Browsing by Author "Can D."
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Item Comparative morphological differences between umbilical cords from chronic hypertensive and preeclamptic pregnancies(2002) Inan S.; Sanci M.; Can D.; Vatansever S.; Oztekin O.; Tinar S.To compare morphological changes in the umbilical cords from chronic hypertensive and preeclamptic patients having normal or pathological umbilical artery Doppler ultrasonographic results. Umbilical cords from 34 normotensive, 31 chronic hypertensive and 70 preeclamptic women with normal and abnormal Doppler flow velocity waveforms (FVW) at 35 40 gestational weeks were studied. Morphological changes in the umbilical cords were examined on formalin-fixed, paraffin-embedded sections. The total umbilical cord area, total vessel area, and wall thickness of umbilical vessels were measured in systematic random samples using unbiased stereology methods. An ANOVA test was used for statistical analysis. In the chronic hypertensive and preeclamptic groups with normal Doppler FVW, the thickness of the umbilical cord vessels remained nearly constant, whereas both the total area and the lumen area were reduced. These changes correlate with the histopathological findings, suggesting a mainly vasoconstrictive effect. By contrast, analysis of the preeclamptic group with pathologic Doppler FVW showed a comparable reduction of all parameters of the umbilical cord. Histopathological findings were related to smaller, contracted smooth muscle cells of the vessel wall, which is suggestive of a predominant hypoplastic mechanism. As a result of reduced uteroplacental perfusion, fetal hypoxia and intrauterine growth retardation become un-avoidable in preeclampsia. The histopathological changes in the umbilical cord between the chronic hypertensive and preeclamptic patients depend on the Doppler results. In conclusion, the umbilical artery Doppler FVW indices provide good values for predicting intrauterine growth retardation in preeclamptic patients.Item Successful desensitization of a case with desferrioxamine hypersensitivity(2006) Gülen F.; Demir E.; Tanaç R.; Aydinok Y.; Gulen H.; Yenigün A.; Can D.Thalassaemia major is a severe chronic hemolytic disease, resulted with iron overload mainly due to regular blood transfusions. Iron overload may lead to serious organ toxicity and even fatal complications, if no iron excretion is achieved by a chelating agent. First introduced in 1976 as s.c. treatment for thalassaemia major, desferrioxamine (DFO) has substantially improved the life expectancy in the disease. While DFO can cause local allergic reactions including redness, itching, pain and lumps, on rare occasion anaphylactic reactions can occur. The mechanism of anaphylaxis like reactions is not well understood. In this case report, we presented a 10 years-old girl with thalassaemia major who had to stop DFO therapy after appearing of systemic allergic reactions with hypotension, tachycardia, pruritus and urticaria against this drug. Serum IgE level was normal, specific IgE and skin prick tests were negative. Intradermal test was resulted with positive reaction to DFO. The patient was hospitalized and desensitization protocol was initiated with rapid s.c. infusions per 15 min. The protocol was stopped at the 17th cycle because of local reaction reappeared. After that, DFO was further diluted and was restarted with lower dosage and longer infusion period. Then, DFO dosage was increased and the dilutions and infusion times were decreased gradually. By this desensitization programme, the patient would continue to use DFO chelation safely for 10 months.Item Sleep quality and depression-anxiety in mothers of children with two chronic respiratory diseases: Asthma and cystic fibrosis(2008) Yilmaz O.; Sogut A.; Gulle S.; Can D.; Ertan P.; Yuksel H.Background: Sleep quality and psychological well being of parents are expected to be influenced by the child's health and disease status. The aim of this study was to compare sleep quality and depression-anxiety parameters in mothers of children with cystic fibrosis (CF) asthma and healthy controls. Methods: The study included mothers of 62 children with asthma, 21 children with CF and 35 healthy children. All mothers filled in the Pittsburgh Sleep Quality Index (PSQI) questionnaire and hospital anxiety depression scale (HADS). Results: Comparison of the three groups with Kruskall Wallis analysis demonstrated that