Retrospective analysis of hemophilia B in Turkey: identifying main characteristics and treatment options
Özet
Background: Hemophilia B (HB), an X-linked recessive inherited bleeding disorder, exhibits a high prevalence among males. Objectives: To present the first national cohort of persons with HB to define the demographics, clinical characteristics, and treatment patterns in Turkey. Methods: This multicenter, retrospective study included 433 alive persons with HB registered in 35 centers between 1961 and 2018. Analyses were performed by age subgroups (0-17 years, 18-64 years, and ≥65 years), disease severity by factor levels (severe, <1 IU/dL; moderate, 1-5 IU/dL; mild, >5 IU/dL). Additionally, patients were stratified based on the initiation year of follow-up at the relevant study center, creating 2 periods: 1993-2006 (referred to as period A) and 2007-2018 (referred to as period B). Results: Predominantly male (98.6%), the median age at data entry was 22.1 years (n = 429). The majority (49.0%) had moderate HB, followed by severe (30.0%) and mild (15.7%) disease. Of the 377 patients with complete treatment details, 209 (55.4%) were under prophylaxis from their diagnosis onwards, while 79 patients (21.0%) only received on-demand treatment. Additionally, 89 patients (23.6%) initially underwent on-demand treatment and later were switched to prophylaxis. Knees were the primary site of bleeding and the most frequently intervened joints. Most of the major (47.5%) and minor (53.3%) orthopedic procedures were carried out in persons with severe HB, while half of radioactive synovectomy procedures were performed on persons with moderate HB. Conclusion: This paper describes the demographics, clinical characteristics, and treatments patterns of a large cohort of alive persons with HB on a national scale.
Anahtar kelimeler
Atıf için künye
ZÜLFİKAR, O. B., Koç, B., Şahin, F., ŞAŞMAZ, H. İ., Kavaklı, K., Balkan, C., Antmen, A. B., Akbayram, S., GÜVENÇ, B., Okan, V., Türkkan, E., Albayrak, C., Albayrak, D., Sarper, N., Celkan, T. T., Ayyıldız, O., Aksu, S., Patıroğlu, T., Şalcıoğlu, Z., … Ar, M. C. (2024). Retrospective analysis of hemophilia B in Turkey: identifying main characteristics and treatment options. Research and Practice in Thrombosis and Haemostasis, 8(7). https://doi.org/10.1016/j.rpth.2024.102588
BibTeX
@article{zulfikar2024-245879,
author = {ZÜLFİKAR, Osman Bülent and Koç, Başak and Şahin, Fahri and ŞAŞMAZ, HATİCE İLGEN and Kavaklı, Kaan and Balkan, Can and Antmen, Ali Bülent and Akbayram, Sinan and GÜVENÇ, BİROL and Okan, Vahap and Türkkan, Emine and Albayrak, Canan and Albayrak, Davut and Sarper, Nazan and Celkan, Tülin Tiraje and Ayyıldız, Orhan and Aksu, Salih and Patıroğlu, Türkan and Şalcıoğlu, Zafer and Güneş, Adalet Meral and Torun, Yasemin Altuner and Çalışkan, Ümran and Tokgöz, Hüseyin and Ay, Yılmaz and Özdemir, Gül Nihal and Sönmez, Mehmet and Ünal, Ekrem and Öner, Ahmet Fayik and Güler, Nil and Küpesiz, Osman Alphan and Ören, Hale and Karaman, Serap and Ünüvar, Ayşegül and Dağlı, Mehmet and Demir, Ahmet Muzaffer and Söker, Murat and Alioğlu, Bülent and Kaya, Zühre and Ayhan, Aylin Canbolat and Bıçakçı, Zafer and Aral, Yusuf Ziya and Ar, Muhlis Cem},
title = {Retrospective analysis of hemophilia B in Turkey: identifying main characteristics and treatment options},
journal = {Research and Practice in Thrombosis and Haemostasis},
year = {2024},
volume = {8},
number = {7},
issn = {2475-0379},
doi = {10.1016/j.rpth.2024.102588},
url = {https://akademik.atauni.edu.tr/yayinlar/245879-retrospective-analysis-of-hemophilia-b-in-turkey-identifying-main}
}