Person: GÖKDEMİR, YASEMİN
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GÖKDEMİR
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YASEMİN
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Publication Open Access Improvements in body mass index of children with cystic fibrosis following implementation of a standardized nutritional algorithm: A quality improvement project(2023-03-01) GÖKDEMİR, YASEMİN; ERDEM ERALP, ELA; ERGENEKON, ALMALA PINAR; YILMAZ YEĞİT, CANSU; KARADAĞ, BÜLENT TANER; GÖKDEMİR Y., ERDEM ERALP E., ERGENEKON A. P., YILMAZ YEĞİT C., Yanaz M., Mursaloglu H., Uzunoglu B., Kocamaz D., Tastan G., Filbrun A., et al.BackgroundA collaboration between the University of Michigan (UM) Cystic Fibrosis Center (CFC) and Marmara University (MU) CFC was initiated in MU through conducting Quality Improvement projects (QIP). The global aim was to improve nutritional status of children with CF (cwCF), with a specific aim to increase the mean BMI percentile (BMIp) for cwCF by 10 percentile points in 12 months. MethodsBody mass index (BMI) percentiles of cwCF were categorized as: nutritionally adequate (BMIp >= 50%); at risk (BMIp 25%-49%); urgently at risk (BMIp 10%-25%); critically at risk (BMIp < 10%). Appropriate interventions were made according to BMIp category every three months. Forced expiratory volume in one-second percent predicted (FEV1pp), and health-related quality of life (HRQoL) were evaluated. ResultsOne hundred and eight-two cwCF with a mean age of 9.1 +/- 4.3 years were included in the project. Baseline BMIp increased from 25.6 to 37.2 at the 12th month (p < 0.001). In the critically at-risk group BMIp increased from 3.6 to 20.5 (p < 0.001), in the urgently at risk group from 15.9 to 30.8 (p < 0.001), in the at risk group from 37.0 to 44.2 (p < 0.079) and in the nutritionally adequate group the increase was from 66.8 to 69.5 (p < 0.301). FEV1pp also improved significantly, from 81.3 +/- 20.6 to 85.9 +/- 20.8 (p < 0.001). Physical functioning, eating problems, and respiratory symptoms domains of the HRQoL evaluation improved (p < 0.05). ConclusionThis project has led to significant improvements in BMIp, FEV1pp and HRQoL of cwCF; similar projects could easily be implemented by centers in other developing countries.Publication Open Access Sweat Testing and Recent Advances(FRONTIERS MEDIA SA, 2021-05-04) KARADAĞ, BÜLENT TANER; Gokdemir, Yasemin; Karadag, Bulent TanerCystic fibrosis (CF) is the most common fatal genetic disease of the Caucasian population. Sweat testing is the principal diagnostic test for CF, and it is used for the evaluation of infants with positive CF newborn screening (NBS) and in patients with clinical findings suggesting CF. This article describes the classical sweat test method in detail and also provides an overwiew of recent advances.Publication Metadata only Novel mutations and deletions in cystic fibrosis in a tertiary cystic fibrosis center in Istanbul(WILEY, 2019) KARADAĞ, BÜLENT TANER; Atag, Emine; Ikizoglu, Nilay Bas; Ergenekon, Almala Pinar; Gokdemir, Yasemin; Eralp, Ela Erdem; Ata, Pinar; Ersu, Refika; Karakoc, Fazilet; Karadag, BulentBackgroundCystic fibrosis (CF) genotyping has garnered increased attention since the discovery of the cystic fibrosis transmembrane conductance regulator (CFTR) gene in 1989 led to the identification of over 1700 mutations on chromosome 7. Yet, little is known about the genetic profile of CF patients in Turkey. This study sought to determine the mutation distribution among CF patients seeking care at Marmara University. MethodsTwo hundred fifty previously diagnosed CF patients were included in the study. CFTR gene exons 1 to 27 were amplified by a polymerase chain reaction and whole DNA sequencing was performed. Duplications and deletions were investigated by the multiplex ligation-dependent probe amplification (MLPA) technique in patients with one or two unidentified mutations in sequence analysis. ResultsCFTR mutation analysis revealed 80 mutations and five large deletions were present in our study population. The five most common mutations were (delta) F508 (c.1521-1523delCTT) (28.4%), 1677delTA (c.1545-1546delTA) (6.4%), 2789+5G->A (c.2657+5G>A) (5.8%), N1303K (c.3909C>G) (2.4%), and c.2183AA->G (c.2051-2052delAAinsG) (4.0%). Large deletions were found in 16 patients. Four novel mutations and two novel deletions were detected in this study. ConclusionsWe have identified four novel mutations and two novel deletions using next-generation DNA sequencing and the MLPA technique and obtained an overall mutation detection rate of 91.4%. Detection of novel variants in CF patients will assist in genetic counseling and in determining appropriate