subjective sleep, sleep efficiency and total PSQI scores were significantly different between the groups (p = 0.02, p = 0.01 and p = 0.04 respectively). Comparisons of the groups in pairs with Mann Whitney U test with Bonferroni correction revealed that subjective sleep quality scores in mothers of children with asthma were significantly higher than the ones in the control group (1.0 ± 0.9 vs 0.6 ± 0.7, p = 0.015). The other PSQI scores as well as the anxiety and depression scores were higher in CF and asthma groups when compared to the control group but did not reach statistical significance. Anxiety and depression scores were significantly correlated with PSQI total score in CF (rho = 0.54 and 0.49 respectively) and asthma groups (rho = 0.45 and 0.60 respectively) but not in the control group. Conclusion: In conclusion, presence of a chronic respiratory disease in a child may be associated with disturbed sleep quality and increased depression and anxiety in mothers. © 2008 European Cystic Fibrosis Society.Item Characteristics and prognosis of childhood atopic dermatitis: A multicenter study in Turkey(2010) Yuksel H.; Can D.; Reisli S.; Uzuner N.; Orhan F.; Cevit O.; Tahan F.; Canitez Y.; Kuyucu S.; Boz A.B.; Akcay A.; Yilmaz O.Background:Childhood atopic dermatitis (AD) is classically accepted as initial finding of atopic march; however, nonatopic cases do not follow this course. The aim of this study was to determine the characteristics and prognosis of AD in childhood in Turkey. Methods:The study included 531 children with AD that presented to pediatric allergy departments in 11 different regions of Turkey. Age at diagnosis, total serum and inhalant-specific immunoglobulin E (IgE) levels and allergen skin prick test results were recorded retrospectively. Clinical characteristics like additional allergic diseases at presentation or during follow-up were recorded as well as duration of follow-up. Results:Mean age at diagnosis was 37.8 ± 36.2 months. Mean IgE level was 318.3 ± 677.8 IU/ml (median 100 IU/ml). Skin prick tests yielded positive results in 47% of children. At presentation, 31.6% of children reported additional allergic disease, while 11.7% developed allergic disease during follow-up. Among all, 46.6% had additional allergic disease at any point. IgE levels were significantly higher in children with additional allergic diseases (p = 0.001). Allergen skin prick test positivity and family history of allergic diseases increased the risk of additional allergic diseases significantly (OR = 3.90, 95% CI = 2.3-6.6 and OR = 1.89, 95% CI = 1.3-2.8, respectively). Conclusions: Allergic sensitization is not present in all cases of AD. Coexistence of additional allergic diseases is not as high as expected but more common in children who have been demonstrated to have atopic sensitization with high IgE levels and allergen skin prick test positivity. Copyright © 2010 S. Karger AG.Item (Un)Awareness of allergy(BioMed Central Ltd, 2011) Kalpaklioglu A.F.; Kalkan I.K.; Akcay A.; Reisli I.; Can D.; Uzuner N.; Yuksel H.; Kirmaz C.; Gulen F.; Ediger D.Background: Allergy is associated with considerable morbidity. Objective: The aim of this multicenter study was to provide insight into allergy knowledge and perceptions among the population. Methods: During the World Allergy Day, several allergy clinics conducted public meetings to encourage the awareness of and education in allergy. At the beginning, participants filled out a questionnaire to assess their knowledge about what is allergy and to determine by whom those symptoms are cared. Results: A total of 256 participants (187 women/69 men, mean age, 31.2 ± 12.5 years) completed the survey. Of the 202 participants with symptoms, 58.9% had physician-diagnosed allergic disease. Among the 19 symptoms evaluated, 56.5% of the symptoms were recognized as related with allergy, and this increased in compliance with education level (r = +0.427; P < 0.001) but not with diagnosed allergy (P = 0.34). Sneezing was the most common symptom thought to be related with allergy-related symptom (77.5%), whereas loss of smell was the least one (37.9%). Participants were more likely to be cared by an allergologist (72.9%) followed by other specialties, when experiencing allergy. Conclusions: Increasing the awareness for allergic symptoms is the key not only for the diagnosis but also for the optimal treatment. Therefore, education is an important component of prevention and control