也门患者地中海贫血表型和相关死亡率的单中心回顾性分析

Thaker A.A. Abdulrahman, Ali Alhaj, Mokhtar E.M. Saif
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引用次数: 0

摘要

目的:回顾性分析也门萨那市寻求医疗保健的也门患者地中海贫血表型和相关死亡率。方法:本回顾性横断面研究于2010年1月至2016年12月在萨那市也门地中海贫血和遗传性血液病学会(YSTGBD)医疗中心进行。从496名地中海贫血患者的记录中检索了有关性别、年龄、原籍省份、地中海贫血类型和地中海贫血相关死亡率的数据。然后将数据输入Excel电子表格,交叉检查准确性,并以频率和比例表示。使用卡方检验或Fisher精确检验分析地中海贫血患者死亡的相关因素。结果:从2010年1月到2016年12月,共有496名患者在萨那市的YSTGBD医疗中心就诊。大多数地中海贫血患者是男性(57.9%),大约一半的患者年龄在10岁或以下,与10岁以上的患者比例相当。大多数地中海贫血患者患有β地中海贫血(92.7%),而7.3%患有α地中海贫血。大多数患者来自哈贾省(16.5%),其次是萨那省(13.1%)和阿姆兰省(11.9%),而其他省份的患者比例较低,从0.2%到8.1%不等。总的地中海贫血患者中有30人死亡,死亡率与反复输注全血显著相关,但与患者的性别和年龄或地中海贫血表型无关。结论:在萨那市寻求医疗保健的大多数地中海贫血患者都患有地中海贫血,其中男性占一半以上,患者在10岁的截止年龄方面几乎分布均匀。寻求医疗保健的也门地中海贫血患者的死亡率低于世界其他地方的报告,死亡率与反复输血显著相关。建议进一步研究以确定与地中海贫血相关的遗传异常,同时需要在也门建立一个国家咨询、研究和监督与地中海贫血和遗传性血液病相关的自愿活动中心。关键词:地中海贫血,表型,死亡率,也门
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Thalassemia Phenotypes and Associated Mortality among Yemeni Patients: A Single-Center Retrospective Analysis
Objective: To retrospectively analyze thalassemia phenotypes and associated mortality among Yemeni patients seeking healthcare in Sana’a city, Yemen. Methods: This retrospective, cross-sectional study was conducted in the medical center of Yemen Society for Thalassemia and Genetic Blood Disorders (YSTGBD) in Sana'a city from January 2010 to December 2016. Data about gender, age, governorate of origin, type of thalassemia and thalassemia-related mortality were retrieved from the records of 496 thalassemia patients. Data were then entered into an Excel spreadsheet, cross-checked for accuracy and presented as frequencies and proportions. Factors associated with deaths among thalassemia patients were analyzed using chi-square or Fisher’s exact tests. Results: A total number of 496 patients attended the medical center of YSTGBD in Sana’a city in the period from January 2010 to December 2016. The majority of thalassemia patients were males (57.9%), and approximately half of the patients were aged 10 years or younger with a comparable proportion to those older than 10 years. Most thalassemia patients had β-thalassemia (92.7%), while 7.3% had α-thalassemia. The majority of patients were from Hajjah governorate (16.5%) followed by those from Sana'a (13.1%) and Amran (11.9%), while lower proportions ranging from 0.2% to 8.1% were recorded for patients from other governorates. Thirty out of the total attending thalassemia patients died, and mortality was significantly associated with recurrent transfusions of whole blood but not with the gender and age of the patients or thalassemia phenotype. Conclusions: The majority of thalassemia patients seeking healthcare in Sana’a city suffer from thalassemia, where males represent more than half of them and the patients are almost equally distributed in relation to the cut-off age of 10 years. The mortality incidence among Yemeni thalassemia patients seeking healthcare is lower than that reported elsewhere in the world, and mortality was significantly associated with recurrent blood transfusions. Further studies to identify the genetic abnormalities associated with thalassemia are recommended together with the need for the launch of a national center for counseling, research and supervision of voluntary activities related to thalassemia and genetic blood disorders in Yemen. Keywords: Thalassemia, Phenotype, Mortality, Yemen
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