in the country.

Background. Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) variants are essential for determining eligibility for CFTR modulator drugs (CFTRms). In contrast to Europe and the USA, the treatment eligibility profile of cystic fibrosis (CF) patients in Türkiye is not known. In this study we aimed to determine the eligibility of CF patients in Türkiye for the CFTRms. Methods. The Cystic Fibrosis Registry of Türkiye (CFrT) data was used to determine the age of patients in the year 2021 and the genetic variants they were carrying. Age- and CFTR-variant appropriate modulator therapies were determined using the Vertex® algorithm. Results. Among a total of 1930 registered patients, CTFR gene analysis was performed on a total of 1841 (95.4%) patients. Mutations were detected in one allele in 10.7% (198 patients), and in both alleles in 79% (1455 patients) of patients. A total of 855 patients (51.7% for whom at least 1 mutation was detected) were eligible for the drugs. The most appropriate drug among genotyped patients was found to be elexacaftor/tezacaftor/ivacaftor for 486 patients (26.4%), followed by ivacaftor for 327 patients (17.7%) and lumacaftor/ivacaftor for 42 patients (2%). Conclusions. Only half of patients registered in CFrT were eligible for CFTRms, which is a significant difference from the CFTR variant profile seen in USA and Europe. However, access to treatment is hampered for some patients whose genes are not analysed. Further studies in CF populations, where rare mutations are relatively more common, will contribute to the field of CFTR modulator treatments for such rare mutations.