of allergic diseases. Copyright © 2011 by World Allergy Organization.Item Anaphylaxis in Turkish children: A multi-centre, retrospective, case study(2011) Orhan F.; Canitez Y.; Bakirtas A.; Yilmaz O.; Boz A.B.; Can D.; Kuyucu S.; Harmanci K.; Tahan F.; Reisli I.; Karakas T.; Baki A.; Cokugras H.; Cakir M.; Yuksel H.Background: Anaphylaxis is a serious and potentially lethal systemic reaction affecting more than one organ or system. Objective: We aimed to describe the demographic characteristics, clinical features, causes, settings, and administered therapy in Turkish children. Methods: This retrospective, case note study included all children referred to the outpatient clinics of the Pediatric Allergy Departments of the participating study centres from 1 July 1999 to 30 June 2009 for investigation of anaphylaxis or who were seen by us at the moment of the reaction during the same period and who met the clinical criteria of anaphylaxis. Results: Two hundred and twenty-four cases of anaphylaxis were reported in 137 children (88 boys, P = 0.0001). The mean ± SD age at the referral was 7.7 ± 4.2 years (range: 4 months-17 years). Ninety-eight episodes (43.8%) occurred at home. The symptoms were cutaneous in 222 (99.1%) episodes, respiratory in 217 (96.9%), neuro-psychiatric in 118 (52.7%), cardiovascular in 92 (41.1%), and gastrointestinal in 88 (39.3%). Biphasic reaction was reported in seven episodes (3.1%, 95% CI: 1.5-6.3). Death occurred in one case (0.4%, 95% CI: 0.08-2.4). Treatment was available in 158 episodes (70.5%). Of them, 148 (93.7%) received antihistamines, 132 (83.5%) corticosteroids, 51 (32.3%) epinephrine, and 17 (10.8%) beta-2-mimetics. The causative agents were foods in 86 (38.4%) episodes, hymenoptera venom in 84 (37.5%), drugs and medications in 47 (21.0%), and latex in 5 (2.2%). In two episodes (0.9%), the causative agent was unidentified. Allergy to the trigger was known prior to anaphylaxis in 116 (51.8%) episodes. An epinephrine auto-injector had been prescribed for 70 children (51.1%). Conclusions and Clinical Relevance: Anaphylaxis was seen significantly more in boys. Most of the reactions occurred at home. Foods were the most frequent cause. Epinephrine, the first-line treatment of anaphylaxis, was administered in only a third of the children. © 2011 Blackwell Publishing Ltd.Item The reliability and validity of Turkish version of Childhood Asthma Control Test(2012) Sekerel B.E.; Soyer O.U.; Keskin O.; Uzuner N.; Yazicioglu M.; Kiliç M.; Artaç H.; Ozmen S.; Can D.; Zeyrek D.; Cokugras H.; Canitez Y.; Aydogan M.; Kuyucu S.; Inal A.; Gurkan F.; Orhan F.; Yilmaz O.; Boz A.B.; Tahan F.; Cevit O.Introduction: The reliability and validity of Turkish version of Childhood Asthma Control Test (C-ACT). Purpose: The management of asthma is an important as well as difficult issue of physician's daily practice particularly in busy clinical settings. C-ACT was created to identify asthma control levels in children aged 4-11 years. Our aim was to evaluate the reliability, validity and responsiveness of C-ACT in a Turkish sample of children with asthma. Method: In this multicenter study, 368 children were enrolled. C-ACT was completed every month by parents and patients who were evaluated in 3 visits within 2 month intervals. At each visit, physicians interpret the control level and decided for the treatment step as established in GINA guidelines. Results: The internal consistency reliability of the Turkish version of C-ACT (C-ACT1 to C-ACT5) was found to be 0.82, 0.83, 0.82, 0.82 and 0.80, respectively (reliability statistics, Cronbach's alpha). Test-retest reliability was 0.71. There was significant correlation between C-ACT and physician's assessment of asthma control at visit 1 (r = 0.65, P <0.001). Conclusions: Turkish version of C-ACT is an accurate and reliable tool to evaluate asthma control in children aged 4-11 years. Its widespread use may facilitate appropriate assessment of asthma control and may lead to decrease the number of uncontrolled patients. © Springer Science+Business Media B.V. 2011.Item Does atopy affect the course of viral pneumonia?