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Öğe The associations between the clinical course of children with tracheostomy and their mothers' depression, burnout, burden, and self-esteem(Wiley, 2023) Gursoy, Tugba Ramasli; Eyuboglu, Tugba Sismanlar; Aslan, Ayse Tana; Pekcan, Sevgi; Buyukkaya, Eda Zeynep; Hangul, Melih; Kose, MehmetAims and Objectives The aim of this study was to evaluate the clinical features of children with tracheostomy and the predictors of psychological status of their primary caregivers in order to determine the associations between the children's clinical course with their caregivers' psychological status. Background The caregivers of children with tracheostomy are responsible for providing basic tracheostomy care at home. All these responsibilities may be associated with significant changes in family members' lifestyles, daily routines and family dynamics. Design This study is a cross sectional study. Methods Data of the family's socioeconomic status and clinical status of children with tracheostomy were noted in four paediatric pulmonology centers. The Beck Depression Inventory, Maslach Burnout Inventory, Zarit Caregiver Burden Scale, and Rosenberg Self-esteem Scale were used for psychological measurements of the caregivers. The STROBE checklist was used for this study. Results Eighty-five children and their primary caregivers were enrolled in the study. The children's median age was 4.1 years. Thirty-eight of them were dependent on home ventilators. Twenty-one had bacterial colonisation. All children's primary caregivers were their mothers. Beck Depression Inventory scores of mothers of children with colonisation were higher. Number of hospitalizations in previous 6 months was related to mothers' emotional exhaustion and depersonalization scores. Duration of children's hospitalizations in previous 6 months was positively correlated to mothers' emotional exhaustion, depersonalization, and Beck Depression Inventory scores. Conclusions Mothers of children with tracheostomy may experience psychological conditions, such as high levels of depression, burnout, burden and low levels of self-esteem. Frequent and long-term hospitalizations of children correlated with mother's depression and burnout. Therefore, primary caregivers should be evaluated and supported psychologically. Relevance to Clinical Practice Preventing mothers of children with tracheostomy from experiencing psychological conditions such as depression, burnout, burden and low self-esteem can also increase the quality of care for children.Öğe Caspase-1 and interleukin-18 in children with post infectious bronchiolitis obliterans: a case-control study(Springer, 2022) Eyuboglu, Tugba Sismanlar; Aslan, Ayse Tana; Gursoy, Tugba Ramasli; Pekcan, Sevgi; Kose, Mehmet; Hangul, Melih; Aral, Latife ArzuThe exact immunological mechanisms of post infectious bronchiolitis obliterans (PIBO) in childhood are not fully known. It has been shown that the inflammasome and IL-18 pathway play important roles in the pathogenesis of lung fibrosis. We aimed to investigate the role of caspase-1, IL-18, and IL-18 components in PIBO. From January to May 2020, children with PIBO, children with history of influenza infection without PIBO, and healthy children were asked to participate in the study in three pediatric pulmonology centers. Serum caspase-1, IL-18, IL-18BP, IL-18R, and INF-gamma levels were measured by ELISA and compared between the 3 groups. There were 21 children in the PIBO group, 16 children in the influenza group, and 39 children in the healthy control group. No differences in terms of age and gender between the 3 groups were found. IL-18 and IL-18BP levels were higher in the healthy control group (p = 0.018, p = 0.005, respectively). IL- 18R was higher in the PIBO group (p = 0.001) and caspase-1 was higher in the PIBO and influenza group than the healthy control group (p = 0.002). IFN-gamma levels did not differ between the 3 groups. IL-18BP/IL-18 was higher in the influenza group than the PIBO group and the healthy control group (p = 0.003). Conclusions: Caspase-1 level was increased in patients with PIBO which suggests that inflammasome activation may have a role in fibrosis; however, IL-18 level was found to be low. Mediators other than IL-18 may be involved in the inflammatory pathway in PIBO. Further immunological studies investigating inflammasome pathway are needed for PIBO with chronic inflammation.