孟加拉国锡尔赫特的地中海贫血和其他血红蛋白病模式

Chowdhury MZR, Benzamin M, Chowdhury T, Jahan A, Dey RK, Sharker MH, Tamal TB, Chowdhury A
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摘要

背景:地中海贫血症和血红蛋白病是孟加拉国最常见的遗传性溶血性先天性疾病:这项回顾性横断面观察研究在孟加拉国锡尔赫特的本扎明博士小儿肝脏研究中心和营养诊所进行。我们查阅了锡尔赫特大众诊断中心和阿多拉山医院血液科 2022 年 3 月至 2023 年 7 月期间的数据记录软件,并收集了所有血红蛋白电泳报告,共评估了 783 名患者的数据,将数据输入 Microsoft Excel,并使用社会科学统计软件包(SPSS)22 版进行分析。研究的目的是确定孟加拉国锡尔赫特地区地中海贫血和其他血红蛋白病的模式:在 783 名患者中,男性 291 人(37.2%),女性 492 人(62.8%),18 岁以下儿童 387 人(44.4%),18 岁以上 396 人(50.6%)。其中,262 例(33.46%)报告患有血红蛋白病。在 6 个月前,10 名患者被建议进行血红蛋白电泳。总体而言,在研究人群中,最常见的血红蛋白病是β地中海贫血特质(118 例,15.1%),其次是 Hb E 特质(45 例,11.6%)、Hb E β地中海贫血(34 例,4.43%)和重型β地中海贫血(8 例,0.9%)。儿科年龄组的趋势相同,β地中海贫血特异性(53,13.7%)、Hb E 塔里特(84,10.7%)、Hb E β地中海贫血(28,7.24%)、重型β地中海贫血(8,1.8%)。在成年组中,β地中海贫血特质(65,16.4%)、Hb E 塔里特(39,9.8%)、Hb E β地中海贫血(6,1.5%%):结论:在锡尔赫特的贫血人群中,血红蛋白病非常常见。β地中海贫血特异性、Hb E tarit、Hb E β地中海贫血、重型β地中海贫血是常见的变种。因此,在进行贫血评估时,应注意血红蛋白病。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Pattern of Thalassemia and other Hemoglobinopathies in Sylhet, Bangladesh
Background: Thalassemias and hemoglobinopathies are the most common inheritant hemolytic congenital disorders in Bangladesh.There is no well precise validated data available about the prevalence of Thalassaemia and related hemoglobin disorders in Bangladesh. Method: This retrospective, cross-sectional observational study was carried out in Dr.Benzamin’s Pediatric Liver Research Centre and Nutrition Clinic, Sylhet, Bangladesh. We reviewed the data record software of the hematology section of the Popular Diagnostic Centre and Mount Adora Hospital, Sylhet, from March, 2022 to July, 2023 and collected all the Hemoglobin electrophoresis reports.A total of 783patients data were evaluated, and the data were entered into Microsoft Excel and analyzed by Statistical Package for Social Sciences (SPSS) software version 22. The aim of the study was to identify the pattern of Thalassemia and other Hemoglobinopathies in Sylhet, Bangladesh. Result: Out of 783 patients, 291(37.2%) were male, 492 (62.8%) female, pediatric population that is under 18 years 387 (44.4%) and 18 years or more 396(50.6%). Among these, 262 (33.46%) reports showed Hemoglobinopathies. Before 6 months, 10 patients were advised for Hb electrophoresis. Overall, in study population, most common hemoglobinopathies were Beta thalassemia trait (118, 15.1%), followed by Hb E trait (45, 11.6%), Hb E Beta thalassemia (34, 4.43%), Beta thalassemia major (8, 0.9%). Same trend followed in pediatric age group, Beta thalassemia trait (53, 13.7%), Hb E tarit (84, 10.7%), Hb E Beta thalassemia (28,7.24%), Beta thalassemia major (8, 1.8 %). In adult age group, Beta thalassemia trait (65, 16.4%), Hb E tarit (39, 9.8%), Hb E Beta thalassemia (6, 1.5%%). Conclusion: Hemoglobinopathies among the anaemic population of Sylhet is very common. Beta thalassaemia trait, Hb E tarit, Hb E Beta thalassemia, Beta thalassemia major are common variants. So, during anaemia evaluation, hemoglobinopathies should be kept in mind
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