(Elsevier Doyma, 2018) Erdem S.B.; Can D.; Girit S.; Çatal F.; Şen V.; Pekcan S.; Yüksel H.; Bingöl A.; Bostancı I.; Erge D.; Ersu R.Background: The presence of atopy is considered as a risk factor for severe respiratory symptoms in children. The objective of this study was to examine the effect of atopy on the course of disease in children hospitalised with viral pneumonia. Methods: Children between the ages of 1 and 6 years hospitalised due to viral pneumonia between the years of 2013 and 2016 were included to this multicentre study. Patients were classified into two groups as mild–moderate and severe according to the course of pneumonia. Presence of atopy was evaluated with skin prick tests. Groups were compared to evaluate the risk factors associated with severe viral pneumonia. Results: A total of 280 patients from nine centres were included in the study. Of these patients, 163 (58.2%) were male. Respiratory syncytial virus (29.7%), Influenza A (20.5%), rhinovirus (18.9%), adenovirus (10%), human metapneumovirus (8%), parainfluenza (5.2%), coronavirus (6%), and bocavirus (1.6%) were isolated from respiratory samples. Eighty-five (30.4%) children had severe pneumonia. Atopic sensitisation was found in 21.4% of the patients. Ever wheezing (RR: 1.6, 95% CI: 1.1–2.4), parental asthma (RR: 1.5, 95% CI: 1.1–2.2), other allergic diseases in the family (RR: 1.8, 95% CI: 1.2–2.9) and environmental tobacco smoke (RR: 1.6, 95% CI: 1.1–3.5) were more common in the severe pneumonia group. Conclusions: When patients with mild–moderate pneumonia were compared to patients with severe pneumonia, frequency of atopy was not different between the two groups. However, parental asthma, ever wheezing and environmental tobacco smoke exposure are risk factors for severe viral pneumonia in children. © 2017 SEICAPItem Patients eligible for modulator drugs: Data from cystic fibrosis registry of Turkey(John Wiley and Sons Inc., 2020) Çobanoğlu N.; Özçelik U.; Çakır E.; Şişmanlar Eyüboğlu T.; Pekcan S.; Cinel G.; Yalçın E.; Kiper N.; Emiralioğlu N.; Şen V.; Şen H.S.; Ercan Ö.; Çokuğraş H.; Kılınç A.A.; Al Shadfan L.M.; Yazan H.; Altıntaş D.U.; Karagöz D.; Demir E.; Kartal Öztürk G.; Bingöl A.; Başaran A.E.; Sapan N.; Çekiç Ş.; Çelebioğlu E.; Aslan A.T.; Gürsoy T.R.; Tuğcu G.; Özdemir A.; Harmancı K.; Yıldırım G.K.; Köse M.; Hangül M.; Tamay Z.; Süleyman A.; Yüksel H.; Yılmaz Ö.; Özcan G.; Topal E.; Can D.; Korkmaz Ekren P.; Çaltepe G.; Kılıç M.; Özdoğan Ş.; Doğru D.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. © 2020 Wiley Periodicals LLCItem Clinical features and accompanying findings of Pseudo-Bartter Syndrome in cystic fibrosis(John Wiley and Sons Inc., 2020) Sismanlar Eyuboglu T.; Dogru D.; Çakır E.; Cobanoglu N.; Pekcan S.; Cinel G.; Yalçın E.; Kiper N.; Sen V.; Selimoglu Sen H.; Ercan O.; Keskin O.; Bilgic Eltan S.; Alshadfan L.; Yazan H.; Altıntas D.U.; Sasihuseyinoglu A.S.; Sapan N.; Cekic S.; Cokugraş H.; Kılınc A.A.; Ramaslı Gursoy T.; Aslan A.T.; Bingol A.; Başaran A.E.; Ozdemir A.; Kose M.; Hangul M.; Emiralioglu N.; Tugcu G.; Yuksel H.; Yılmaz O.; Orhan F.; Gayretli Aydın Z.G.; Topal E.; Tamay Z.; Suleyman A.; Can D.; Bal C.M.; Caltepe G.; Ozcelik U.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. © 2020 Wiley Periodicals, Inc.Item Cystic fibrosis in Turkey: First data from the national registry(John Wiley and Sons Inc., 2020) Dogru D.; Çakır E.; Şişmanlar T.; Çobanoğlu N.; Pekcan S.; Cinel G.; Yalçın E.; Kiper N.; Şen V.; S. Şen H.; Ercan Ö.; Keskin Ö.; B. Eltan S.; Al Shadfan L.M.; Yazan H.; Altıntaş D.U.; Şaşihüseyinoğlu Ş.; Sapan N.; Çekiç Ş.; Çokuğraş H.; A. Kılınç A.; R. Gürsoy T.; Aslan A.T.; Bingöl A.; Başaran A.E.; Özdemir A.; Köse M.; Hangül M.; Emiralioğlu N.; Tuğcu G.; Yüksel H.; Yılmaz Ö.; Orhan F.; Gayretli Aydın Z.G.; Topal E.; Tamay Z.; Süleyman A.; Can D.; Bal C.M.; Çaltepe G.; Özçelik U.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. © 2019 Wiley Periodicals, Inc.Item The success of the Cystic Fibrosis Registry of Turkey for improvement of patient care(John Wiley and Sons Inc, 2022) Asfuroglu P.; Sismanlar Eyuboglu T.; Aslan A.T.; Gursoy T.R.; Emiralioglu N.; Yalcin E.; Kiper N.; Sen V.; Sen H.S.; Altintas D.U.; Ozcan D.; Kilinc A.A.; Cokugras H.; Baskan A.K.; Yazan H.; Erenberk U.; Dogan G.; Unal G.; Yilmaz A.I.; Keskin O.; Arik E.; Kucukosmanoglu E.; Irmak I.; Damadoglu E.; Ozturk G.K.; Gulen F.; Basaran A.E.; Bingol A.; Cekic S.; Sapan N.; Kilic G.; Harmanci K.; Kose M.; Ozdemir A.; Tugcu G.D.; Polat S.E.; Hangul M.; Ozcan G.; Aydin Z.G.G.; Yuksel H.; Topal E.; Ozdogan S.; Caltepe G.; Suleyman A.; Can D.; Ekren P.K.; Bal C.M.; Kilic M.; Cinel G.; Cobanoglu N.; Pekcan S.; Cakir E.; Ozcelik U.; Dogru D.Background: Cystic fibrosis (CF) registries play an essential role in improving disease outcomes