Person: GÖKDEMİR, YASEMİN
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GÖKDEMİR
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YASEMİN
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Publication Metadata only Akciğer transplantlı kistik fibroz hastasında kardiyopulmoner egzersiz testi(2022-05-12) GÖKDEMİR, YASEMİN; ERDEM ERALP, ELA; KASIMAY ÇAKIR, ÖZGÜR; KAHRAMAN, MERVE MERİÇ; Kahraman M. M., Mermer K. S., Gökdemir Y., Erdem Eralp E., Kasımay Çakır Ö.Giriş: G2P1A0 anneden term doğan, ablası kistik fibrozdan(KF) 6 yaşında ex, 50 günlükkentanı alan hasta. Anne baba sağ sağlıklı. Mayıs 2019’da 14 yaşında Amerika’da bilateralakciğer transplantı olmuş. Ek hastalığı yok.Amaç: Hasta polikliniğimize kardiyopulmoner egzersiz testi için başvurdu.Yöntem: Hastaya koşu bandı ergometresinde modifiye Bruce protokolü ile kademeli artankardiyopulmoner egzersiz testi(KPET) uygulanmıştır. Otomatik bir tansiyon aleti ile kanbasıncı ölçülmüştür. Hastanın yüzüne yerleştirilen maske ve önündeki türbün aracılığıyla hersoluk havasında tüketilen oksijen, üretilen karbondioksit, ventile edilen hava miktarı, solunumsıklığı, solunum rezervi, ventilatuar eşik değeri, nabız oksijeni, kalp hızı, hız-basınç ürünügibi kardiyopulmoner sağlamlığı gösteren belirteçler değerlendirilmiştir. Hastanınantropometrik ölçümleri Tanita 418 cihazıyla alınmıştır.Bulgular: Hastanın ölçümlerinde boyu 165 cm(10.p), kilosu 69.5 kg(50-75.p), BMI:25.5(85-95 p) olarak kaydedilmiştir. Hastanın yağ yüzdesi %25.9, yağ kütlesi 18 kg, yağsız vücutkütlesi ise 51.5 kg olarak ölçülmüştür. Egzersiz testinde VO2pik 30 ml/kg/dk(%49) olarak hesaplanmıştır. Maksimum kalp hızı 148 vuru/dk’dır(%81). Dakikadaki ventilasyonu 89.3 lt,soluk sayısı 48/dk, RER değeri 1.22dir. VE/VO2 değeri 39.5. VE/VCO2 değeri ise 32.4 olaraktespit edilmiştir. Ventilatuar eşik sırasındaki tüketilen oksijen 17 ml/kg/dk (beklenenin%34’ü, ulaşılanın %57’si), kalp hızı 125 vuru/dk’dır(%68).Tartışma: KPET KF hastalarında diagnostik, prognostik ve fonksiyonel verileri göstermesiaçısından çok kıymetlidir. VO2maks, VE/VO2, VE/VCO2 değerleri özellikle 10 yıllık izlemdeölüm ya da transplanta gidişle ilişkilendirilmiştir. KF hastalarında yapılan çalışmada dahayüksek aerobik sağlamlığa sahip olanlar diğer risk faktörlerinin ayarlaması yapıldıktan sonradahi daha düşük aerobik sağlamlığa sahip olanlarla karşılaştırıldıklarında hayatta kalmaşansları 3 kata kadar fazla bulunmuş. 27 pediatrik KF hastasının katıldığı başka çalışmadaVO2pik 32 ml/kg/dk’dan düşük olan hastalar yüksek mortaliteyle ilişkilendirilirken, pik VO2değeri 45 ml/kg/dk olanlar artmış yaşam süresiyle ilişkilendirilmiştir. Pre ve post-transplantdöneminde KPET’in kullanımı için rehberler eksiktir. Özellikle post-transplant dönemindetestin zamanının optimizasyonu ve çıktılarının prognostik değerleri açısından daha çok veriyeihtiyaç vardır.Anahtar Sözcükler: Kardiyopulmoner Egzersiz Testi, Kistik Fibroz, VO2maksPublication 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 Collaboration between two CF centers; one in USA and one in Turkey before and during CoV2 pandemic(2022-10-01) GÖKDEMİR, YASEMİN; ERDEM ERALP, ELA; Nasr S. Z., Gökdemir Y., Erdem E., Karakoc F., Ergenekon P., Tapley C., Dagher S., Bouma S., Coşkun Ö. K., Kocamaz D., et al.To address the discrepancy in the quality of care and outcomes between cystic fibrosis centers (CFCs) in high-income countries and limited resources countries (LRCs), a collaboration between our team at the University of Michigan CFC (UMCFC) and a CF center in Turkey (Marmara University CFC [MUCFC], Istanbul) was established. The collaboration included evaluation of all aspects of care and initiation of quality improvement (QI) measures. Teaching and implementing QI tools has led to start of improvement in MUCFC care. Close monitoring and sharing