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 Metadata only WS07.04Electronic home monitoring of children with cystic fibrosis to detectand treat acute pulmonary exacerbations and their effect on one yearFEV1 loss(2022-06-16) ERGENEKON, ALMALA PINAR; GÖKDEMİR, YASEMİN; ERDEM ERALP, ELA; CENK M., YILMAZ YEĞİT C., Guliyeva A., Kalyoncu M., SELÇUK M., Uzunoğlu B., Taştan G., ERGENEKON A. P., GÖKDEMİR Y., ERDEM ERALP E., et al.Publication Metadata only Coping and psychopathology in children with malignancy and bronchiectasis(WILEY, 2020) PERDAHLI FİŞ, NEŞE; Bikmazer, Alperen; Orengul, Abdurrahman Cahid; Buyukdeniz, Ayse; Okur, Fatma Visal; Gokdemir, Yasemin; Fis, Nese PerdahliAim: We aimed to evaluate the coping styles and social support perceived by the children with two different chronic diseases (cancer and bronchiectasis), their mothers' coping styles and compare them with a control group without any chronic physical or psychiatric disorder. Methods: Our sample consisted of 114 children and adolescents, with an age range from 9 to 15 years. The data were collected by using schedule for affective disorders and schizophrenia for school-age children-present and lifetime version, kid-coping orientation to problems experienced (Kid-COPE), social support appraisals scale (SSAS), and COPE. Results: All three groups were similar with respect to age and sex distribution. Around 50% to 60% of the children in both patient groups had a psychiatric diagnosis. Remarkably, 30% of the children had an internalizing disorder. The most commonly used coping style by the mothers was religious coping in all groups. Kid-COPE scores did not significantly differ between groups. The scores on Family and Friend subscales of SSAS in the bronchiectasis group were significantly lower when compared with those of participants in hematology-oncology and control groups. Conclusion: Chronic medical illnesses may have a similar psychological impact on children regardless of disease-specific clinical presentations and outcomes. Future studies need to focus on identifying protective and risk factors that potentially mediate psychosocial well-being.Publication Metadata only Bronchoscopic evaluation of unexplained recurrent and persistent pneumonia in children(WILEY-BLACKWELL, 2013) KARADAĞ, BÜLENT TANER; Gokdemir, Yasemin; Cakir, Erkan; Kut, Arif; Erdem, Ela; Karadag, Bulent; Ersu, Refika; Karakoc, Faziletd Background: Persistent or recurrent pneumonia in children can pose a significant challenge to paediatricians and respiratory physicians. Aim: The aim of this study is to determine the role of flexible bronchoscopy (FB) in evaluation of recurrent or persistent pneumonia that remain otherwise unexplained by non-invasive diagnostic tests in children. Methods: Retrospective evaluation of patients who underwent FB with an indication of recurrent or persistent pneumonia from 1997 to 2011. Results: Among 2600 FB procedures, 434 (17%) were performed with the indication of recurrent or persistent pneumonia. There were 237 (54%) boys. Median age at presentation was 84 months, and median duration of symptoms was 9 months. FB led to specific diagnosis in 33% of the cases. The most common diseases diagnosed by FB were malacia disorders (n: 32, 7%), aspirated foreign body (n: 30, 7%), endobronchial tuberculosis (n: 20, 5%), congenital airway anomalies (n: 14, 3%), mucus plugs (n: 14, 3%), pulmonary haemosiderosis (n: 12, 3%) and middle lobe syndrome (n: 11, 3%). During FB, only 6% of the patients had minor complications such as transient hypoxia, stridor and tachycardia. Conclusions: In our study, FB proved to be a safe and effective tool in evaluation of children with persistent or recurrent pneumonia. FB is indicated for children with recurrent or persistent pneumonia where the underlying diagnosis remains unclear even after non-invasive diagnostic tests.Publication Open Access A Rare Cause of Hypophosphatemia: Raine Syndrome Changing Clinical Features with Age(SPRINGER, 2020-07) DAĞÇINAR, ADNAN; Eltan, Mehmet; Alavanda, Ceren; Yavas Abali, Zehra; Ergenekon, Pinar; Yalindag Ozturk, Nilufer; Sakar, Mustafa; Dagcinar, Adnan; Kirkgoz, Tarik; Kaygusuz, Sare Betul; Gokdemir, Yasemin; Elcioglu, Huriye Nursel; Guran, Tulay; Bereket, Abdullah; Ata, Pinar; Turan, SerapRaine Syndrome (RS) is caused by biallelic loss-of-function mutations in FAM20C gene and characterized by hypophosphatemia, typical facial and skeletal features. Subperiosteal bone formation and generalized osteosclerosis are the most common radiological findings. Here we present a new case with RS. A 9-month-old male patient on a home-type ventilator was referred for hypophosphatemia. He was born with a weight of 3800 g to non-consanguineous parents. Prenatal ultrasound had demonstrated nasal bone agenesis. A large anterior fontanel, frontal bossing, exophthalmos, hypoplastic nose, high arched palate, low set ears, triangular mouth, and corneal opacification were detected on physical examination. Serial skeletal X-rays revealed diffuse osteosclerosis at birth which was gradually decreased by the age of 5 months with subperiosteal undermineralized bone formation and medullary space of long bone could be distinguishable with bone-within-a-bone appearance. At 9 months of age, hand X-ray revealed cupping of the ulna with loose radial bone margin with minimal fraying and osteopenia. Cranial computed tomography scan showed bilateral periventricular calcification and hydrocephalus in progress. The clinical, laboratory, and radiological examinations were consistent with RS. Molecular analyses revealed a compound heterozygous mutation in FAM20C gene (a known pathogenic mutation, c.1645C > T, p.Arg549Trp; and a novel c.863 + 5 G > C variant). The patient died due to respiratory failure at 17 months of age. This case allowed us to demonstrate natural progression of skeletal features in RS. Furthermore, we have described a novel FAM20C variant causing RS. Previous literature on RS is also reviewed.Publication Metadata only Evaluation of caregiver burden, somatization and sleep quality in mothers of children with cystic fibrosis(2022-12-08) KARAVUŞ, MELDA; GÖKDEMİR, YASEMİN; ERDEM ERALP, ELA; ERGENEKON, ALMALA PINAR; KARADAĞ, BÜLENT TANER; AYVAZ, İLKNUR; HIDIROĞLU, SEYHAN; Ayvaz İ., Karavuş M., Hıdıroğlu S., Atasoy A., Karagöz D. C. , Baştuğ R. E. , Ladikli Ş. B. , Gökdemir Y., Erdem Eralp E., Ergenekon A. P. , et al.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 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 Metadata only Ev içi temaslı beş çocuk olguda çoklu ilaca dirençli tüberkülozun yönetimi(2022-03-17) ERDEM ERALP, ELA; GÖKDEMİR, YASEMİN; KEPENEKLİ KADAYİFCİ, EDA; Parlak B., Yılmaz D., Ergenç Z., Yılmaz S., Aslan S., Babalık A., Erdem Eralp E., Gökdemir Y., Kepenekli Kadayifci E.