of people with CF. This study aimed to evaluate the association of newly established CF registry system in Turkey on follow-up, clinical, growth, treatment, and complications of people with this disease. Methods: Age at diagnosis, current age, sex, z-scores of weight, height and body mass index (BMI), neonatal screening results, pulmonary function tests, history of meconium ileus, medications, presence of microorganisms, and follow-up were evaluated and compared to data of people with CF represented in both 2017 and 2019 registry data. Results: There were 1170 people with CF in 2017 and 1637 in 2019 CF registry. Eight hundred and fourteen people were registered in both 2017 and 2019 of whom z-scores of heights and BMI were significantly higher in 2019 (p = 0.002, p =0.039, respectively). Inhaled hypertonic saline, bronchodilator, and azithromycin usages were significantly higher in 2019 (p =0.001, p = 0.001, p = 0.003, respectively). The percent predicted of forced expiratory volume in 1 sec and forced vital capacity were similar in 2017 and 2019 (88% and 89.5%, p = 0.248 and 84.5% and 87%, p =0.332, respectively). Liver diseases and osteoporosis were significantly higher, and pseudo-Bartter syndrome (PBS) was significantly lower in 2019 (p = 0.011, p = 0.001, p = 0.001, respectively). Conclusions: The z-scores of height and BMI were higher, the use of medications that protect and improve lung functions was higher and incidence of PBS was lower in 2019. It was predicted that registry system increased the care of people with CF regarding their follow-up. The widespread use of national CF registry system across the country may be beneficial for the follow-up of people with CF. © 2022 Wiley Periodicals LLC.Item Clinical findings of patients with cystic fibrosis according to newborn screening results(John Wiley and Sons Inc, 2022) Ramasli Gursoy T.; Aslan A.T.; Asfuroglu P.; Sismanlar Eyuboglu T.; Cakir E.; Cobanoglu N.; Pekcan S.; Cinel G.; Dogru D.; Ozcelik U.; Yalcin E.; Sen V.; Ercan O.; Kilinc A.A.; Yazan H.; Altintas D.U.; Kartal Ozturk G.; Bingol A.; Sapan N.; Celebioglu E.; Tugcu G.D.; Ozdemir A.; Harmanci K.; Kose M.; Emiralioglu N.; Tamay Z.; Yuksel H.; Ozcan G.; Topal E.; Can D.; Korkmaz Ekren P.; Caltepe G.; Kilic M.; Ozdogan S.Background: Cystic fibrosis (CF) is a lethal recessive genetic disease caused by loss of function associated with mutations in the CF trans-membrane conductance regulator. It is highly prevalent (approximately 1 in 3,500) in Caucasians. The aim of this study was to compare demographic and clinical features, diagnostic tests, treatments, and complications of patients with CF whose newborn screening (NBS) with twice-repeated immune reactive trypsinogen testing was positive, normal, and not performed. Methods: In this study, 359 of all 1,488 CF patients recorded in the CF Registry of Turkey in 2018, who had been born through the process of NBS, were evaluated. Demographic and clinical features were compared in patients diagnosed with positive NBS (Group 1), normal (Group 2), or without NBS (Group 3). Results: In Group 1, there were 299 patients, in Group 2, there were 40 patients, and in Group 3, there were 20 patients. Among all patients, the median age at diagnosis was 0.17 years. The median age at diagnosis was higher in Groups 2 and 3 than in Group 1 (P = 0.001). Fecal elastase results were higher in Group 2 (P = 0.033). The weight z-score was lower and chronic Staphylococcus aureus infection was more common in Group 3 (P = 0.017, P = 0.004, respectively). Conclusions: Frequency of growth retardation and chronic S. aureus infection can be reduced with an early diagnosis using NBS. In the presence of clinical suspicion in patients with normal NBS, further analyses such as genetic testing should be performed, especially to prevent missing patients with severe mutations. © 2021 Japan Pediatric Society.Item Patients with cystic fibrosis who could not receive the CFTR modulator treatment: What did they lose in 1 year?(John Wiley and Sons Inc, 2023) Uytun S.; Cinel G.; Eryılmaz Polat S.; Özkan Tabakçı S.; Kiper N.; Yalçın E.; Ademhan Tural D.; Özsezen B.; Şen V.; Selimoğlu Şen H.; Ufuk Altıntaş D.; Çokuğraş H.; Kılınç A.A.; Başkan A.K.; Yazan H.; Çollak A.; Uzuner S.; Ünal G.; Yılmaz A.