Bibliographic Details
| Title: |
A National Registry of Thalassemia in Turkey: Demographic and Disease Characteristics of Patients, Achievements, and Challenges in Prevention |
| Authors: |
Yeşim Aydınok, Yeşim Oymak, Berna Atabay, Gönül Aydoğan, Akif Yeşilipek, Selma Ünal, Yurdanur Kılınç, Banu Oflaz, Mehmet Akın, Canan Vergin, Melike Sezgin Evrim, Ümran Çalışkan, Şule Ünal, Ali Bay, Elif Kazancı, Talia İleri, Didem Atay, Türkan Patıroğlu, Selda Kahraman, Murat Söker, Mediha Akcan, Aydan Akdeniz, Mustafa Büyükavcı, Güçhan Alanoğlu, Özcan Bör, Nur Soyer, Nihal Özdemir Karadaş, Ezgi Uysalol, Meral Türker, Arzu Akçay, Süheyla Ocak, Adalet Meral Güneş, Hüseyin Tokgöz, Elif Ünal, Naci Tiftik, Zeynep Karakaş |
| Source: |
Turkish Journal of Hematology, Vol 35, Iss 1, Pp 12-18 (2018) |
| Publisher Information: |
Galenos Publishing House, 2018. |
| Publication Year: |
2018 |
| Collection: |
LCC:Diseases of the blood and blood-forming organs |
| Subject Terms: |
thalassemia, hemoglobinopathies, splenectomy, registries, iron chelators, β, -thalassemia mutations, turkey, Diseases of the blood and blood-forming organs, RC633-647.5 |
| More Details: |
Objective: The Turkish Society of Pediatric Hematology set up a National Hemoglobinopathy Registry to demonstrate the demographic and disease characteristics of patients and assess the efficacy of a hemoglobinopathy control program (HCP) over 10 years in Turkey. Materials and Methods: A total of 2046 patients from 27 thalassemia centers were registered, of which 1988 were eligible for analysis. This cohort mainly comprised patients with β-thalassemia major (n=1658, 83.4%) and intermedia (n=215, 10.8%). Results: The majority of patients were from the coastal areas of Turkey. The high number of patients in Southeastern Anatolia was due to that area having the highest rates of consanguineous marriage and fertility. The most common 11 mutations represented 90% of all β-thalassemia alleles and 47% of those were IVS1-110(G->A) mutations. The probability of undergoing splenectomy within the first 10 years of life was 20%, a rate unchanged since the 1980s. Iron chelators were administered as monotherapy regimens in 95% of patients and deferasirox was prescribed in 81.3% of those cases. Deferasirox administration was the highest (93.6%) in patients aged |
| Document Type: |
article |
| File Description: |
electronic resource |
| Language: |
English |
| ISSN: |
1308-5263 |
| Relation: |
https://jag.journalagent.com/z4/download_fulltext.asp?pdir=tjh&un=TJH-10821; https://doaj.org/toc/1308-5263 |
| DOI: |
10.4274/tjh.2017.0039 |
| Access URL: |
https://doaj.org/article/ddb5427c3e23457d87b7b6e9ef919f94 |
| Accession Number: |
edsdoj.b5427c3e23457d87b7b6e9ef919f94 |
| Database: |
Directory of Open Access Journals |
