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Öğe Acquired Hemophilia A In Adults: A Multicenter Study from Turkey(Springer India, 2022) Davulcu, Eren Arslan; Demirci, Zuhal; Yilmaz, Umut; Ar, Muhlis Cem; Teke, Hava Uskudar; Karakus, Volkan; Ciftciler, RafiyeAcquired hemophilia A (AHA) is a rare disease caused by autoantibodies inhibiting factor VIII (FVIII) activity. Although the conditionis usually idiopathic, there may be other underlying diseases. Treatment consists of two steps: treatment of acute bleeding and immunosuppression. In this multicenter study, we aimed to demonstrate the clinical characteristics, management details, and survival of AHA patients in Turkey. Data was collected from eleven centers in Turkey. aPTT, FVIII, FVIII inhibitor, and hemoglobin (HB) levels, mixing test results, and demographics at diagnosis, treatment information, adverse events, bleeding episodes during follow-up, relapses, and outcome were analyzed. Twenty-nine patients were analyzed (58.6% female). No underlying disorder could be detected in 14 patients. The most prevalent etiologies were pregnancy, malignancy and infections. The median FVIII activity and FVIII inhibitor titer at diagnosis were 0.7% (0.0-29.4%) and 32.6 BU (0.6-135.6 BU) respectively. Bleeding was severe in 44.8% of patients. The HB value was significantly lower in patients with severe bleeding. Most of the patients (n = 25, 86.2%) had only one bleeding episode without relapse, three patients (10.3%) had two bleeding episodes, and one patient had more than three bleedings. 21 (75%) patients received hemostatic therapy. The use of recombinant FVIIa was slightly higher than activated prothrombin complex concentrate (15 versus 10 patients). Immunosuppressive treatment was initiated in 26 (93%) patients. Regimens containing steroid, cyclophosphamide, and rituximab in different combinations were the most preferred. The median follow-up period was 13 months (2-156 months). Median overall survival was 154.97 months. Four and six-year survival were 90.9 +/- 0.8% and 77.9 +/- 14.1% respectively. This is a unique study that investigated the demographic characteristics, treatment approaches, and patient survival of AHA in Turkey.Öğe Evaluation of bone mineral density and related parameters in patients with haemophilia: a single center cross-sectional study(E-Century Publishing Corp, 2017) Unal, Hatice Demet Kiper; Ozkan, Melda Comert; Atilla, Fatos Dilan; Demirci, Zuhal; Soyer, Nur; Simsir, Ilgin Yildirim; Omur, Ozgur; Capaci, Kazim; Saydam, Güray; Sahin, FahriHaemophilia has been associated with low bone mineral density (BMD) probably due to some predisposing factors. The aim of this study was to evaluate the relationship between BMD and potential clinical predictors in adult haemophilic patients. Fortynine patients with moderate and severe haemophilia were enrolled. BMD was measured by Dual Energy X-Ray Absorptiometry (DXA) and blood tests were performed for vitamin D, calcium, phosphore, alkaline phosphatase and parathormone levels. Functional Independence Score in Haemophilia (FISH) and Haemophilia Joint Health Score (HJHS) were used to assess musculoskeletal functions. Body mass index (BMI), Hepatitis C virus (HCV)/Human immunodeficiency virus (HIV) seropositivity and smoking status were also recorded. BMD was found lower than expected for reference age in 34.8% of patients of less than 50 years old. In patients older than 50 years, 66.6% of them had osteoporosis and 33.3% of them had normal BMD. FISH score was statistically significant correlated with BMD of total hip (TH) and femur neck (FN) but not with lumbar spine (LS). In eligible patients, there was also a statistically significant correlation between BMD of TH and HJHS. Vitamine D deficiency was common and found in 77.5% of patients, although there was no significant correlation with BMD. Also no correlation was found between BMD and blood tests, HCV/HIV status, BMI and smoking. This study confirmed that patients with haemophilia have an increased prevelance of low BMD even in younger group. Our results showed that there are significant correlations between FISH score and BMD of TH and FN and also between HJHS score and BMD of TH. Thus, using scoring systems may be beneficial as a simple predictors of BMD to reflect the severity of haemophilic arthropathy.