新生儿G6PD缺乏症并发高胆红素血症72例临床分析

Clinical Analysis of 72 Cases of the Lack of G6PD Complicated by Neonatal Hyperbilirubinemia

目的探讨新生儿期G6PD缺乏症并发高胆红素血症的发病诱因、临床特点和预后。方法回顾性分析我院新生儿科2011年至2015收治的72例新生儿G6PD缺乏症并发高胆红素血症患儿的临床资料。结果新生儿期G6PD缺乏并发高胆红素血症的诱因主要为感染和母孕后期或生产时有异常情况。性别比例为男:女为4∶1。高胆红素血症出现时间主要发生在生后7d内(75%)。伴贫血22例(30.6%),胆红素脑病发生2例(2.8%)。经治疗57例治愈出院(79.2%),15例(20.8%)好转出院。回归分析显示血清总胆红素与G6PD酶活性浓度及Hb水平均无明显相关性(r=-0.167,-0.175;P=0.568,0.583)。结论新生儿期G6PD缺乏症并发高胆红素血症黄疸发病时间早、进展快且严重,感染和母亲生产时胎膜早破为其主要诱因,G6PD酶活性浓度和Hb水平不能完全反应黄疸的严重程度。对新生儿期Hb无明显下降的高胆红素血症患儿应常规行G6PD活性检查,并给与积极的抗感染和退黄治疗,预防胆红素脑病的发生。

Objective To investigate the predisposing factors,clinical features,and prognosis of neonatal G6PD deficiency complicated with hyperbilirubinemia.Methods A retrospective analysis of 72 cases of neonatal G6PD deficiency complicated with hyperbilirubinemia in neonatal department of our hospital from 2011 to 2015.Results Neonatal G6PD deficiency complicated with hyperbilirubinemia was mainly induced by infection and abnormal situation during pregnancy or later production.Sex ratio was that male： female was 4： 1. The emergence time of hyperbilirubinemia was mainly within 7d after birth（ 75%）. There were 22 cases（ 30. 6%） with anemia,and 2 cases（ 2. 8%） with bilirubin encephalopathy.The prognosis was that 57 patients cured and discharged（ 79. 2%）,and15 cases（ 20. 8%） improved and discharged.Regression analysis showed that there was no significant correlation between serum total bilirubin and G6PD enzyme activity and Hb levels（ r =- 0. 167,-0. 175; P = 0. 568,0. 583）. Conclusion Neonatal G6PD deficiency disease complicated with hyperbilirubinemia jaundice is with early incidence time,rapid progression and it is serious.Infection and premature rupture of membranes during maternal production are the main causes of it.G6PD enzyme activity concentration and Hb levels can not completely reflect the severity of jaundice.Newborn with neonatal hyperbilirubinemia which has not significant decrease in Hb levels should be routinely performed G6PD biopsy,and given positive anti infection and jaundice treatment,in order to prevent the occurrence of bilirubin encephalopathy.