Epidemiological and Clinical Profile of Iraqi Patients with β-Thalassemia Major

Ebtissam Yasseen Ali, M. M. Abdulla
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Abstract

Thalassemia is an autosomal recessive inherited blood disorder due to hemoglobin-production abnormalities. Over the past three decades, hyper-transfusion therapy in these patients has shown significant increase in life expectancy and quality of life. unfortunately, this type of therapy also increased the frequency of complications due to iron overload. The study aims to identify the Sociodemographic and clinical characteristics of patients with thalassemia, and to assess transfusion related complication among them. A descriptive, cross-sectional study was conducted in Thalassemia Center in Al-Najaf province in Iraq, during the period from the 1st of April to the 31st of August 2018. Data were collected through direct interview with patients/parents using a specially designed questionnaire form. A total of 175 transfusion dependent thalassemia major patients were included, the mean age 10. 5 years ranging from 5 m to 34years. Patients under 10 years of age represented the highest rate with Male: Female ratio 1.2:1. 60% were from rural area with high percentage of parental consanguinity (70%). The study documented the relationship between iron over load and appearance of complications. Development of preventive measures as genetic counseling, prenatal diagnosis, pre-marital screening are the best ways to decrease the incidence of disease, in addition to regular blood transfusion with optimum chelation therapy.
伊拉克β-地中海贫血患者的流行病学及临床分析
地中海贫血是一种常染色体隐性遗传血液疾病,由于血红蛋白产生异常。在过去的三十年中,这些患者的超输血治疗显示出预期寿命和生活质量的显著提高。不幸的是,这种类型的治疗也增加了铁超载引起的并发症的频率。该研究旨在确定地中海贫血患者的社会人口学和临床特征,并评估其中输血相关并发症。2018年4月1日至8月31日期间,在伊拉克纳杰夫省地中海贫血中心进行了一项描述性横断面研究。采用特别设计的问卷形式对患者/家长进行直接访谈,收集数据。共纳入175例输血依赖型地中海贫血重症患者,平均年龄10岁。5年,5至34年不等。10岁以下患者发病率最高,男女比例为1.2:1。60%来自农村,亲本血缘比例高(70%)。该研究记录了铁超载与并发症出现之间的关系。制定预防措施,如遗传咨询、产前诊断、婚前筛查,除了定期输血和最佳的螯合治疗外,是减少疾病发病率的最佳途径。
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