Öğe EVALUATION OF SOCIO-DEMOGRAPHIC FACTORS AND COMORBIDITIES IN ADULT HEMOPHILIA PATIENTS(Dokuz Eylul Univ Inst Health Sciences, 2024) Karadag, Fatma Keklik; Demirci, Zuhal; Koseoglu, Fatos Dilan; Saydam, Guray; Sahin, FahriPurpose: The association between socio-demographic factors and hemophilia status with the prevalence of comorbidities was evaluated. Material and Methods: Patients with hemophilia A (n=111) and B (n=24) who completed the questionnaire form about their socio-demographic factors were included in our study. Factor and inhibitor levels, comorbidities, factor replacement therapies, hemophilic arthropathy, viral status and annual bleeding episodes were recorded. Results: The median age was 39 years among 135 hemophilia patients, and 63.1% of all patients had severe hemophilia, which was significantly higher among hemophilia A (p=0.002). Most patients (74.8%) were treated with prophylactic factor replacement therapy. The inhibitor status was positive in 8.9% of all patients. The unemployment rate was found to be 33.3%. Annual bleeding episodes were higher in workers. Most patients (60%) had graduated from at least high school. Patients with severe hemophilia were significantly less educated than those with moderate to mild hemophilia (p=0.045). The prevalence of cardiovascular disease, hypertension, diabetes mellitus, and obesity was 6.7%; 17.8%, 13.3%, and 11.9% respectively. Although there was no association between obesity and annual bleeding episodes, right ankle was the most affected joint in overweight/obese patients. Conclusion: Age -related comorbidities and the relationship between hemophilia status and social life need further investigation.Öğe Hematuria in Adult Hemophilic Individuals: Is It the Only Cause of Hemophilia? A Single-Center Experience(Cig Media Group, Lp, 2023) Tüzün, Betül Kübra; Demirci, Zuhal; Sevgili, Bahar; Saydam, Güray; Şahin, Fahri[No abstract available]Öğe Hemophilia and Gastrointestinal Bleeding: Beyond Hemarthrosis(Cig Media Group, Lp, 2023) Sevgili, Bahar; Demirci, Zuhal; Tekin, Fatih; Saydam, Güray; Sahin, Fahri[No abstract available]Öğe Prognostic impact of next-generation sequencing on myelodysplastic syndrome: A single-center experience(Lippincott Williams & Wilkins, 2024) Bulbul, Hale; Kaya, Ozge Ozer; Karadag, Fatma Keklik; Olgun, Aybuke; Demirci, Zuhal; Ceylan, CengizMyelodysplastic syndromes (MDS) are clinically heterogeneous disorders characterized by peripheral blood cytopenias, poor differentiation, clonal hematopoiesis, and increased risk of developing acute myeloid leukemia (AML). While somatic mutations do not currently feature in prognostic scoring systems, they may impact the clinical phenotype. In recent years, next-generation sequencing (NGS) has enabled the opportunity to identify an increasing number of genetic abnormalities, including recurrent modifications in the TP53, DNMT3A, NRAS, NPM1, RUNX1, and FLT3 genes. Bone marrow aspirate samples of 56 patients with MDS were investigated for mutations using NGS. We compared the relationship between gene mutation status and laboratory characteristics, such as certain cytopenias, the revised international prognostic scoring system, MDS subtypes, karyotypes, AML development, and overall survival. Twenty-one genes were found to have gene mutations, including ASXL1, TET2, SRSF2, EZH2, CSF3R, NRAS, ETV6, SETBP1, RUNX1, DDX41, U2AF1, JAK2, FLT3ITD, SF3B1, DNAMT3A, PHF6, TP53, CEBPA, CBL, IDH2, and GATA2. At least one point mutation occurred in 64.2% of all patients, including 58.3% of those with normal cytogenetics. Thrombocytopenia (P = .016), anemia (P = .018), decreased overall survival (P = .017), and increased AML transformation (P = .023) have been revealed to be linked to non-SF3B1 mutations. MDS are frequently associated with somatic point mutations. According to early findings, NGS panels are extremely effective instruments that provide an entirely new viewpoint on the disease for particular individuals. Future prognostications will depend more on NGS because those who exhibit normal cytogenetics may additionally have gene mutations.Öğe Rare bleeding disorders; Improvement on the amount of collected data as a result of increasing awareness(Wiley, 2018) Atilla, Fatos Dilan; Demirci, Zuhal; Saydam, Güray; Sahin, FahriÖğe Sleeve Gastrectomy in a Severe Hemophilia A Patient: One of the Very Rare Cases(Galenos Yayincilik, 2020) Davulcu, Eren Arslan; Demirci, Zuhal; Firat, Ozgur; Saydam, Guray; Sahin, Fahri[No Abstract Available]