patients for new therapies.Publication Open Access Performance Evaluation of a New Coulometric Endpoint Method in Sweat Testing and Its Comparison With Classic Gibson&Cooke and Chloridometer Methods in Cystic Fibrosis(FRONTIERS MEDIA SA, 2018-05-22) KARADAĞ, BÜLENT TANER; Gokdemir, Yasemin; Vatansever, Pinar; Karadag, Bulent; Seyrekel, Tuncay; Baykan, Ozgur; Ikizoglu, Nilay Bas; Ersu, Refika; Karakoc, Fazilet; Haklar, GoncagulBackground: The objective of the study was to assess the diagnostic efficacy of the coulometric endpoint method and compare it with classic Gibson&Cooke and chloridometer methods. Methods: This study is a prospective clinical study comparing two conventional sweat testing methods with the coulometric endpoint method in previously diagnosed cystic fibrosis (CF) patients and a non-CF control group. All individuals underwent two simultaneous sweat collections. One sample of sweat, collected by the CF Delta collector coil system, was analyzed by two methods: the titrimetric Cl- measurement (Sherwood (R) Chloridometer 926S, Sherwood Scientific Ltd., Cambridge, UK) and the coulometric endpoint method (CF Delta Collection System (R), UTSAT/Turkey); the second sample was collected from the other forearm by the Gibson&Cooke method and the collected sweat was analyzed by manual titration in accordance with the Schales&Schales method. Within-run and between-run imprecisions were evaluated via Cl- concentrations of 40, 70, and 130 mmol/L samples. Results: One hundred and seventy (60 CF and 110 controls) subjects were included in the study. All three sweat test methods discriminated CF subjects from the healthy individuals. The mean difference between the coulometric endpoint and titrimetric Cl - measurement methods was -1.5 mmol/L, (95% confidence limits of agreement, ranging from -8.9 to 15.9 mmol/L); the mean difference between manual titration vs. coulometric endpoint methods was 12.8 mmol/L, (95% confidence limits of agreement ranging from -9.7 to 45.3 mmol/L) and the mean difference between the manual titration and titrimetric Cl - measurement methods was 11.3 mmol/L, (95% confidence limits of agreement ranging from -7.8 to 40.5 mmol/L) based on a Bland-Altman analysis. In the Receiver operating characteristic (ROC) analysis, made on the basis that Cl- concentration values <40 mmol/L exclude the CF diagnosis, the coulometric endpoint method resulted in 96.7% sensitivity and 100% specificity for a cut-off value of 58.5 mmol/L (AUC: 0.994; 95% CI = 0.986-1.000; p < 0.001). Conclusions: The coulometric endpoint method can be as reliable as quantitative sweat Cl- analysis and may be considered as a definitive diagnostic tool for CF.Publication Metadata only Cumulative Antimicrobial Susceptibility Data of Pseudomonas Aeruginosa Isolates from Cystic Fibrosis Patients: 4-Year Experience(GEORG THIEME VERLAG KG, 2021) KARAHASAN, AYŞEGÜL; Fidan, Ebru; Alci, Gamze; Koldas, Seda Sevilay; Gokdemir, Yasemin; Karadag, Bulent; Eralp, Ela Erdem; Yagci, Aysegul KarahasanObjective Pseudomonas aeruginosa is the most important cause of lung infection among cystic fibrosis (CF) patients, and to reduce the severity of the infection, facility-specific cumulative antibiograms could help clinicians in empirical treatment. Methods Respiratory samples of CF patients between January 2015 and December 2018 were scanned through Laboratory Operating System retrospectively. Demographical data of patients, culture results, and antibiotic susceptibilities are recorded using Microsoft Excel 2010. Cumulative antibiogram data were obtained according to the CLSI M39A4 document. Results The number of registered patients has increased in 4 years from 154 to 253. The mean age of patients varied from 9 to 11.7 (range, 2-42). The ratio of patients with a positive culture for P. aeruginosa increased from 32 to 40%, and the mean patients' age decreased from 16.6 to 11.1 (p < 0.05). A total number of 4,146 respiratory samples were analyzed. Sputum samples consisted of 42.5% (n: 1,767) of the samples with a 58.4% isolation rate of P. aeruginosa (n: 1,034). A notable increase of resistance was seen almost all antimicrobials tested by years. The ratio of multidrug-resistant (MDR) P. aeruginosa was 4.1, 10.2, 4.5, and 8.6% in 2015, 2016, 2017, and 2018. Conclusion Antimicrobial resistance is a challenging problem in CF patients, and surveillance should be done regularly.Publication Metadata only The effects of nebulizer hygiene training on the practices of cystic fibrosis patients and caregivers(WILEY, 2021) ERGENEKON, ALMALA PINAR; Yilmaz Yegit, Cansu; Ergenekon, Almala Pinar; Mursaloglu, Huseyin Hakan; Cenk, Muruvvet; Uzunoglu, Burcu