CAJ | 학술논문

目的评价法国Sebia公司Capillarys2全自动毛细管电泳仪在珠蛋白生成障碍性贫血筛查中的应用。方法采集2011年8～12月经血分析（红细胞平均体积小于80fL，红细胞平均血红蛋白浓度小于26pg的8岁以上患者）筛选后的292份标本用法国Sebia公司Capillarys2全自动毛细管电泳仪对入选标本进行血红蛋白（Hb）毛细管电泳、血清铁、铁蛋白检查，同时作α-及β-珠蛋白生成障碍性贫血基因检查。结果（1）共检测了292例临床标本，符合α-珠蛋白生成障碍性贫血表型者36例，β-珠蛋白生成障碍性贫血表型者135例。同时出现α-及β-珠蛋白生成障碍性贫血表型者2例。（2）高压毛细管电泳法用于α-珠蛋白生成障碍性贫血诊断的灵敏度为86．11％，特异度为98．37％，准确度为95．60％，阳性预测值为93．94％，阴性预测值为96．03％。（3）高压毛细管电泳法用于β-珠蛋白生成障碍性贫血诊断的灵敏度为97．04％，特异度为98．35％，准确度为97．66％，阳性预测值为98．50％，阴性预测值为96．75％。HbA2值大于4．0％时，β-珠蛋白生成障碍性贫血基因法检测阳性率达100．00％。结论法国Sebia公司Capillarys2全自动毛细血管电泳仪对Hb区带分辨及定量准确度高，可为Hb病（包括珠蛋白生成障碍性贫血及异常Hb病）的初筛提供快速的诊断依据。
목적평개법국Sebia공사Capillarys2전자동모세관전영의재주단백생성장애성빈혈사사중적응용。방법채집2011년8～12월경혈분석（홍세포평균체적소우80fL，홍세포평균혈홍단백농도소우26pg적8세이상환자）사선후적292빈표본용법국Sebia공사Capillarys2전자동모세관전영의대입선표본진행혈홍단백（Hb）모세관전영、혈청철、철단백검사，동시작α-급β-주단백생성장애성빈혈기인검사。결과（1）공검측료292례림상표본，부합α-주단백생성장애성빈혈표형자36례，β-주단백생성장애성빈혈표형자135례。동시출현α-급β-주단백생성장애성빈혈표형자2례。（2）고압모세관전영법용우α-주단백생성장애성빈혈진단적령민도위86．11％，특이도위98．37％，준학도위95．60％，양성예측치위93．94％，음성예측치위96．03％。（3）고압모세관전영법용우β-주단백생성장애성빈혈진단적령민도위97．04％，특이도위98．35％，준학도위97．66％，양성예측치위98．50％，음성예측치위96．75％。HbA2치대우4．0％시，β-주단백생성장애성빈혈기인법검측양성솔체100．00％。결론법국Sebia공사Capillarys2전자동모세혈관전영의대Hb구대분변급정량준학도고，가위Hb병（포괄주단백생성장애성빈혈급이상Hb병）적초사제공쾌속적진단의거。
Objective To evaluate the French Sebia Capillarys 2 automatic capillary electrophoresis in screen-ing for thalassemia .Methods From August to December in 2011 ,292 specimens of blood screening to detect capil-lary electrophoresis of hemoglobin ,serum iron and ferritin by French Sebia Capillarys 2 automatic capillary electro-phoresis (mean corpuscular volume less than 80 fL ,MCHC<26 pg in patients over 8 years old) ,while for α-andβ-thalassemia gene detection .Results (1) Detected 292 cases of clinical specimens ,in which there were 36 cases of α-thalassemia phenotype ,135 cases of β-thalassemia phenotype ,2 cases bothα-and β-thalassemia phenotype .(2) The high-pressure capillary electrophoresis method forα-thalassemia diagnosis sensitivity was 86 .11% ,the specificity was 98 .37% ,accuracy was 95 .60% ,the positive predictive value was 93 .94% ,the negative predictive value was 96 .03% .(3)The high-pressure capillary electrophoresis method for β-thalassemia diagnosis sensitivity was 97 .04% , the specificity was 98 .35% ,accuracy was 97 .66% ,the positive predictive value was 98 .50% ,the negative predictive value was 96 .75% .The HbA2 value was greater than 4 .00% ,β-thalassemia gene detection positive rate was up to 100 .00% .Conclusion French Sebia Capillarys 2 automatic capillary electrophoresis with high accuracy for zone reso-lution and quantification of hemoglobin ,which provides rapidly diagnostic basis for preliminary screening of hemoglo-binopathy (including thalassemia and abnormal hemoglobinopathy ) .