Öğe Childhood sarcoidosis in the middle Anatolia of Turkey(Wiley, 2023) Hangul, Melih; Kose, Mehmet; Pekcan, Sevgi; Ersoy, Ali; Unal, Gokcen; Caglar, Hanife TugceObjectiveThis retrospective study aimed to describe the clinical and radiological features, diagnostic methods, laboratory findings, organ involvement, and treatment strategies of 22 patients who followed up with late-onset childhood sarcoidosis and compare them with the literature. Material and MethodThis retrospective multicenter study reviewed the medical records of 22 children with sarcoidosis who applied to the Pediatric pulmonology department of Erciyes University Faculty of Medicine and Necmettin Erbakan Faculty of Medicine in 2012 and 2022. ResultsThe mean age of the patients at the time of diagnosis was 13.1/year (interquartile range [IQR]1:6.3-[IQR]3:15.7). The most common first presenting symptom was cough 40.9% (n = 9), weight loss 31.8% (n = 7), and dyspnea 22.7% (n = 5). There were elevated levels of C-reactive protein (CRP; 59%), angiotensin-converting enzyme (ACE; 54.5%), erythrocyte sedimentation rate (ESR; 54.5%), and immunoglobulin G (IgG; 54.5%). Twenty patients (90%) received systemic steroid treatment. Eighteen (81.8%) of these patients responded positively to treatment. Two patients had a recurrence. ConclusionThe incidence of sarcoidosis in children in Turkey is currently unknown. However, a regional average of 2.2 cases per year has been documented for the first time. Contrary to previous studies, a significant prevalence of consanguineous marriage was observed in our study. While constitutional symptoms were most common in other studies, the cough was the most common symptom in our study. To our knowledge, this is one of the Turkish studies with the highest number of sarcoidosis in children and one of the few European studies on sarcoidosis in children.Öğe Comparison of refugee patients with cystic fibrosis and their counterpart children from Turkey during the war(Springer, 2024) Yilmaz, Asli Imran; Pekcan, Sevgi; Eyuboglu, Tugba Sismanlar; Hangul, Melih; Arslan, Huseyin; Kilinc, Ayse Ayzit; Cokugras, HalukSince 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:center dot Children who have chronic diseases are the group that is most affected by wars.center dot The outcome gets better with early diagnosis and treatment in patients with Cystic Fibrosis (CF).What is New:center dot 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. center dot 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.Öğe The Genetic Analysis of Cystic Fibrosis Patients with Seven Novel Mutations in the CFTR Gene in the Central Anatolian Region of Turkey(Galenos Publ House, 2021) Erdogan, Murat; Kose, Mehmet; Pekcan, Sevgi; Hangul, Melih; Balta, Burhan; Kiraz, Aslihan; Gonen, Gizem AkinciBackground: Cystic fibrosis, a pulmonary disease which is an autosomal recessive, inherited, multisystemic genetic disease commonly seen in the Caucasian race, is the most frequent cause of mortality and morbidity. So far, more than 2000 disease-causing gene variants have been found and this number has been increasing with the studies conducted. Although there is not yet enough data that include the Turkish population, the recent increase of studies is noteworthy. Aims: To discover the genetic variation in patients diagnosed with cystic fibrosis in the Central Anatolian region. Study Design: Cross-sectional study. Methods: The study was carried out in the Central Anatolian region in 3 pediatric pulmonology departments (Kayseri, Konya, and Ankara) in Turkey between July 2014 and December 2017. The Sanger and Next Generation Sequence analyses were used for exon and exon-intron boundaries in the cystic fibrosis transmembrane conductance regulatory (CFTR) gene, and in selected patients, mutation analysis was performed using the Multiplex Ligation-dependent Probe Amplification technique for large deletions and duplications. Results: CFTR gene analysis was performed for 316 patients and 215 of them were genetically diagnosed with cystic fibrosis. Sixty-three different variants were defined in these patients and 7 of these were large deletions/duplications detected with the MLPA method. The most frequent variants were F508del (29.6%), G85E (8.2%), N1303K (8.2%), Y515* (7.5%), and G542* (3.4%). Conclusion: Using sequencing and Multiplex Ligation-dependent Probe Amplification methods, the identification of seven new mutations that were not previously reported in the literature contributes to a better understanding of the heterogeneous nature of CFTR mutations in the Turkish population. When no mutations are detected (pathogenic/probably pathogenic) in clinically compatible cases, Multiplex Ligationdependent Probe Amplification analysis contributes significantly to the diagnosis.