广东地区地中海贫血患儿血清可溶性转铁蛋白受体水平的研究

Study on Soluble transferrin receptor in children with thalassemia in GuangDong Province

目的研究广东地区不同类型地中海贫血(地贫)患儿血清可溶性转铁蛋白受体(sTfR)水平与患儿临床表现的关系。方法对14例静止型α地贫,47例标准型α地贫,27例血红蛋白H(HbH)病,14例异常血红蛋白Constant Spring(HbCS)复合HbH病,59例β地贫杂合子,31例β地贫纯合子或双重杂合子和50例正常健康儿童的血红蛋白(Hb)含量,血清铁(SI)水平及sTfR水平进行检测,并对结果进行统计学分析。结果静止型α地贫患儿的sTfR水平与正常对照相比无显著性差异,标准型α地贫患儿与轻型β地贫患儿sTfR水平略高于正常对照,以上三类地贫患儿Hb含量轻度减低或正常。血红蛋白H病患儿Hb含量低于正常对照,sTfR水平高于正常对照。重型β地贫患儿Hb含量明显低于正常对照,sTfR水平则明显高于正常对照与轻型α及β地贫患儿。结论 1广东地区不同类型地贫患儿血清sTfR水平升高程度不同,其与临床表现严重程度一致。2sTfR可作为一项监测地贫患儿体内红系增生程度及重型β地贫治疗效果的指标。

Objective To investigate the relationship of soluble transferrin receptor（sTfR）value and clinical manifestation in children with thalassemia in GuangDong Province.Methods 192 Blood samples from 14 chidren with silentαthalassemia,47 children with standardαthalassemia,27 children with HbH disease,14 children with HbH-CS disease,59 children withβthalassemia trait,31 children withβthalassemia major and 50 normal individuals were detected for the values of hemoglobin（Hb）,serum iron（SI）and sTfR,and the results were statistically analyzed.ResultsThere was no distinct difference between the chidren with silentαthalassemia and normal controls on the values of sTfR.The values of sTfR in chidren with standardαthalassemia andβthalassemia trait were slightly higher than those of normal controls.The values of sTfR in chidren with HbH disease were higher than those of normal controls.And the values of sTfR in chidren withβthalassemia major were significantly higher than those of other groups.Conclusion ①Children with different genotype thalassemia have different sTfR values and sTfR values are associated with clinic manifestation.②The sTfR level could be used as an index of clinic treatment effects in children with thalassemia major.