新生儿G6PD缺乏症并发高胆红素血症的临床分析

目的：回顾性分析新生儿GBPD缺乏症并发新生儿高胆红素血症的临床特点，探讨G6PD缺乏症在新生儿期发病的诱因、临床进程及诊治效果。方法：2007—10—201206收治的12例新生儿G6PD缺乏症并发高胆红素血症患儿，对其病因、临床表现及转归进行分析。结果：黄疸高峰出现在生后第2～7天，血清总胆红素（STB）峰值为（417．38±154．73）μmol／L，STB〉342μmol／L占66．7％。血红蛋白（Hb）为52～164g／L，平均为（123．37±34．54）g／L，Hb〈140g／L占66．7％；血网织红细胞（Ret）为1．31％～4．22％，均值（2．50±0．94）％。G6PD／6PGD比值平均为（0．44±0．18）。回归分析显示STB与G6PD／6PGD比值、HB和Ret均无明显相关性（r=-0．175，-0．180，0．272；P=0．587，0．576，0．393）。合并疾病及并发症：感染4例，窒息缺氧1例，ABO溶血病2例，核黄疸2例。结论：G6PD缺乏所致新生儿高胆红素血症发病早，黄疸进展快而严重，大多数患儿无明显诱因；G6PD活性和Hb水平不能完全反映黄疸的严重性或溶血程度。对新生儿早期重度黄疸应提高对本病的认识，常规行G6PD活性的检查，并给予积极治疗能有效预防胆红素脑病的发生。

Clinical analysis of neonatal G6PD deficiency complicated with hyperbilirubinemia

Objective：To analyze the clinical characteristics of neonatal G6PD deficiency complicated with hy- perbilirubinemia, and study the predisposing factor, clinical process, diagnosis,and treatment outcome of G6PD de- ficiency disease during neonatal period. Method： 12 neonates of G6PD deficiency complicated with hyperbilirubinemia were reviewed from Oct. 2007 to J un. 2012, about the etiology, clinical features and outcomes. Result：The peak time of hyperbilirubinemia ranged from 2 to 7 days alter birth. The maximum serum total bilirubin （STB） was （417.38±154.73） μmol/L,and eight of patients with STB〉342 μmol/L（66.7 ~ ）. Blood hemoglobin level （Hb） range was 52-164 g/L,mean Hb level was （123.37±34.54） g/L and Hb〈140 g/L in 66.7% of patients. Blood reticulocyte （Ret） counted 1.31-4.22% and （2.50±0.94）% as average value. There was no significant correla- tion between STB and either G6PD/6PGD ratio,Hb or Ret （r=-0. 175,-0. 180,0. 272;P=0. 587,0. 576,0. 393, respectively）. The comorbidities：4 cases of infectious diseases,and 1 case of asphyxia, 2 cases of ABO hemo lytic disease,2 cases of kernicterus. Conclusion：This study showed that neonatal jaundice induced by G6PD deficiency was characterized by early onset, rapid progression and severe hyperbilirubinemia. Most babies had no obvious trigger factors. G6PD activity and Hb level can not fully reflect the severity of jaundice or the degree of hemolysis. For neonates with early severe jaundice,we suggest that G6PD activity should be examined to exclude G6PD deficiency.