Suzer; Tastan, Gamze; Gokdemir, Yasemin; Erdem Eralp, Ela; Karakoc, Fazilet; Nasr, Samya Z.; Karadag, BulentBackground Nebulizers can be contaminated with microorganisms and may be a source of infection in the lower airways in patients with cystic fibrosis (CF). Objective Primary aim of this study was to determine the level of knowledge regarding nebulizer hygiene and adherence to CF foundation infection prevention and control (IPC) measures of CF patients in our center. We also evaluated the effect of a standardized training program on nebulizer cleaning and disinfection practises with pre and posttest. Methods Caregivers of 173 CF patients followed at Marmara University CF Center filled a questionnaire (pretest) regarding nebulizer hygiene and received didactic education including pictures and videos based on the cystic fibrosis foundation (CFF) IPC guidelines, patients were also provided educational materials. Posttest was performed 1-3 months after the education session. Results Following standardized training, usage of appropriate methods according to CFF IPC guidelines improved significantly. Frequency of nebulizer cleaning after each use increased from 58.4% to 78% (p < .01) and disinfection frequency after each/daily usage increased from 33.6% to 75.7% (p < .01). Additionally, methods of cleaning and storage of the nebulizer, also improved significantly (p < .01, p < .01). Conclusion Education was highly effective to increase the rate of proper practices for nebulizer hygiene. The necessity of cleaning, disinfection, careful drying, correct storage of the nebulizer parts, and changing the nebulizer equipment within recommended time should be emphasized to CF families regularly.Publication Metadata only Hypoglycemia is common in children with cystic fibrosis and seen predominantly in females(WILEY, 2017) BEREKET, ABDULLAH; Haliloglu, Belma; Gokdemir, Yasemin; Atay, Zeynep; Abali, Saygin; Guran, Tulay; Karakoc, Fazilet; Ersu, Refika; Karadag, Bulent; Turan, Serap; Bereket, AbdullahObjective: To determine the prevalence of hypoglycemia in children and adolescents with cystic fibrosis (CF) in 2-hour oral glucose tolerance test (OGTT) and continuous glucose monitoring (CGM) under free-living conditions. Research Design and Methods: Height, weight, body mass index (BMI), hemoglobin A1c (HbA1c), and Forced expiratory volume (FEV1%) were measured in children with CF (aged 5-18 years). Following OGTT, CGM was installed for 3 days. The total hypoglycemic and hyperglycemic time (%) during 3 days was measured. Subjects were categorized according to hypoglycemic time < 3% (hypo -) and = 3% (hypo +). Each category was further divided according to hyperglycemic time < 3% (hyper -) or = 3% (hyper +). Results: OGTT and CGM were sequentially performed in 45 CF patients. The frequency of hypoglycemia in OGTT and hypoglycemic time >= 3% of CGM were 13.3% and 27.5%, respectively. After 5 cystic fibrosis-related diabetes (CFRD) subjects were excluded, the number of subjects in each subgroup was 17 (hypo-/hyper-), 12 (hypo-/hyper+), 6 (hypo+/hyper-), and 5 (hypo+/hyper+). Significantly higher insulin at 120 minutes was observed in OGTT in (hypo +/hyper-), as compared with subgroup (hypo-/hyper-) (P = .018). Total insulin levels were also significantly higher in (hypo+/hyper-), than (hypo-/hyper-), but were similar to those in the healthy control group (P = .049 and P =.076, respectively). There was a female predominance in hypoglycemic subjects both in OGTT and subgroup (hypo+/hyper-) in the CGM group (P = .033 and P = .033, respectively). FEV1 was significantly lower in hypo + group as a whole, and (hypo+/hyper+) subgroup than in (hypo-/hyper-), (P = .044 and P = .042, respectively); the difference was independent of body mass index-standard deviation score (BMI-SDS) (P = .15 and P = .12, respectively). Conclusion: The frequency of hypoglycemia in children with CF was higher in CGM than that in OGTT. Insulin secretion was delayed and total insulin levels increased in the hypoglycemic patients. Glucose instability/hypoglycemia is associated with poorer lung function in patients with CF, independent of nutritional status.Publication Metadata only Caregiver burden in children with cystic fibrosis and primary ciliary dyskinesia(WILEY, 2019) KARADAĞ, BÜLENT TANER; Coskun, Ozge Kenis; Atalay, Kardelen Gencer; Erdem, Ela; Karadag-Saygi, Evrim; Gokdemir, Yasemin; Karadag, BulentIntroduction Caregiver burden impacts both the social and economic framework of society. Cystic fibrosis (CF) causes significant caregiver burden, but the current data is scarce. In the case of primary ciliary dyskinesia (PCD), even less is known. This study aims to compare the caregiver