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  • Küçük Resim Yok
    Öğe
    Assessment of right ventricular function in patients with pulmonary arterial hypertension-congenital heart disease and repaired and unrepaired defects: Correlation among speckle tracking, conventional echocardiography, and clinical parameters
    (2020) Kemal, Hatice S.; Kayıkçıoğlu, Meral; Nalbantgil, Sanem; Can, Levent Hürkan; Moğulkoç, Nesrin; Kültürsay, Kadir Hakan
    Objective: The purpose of this study is to compare the analysis of right ventricular (RV) free wall strain via 2D speckle tracking echocardiography with conventional echocardiography and clinical parameters in patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) receiving specific treatment. This study also aims to describe the differences between patients with repaired and unrepaired defects. Methods: This prospective study included 44 adult patients with PAH-CHD who were receiving PAH-specific treatment in a single center. This study excluded patients with complex congenital heart disease. The authors studied the conventional echocardiographic parameters, such as RV fractional area change (FAC), tricuspid annular plane systolic excursion (TAPSE), right atrial (RA) area, Tricuspid S', and hemodynamic parameters, such as functional class, 6-minute walking distance (6MWD), and N-terminal pro-brain natriuretic peptide (NT-proBNP) levels. Results: The mean age of participants was 33.8±11.6 years, and 65.9% of participants were female. The mean RV free wall strain was ?14.8±4.7%. Majority of the patients belonged to WHO functional class 2 (61.4%) with a mean NT-proBNP level of 619.2±778.4 and mean 6MWD of 400.2±86.9 meters. During the follow-up of 30.8±9.0 months, 6 patients (13.6%) developed clinical right heart failure, whereas 9 (20.5%) of them died. There was a positive and significant correlation between RV free wall strain and WHO functional class (r=0.320, p=0.03), whereas there was a negative correlation between RV free wall strain and FAC (r=?0.392, p=0.01), TAPSE (r=?0.577, p=0.0001), and Tricuspid S' (r=?0.489, p=0.001). There was no significant correlation of RV free wall strain with either RA area or 6MWD. Patients with repaired congenital heart defects had worse RV functional parameters and RV free wall strain than patients with unrepaired defects. Conclusion: The assessment of RV free wall strain via 2D speckle tracking echocardiography is a feasible method and correlates well with conventional echocardiography and clinical parameters in patients with PAH-CHD receiving specific treatment.
  • Küçük Resim Yok
    Öğe
    Klinik ve subklinik alveolitis
    (Ege Üniversitesi, 1991) Moğulkoç, Nesrin
    ÖZET İnterstisyel akciğer hastalıklarının başlangıç dönemi olarak kabul edilen "alveolitis" son 10 yıl içinde tanımlanmış bir kavramdır. Alveolitis, alveol inflamasyonu anlamına gelmekte ve alveoller içinde, nötrofil, lenfosit ve eosinofil gibi infiamatuar hücrelerin sayı ve aktivitelerinin artışı ile karakterize edilmektedir. Bronkoalveoler Lavaj (BAL) sıvısı incelemeleri ile ortaya koyulan bu tanımlama, in- testisyel akciğer hastalıklarının patogenez, tanı ve izlenmelerine yeni ve önemli boyutlar getirmiştir. Daha yeni bir kavram olan "subklinik alveolitis" ise, akciğerlerin rutin radyolojik incelemeler, fizik muayene, solunum fonksiyon testleri ve arteryel kan gazlan an alizi ile normal bulunmasına rağmen, BAL incelemeleri ile alveolitis saptanması anlamına gelmektedir. Başka dokuları veya organları tu tan bir çok sistemik hastalıkta, akciğerlerin de olaya inflamasyon düzeyinde katıldığını gösteren "subklinik alveolitis" bulgusunun, ge lecekteki bir kronik interstisyel akciğer hastalığının ön belirtisi olup olmadığı henüz araştırma aşamasındadır. Kendi toplumumuzdaki, interstisyel akciğer hastalığı bulunan veya bu hastalığı oluşturma potansiyeline sahip bir sistemik hastalığı olan olgulardaki klinik ve subklinik alveolitis! göstermek amacıyla yapılan araştırmamızda ayrıca 12 sağlıklı kişinin BAL sıvısı hücre yüzdeleri saptanarak normal değerlerimize bir örnek verilmiştir. E.