Zhong Dan-Ni, Gao Zong-Yan, Liu You-Nan, Liu Yi, Wei Lu-Ming
Department of Pediatrics, First Affiliated Hospital, Guangxi Medical University, Nanning 530021, China.
Zhongguo Dang Dai Er Ke Za Zhi. 2009 Dec;11(12):970-2.
To study the correlation between glucose-6-phosphate dehydrogenase (G-6-PD) activities and three common mutations of G-6-PD gene G1388A, G1376T and A95G and investigate the effects of G-6-PD gene mutations on neonatal jaundice in Nanning, Guangxi.
One hundred and twenty-four neonates from Nanning, Guangxi, with hyperbilirubinemia were enrolled. The ARMS-PCR and PCR/REA methods were used to determine G-6-PD gene mutations. G-6-PD activities were measured using the NBT method. The incidence of acute bilirubin encephalopathy and the peak bilirubin concentration 72 hrs after birth were compared between the neonates with different genotypes and between the G-6-PD mutation and normal groups. The risk of blood serum bilirubin >340 mumol/L was evaluated by logistic regression analysis.
Of the 124 cases, gene mutations were found in 37 cases, including G1388A (n=20), G1376T (n=14), A95G (n=4) and G1388A+A95G (n=1). Five cases (25%) showed normal G-6-PD activities in the G1388A gene mutation group and 4 (29%) had normal G-6-PD activities in the G1376T G1388A gene mutation group. All of 4 cases of A95G G1388A gene mutation showed a deficiency of G-6-PD activities. There were no significant differences in the incidence of acute bilirubin encephalopathy and the peak bilirubin concentration 72 hrs after birth between the G1388A and G1376T G1388A gene mutation groups. The incidence of acute bilirubin encephalopathy, the peak bilirubin concentration 72 hrs after birth and the risk of serum bilirubin >340 micromol/L in the G-6-PD mutation group were not different from the normal group.
G1388A, G1376T and A95G are common G-6-PD gene mutations in Nanning, Guangxi. The false negative results may be received when the NBT method is used for diagnosis of G-6-PD deficiency. There are similar effects on the incidence of acute bilirubin encephalopathy and the peak bilirubin concentration 72 hrs after birth between different gene mutation groups. G-6-PD gene mutations alone may not contribute to the development of acute bilirubin encephalopathy and the changes of peak bilirubin concentration 72 hrs after birth and the risk of serum bilirubin >340 micromol/L.
研究葡萄糖-6-磷酸脱氢酶(G-6-PD)活性与G-6-PD基因G1388A、G1376T和A95G三种常见突变之间的相关性,并探讨G-6-PD基因突变对广西南宁新生儿黄疸的影响。
纳入广西南宁124例高胆红素血症新生儿。采用扩增阻滞突变系统聚合酶链反应(ARMS-PCR)和聚合酶链反应/限制性内切酶分析(PCR/REA)方法检测G-6-PD基因突变。采用NBT法测定G-6-PD活性。比较不同基因型新生儿以及G-6-PD突变组与正常组急性胆红素脑病的发生率及出生后72小时胆红素峰值浓度。通过逻辑回归分析评估血清胆红素>340μmol/L的风险。
124例中,37例发现基因突变,包括G1388A(n=20)、G1376T(n=14)、A95G(n=4)和G1388A+A95G(n=1)。G1388A基因突变组中有5例(25%)G-6-PD活性正常,G1376T G1388A基因突变组中有4例(29%)G-6-PD活性正常。4例A95G G1388A基因突变者G-6-PD活性均缺乏。G1388A和G1376T G1388A基因突变组急性胆红素脑病的发生率及出生后72小时胆红素峰值浓度差异无统计学意义。G-6-PD突变组急性胆红素脑病的发生率、出生后72小时胆红素峰值浓度及血清胆红素>340μmol/L的风险与正常组无差异。
G1388A、G1376T和A95G是广西南宁G-6-PD基因的常见突变。采用NBT法诊断G-6-PD缺乏时可能出现假阴性结果。不同基因突变组对急性胆红素脑病的发生率及出生后72小时胆红素峰值浓度的影响相似。单纯G-6-PD基因突变可能与急性胆红素脑病的发生、出生后72小时胆红素峰值浓度的变化及血清胆红素>340μmol/L的风险无关。