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广西地中海贫血112例临床分析
  • ISSN号:1001-5639
  • 期刊名称:《蛇志》
  • 时间:0
  • 分类:R714.254[医药卫生—妇产科学;医药卫生—临床医学]
  • 作者机构:[1] 广西医科大学第一附属医院 第一临床医学院血液内科,广西南宁530021, [2] 中国人民解放军三○三医院血液内科,广西南宁530021
  • 相关基金:广西医疗卫生重点科研课题(重2012077)
中文摘要:

目的:通过对广西112例地中海贫血病例的回顾性分析,了解地中海贫血患者的临床特点、治疗现状和并发症。方法用回顾性分析方法对广西112例地中海贫血患者(α中间型16例,β中间型30例,β重型66例)的临床特点、治疗现状和并发症进行分析。结果(1)52例β重型地贫患者和所有需长时间频繁输血的中间型地贫患者输血前H b<90 g/L。(2)不规范祛铁治疗的β中间型和β重型患者分别为84.0%和89.8%。(3)112例患者中,74例(67.9%)存在铁过载,2例α中间型,19例β中间型,53例β重型。(4)2例β中间型和1例β重型患者曾发生心力衰竭;2例β重型患者有继发性糖尿病;17例β中间型和46例β重型患者身材矮小,身材矮小与铁蛋白、输血前血红蛋白量相关( r=0.336,P<0.001;r=-0.243,P=0.011)。结论本组β中间型地贫和β重型地贫患者输血和去铁治疗不规范,铁过载严重,身材矮小、心力衰竭等并发症发生率高。

英文摘要:

Objective Through the retrospective analysis of 112 cases of thalassemia in Guangxi province ,explore the clinical features ,treatment status quo and complication in patients with thalassemia . Methods Analysis of the clinical characteristics ,treatment and complication of 112 thalassemia patients(16α-thalassemia intermediate ,30β-thalassemia intermediate ,66β-thalassemia major) in Guangxi with a retrospective analyzed method . Results (1)52β-thalassemia major patients and all long time frequent blood transfusions patients of β-thalassemia interme-dia with pre-transfusion Hb〈90 g/L .(2)Iron chelation therapy was used irregularly in 84% patients of β-thalasse-mia intermediate and 89.8% patients of β-thalassemia major .(3)In 112 thalassemia patients ,74 patients (67.9% ) with iron overload ,including two α-thalassemia intermediate patients ,19 β-thalassemia intermediate patients ,53 β-thalassemia major patients .(4)Two β-thalassemia intermediate patients and one β-thalassemia major patient with severe heart failure ;two β-thalassemia major patients with secondary diabetes mellitus ;17β-thalassemia intermedi-ate patients and 46β-thalassemia major patients with severe short stature ,short stature was correlated with levels of ferritin and pre-transfusion Hb (r=0.336 ,P〈0.001 ;r= -0.243 ,P=0.011) . Conclusion Our data showed that ,blood transfusion and iron chelation therapy were not standardized in the β-thalassemia intermedia and β-thalassemia major patients ,iron overload severely ,a high incidence of complications such as heart failure ,diabetes mellitus and short stature .

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期刊信息
  • 《蛇志》
  • 主管单位:广西省科协
  • 主办单位:中国蛇协
  • 主编:李其斌 蓝海
  • 地址:广西南宁市仙葫家福路6号
  • 邮编:530222
  • 邮箱:shezhi771@163.com
  • 电话:0771-5330221
  • 国际标准刊号:ISSN:1001-5639
  • 国内统一刊号:ISSN:45-1168/R
  • 邮发代号:48-52
  • 获奖情况:
  • 国内外数据库收录:
  • 被引量:4336