摘要
目的 :了解不同基因型地中海贫血 (地贫 )患儿并发铁缺乏的发生率。方法 :对 2 33例临床诊断为地中海贫血的患儿进行点阵列杂交技术基因分析和铁指标检测。结果 :2 33例患儿中并发缺铁的有 5 9例(2 5 .3% ) ;30例α 地贫并发缺铁 9例 (30 % ) ,2 0 3例 β 地贫并发缺铁 5 0例 (2 4 .6 % ) ,差异无统计学意义 (P >0 .2 5 )。HbH病患儿中 ,基因缺失型的缺铁发生率为 33.3% (5 / 15 ) ,非缺失型的缺铁发生率为 12 .5 % (1/ 8) ,前者高于后者 ,但差异无统计学意义 (P >0 .10 )。双重杂合子 β 地贫无缺铁病例 ,而杂合子 β 地贫铁缺乏率为2 6 .7% (5 0 / 16 5 ) ,两者差异有统计学意义 (P <0 .0 1)。结论 :本文发现地贫患儿可并发铁缺乏 ,尤其是杂合子 β 地贫及除非缺失型HbH病外的α 地贫患儿的铁缺乏发生率较高。因此有必要对其进行适当治疗。
Objective:To investigate the incidence of iron-deficiency(ID) in different genotrpeed thalassemias.Method:233 children with Thalassemia were genotyped by microarray and detected the iron indices.Result:Among the 233 thalassemia patients, 59( 25.3%)were combined with ID. In 30 cases of α-Thalassemia patients, 9 cases(30%) complicated with ID. Among 203 cases genotyped as β-Thalassemia, 50 cases( 24.6%) were combined with ID. The incidence of iron-deficency has no statistic significance between α-thalassemia and β-thalassemia group.In HbH patients, the ID incidence in gene-deletion type was 33.3%(5/15) and that of 12.5%(1/8) in non-gene-deletion type. 26.7%(50/165) in heterozygote β-Thalassemia were found ID. NO ID occurred in double-heterozygote β-Thalassemia .Between them, there was highly statistics significance(P< 0.005).Conclusion:Thalassemia patients, especially for heterozygote β-Thalassemia to α-Thalassemia except non-gene-deletion HbH, combined with ID were necessary to iron therapy.
出处
《临床血液学杂志》
CAS
2004年第3期141-142,共2页
Journal of Clinical Hematology
关键词
贫血
基因型
铁缺乏
Thalassaemia
Gene-type
Iron-deficiency