resources like UMCFC algorithms, protocols, and QI processes were done.Publication Open Access Depression, anxiety, and sleep quality of caregivers of children with spinal muscular atrophy(2023-03-01) ERGENEKON, ALMALA PINAR; YILMAZ YEĞİT, CANSU; SELÇUK, MERVE; KARABULUT, ŞEYDA; ÖZTÜRK THOMAS, GÜLTEN; ERDEM ERALP, ELA; ÜNVER, OLCAY; KARADAĞ, BÜLENT TANER; GÖKDEMİR, YASEMİN; ERGENEKON A. P., Gumus Z., YILMAZ YEĞİT C., Cenk M., Gulieva A., Kalyoncu M., SELÇUK M., KARABULUT Ş., ÖZTÜRK G., ERDEM ERALP E., et al.BackgroundThe aim of this study was to evaluate the prevalence of anxiety, depression, sleep, and associated factors in caregivers of children with spinal muscular atrophy (SMA). Materials and MethodsBeck Depression Inventory (BDI), the State-Trait Anxiety Inventory-State (STAI-S), the State-Trait Anxiety Inventory-Trait (STAI-T), and Pittsburgh Sleep Quality Index (PSQI) were used to assess the anxiety, depression, and sleep quality of the caregivers of children with SMA. Higher scores indicated worse outcome for all three questionnaires. ResultsFifty-six caregivers of children with SMA were included in the study. Median age of children was 6 (3.2-10) years and mean age of the caregivers was 37.0 +/- 6.5 years. Median scores of the BDI, STAI-S, STAI-T, and PSQI were 12 (7.2-17), 35.5 (31-44), 40.5 (35-48), and 7.0 (5.0-10.0), respectively. There was a positive correlation between BDI and PSQI scores (p < 0.05). There was a negative correlation between the age of the caregivers and PSQI, BDI, STAI-T scores (p = 0.01, r = -0.341; p = 0.006, r = -0.364; p = 0.003, r = -0.395, respectively). There was a negative correlation between the age of the patients and the PSQI scores of the caregivers (p = 0.01, r = -0.33). There was a negative correlation between BDI scores and household income (p = 0.01, r = -0.34). ConclusionCaregivers of children with SMA had elevated depression and anxiety levels and they also had decreased sleep quality. Economic and social support resources are needed to help caregivers of those children.Publication Open Access Electronic home monitoring of children with cystic fibrosis to detect and treat acute pulmonary exacerbations and its effect on 1-year FEV1(2023-01-01) UZMANOĞLU, MUSTAFA SELÇUK; ERGENEKON, ALMALA PINAR; GÖKDEMİR, YASEMİN; ERDEM ERALP, ELA; Yanaz M., Yilmaz Yegit C., Gulieva A., Kalyoncu M., UZMANOĞLU M. S., Uzunoglu B., Tastan G., ERGENEKON A. P., GÖKDEMİR Y., ERDEM ERALP E., et al.Background: We aimed to investigate the effect of the use of electronic home spirometry in children with cystic fibrosis (CF) on 1-year FEV1 (% predicted, pp) change. Methods: This is a randomised, one-year prospective study including children with CF between 6 and 18 years of age. Subjects were randomised into home spirometry group (HSG) and usual care group (UCG). Children in HSG performed two pulmonary function tests (PFT) per week. Data regarding acute pulmonary exacerbations (PEx) was obtained from patients’ records. At baseline and 12th month, health related quality of life questionnaire for CF patients (CFQ-R) and lung clearance index (LCI) were performed. Results: Sixty children were recruited with a median (IQR) age of 13.3 (11.4–15.4) years. Absolute change in FEV1pp from baseline to 12th month as median (IQR) was +1% (-6.75–9.75) in HSG and -2.50% (-7.50–3.25) in UCG (p = 0.10). Sensitivity analysis including only adherent children in HSG (n = 22), yielded an increase of 5% (-3.50–12) in HSG and a decrease of 2.50% (-7.50–3.25) in UCG (p = 0.009). A total of 29 (96.7%) subjects in HSG and 23 (76.7%) in UCG had PEx (p = 0.05). Absolute change in median (IQR) LCI2.5 from baseline to the 12th month was -1.6 [-2.9–0] (p<0.001) in HSG and -1.5 [-2.8-(-0.6)] (p<0.001) in UCG (p = 0.94). There was a significant increase in the social domain