İ.; Çağlar H.T.; Damadoğlu E.; Irmak İ.; Demir E.; Kartal Öztürk G.; Bingöl A.; Başaran E.; Sapan N.; Canıtez Y.; Tana Aslan A.; Asfuroğlu P.; Harmancı K.; Köse M.; Hangül M.; Özdemir A.; Çobanoğlu N.; Özcan G.; Keskin Ö.; Yüksel H.; Özdoğan Ş.; Topal E.; Çaltepe G.; Can D.; Korkmaz Ekren P.; Kılıç M.; Emiralioğlu N.; Şişmanlar Eyüboğlu T.; Pekcan S.; Çakır E.; Özçelik U.; Doğru D.Background: Cystic fibrosis (CF) is an autosomal recessive disorder caused by CF transmembrane conductance regulator (CFTR) genetic variants. CFTR modulators improve pulmonary function and reduce respiratory infections in CF. This study investigated the clinical and laboratory follow-up parameters over 1 year in patients with CF who could not receive this treatment. Methods: This retrospective cohort study included 2018 and 2019 CF patient data from the CF registry of Turkey. Demographic and clinical characteristics of 294 patients were assessed, who had modulator treatment indications in 2018 but could not reach the treatment. Results: In 2019, patients younger than 18 years had significantly lower BMI z-scores than in 2018. During the 1-year follow-up, forced expiratory volumes (FEV1) and FEV1 z-scores a trend toward a decrease. In 2019, chronic Staphylococcus aureus colonization, inhaled antipseudomonal antibiotic use for more than 3 months, oral nutritional supplement requirements, and oxygen support need increased. Conclusions: Patients who had indications for modulator treatments but were unable to obtain them worsened even after a year of follow-up. This study emphasized the importance of using modulator treatments for patients with CF in our country, as well as in many countries worldwide. © 2023 Wiley Periodicals LLC.Item Clinical characteristics of patients requiring lung transplantation referral in national cystic fibrosis registry data(Turkish National Pediatric Society, 2023) Onay Z.R.; Eyüboğlu T.Ş.; Aslan A.T.; Gürsoy T.R.; Yalçın E.; Kiper N.; Emiralioğlu N.; Şen H.S.; Şen V.; Ünal G.; Yılmaz A.İ.; Kılınç A.A.; Çokuğraş H.; Başkan A.K.; Yazan H.; Çollak A.; Uzuner S.; Şasihüseyinoğlu A.Ş.; Özcan D.; Altıntaş D.U.; Öztürk G.K.; Demir E.; Bingöl A.; Başaran E.; Çekiç Ş.; Sapan N.; Irmak İ.; Damadoğlu E.; Tuğcu G.D.; Polat S.E.; Özdemir A.; Harmancı K.; Kılıç G.; Hangül M.; Köse M.; Tamay Z.; Yüksel H.; Özcan G.; Topal E.; Can D.; Korkmaz P.; Çaltepe G.; Kılıç M.; Özdoğan Ş.; Çakır E.; Çobanoğlu N.; Pekcan S.; Cinel G.; Özçelik U.; Doğru D.Background. We aimed to determine the number of cystic fibrosis (CF) patients recorded in the Cystic Fibrosis Registry of Türkiye (CFRT) who were in need of lung transplantation (LT) referral and examine clinical differences between patients who were LT candidates due to rapid forced expiratory volume in one second (FEV₁) decline and LT candidates without rapid FEV₁ decline in the last year to identify a preventable cause in patients with such rapid FEV₁ decline. Methods. All CF patients recorded in the CFRT in 2018 were evaluated in terms of LT. Patients were divided into those with FEV₁ below 50% and in need of LT due to a decrease of 20% or more in the previous year (Group 1) and those who did not have FEV₁ decline of more than 20% in the previous year but had other indications for LT (Group 2). Demographic and clinical features were compared between the two groups. Results. Of 1488 patients registered in CFRT, 58 had a need for LT. Twenty patients were included in Group 1 and others in Group 2. Our findings did not reveal any significant variations in treatment, chronic infection status, or complications between the two groups. The average weight z-score was significantly higher in Group 1. Positive correlations were detected between weight z-score and FEV₁ in 2017 in Group 1 and between FEV₁ values in 2017 and 2018 in Group 2. Conclusions. There appears to be a relationship between the nutritional status and weight z-scores of CF patients and pulmonary function, which may indirectly affect the need for lung transplantation referral. © 2023, Turkish National Pediatric Society. All rights reserved.Item Factors associated with pulmonary function decline of patients in the cystic fibrosis registry of Turkey: A retrospective cohort study(John Wiley and Sons Inc, 2024) Emiralioğlu N.; Çakır B.; Sertçelik A.; Yalçın E.; Kiper N.; Şen V.; Altıntaş D.U.; Serbes M.; Çokuğraş H.; Kılınç A.A.; Başkan A.K.; Hepkaya E.; Yazan H.; Türel Ö.; Kafi H.M.; Yılmaz A.İ.; Ünal G.; Çağlar T.; Damadoğlu E.; Irmak İ.; Demir E.; Öztürk G.; Bingöl A.; Başaran E.; Sapan N.; Aslan A.T.; Asfuroğlu P.; Harmancı K.; Köse M.; Hangül M.; Özdemir A.; Tuğcu G.; Polat S.E.; Özcan G.; Gayretli Z.G.; Keskin Ö.; Bilgiç S.; Yüksel H.; Özdoğan Ş.; Topal E.; Çaltepe G.; Can D.; Ekren P.K.; Kılıç M.; Süleyman A.; Eyüboğlu T.