3 research outputs found
Evaluation of T-cell repertoire by flow cytometric analysis in primary immunodeficiencies with DNA repair defects
No full textThe group of patients with DNA-repair-defects increases susceptibility to infections due to impaired repertoire diversity. In this context, we aimed to investigate the TCRv beta-repertoire by flow cytometric analysis and its correlation with clinical entities in a group of IEI patients with DNA repair defects. Peripheral lymphocyte subset and TCRv beta-repertoire analyses were performed by flow cytometric analysis. The aim was to explore the changing TCR-V beta-repertoire that can predict some clinical entities by investigating the repertoire using flow-cytometric-analysis-based TCR-V beta and its interaction with clinical entities in a group of IEI patients with DNA repair defects. TCR-repertoire of the patients with DNA-repair-defects and healthy controls was analysed with flow-cytometer. The potential of flow-cytometric analysis of the TCR repertoire as a practical and easily accessible clinical prediction method was investigated. Thirty-nine-IEI patients with DNA-repair-defects and 15 age-matched healthy-controls were included in this study. Peripheral lymphocyte subset and TCR-V beta repertoire analyses were performed by flow cytometry. Compared to the control group, 9 out of 24 clones (37.5%) exhibited a statistically significant reduction, while only 3 clones showed a statistically significant increase (p < 0.05). Preferential use of v beta-genes was associated with some clinical entities. Lower TCR-v beta-9 and TCR-v beta 23, higher TCR-v beta 7.2 were found in the patients with pneumonia (n = 13) (p = 0.018, p = 0.044 p = 0.032). AT patients with pneumonia had lower TCR-v beta-9 clone than patients without pneumonia (p = 0.008). Skewed proliferation of most TCR-v beta clones was seen DNA-repair-defects, especially AT. In addition, this study showed that preferential use of TCR-v beta genes could be predictive for some clinical entities.Istanbul Universitesi-CerrahpasaIstanbul Universitesi-Cerrahpas
An ıdadı Arabıc notebook whıch was wrıtten ın the last Ottoman perıod (h. 1323 / gc. 1905-1906)
No full textBu çalışmada 20. yüzyıl başlarında imla edilmiş bir Arapça öğrenci defteri ve onun Arapça tedris usulü içerisindeki yeri incelenmiştir. Araştırma somut bir örnek sunması ve döneme ışık tutması bakımından önem arz etmektedir. Çalışmada Osmanlı klasik dönem Arapça eğitiminden modern eğitime geçilen süreç adım adım ele alınmıştır. İncelemesi yapılan defter H. 1323/ M.1905-1906 yıllarında, Mercan İdadîsinde eğitim görmekte olan Mehmed adlı 3. sınıf öğrencisine aittir. İnceleme sonunda dönemin Arapça eğitimine dair çıkarımlar yapılmış, o dönemin ders kitapları ve ders anlatım metoduyla defter arasındaki ilişki araştırılmıştır. Bu bağlamda ders işleniş şekil ve sürecine ışık tutulmuştur. Araştırmanın gelecekteki araştırmacılara, eğitmenlere ve dilcilere yol göstereceği ve kaynak niteliğinde olacağı düşünülmektedir.This study discusses an Arabic student notebook written in the early 20th century and its place in the Arabic teaching method of the period. The research is essential in presenting a concrete example and shedding light on the period. The process from classical Ottoman period Arabic education to Modern Education has been discussed step by step. The analyzed notebook belongs to a 3rd-grade student named Mehmed, who was studying at Mercan High School in H. 1323/ M.1905-1906. Inferences were made about the Arabic education of that period, the relationship between the textbooks of that period and the lecture method, and the notebook investigated. In this context, the author aimed to shed light on the way and process of teaching the course. The research will hopefully guide and be a resource for future researchers, educators, and linguists
Malignancies and lymphoproliferations in children with primary immune deficiency-a single-center experience
No full textPrimary immune deficiencies (PIDs) are rare genetic disorders characterized by impaired immune function, leading to frequent infections and immune dysregulation. Studies have shown that individuals with PID are at an increased risk of developing malignancies and lymphoproliferative disorders compared with the general population. In this single-center study, we aimed to analyze the occurrence of malignancies and lymphoproliferations in children diagnosed with PID. We retrospectively analyzed the medical records of 550 pediatric patients diagnosed with PIDs at our center. Among them, 17 (3,0%) patients were identified with malignancy and/or benign lymphoproliferation. Eight of the 17 patients (47.0%) had immune dysregulatory diseases, whereas ataxia-telangiectasia was the second most common PID associated with malignancy and/or benign lymphoproliferation (n = 5, 29.4%). Lymphoma was the predominant malignancy (n = 11, 64.7%), and Epstein-Barr virus was identified as the most common viral agent associated with malignancy and/or benign lymphoproliferation in patients with PID (n = 8, 47.0%). Our study highlights the association between PID and malignancies/lymphoproliferations, with immune dysregulation syndromes being the most common subclass associated with malignancies/lymphoproliferations. Early diagnosis, multidisciplinary management, and regular surveillance are crucial in improving patient outcomes and saving lives
