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There exists insufficient information about the natural course of incidental pulmonary nodules (IPN) determined on tomography in children. The aim was to determine the characteristic features and factors affecting the course of IPN.This retrospective study included patients who presented at the Pediatric Pulmonology, AllergyImmunology Section of Akdeniz University Hospital between January 2014-2020, and were determined with pulmonary nodules on high-resolution computed tomography (HRCT). The patients were separated into two groups as those with a nodule decreased in size or which had disappeared on the follow-up HRCT (Group 1) and those with a nodule which had remained at the same size (Group 2). These two groups were compared in respect to demographic data, nodule size and characteristics, and accompanying findings on HRCT.A total of 177 nodules were determined in the 66 patients included in the study. A follow-up HRCT was taken within mean 16.29±11.38 months in 27 patients. In these patients, 78 nodules were determined on the initial HRCT. On the follow-up, twelve of the nodules were seen to have shrunk or disappeared compared to the initial images, 66 had remained the same size, and none had grown. The mean age of the patients in Group 1 was statistically significantly lower than that of patients in Group 2 (p0.001). The rates of an accompanying mosaic attenuation pattern (p0.001) on HRCT and subsolid density (p=0.011) of the nodules in Group 1 were statistically significantly higher compared to Group 2 and the rate of calcification content was statistically significantly lower (p=0.002). No suspicious or confirmed malignancy was observed in any case throughout the mean follow-up period of 38.33±16.5 months after the initial HRCT.The young age of patients, subsolid structure of nodules, calcification content and the presence of an accompanying mosaic attenuation pattern on HRCT, could be useful factors in the estimation of size in the follow-up of nodules.

Background: There are a limited number of studies investigating the relationship of sighing dyspnea (SD) with psychiatric disorders and various mental states. Methods: This cross-sectional study enrolled 39 pediatric patients with SD, but free from apparent cardiopulmonary diseases, who presented to a pediatric pulmonology policlinic between June and December 2019 and age-gender matched 34 healthy children were recruited for comparison. Patient characteristics, psychological or physical trauma history, and cigarette smoke exposure were recorded. All the participants completed the “NOSE scale,” “Modified Borg Scale,” “Children Depression Inventory,” “Screen for Child Anxiety Related Disorders,” “Pediatric Quality of Life Inventory,” “Stress Coping Style Scale,” “Family Assessment Device,” and “Parents Attitude Scale.” Results: Compared to the control group, there was a statistically significantly higher rate in the SD group of physical or psychological trauma history (35.9% vs. 14.7%, p = .04) and passive cigarette smoking (60.5% vs.27.3%, p = .005). When the groups were compared in respect of depression and anxiety levels, the depression score, total anxiety level, somatic/panic, generalized anxiety and fear of school points were determined to be statistically significantly higher in the SD group. The QoL scores (total, physical, and psychosocial) in the SD group were found to be statistically significantly lower than those of the control group. Conclusion: To the best of our knowledge, this is the first study to have examined the psychological profiles of SD patients and their families in detail, and to have evaluated the effect of SD on quality of life.

OBJECTIVE: To compare class I/II cystic fibrosis transmembrane conductance regulator (CFTR) mutations to class III-V mutations with regards to cystic fibrosis disease severity markers in children. MATERIAL AND METHODS: This study was designed as a cross-sectional study in Antalya province, located on the south coast of Turkey. The study included 38 cystic fibrosis patients aged between 0.6 and 18 years. The CFTR genotype of the patients was categorized into 2 groups based on the presence or absence of class I or class II mutations in any of the alleles. Group I comprised 8 homozygous, 8 with unknown alleles, and 8 compound heterozygous patients, and group II comprised 11 homozygous and 3 compound heterozygous patients. The groups were analyzed in respect of cystic fibrosis disease severity markers, such as spirometry, ShwachmanKulczycki score, body mass index (BMI), sweat chloride concentration, chronic Pseudomonas aeruginosa infection, annual exacerbation frequency, and severe exacerbations requiring hospitalization during the previous year. RESULTS: In the comparison of group I and group II patients, a significant difference was observed in pancreas insufficiency (83.3% vs. 35.7%; P = .005), chronic P. aeruginosa infection (58.3% vs. 7.1%; P = .002), cough severity score (1.7 ± 1.1 vs. 0.9 ± 1.5; P = .029), number of severe exacerbations requiring hospitalization during the previous year (0.9 ± 1 vs. 0.3 ± 0.8; P = .03), and sweat chloride levels (76.7 ± 15.2 vs. 61 ± 22.3; P = .02). All these values were higher in group I patients. The mean BMI values (15.8 ± 2.2 vs. 17.6 ± 2.8; P = .03) were lower in group I patients. CONCLUSION: There seems to be a difference between class I/II CFTR mutations and class III-V mutations on the severity of the disease in cystic fibrosis patients.

