CAJ | 학술논문

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 Con-stant 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。
목적：연구엄동지구불동류형지중해빈혈（지빈）환인혈청가용성전철단백수체（sTfR ）수평여환인림상표현적관계。방법대14례정지형α지빈，47례표준형α지빈，27례혈홍단백 H（HbH ）병，14례이상혈홍단백 Con-stant Spring（HbCS ）복합 HbH 병，59례β지빈잡합자，31례β지빈순합자혹쌍중잡합자화50례정상건강인동적혈홍단백（Hb）함량，혈청철（SI）수평급 sTfR 수평진행검측，병대결과진행통계학분석。결과정지형α지빈환인적sTfR 수평여정상대조상비무현저성차이，표준형α지빈환인여경형β지빈환인 sTfR 수평략고우정상대조，이상삼류지빈환인 Hb 함량경도감저혹정상。혈홍단백 H 병환인 Hb 함량저우정상대조，sTfR 수평고우정상대조。중형β지빈환인 Hb 함량명현저우정상대조，sTfR 수평칙명현고우정상대조여경형α급β지빈환인。결론①엄동지구불동류형지빈환인혈청 sTfR 수평승고정도불동，기여림상표현엄중정도일치。②sTfR 가작위일항감측지빈환인체내홍계증생정도급중형β지빈치료효과적지표。
Objective To investigate the relationship of soluble transferrin receptor (sTfR )value and clinical mani-festation in children with thalassemia in GuangDong Province.Methods 192 Blood samples from 14 chidren with silentαthalassemia,47 children with standardα thalassemia,27 children with HbH disease,14 children with HbH-CS dis-ease,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.Results There was no distinct difference between the chidren with silent α thalassemia 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 man-ifestation.②The sTfR level could be used as an index of clinic treatment effects in children with thalassemia major.