Ü.T.F. Göğüs Hastalıkları Ana Bilim Dalı ve Romatoloji bilim dalınca tetkik edilerek tanılan koyulan beş değişik hastalık grubunu 37(sarkoidosis, İAF, RA, SLE ve PSS) kapsayan olgular, BAL sıvısı hücre analizi ve beraberinde klinik, radyolojik ve fonksiyonel değerlendirmelerle incelenmişlerdir. Bu olgular arasında belirlenen 107 klinik ve 36 subklinik alveolitisli hastamızın BAL sıvısı hücre yüzdeleri bildirilmiştir. Ayrıca her iki grup arasında, hücre yüzdeleri karşılaştırılmıştır. Sonuçta alveolit tanısı koyduran inflamatuar hücre oranındaki artışın subklinik olgularda da hemen klinik alveo litisli olgular düzeyindeki kadar yüksek olduğu dikkati çekmiştir. immünolojik sistemik hastalığı bulunan kişilerde belirlene cek bir subklinik alveolitisin, gelecekte oluşacak interstisyel akciğer hastalığının habercisi olma potansiyeli vardır. Ancak bu ko nudaki kesin bilgiler, subklinik alveolitisli olguların yakından izlen mesi ile elde edilebilecektir. 38
  • Küçük Resim Yok
    Öğe
    Sequencing of mutations in the serine/threonine kinase domain of the bone morphogenetic protein receptor type 2 gene causing pulmonary arterial hypertension
    (2016) Mutlu, Zeynep; Kayıkçıoğlu, Meral; Nalbantgil, Sanem; Vuran, Özcan; Kemal, Hatice; Moğulkoç, Nesrin; Onay, Hüseyin
    Objective: Germline mutations in the bone morphogenetic protein receptor type-2 (BMPR2) gene are considered to be a major risk factor for pulmonary arterial hypertension (PAH). BMPR2 mutations have been reported in 10%-20% of idiopathic PAH and in 80% of familial PAH cases. the aim of this study was to evaluate the frequency of mutations in the serine/threonine kinase domain of the BMPR2 gene in a group of patients from a single PAH referral center in Turkey.Methods: This cross-sectional study used a DNA-sequencing method to investigate BMPR2 mutations in the serine-threonine-kinase domain in 43 patients diagnosed with PAH [8 with idiopathic PAH and 35 with congenital heart disease (CHD)] from a single PAH referral center. Patients were included if they had a hemodynamically measured mean pulmonary arterial pressure of >25 mm Hg with a mean pulmonary capillary wedge pressure of <=15 mm Hg. Patients with severe left heart disease and/or pulmonary disease that could cause pulmonary hypertension were excluded. Associations between categoric variables were determined using the chi-square test. Differences between idiopathic and CHD-associated PAH groups were compared with the unpaired Student's t-test for continuous variables. Results: We detected a missense mutation, [p.C347Y (c.1040G>A)], in one patient with idiopathic PAH in exon 8 of the BMPR2 gene. the mutation was detected in a 27-year-old female with a remarkable family history for PAH. She had a favorable response to endothelin receptor antagonists. No mutations were detected in the exons 5-11 of the BMPR2 gene in the PAH-CHD group.Conclusion: A missense mutation was detected in only one of the eight patients with idiopathic PAH. the BMPR2 missense mutation rate of 12.5% in this cohort of Turkish patients with idiopathic PAH was similar to that seen in European registries. the index patient was a young female with a family history remarkable for PAH; she had a good long-term response to PAH-specific treatment, probably due to the early initiation of the treatment. Genetic screening of families affected by PAH might have great value in identifying the disease at an early stage.