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目的研究铁缺乏对β-珠蛋白生成障碍性贫血(β-地贫)患者血红蛋白A2(HbA2)的影响。方法标本来自2008年5月-2010年5月在桂林医学院附属医院儿科、产科门诊及重型β-地贫家系,对所有标本进行血常规检查、血红蛋白电泳并定量测定HbA2值、免疫荧光法测定血清铁蛋白(SF)、Multi-PCR法确定3种缺失型α-地贫基因突变、PCR结合反向斑点杂交法确定17种β-地贫基因突变。结果 408份标本中,A组304例,B组26例,C组56例,D组22例,将各组进行计量资料两样本均数的比较,其中A组与B组比较q=5.074 7,P<0.05,A组与C组比较q=37.650 8,P<0.05,A组与D组比较q=16.043 0,P<0.05,C组与D组比较q=7.682 9,P<0.05,B组与D组比较q=15.806 6,P<0.05。4组的SF和HbA2的相关性检测均无显著性差异。结论 1.铁缺乏会使健康人和轻型β-地贫患者血红蛋白电泳时HbA2值下降;2.轻型β-地贫合并铁缺乏时HbA2值较单纯轻型β-地贫下降显著,但仍较健康人群高,差异有统计学意义,可见即使合并铁缺乏,HbA2仍可以作为临床诊断轻型β-地贫的可靠指标。
Objective To investigate the effect of iron deficiency on hemoglobin A2 (HbA2) in patients with β-thalassemia. Methods The specimens were collected from pediatric department, obstetric clinic and severe β-thalassemia family in the Affiliated Hospital of Guilin Medical College from May 2008 to May 2010, HbA2 was determined by hemoglobin electrophoresis and quantitative determination of HbA2 in all specimens. Immunofluorescence Serum ferritin (SF) and multi-PCR were used to identify three kinds of deletional α-thalassemia mutations. 17 kinds of β-thalassemia mutations were identified by PCR and reverse dot blot hybridization. Results Among the 408 specimens, there were 304 cases in group A, 26 cases in group B, 56 cases in group C and 22 cases in group D, and the mean of two samples in each group was compared. The comparison between group A and group B was q = 5.074 7 , P <0.05, A group and C group compared q = 37.6508, P <0.05, A group and D group compared q = 16.043 0, P <0.05, C group and D group compared with q = 7.682 9, P < There was no significant difference in the correlation between SF and HbA2 in group B and group D (q = 15.806 6, P <0.05.4). HbA2 decreased in hemoglobin electrophoresis in healthy people and patients with mild β-thalassemia due to iron deficiency.2.HbA2 decreased significantly in mild β-thalassemia combined with iron deficiency compared with simple thalassemia, but still more healthy High population, the difference was statistically significant, showing that even with iron deficiency, HbA2 can still be used as a reliable clinical diagnosis of light β-thalassemia indicators.