Öğe The Incidence of Cystic Fibrosis in the Central Region of Anatolia in Turkey Between 2015 and 2016(Galenos Publ House, 2019) Hangul, Melih; Pekcan, Sevgi; Kose, Mehmet; Acican, Deniz; Sahlar, Tuba Esra; Erdogan, Murat; Kendirci, MustafaBackground: Cystic fibrosis is the most common metabolic chronic disease among European Caucasian children. Cystic fibrosis incidence in Northern Europeans countries is approximately 1 in 3000 births while the worldwide prevalence varies considerably. Aims: To determine the incidence of cystic fibrosis in the central region of Anatolia in Turkey using the newborn screening program data. Study Design: Cross-sectional study. Methods: We used the records of the newborn screening program which is implemented by the Konya and Kayseri Provincial Health Directories. Between January 2015 and December 2016, there were a total of 119006 live births in Konya and Kayseri. The newborn screening test was applied to all these babies. Results: During this period, there were 22 live born babies diagnosed with cystic fibrosis in Konya with an incidence of 2.9 per 10000 live births and 13 live born babies diagnosed with cystic fibrosis in Kayseri with an incidence of 2.8 per 10000 live births. In genetic of 30 patients, fifteen patients were homozygous, and 15 patients were a compound heterozygote. Twenty-one different gene variants were detected and the most common mutation was F508del (17/30) Conclusion: We found the incidence of cystic fibrosis in central Anatolia similar to northern European countries.Öğe Misdiagnosis of Asthma May Delay the Post Infectious Bronchiolitis Obliterans Diagnosis(European Respiratory Soc Journals Ltd, 2019) Onay, Zeynep Reyhan; Gursoy, Tugba Ramasli; Aslan, Ayse Tana; Eyuboglu, Tugba Sismanlar; Kibar, Busra Sultan; Pekcan, Sevgi; Hangul, Melih[Abstract Not Availabe]Öğe Passive Smoking and Disease Severity in Childhood Pneumonia Under 5 Years of Age(Oxford Univ Press, 2020) Eyuboglu, Tugba Sismanlar; Aslan, Ayse Tana; Kose, Mehmet; Pekcan, Sevgi; Hangul, Melih; Gulbahar, Ozlem; Cingirt, MehmetObjective: To objectively investigate the effect of passive smoking on pneumonia and disease severity in children aged less than 5 years by using cotinine as an indicator of passive smoking. Methods: Between December 2015 and April 2016, children aged less than 5 years with pneumonia and age-matched healthy controls were included in this study, which was conducted at three tertiary pediatric pulmonology centers. A questionnaire was given to the parents regarding demographic data and smoking status at home. Urinary cotinine/creatinine ratio (CCR) was measured. The data from the pneumonia and control groups, as well as children with mild and severe pneumonia within the pneumonia group, were compared. Results: A total of 227 subjects were included in the study; there were 74 children in the pneumonia group and 153 in the control group. The mean age of all the children was 33.461.28 months. Of all subjects, 140 were male and 102 were exposed to passive smoking by their parents at home. There were statistically significant differences in age, number of people in the home, and mother's and father's age between the control and pneumonia groups (p< 0.05). No difference was found in the CCR in the control and pneumonia group (p> 0.05). Age and urinary CCR were significantly different between children with mild and severe pneumonia (p< 0.05). Conclusion: We showed that passive smoking exposure was associated with the development of severe pneumonia in children. Further studies are needed to examine the underlying cause in detail.Öğe Postinfectious bronchiolitis obliterans masked by misdiagnosis as asthma(Wiley, 2020) Onay, Zeynep R.; Gursoy, Tugba Ramasli; Aslan, Ayse T.; Eyuboglu, Tugba Sismanlar; Kibar, Busra S.; Pekcan, Sevgi; Hangul, MelihObjectives Asthma and postinfectious bronchiolitis obliterans (PIBO) have similar clinical findings, and PIBO may be misdiagnosed with asthma. This study aimed to determine the clinical features of PIBO in children and the causes of delay in its diagnosis. Methods We retrospectively evaluated all patients diagnosed with PIBO in four pediatric pulmonology centers between 2007 and 2018. In total, 64 PIBO patients were retrospectively reviewed. We compared the clinical and laboratory differences between PIBO patients who had initially been misdiagnosed with asthma and correctly diagnosed with PIBO. Results Of the 64 patients, 22 (34.4%) had initially been misdiagnosed with asthma. Adenovirus was the most common infectious agent in children. The age upon diagnosis was older, and the symptom duration was significantly longer in patients misdiagnosed with asthma (P < .05). There were no statistical differences in terms of sex, history of prematurity, duration of hospitalization, treatment, history of oxygen or mechanical ventilation support, pulmonary function test (PFT) results and asthma-predisposing findings between the two groups (P > .05). Conclusions Patients with PIBO who had initially been misdiagnosed with asthma were correctly diagnosed at older ages and had longer symptom duration. Asthma may mask PIBO diagnosis by the similarity of symptoms and the clinical response to inhaled beta 2-agonist or steroid treatment. PFTs may not help clinicians because of the age of children. The delay in the diagnosis of PIBO is probably attributable to the fact that some clinicians fail to include PIBO in the differential diagnosis when there is no clinical response to asthma medication.