Background: Cystic Fibrosis Registry of Turkey shows various CFTR mutations due to the geographical location and historical background of our country, and also high prevalence of consanguineous marriages. Method:All mutations detected in the Cystic Fibrosis Registry of Turkey 2017 (CFRT2017) data were screened in CFTR1 and CFTR2 databases. Mutations which were not found in both were identified and characteristics of these patients were compared with F508del homozygous patients. Results: Among 1170 registered patients, 978 were genotyped and 200 different mutations were shown in 1270 alleles.29 mutations were not reported in both databases; 58 mutations have been reported in CFTR1 but not in CFTR2. Demographic and phenotypic characteristics of the 112 patients with 87 different alleles those were not previously reported in the CFTR2 database (nonCFTR2 group) were compared with F508del homozygous 103 patients in CFRT2017. In the nonCFTR2 group, mean age was younger (5.81 vs 7.69; p:0.015), mean age at diagnosis was older (1.88 vs 1.08; p:0.041), sweat test was lower (75.12 vs 98.28; p Conclusion: We suggest that patients in the nonCFTR2 group have a mild clinical course, but in some patients, further investigations and functional studies are required for the exact diagnosis.

Sismanlar Eyuboglu, Tugba/0000-0001-7284-4999; Dogru, Deniz/0000-0001-9931-9473; Aslan, Ayse Tana/0000-0002-5360-8517; COBANOGLU, NAZAN/0000-0002-3686-2927; ORHAN, FAZIL/0000-0002-4850-932X WOS: 000530442700001 PubMed: 32364312 Background Pseudo-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. Methods The 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. Results Totally 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. Conclusion PBS 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.

OBJECTIVES The aim of this study was to evaluate radiological, clinical, and demographic data of patients with noncystic fibrosis bronchiectasis and to compare high-resolution computed tomography (HRCT) scores based on the demographic and clinical characteristics. MATERIALS AND METHODS A total of 34 patients (18 male, 16 female) were assessed in terms of age at symptom onset, age at diagnosis, annual attack frequency, cough severity score, physical examination findings, and pulmonary function test results. Modified Bhalla scoring system (B total) and anatomical prevalence degree score (D total) were used for HRCT examination. RESULTS There was a strong negative correlation between forced expiratory volume at first second (FEV1) and bronchial dilatation degree (SBRDIL). There was a moderate negative correlation of FEV1, forced vital capacity (FVC), and maximum mid-expiratory flow rate (MEF; 25-75) with bronchiectasis degree (EXBRNC), bronchial wall thickness degree (SBWTHICK), and mucus accumulation in the major airways (PMPLA). The B total, D total, EXBRNC, and SBRDIL scores were significantly higher in patients with hemoptysis and sputum. In comparing B and D total scoring systems, B total provided better results in terms of rale, annual exacerbation frequency (AEF), cough severity score (CSS), and FEV1 values. CONCLUSION As it is proved using HRCT, pulmonary function impairment, sputum production, hemoptysis, and increase in AEF strongly correlating with objective HRCT scoring can be accepted as markers for pathological changes due to bronchiectasis.