Ş.; Cinel G.; Pekcan S.; Çobanoğlu N.; Çakır E.; Özçelik U.; Doğru D.Background: The decline in pulmonary function is a predictor of disease progression in patients with cystic fibrosis (CF). This study aimed to determine the decline rate of percent predicted forced expiratory volume in 1 s (ppFEV1) based on the data of the CF Registry of Turkey. The secondary aim was to investigate the risk factors related to the decline in ppFEV1. Methods: A retrospective cohort study of CF patients over 6 years old, with pulmonary function data over at least 2 years of follow-up was extracted from the national CF registry for years 2017–2019. Patients were classified according to disease severity and age groups. Multivariate analysis was used to predict the decline in ppFEV1 and to investigate the associated risk factors. Results: A total of 1722 pulmonary function test results were available from 574 patients over the study period. Mean diagnostic age was older and weight for age, height for age, and body mass index z scores were significantly lower in the group of ppFEV1 < 40, while chronic Pseudomonas aeruginosa (p <.001) and mucoid P. aeruginosa colonization (p <.001) were significantly higher in this group (p <.001). Overall mean annual ppFEV1 decline was −0.97% (95% confidence interval [CI] = −0.02 to −1.92%). The mean change of ppFEV1 was significantly higher in the group with ppFEV1 ≥ 70 compared with the other (ppFEV1 < 40 and ppFEV1: 40–69) two groups (p =.004). Chronic P. aeruginosa colonization (odds ratio [OR] = 1.79 95% CI = 1.26–2.54; p =.01) and initial ppFEV1 ≥ 70 (OR = 2.98 95% CI = 1.06-8.36), p =.038) were associated with significant ppFEV1 decline in the whole cohort. Conclusions: This data analysis recommends close follow-up of patients with normal initial ppFEV1 levels at baseline; advocates for early interventions for P. aeruginosa; and underlines the importance of nutritional interventions to slow down lung disease progression. © 2024 Wiley Periodicals LLC.Item Comparison of clinical features of cystic fibrosis patients eligible but not on CFTR modulators to ineligible for CFTR modulators(John Wiley and Sons Inc, 2024) Nayır Büyükşahin H.; Emiralioğlu N.; Yalçın E.; Şen V.; Selimoğlu Şen H.; Arslan H.; Başkan A.K.; Çakır F.B.; Koray C.F.; Yılmaz A.İ.; Ercan F.; Altıntaş D.U.; Serbes M.; Keskin Ö.; Arık E.; Gülen F.; Barlık M.; Karcıoğlu O.; Damadoğlu E.; Köse M.; Ersoy A.; Bingöl A.; Başaran E.; Çakır E.P.; Aslan A.T.; Canıtez Y.; Korkmaz M.; Özdemir A.; Harmancı K.; Soydaş Ş.S.; Hangül M.; Yüksel H.; Özcan G.; Korkmaz P.; Kılıç M.; Gayretli Aydın Z.G.; Çaltepe G.; Can D.; Doğru S.; Kartal Öztürk G.; Süleyman A.; Topal E.; Özsezen B.; Hızal M.; Demirdöğen E.; Ogun H.; Börekçi Ş.; Yazan H.; Çakır E.; Şişmanlar Eyüboğlu T.; Çobanoğlu N.; Cinel G.; Pekcan S.; Özçelik U.; Doğru D.Introduction: Cystic fibrosis transmembrane conductance regulator (CFTR) modulator drugs target the underlying defect and improve CFTR function. They are a part of standard care in many countries, but not all patients are eligible for these drugs due to age and genotype. Here, we aimed to determine the characteristics of non-eligible patients for CFTR modulators in the CF registry of Turkey (CFRT) to highlight their clinical needs. Methods: This retrospective cohort study included CF patient data from the CFRT in 2021. The decision of eligibility for the CFTR modulator was determined according to the ‘Vertex treatment-Finder' on the Vertex® website. Demographic and clinical characteristics of patients were compared between eligible (group 1) and ineligible (group 2) groups for CFTR modulators. Results: Among the study population (N = 1527), 873 (57.2%) were in group 1 and 654 (42.8%) were in group 2. There was no statistical difference between groups regarding sex, meconium ileus history, diagnoses via newborn screening, FEV1 z-score, CF-associated complications, organ transplant history, and death. Patients in group 2 had a higher incidence of pancreatic insufficiency (87.7% vs. 83.2%, p =.010), lower median height z-scores (−0.87 vs. −0.55, p <.001), lower median body mass index z-scores (−0.65 vs. −0.50, p <.001), longer days receiving antibiotics due to pulmonary exacerbation (0 [interquartile range, IQR: 0–2] vs. 0 [IQR: 0–7], p = 0.001), and more non-invasive ventilation support (2.6% vs. 0.9%, p = 0.008) than patients in group 1. Conclusion: The ineligible group had worse clinical outcomes than the eligible group. This highlights their need for life-changing drugs to improve clinical outcomes. © 2024 Wiley Periodicals LLC.Item Eligibility of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) modulator therapies: cohort of cystic fibrosis registry of Türkiye(Turkish National Pediatric Society, 2025) Erdal M.A.; Büyükşahin H.N.; Şen V.; Kılınç A.A.; Çokuğraş H.; Doğan G.; Yılmaz A.