of the CFQ-R in HSG (from 59.1 to 76.2, p = 0.01). Conclusions: Electronic home monitoring of children with CF by spirometry may result in improvement in lung function.Publication Metadata only Nöromüsküler Hastalığı olan çocuklarda solunum sisteminin değerlendirilmesinde kullanılan testler(Özel Ofset Basın Yayın Matbaa, 2022-09-01) GÖKDEMİR, YASEMİN; Yılmaz Yeğit C., Gökdemir Y., Karakoç F.Publication Metadata only The utility of risk assessment tools for acute pulmonary embolism in children(2022-09-01) ERGENEKON, ALMALA PINAR; YILMAZ YEĞİT, CANSU; SELÇUK, MERVE; TRUE, ÖMER; ERDEM ERALP, ELA; GÖKDEMİR, YASEMİN; KARADAĞ, BÜLENT TANER; ERGENEKON A. P. , YILMAZ YEĞİT C., Cenk M., Gulieva A., Kalyoncu M., SELÇUK M., DOĞRU Ö., ERDEM ERALP E., GÖKDEMİR Y., Karakoc F., et al.Background and Aim Pulmonary embolism (PE) is a potentially life-threatening disease in children. The objective of the study is to evaluate the utility of adult-based pulmonary embolism rule-out criteria (PERC), Pediatric PE Model, and D-dimer in the diagnosis of PE in children. Material and Methods The study consisted of patients under 18 years of age who were consulted to the Pediatric Pulmonology Clinic for the evaluation of PE. Patients were divided into two groups based on the confirmation of PE. The group with the presence of PE (n = 20) consisted of children who were diagnosed with PE. The group with the absence of PE (n = 28) consisted of children with clinically suspected PE but negative diagnostic imaging. Adult validated clinical decision PERC rule and Pediatric PE Model were retrospectively applied to the patients. Results In the study, PERC demonstrated a sensitivity of 60% and a specificity of 46% for the diagnosis of PE in children. When PE Model was evaluated for the children, it was found a 50% sensitivity and 75% specificity. Combining PE Model and PERC rule with D-dimer did not increase the specificity and sensitivity. Smoking was found to be relevant for PE in the childhood. Twenty-five percent of the patients had a genetic tendency for PE. All of the patients had an underlying disease as well. Conclusion None of the current risk assessment tools (PE Model, PERC, D-dimer) were found to be accurate in predicting PE. Further larger population studies are still required to develop a better diagnostic approach.Publication Open Access The outcome of two SMA cases treated with nusinersen at seven hours and at three days of life: the earliest ever(2022-07-01) ÜNVER, OLCAY; ESİM BÜYÜKBAYRAK, ESRA; GÖKDEMİR, YASEMİN; MEMİŞOĞLU, ASLI; Ünver O., Çelik T., Memişoğlu A., Büyükbayrak E., Tülin Şimşek F., Öztürk G., Eser G., Saygı E. K., Gökdemir Y., Aktekin B., et al.New molecular therapies are available for the treatment of spinal muscular atrophy (SMA) but early intervention is required. We report two cases that were diagnosed prenatally, where treatment with nusinersen was initiated within 7 h and three days respectively. The children were followed up for 13 months and almost six years respectively. Both children have developed within entirely normal centiles, indicating that initiating treatment immediately after birth, as in these cases, is essential for a good outcome.Publication Open Access Is the pediatric sleep questionnaire sensitive for sleep-disordered breathing in children with complex chronic disease(2023-01-01) GÖKDEMİR, YASEMİN; ERDEM ERALP, ELA; Kalyoncu M., Namlı N., Yegit C. Y., Yanaz M., Gulieva A., Ergenekon A. P., Selçuk M., Atağ E., İkizoğlu N. B., Sabancı M., et al.Purpose: Sleep-disordered breathing (SDB) is a disease defined by breathing or breathing irregularities while asleep. The current study examines the association between results of polysomnography (PSG) and the Pediatric Sleep Questionnaire (PSQ), and the specificity and sensitivity of the PSQ for obstructive sleep apnea (OSA) in patients with chronic illnesses. Methods: Demographic and clinical attributes, in addition to PSQ and PSG outcomes were examined retrospectively among patients who underwent polysomnography (PSG) at our facility between 2012 and 2021. Results: Of 745 patients included in the study, 462 (62%) were male. The median age was 81 months (34–151 months). 