burden of the parents of patients with CF and PCD. Methods Patients with CF and PCD between the ages of 6 to 13 and their parents were included. Patients' clinical information and parents' demographics were recorded. Caregiver burden was measured with Zarit Caregiver Burden Scale (ZCB), while the quality of life (QOL) was measured with CFQOL-revised (CFQOL-R) and PCD QOL questionnaire as the patients' age and diagnosis indicated. Results A total of 63 patients, 44 with CF (69%) and 85 caregivers (35 mothers, 6 fathers, and 22 mother-father dyads) participated in the study. Caregiver burden was significantly higher in mothers of the CF group with a mean ZCB of 30.5 +/- 10.7 when compared to the PCD group with a mean ZCB of 21.93 +/- 8.26 (P = .006). This was similar in fathers with mean ZCB of 27.5 +/- 9.21 in the CF group and 20.36 +/- 7.43 in the PCD group (P = .03). In correlation analyses, mothers' caregiver burden moderately and inversely correlated with CFQOL-R subscales in the CF population. Conclusion Caregiver burden is significantly higher in the CF population when compared to PCD. It is correlated with pulmonary functions and QOL in patients with CF.Publication Metadata only Screening of depression and anxiety in adolescents with cystic fibrosis and caregivers in Turkey by PHQ-9 and GAD-7 questionnaires(WILEY, 2021) KARADAĞ, BÜLENT TANER; Mursaloglu, H. Hakan; Yilmaz Yegit, Cansu; Ergenekon, Almala P.; Gokdemir, Yasemin; Eralp, Ela E.; Karakoc, Fazilet; Nasr, Samya Z.; Karadag, Bulent T.Background Depression and anxiety symptoms in patients with cystic fibrosis (CF) and their caregivers are 2-3 times higher than in the normal population. This study aims to evaluate the frequency and severity of depression and anxiety symptoms and to determine possible risk factors in CF patients and their mother and/or fathers at Marmara University CF center. Methods The study included 132 CF patients who were followed up at our CF center. Patient Health Questionnaire (PHQ-9) and the Generalized Anxiety Disorder Questionnaire (GAD-7) were used to screen depression and anxiety. The questionnaires were completed by 50 CF patients (aged 12-17 years) and 132 parents of patients (aged 0-17 years). Results While moderate to severe depressive symptoms were seen in 26% of patients, 33.7% of mothers and 14.6% of fathers; moderate to severe anxiety were present in 18%, 21.8% and 8.5%, respectively. None of the demographic characteristics was identified as a predictor of depression or anxiety. GAD-7 scores have shown a higher prevalence of anxiety in mothers of patients with chronic methicillin-resistant Staphylococcus aureus (p = .034). Additionally, hospitalization in the last 12 months was significantly correlated with higher PHQ-9 scores in fathers (p = .043). Analysis of patients' adherence to medical treatment and airway clearance showed higher depression and anxiety in mothers of the nonadherent group (p = .002). Conclusion Depression and anxiety were common in CF patients and their parents. These results illustrate the importance of depression/anxiety screening and psychosocial support for the CF patient and their parents.Publication Metadata only Other risk factors associated with mortality in moderate and severe cystic fibrosis patients(TURKISH PEDIATRICS ASSOC, 2012-12-15) KARADAĞ, BÜLENT TANER; Gokdemir, Yasemin; Erdem, Ela; Akpinar, Ihsan Nuri; Ersu, Refika; Karadag, Bulent; Karakoc, FaziletAim: Severe pulmonary disease is responsible for over 80% of deaths associated with cystic fibrosis. Our aim was to evaluate the other risk factors associated with mortality in patients with cystic fibrosis who have moderate and severe lung disease. Material and Method: Among 200 patients with cystic fibrosis who were followed up in our clinic, 35 patients with moderate and severe lung disease (%FEV1 predicted <= 60) were included in the study. Demographic data, pulmonary function tests, high resolution thorax tomography, modified Bhalla score, blood gas analysis and nutritional status of the patients were evaluated. Results: Requirement for respiratory support was 31.4% in moderate and severe lung disease. Lower pulmonary function, requirement for non invasive ventilation, presence of diabetes mellitus, a SaO(2) value of <95, a value of pCO(2) >50 mm Hg and requirement for frequent IV antibiotics were significantly related with increased mortality. Conclusions: This is a single centre study conducted in patients with cystic fibrosis who had moderate and severe lung disease and who had not received lung transplantation. We evaluated the clinical outcome and other factors that were associated with mortality. (Turk Arch Ped 2012; 47:267-71)