İ.; Ünal G.; Serbes M.; Altıntaş D.U.; Arık E.; Keskin Ö.; Özaslan M.M.; Karcıoğlu O.; Köse M.; Başaran A.E.; Çakır E.P.; Canıtez Y.; Özdemir A.; Harmancı K.; Uytun S.; Polat S.E.; Hangül M.; Yüksel H.; Özcan G.; Korkmaz P.; Kılıç M.; Aydın Z.G.G.; Çaltepe G.; Can D.; Doğru S.; Öztürk G.K.; Süleyman A.; Topal E.; Özsezen B.; Hızal M.; Demirdöğen E.; Ogun H.; Börekçi Ş.; Yazan H.; Şen H.S.; Demir A.D.; Çakır E.; Eyüboğlu T.Ş.; Emiralioğlu N.; Pekcan S.; Özçelik U.; Doğru D.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. © 2025 The Author(s).Item Childhood interstitial lung disease survivors in adulthood: a European collaborative study(2025) Manali E.D.; Griese M.; Nathan N.; Uzunhan Y.; Borie R.; Michel K.; Schwerk N.; Fijolek J.; Radzikowska E.; Chua F.; Pabary R.; Mogulkoc N.; McCarthy C.; Kallieri M.; Papaioannou A.I.; Kiper N.; Koziar Vasakova M.; Lacina L.; Molina-Molina M.; Torrent-Vernetta A.; Tsiligiannis T.; Karadag B.; Kokosi M.; Renzoni E.A.; van Moorsel C.H.M.; Campo I.; Bendstrup E.; Prior T.S.; Prasse A.; Bonella F.; Cottin V.; Diesler R.; Froidure A.; Kolilekas L.; Fotis L.; Douros K.; Kaditis A.G.; Jeny F.; Chauveau S.; Nunes H.; Dahbia A.; Mariani F.; van der Vis J.J.; Groen K.; Erdem Eralp E.; Gokdemir Y.; Kocakaya D.; Olgun Yildizeli S.; Yalçın E.; Emiralioğlu N.; Nayir Buyuksahin H.; O'Brien H.; Karcıoglu O.; Can D.; Ezircan A.; Kartal Ozturk G.; Ocal N.; Yuksel H.; Narin Tongal S.; Safrankova M.; Kourtesi K.; Louvrier C.; Kannengiesser C.; Fabre A.; Legendre M.; Crestani B.; Pohunek P.; Bush A.; Papiris S.A.BACKGROUND: Interstitial lung disease is rarer in children than adults, but, with increasing diagnostic awareness, more cases are being discovered. The prognosis of childhood interstitial lung disease is often poor, but increasing numbers are now surviving into adulthood. AIM: To characterise childhood interstitial lung disease survivors and identify their impact on adult interstitial lung disease centres. METHODS: This was a European study (34 adult and childhood interstitial lung disease centres) reporting incident/prevalent cases of childhood interstitial lung disease survivors from January to July 2023. Epidemiological, clinical, physiological and genetic data were collected. RESULTS: 244 patients were identified with a median (interquartile range) age at diagnosis of 12.5 years (6-16 years) and age at study inclusion of 25 years (22-33 years), with 51% male, 86% nonsmokers and a median (interquartile range) % predicted forced vital capacity of 70% (47-89%) and diffusing capacity of the lungs for carbon monoxide of 48% (32-75%). 32% were prescribed long-term oxygen and 227 (93%) were followed up in adult centres whereas 17 (7%) never transitioned. The commonest diagnoses (82%) were childhood interstitial lung disease category B1 (sarcoidosis, hemosiderosis, connective tissue disorders, vasculitis) at 35%, A4 (surfactant-related) at 21%, B2 (bronchiolitis obliterans, hypersensitivity pneumonitis) at 14% and Bz (unclassified interstitial lung disease) at 13%. Bz patients had the worst functional status. 60% of all patients were still being prescribed corticosteroids. Re-specification of diagnosis and treatment were made after transition for 9.8% and 16% of patients, respectively. Not all childhood interstitial lung disease diagnoses were recognised in adult interstitial lung disease classifications. CONCLUSION: Childhood interstitial lung disease survivors are seen in most adult interstitial lung disease centres and only a minority continue follow-up in paediatric centres. Survivors have a significant loss of lung function. The heterogeneity of their aetiologies and therapeutic requirements has a real impact on adult interstitial lung disease centres. Re-specification of diagnosis and treatment may contribute to precision and personalisation of management. Copyright ©The authors 2025. For reproduction rights and permissions contact permissions@ersnet.org.