Harmanci, Koray/0000-0002-8494-648X; Sismanlar Eyuboglu, Tugba/0000-0001-7284-4999; Aslan, Ayse Tana/0000-0002-5360-8517 WOS: 000535323700001 PubMed: 32453906 Background A better understanding of cystic fibrosis transmembrane conductance regulator biology has led to the development of modulator drugs such as ivacaftor, lumacaftor-ivacaftor, tezacaftor-ivacaftor, and elexacaftor-tezacaftor-ivacaftor. This cross-sectional study evaluated cystic fibrosis (CF) patients eligible for modulator drugs. Methods Data for age and genetic mutations from the Cystic Fibrosis Registry of Turkey collected in 2018 were used to find out the number of patients who are eligible for modulator therapy. Results Of registered 1488 CF patients, genetic analysis was done for 1351. The numbers and percentages of patients and names of the drugs, that the patients are eligible for, are as follows: 122 (9.03%) for ivacaftor, 156 (11.54%) for lumacaftor-ivacaftor, 163 (11.23%) for tezacaftor-ivacaftor, and 57 (4.21%) for elexacaftor-tezacaftor-ivacaftor. Among 1351 genotyped patients total of 313 (23.16%) patients are eligible for currently licensed modulator therapies (55 patients were shared by ivacaftor and tezacaftor-ivacaftor, 108 patients were shared by lumacaftor-ivacaftor and tezacaftor-ivacaftor, and 22 patients were shared by tezacaftor-ivacaftor and elexacaftor-tezacaftor-ivacaftor groups). Conclusions The present study shows that approximately one-fourth of the registered CF patients in Turkey are eligible for modulator drugs. As, frequent mutations that CF patients have in Turkey are different from North American and European CF patients, developing modulator drugs effective for those mutations is necessary. Furthermore, as modulator drugs are very expensive currently, financial support of the government in developing countries like Turkey is noteworthy.

Better understanding of CFTR biology has led to the development of modulator drugs. Lumacaftor/Ivacaftor (L/T) is approved for people ages ≥2 who have two copies of the F508del mutation. Tezacaftor/Ivacaftor (T/I) is approved for people ages ≥12 who have two copies of the F508del mutation and also approved for people who have a single copy of one of 26 specific mutations. Ivacaftor (I) is approved for people ages ≥1 who have a single copy of one of the mutations approved for T/I and additionally for 12 specific mutations. We aimed to find out the number of patients recorded in Cystic Fibrosis Registry of Turkey (CFRT) in 2018 who are eligible for modulator therapy. Of 1170 patients, 128 are homozygote F508del (22 mths-36 yrs), and 123 (10.51%) are aged ≥2 yrs and eligible for L/T. Among 128 patients with homozygote F508del, 48 are aged ≥12 yrs and among 42 patients (1-31 yrs) who have one or two copies of 3849+10kbC→T, A455E, D110H, D579G, F1052V, R74W, R347H, S945L, 13 are aged ≥12 yrs, and totally 61 (5.21%) patients are eligible for T/I. Finally, total 68 (5.81%) patients (1-31 yrs) have one or two copies of D110H, G178R, G1069R, G1349D, R117H and S549R mutations in addition to the mutations stated in T/I group and all of them are older than one year old and eligible for I therapy. According to the data, approximately one fifth of CF patients are eligible for modulator drug therapy. Compared to North European and North American CF patients, less Turkish patients are eligible for modulator therapies, so new treatment modalities are necessary for them.

Can, Demet (Balikesir Author), 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.