Öğe Psychological status of mothers of children with cystic fibrosis and primary ciliary dyskinesia(Wiley, 2022) Gursoy, Tugba Ramasli; Eyuboglu, Tugba Sismanlar; Aslan, Ayse T.; Emiralioglu, Nagehan; Pekcan, Sevgi; Buyukkaya, Eda Z.; Hangul, MelihObjectives We aimed to investigate depression, burnout, attitude, and burden of caregivers of children with cystic fibrosis (CF), and especially caregivers of children with primary ciliary dyskinesia (PCD) due to limited number of studies on this topic, and to compare them according to their children's clinical status. Methods Clinical features and demographic data of children and their families were asked from caregivers in four pediatric pulmonology centers. Beck Depression Inventory, Maslach Burnout Inventory, Zarit Caregiver Burden Scale, and Parental Attitude Research Instrument were administered to caregivers in both groups. Results were compared between the two groups. Results In total, 131 children with CF and 39 with PCD and their caregivers were involved in the study. All primary caregivers were mothers in both groups. Depression, burnout, and burden scores of mothers of children with CF were significantly higher than mothers of children with PCD (p = 0.017, p = 0.024, p = 0.038, respectively). Burnout was higher in both CF and PCD groups with low family income (p = 0.022, p = 0.034). Number of hospital visits in the previous 6 months was correlated with burnout in both CF and PCD groups (r = 0.207, p = 0.034; r = 0.352, p = 0.044). Conclusions Although mothers with children with CF have higher levels of depression, burnout, burden, and negative attitudes toward children than mothers with children with PCD, these are also significantly high in mothers with children with PCD. Psychological problems of mothers of children with CF and PCD may increase with frequent hospital visits, hospitalizations, low family income, number of children, and chronic disease in another child.Öğe Psychosocial status of primary caregivers of children with tracheostomy(European Respiratory Soc Journals Ltd, 2018) Eyuboglu, Tugba Sismanlar; Gursoy, Tugba Ramasli; Aslan, Ayse Tana; Pekcan, Sevgi; Buyukkaya, Zeynep; Hangul, Melih; Kose, Mehmet[Abstract Not Availabe]Öğe Pulmonary Embolism in Childhood: A Multicenter Experience from Turkey(Galenos Publ House, 2022) Hangul, Melih; Kose, Mehmet; Pekcan, Sevgi; Caliskan, Umran; Tokgoz, Huseyin; Aslan, Ayse Tana; Eyuboglu, Tugba SismanlarBackground: Pulmonary embolism is a clinical condition caused by the obstruction of the pulmonary artery and its branches with endogenous, exogenous embolism, or local thrombus formation. It is a rare but potentially life-threatening event in the pediatric population. Pediatric pulmonary embolism has many unknown characteristics.Aims: To evaluate clinical features, genetic and acquired risk factors, diagnostic imaging, and treatment strategies with long-term results in children with pulmonary embolism.Study Design: A retrospective multicenter clinical trialMethods: Patients aged 0-18 years who were diagnosed with pulmonary embolism with computed tomography pulmonary angiography (CTPA) findings (intraluminal filling defect in the lobar or main pulmonary artery) in 3 university hospitals between 2006 and 2021 were included in the study. A form was created for data standardization, and variables were collected retrospectively through medical record review. In addition to the features given above, we also evaluated in situ pulmonary artery thrombosis (ISPAT) and patients' Wells scores. Follow-up CTPA results were evaluated for patient response to treatment. Complete recovery means that there were no lesions, incomplete recovery if there was still embolism, and no response if there was no change.Results: Twenty-four patients (female:13, male:11) were included in the study. The mean age was 13.5 years. All patients but one had at least one or more genetic or acquired risk factors. Factor V Leiden mutation (16.6%) was the most common genetic risk factor. Six of 16 patients with Doppler ultrasonography were diagnosed with ISPAT because there was no sign of thromboembolic thrombosis. Nine (41.6%) patients had a Wells score of >4 (pulmonary embolism clinically strong), and 15 (58.4%) patients scored <4 (pulmonary embolism clinically likely weak), indicating that an alternative diagnosis was more likely than pulmonary embolism (sensitivity %37.5). The mean follow-up period was 23 (+/- 17) months. Complete and incomplete recovery was observed in 15 (62.5%) and 7 (29.1%) patients, respectively, among the patients who underwent follow-up evaluation. No response was obtained in 2 patients (8.3%) who died.Conclusion: The Wells scoring system seems insufficient to diagnose pulmonary embolism in children and should be improved by adding new parameters. ISPAT may be more common in children with congenital heart disease and systemic disease.Öğe Response to Letter to Editor: It takes a village to raise a child with a tracheostomy: Translating principles into practice(Wiley, 2023) Ramasli Gursoy, Tugba; Sismanlar Eyuboglu, Tugba; Aslan, Ayse Tana; Pekcan, Sevgi; Buyukkaya, Eda Zeynep; Hangul, Melih; Kose, Mehmet[Abstract Not Availabe]