高胆红素血症新生儿血红蛋白A、F与G-6-PD表达的相关性

The correlation between hemoglobin A, F and G-6-PD expression in neonates with hyperbilirubinemia

目的:分析高胆红素血症新生儿的血红蛋白A(HbA)、F(HbF)和葡萄糖-6-磷酸脱氢酶(G-6-PD)的表达特点,探讨HbA、HbF及G-6-PD的浓度与新生儿高胆红素血症之间的相关性.方法:回顾性分析新生儿科住院的2932例新生儿的性别、G-6-PD活性、Hb、HbA和HbF的质量浓度与高胆红素血症之间的相关性.结果:住院新生儿中G-6-PD缺乏患病率为10.0%;G-6-PD缺乏患儿中高胆红素血症患病率男性(5.3%)明显高于女性(2.3%);G-6-PD重度缺乏新生儿血清TBIL浓度(245.0±126.9)μmol/L明显高于正常新生儿的血清TBIL浓度(224.1±114.0)μmol/L(P=0.0162),而中度缺乏新生儿的血清TBIL浓度(217.5±126.7)μmol/L与G-6-PD重度缺乏新生儿和正常新生儿之间无统计学差异(P=0.0989,P=0.6570);高胆红素血症患儿HbA质量浓度明显高于胆红素正常组(P<0.0001),而Hb和HbF浓度则明显低于正常组(P<0.0001,P<0.0001).结论:住院新生儿G-6-PD缺乏具有很高的发病率,其中男性新生儿G-6-PD缺乏发病率明显高于女性新生儿,并且男性更易发生高胆红素血症;高胆红素血症新生儿的Hb及其组分HbF质量浓度明显减低.G-6-PD重度缺乏不一定会引起新生儿高胆红素血症,但G-6-PD重度缺乏患儿体内会发生轻微的红细胞破坏.

Objective:By analyzing the expression characteristics of hemoglobin A (HbA),F (HbF) and glucose-6-phosphate dehydrogenase (G-6-PD) in children with hyperbilirubinemia,the correlation between neonatal hyperbilirubinemia and the content of HbA,HbF and G-6-PD was explored Method:The correlation between hyperbilirubinemia and gender,G-6-PD activity,Hb,HbA and HbF level was analyzed retrospectively in 2 932 neonates admitted to the Neonatology Department from January 2014 to October 2018. Results:The prevalence of G-6-PD deficiency in hospitalized neonates was 10.0%.The prevalence of hyperbilirubinemia in children with G-6-PD deficiency was significantly higher in male (5.3%) than in female (2.3%).The serum total bilirubin (TBIL) content of neonates with severe G-6-PD deficiency (245.0±126.9)μmol/L was significantly higher than that of normal neonates (224.1±114.0)μmol/L ( P =0.016 2),while there was no significant difference in TBIL content between neonates with moderate G-6-PD deficiency (217.5±126.7)μmol/L and those with severe G-6-PD deficiency or normal neonates ( P =0.098 9 and P =0.657 0),respectively.The content of HbA in neonates with hyperbilirubinemia was significantly higher than that in normal group ( P <0.000 1),while the content of Hb and HbF was significantly lower than that in normal group ( P <0.000 1 and P <0.000 1). Conclusion:The incidence of neonatal G-6-PD deficiency in our hospital was very high.The incidence of male neonatal G-6-PD deficiency was significantly higher than that of female,and male neonatal patients were more prone to develop hyperbilirubinemia.The content of Hb and its component HbF in neonates with hyperbilirubinemia decreased significantly.Severe deficiency of G-6-PD may not necessarily lead to neonatal hyperbilirubinemia,but it may cause slight erythrocyte damage in neonates.