Background/ aim: Since January 2015, the Cystic Fibrosis Newborn Screening (CFNS) program has been implemented in Turkey. We aimed to evaluate the demographic, clinical, and laboratory data of cases referred from the CFNS program and to determine the most suitable cut-off value for immunoreactive trypsinogen (IRT)-1 and immunoreactive trypsinogen (IRT-2) that are used in the CFNS program in Turkey. Materials and methods: A total of 156 Turkish Caucasian subjects were determined as positive cases during 3 years, from January 2015 to January 2018, and were referred to the pediatric pulmonology clinics of Akdeniz University Hospital, Antalya, Turkey, for the national CFNS program. The evaluation was made considering the IRT-1 and IRT-2 values, demographic characteristics, sweat test results, CFTRgenotypes, and diagnoses. Results: Nine patients were diagnosed with cystic fibrosis (CF). Eight were diagnosed with CF-related metabolic syndromes and three were determined to be CF carriers. The ratio of CF to CF-related metabolic syndrome was determined as 1.1:1. Considering the limits of the present CFNS program and the IRT method, the positive predictive value (PPV) for the referred cases was determined as 5.8%. When a cut-off value of 105.6 ng/mL was taken for IRT-1, sensitivity was 100%, specificity was 59%, and PPV was 12.8%. For a cut-off value of 88.75 ng/mL for IRT-2, sensitivity was determined as 90%, specificity as 65%, and PPV as 15.2%.Conclusion: This is the first detailed clinical study to evaluate the data from the CFNS program along the Mediterranean coast of Turkey. As false positive results are extremely high in Turkey, there is an urgent need for revision of the IRT-1 and IRT-2 limits by evaluating the data of the whole country.

Objective: It is important to detect allergens that cause sensitization to decrease the morbidity of allergic diseases and to prevent or to delay the development of the disease in already sensitized patients. In this study, we aimed to evaluate which aeroallergens cause sensitization in allergic patients in the Akdeniz Region, Turkey, as well as their frequency of occurrence.Materials and Methods: The demographic properties, diagnoses, total IgE levels, sensitization against aeroallergens detected via EPT (Epidermal prick test), the dates of EPT application and peripheral eosinophil counts at the time of EPT application were analyzed in 510 patients aged 2-18 years who presented to the Akdeniz University Pediatric Allergy – Immunology and Pulmonology Departments and Antalya Education and Research Hospital Pediatric Allergy - Immunology Department with an allergic history or diagnosis between January 2014 and July 2015. All patients underwent EPT during their follow-up and sensitivity against at least one aeroallergen was detected. The seasonal distribution of these aeroallergens was also analyzed.Results: A total of 1180 children to which an allergic skin test had been applied between January 2014 and July 2015 were included in this study. It was seen that 510 patients (43.2%) had a positive response to at least one aeroallergen and 402 patients among them (78.8%) had a positive response to more than one aeroallergen. Males made up 58.4% and females 41.6% of the patients who showed a positive EPT. Sensitivity was most frequently (69%) against house dust mites, followed by tree pollen mix (54.9%) and grass/weed mix (52.5%). Other allergens were animal dander (45.3%), fungi mix (43.4%), herb/pollen mix (34.9%), cockroach (16.9%) and olea (6%). Further, by analyzing the seasonal distributions of allergens, it was seen that sensitivity was most frequently against house dust in all seasons.Conclusion: Avoiding specific allergens is important in eliminating and managing the symptoms in allergic patients. Determining the regional differences regarding allergen sensitivity is therefore critical.

Basaran AE, Karatas-Torun N, Maslak IC, Bingol A, Alper OM. Normal sweat chloride test does not rule out cystic fibrosis. Turk J Pediatr 2017; 59: 68-70. A 5-month-old patient presented with complaints of fever and cough. He was hospitalized with the diagnosis of bronchopneumonia and pseudo-Bartter's syndrome. Patient was further investigated for diagnosis of cystic fibrosis. The chloride (Cl) level in sweat was determined within the normal range (25.1 mmol/L, 20.3 mmol/L). CFTR (Cystic Fibrosis Transmembrane Regulator gene; NM_000492.2) genotyping results were positive for p.E92K; p.F1052V mutations. The patient was diagnosed with cystic fibrosis. In our patient, with features of CF and normal sweat test, mutation analysis was helpful for the diagnosis of cystic fibrosis.