117 of the patients (15/8%) had chronic lung disease, and 80 (10.7%) had cerebral palsy. The most common indications for PSG were symptoms of OSA (n = 426; 57.1%). According to obstructive apnea-hypopnea index (AHI), 361 patients (48.5%) had normal PSG. The median PSQ score was 0.40 (0.22–0.57). The sensitivity and specificity of the PSQ were 71.8% and 40.4%, respectively, for individuals aged 2 to 18 years. Among the disease subgroups, the cerebral palsy group had the highest sensitivity of PSQ (88.8%) for diagnosis of OSA. Conclusion: Questionnaires for evaluating SDB are not sensitive or specific for identification of OSA in children with chronic conditions, and PSG remains the best method.Publication Open Access Childhood interstitial lung disease in Turkey: first data from the national registry(2023-01-01) GÖKDEMİR, YASEMİN; KARADAĞ, BÜLENT TANER; Nayır-Büyükşahin H., EMİRALİOĞLU ORDUKAYA N., KILINÇ SAKALLI A. A., Girit S., Yalçın E., ŞİŞMANLAR EYÜBOĞLU T., ÇOBANOĞLU F. N., Cinel G., Pekcan S., GÖKDEMİR Y., et al.Abstract: The childhood interstitial lung diseases (chILD) Turkey registry (chILD-TR) was established in November 2021 to increase awareness of disease, and in collaboration with the centers to improve the diagnostic and treatment standards. Here, the first results of the chILD registry system were presented. In this prospective cohort study, data were collected using a data‐entry software system. The demographic characteristics, clinical, laboratory, radiologic findings, diagnoses, and treatment characteristics of the patients were evaluated. Clinical characteristics were compared between two main chILD groups ((A) diffuse parenchymal lung diseases (DPLD) disorders manifesting primarily in infancy [group1] and (B) DPLD disorders occurring at all ages [group 2]). There were 416 patients registered from 19 centers. Forty-six patients were excluded due to missing information. The median age of diagnosis of the patients was 6.05 (1.3–11.6) years. Across the study population (n = 370), 81 (21.8%) were in group 1, and 289 (78.1%) were in group 2. The median weight z-score was significantly lower in group 1 (− 2.0 [− 3.36 to − 0.81]) than in group 2 (− 0.80 [− 1.7 to 0.20]) (p < 0.001). When we compared the groups according to chest CT findings, ground-glass opacities were significantly more common in group 1, and nodular opacities, bronchiectasis, mosaic perfusion, and mediastinal lymphadenopathy were significantly more common in group 2. Out of the overall study population, 67.8% were undergoing some form of treatment. The use of oral steroids was significantly higher in group 2 than in group 1 (40.6% vs. 23.3%, respectively; p = 0.040). Conclusion: This study showed that national registry allowed to obtain information about the frequency, types, and treatment methods of chILD in Turkey and helped to see the difficulties in the diagnosis and management of these patients. What is Known: • Childhood interstitial lung diseases comprise many diverse entities which are challenging to diagnose and manage. What is New: • This study showed that national registry allowed to obtain information about the frequency, types and treatment methods of chILD in Turkey and helped to see the difficulties in the diagnosis and management of these patients. Also, our findings reveal that nutrition should be considered in all patients with chILD, especially in A-DPLD disorders manifesting primarily in infancy.