光疗对G-6-PD缺陷新生儿溶血的效应

目的　探讨光疗是否有加重红细胞葡萄糖6-磷酸脱氢酶(G - 6-PD)缺陷的黄疸儿溶血的作用,为制定光疗指征提供参考。方法　将G - 6-PD缺陷轻度黄疸新生儿71例分成积极光疗组和对照组,积极光疗组早期进行光疗,对照组尽可能不进行光疗,比较两组出现继续溶血情况。结果　积极光疗组3 7例,出现继续溶血9例( 9/ 3 7) ;对照组3 4例,出现继续溶血3例( 3 / 3 4)。积极光疗组继续溶血倾向明显高于对照组(P <0 0 1 ,U =2 75 )。结论　光疗可加重G - 6-PD缺陷的黄疸新生儿溶血,应从严掌握该类患儿的光疗指征     

不同G-6-PD活性新生儿光疗致溶血机制及其预防

目的 探讨不同G-6-PD活性新生儿光疗溶血机制及预防。方法将G-6-PD正常与缺陷光疗患儿随机分为维生素E干预组和对照组，测定比较超氧化物歧化酶（SOD）、丙二醛（MDA）、活性氧（ROS）、总胆红素（TB）、血红蛋白（Hb）及光疗指数。结果光疗前G-6-PD缺陷组比正常组SOD和Hb低，ROS高；光疗中G-6-PD缺陷干预组比正常干预组SOD高，MDA低，光疗指数小．G-6-PD缺陷对照组比正常对照组ROS、MDA高，光疗指数大（各组比较均P〈0．01或P〈0．05）。光疗后G-6-PD缺陷对照组Hb下降，并比干预组低，G-6-PD正常两组Hb均下降，干预组比对照组高（各组比较均P〈0．01或P〈0．05）。结论光疗可致抗氧化能力下降，脂质过氧化损伤致G-6-PD缺陷光疗者溶血更突出，维生素E干预更有效。     

不同G-6-PD活性新生儿光疗致溶血机制及其预防

目的探讨不同G6PD活性新生儿光疗溶血机制及预防。方法将G6PD正常与缺陷光疗患儿随机分为维生素E干预组和对照组,测定比较超氧化物歧化酶(SOD)、丙二醛(MDA)、活性氧(ROS)、总胆红素(TB)、血红蛋白(Hb)及光疗指数。结果光疗前G6PD缺陷组比正常组SOD和Hb低,ROS高;光疗中G6PD缺陷干预组比正常干预组SOD高,MDA低,光疗指数小,G6PD缺陷对照组比正常对照组ROS、MDA高,光疗指数大(各组比较均P<0.01或P<0.05)。光疗后G6PD缺陷对照组Hb下降,并比干预组低,G6PD正常两组Hb均下降,干预组比对照组高(各组比较均P<0.01或P<0.05)。结论光疗可致抗氧化能力下降,脂质过氧化损伤致G6PD缺陷光疗者溶血更突出,维生素E干预更有效。     

光疗对G-6-PD缺陷新生儿溶血的效应

目的 探讨光疗是否有加重红细胞葡萄糖6-磷酸脱氢酶(G-6-PD)缺陷的黄疸儿溶血的作用，为制定光疗指征提供参考。方法 将G-6-PD缺陷轻度黄疸新生儿71例分成积极光疗组和对照组，积极光疗组早期进行光疗，对照组尽可能不进行光疗，比较两组出现继续溶血情况。结果 积极光疗组37例，出现继续溶血9例(9／37)；对照组34例，出现继续溶血3例(3/34)。积极光疗组继续溶血倾向明显高于对照组(P<0．01，U=2．75)。结论 光疗可加重G-6-PD缺陷的黄疸新生儿溶血，应从严掌握该类患儿的光疗指征。     

新生儿G-6-PD缺陷症合并巨细胞病毒感染的研究

了解新生儿G-6-PD缺陷症合并巨细胞病毒感染的情况.方法对新生儿高胆红素血症患儿进行G-6-PD活性、PCR HCMV及其他相关检查.结果新生儿G-6-PD缺陷症病人合并HCMV(+)者其ALT、IBIL、DBIL、血红蛋白、胆汁酸、G-6-PD活性与新生儿G-6-PD缺陷症HCMV(-)者、非G-6-PD缺陷症HCMV(+)者、非G-6-PD缺陷症HCMV(-)者比较差异均有显著性.结论新生儿G-6-PD缺陷症合并巨细胞病毒感染会加重肝脏、血液系统的损害,巨细胞病毒感染可降低新生儿G-6-PD活性.     

G-6-PD缺陷症患儿急性溶血时白细胞计数与病情程度的关系探讨

G-6-PD缺陷症是全世界最常见的一种遗传性红细胞酶缺陷病，在我国该病常见于华南和西南地区。本文收集我院于2000年2月～2004年5月共收治的G-6-PD缺陷症引起急性溶血性贫血64例，现报告如下。     

早期治疗对G-6-PD缺乏并发胆红素脑病的影响

新生儿红细胞葡萄糖-6-磷酸脱氢酶(G-6-PD)缺乏在围产期可在一些因素下引起高胆红素血症,早已为国内外文献所证实,且常因诊断过晚导致凶险胆红素脑病。本文通     

Leucocyte glucose-6-phosphate dehydrogenase (G-6-PD) activity in G-6-PD deficient subjects

The activity of glucose-6-phosphate dehydrogenase (G-6-PD) in leucocytes was studied in the following groups of Greek people.Group 1: 43 male children and 16 male students with mean values of enzyme activity of 27.7±16.6 units and 24.6±5.6 units, respectively.Group 2: 15 G-6-PD deficient male children who had never experienced an acute haemolytic episode with a mean value of 10.8±4.6 units.Group 3: 19 G-6-PD deficient male children during favism and 3 months after the haemolytic crisis with mean values of 8±4 units and 9.2±1.9 units, respectively.Group 4: 19 mothers of children from group 3 who by definition were carriers of G-6-PD deficiency had a mean value of 18.2±8.2 units.The difference between means for group 1 and groups 2, 3 and 4 is highly significant (P<0.001).Therefore the enzymatic defect in Greek people is not limited to the erythrocytes but can be also demonstrated in leucocytes.     

葡萄糖-6-磷酸脱氢酶缺乏症患儿G-6-PDmRNA表达的研究

目的：检测葡萄糖-6-磷酸脱氢酶(G-6-PD)缺乏症患者及其家系成员的G-6-PDmRNA表达水平,从转录水平探讨其可能的发病机制。方法：提取G-6-PD缺乏症患者及其直系家属(患者父亲和/或母亲等)外周血RNA,采用逆转录方法形成cDNA后,运用逆转录实时定量PCR(QuantitativeReal-TimePCR,QRT-PCR)技术,测定G-6-PDmRNA的表达量。使用SPSS10.0统计分析软件将3组进行组间两两比较。结果：G-6-PD缺乏症患儿组mRNA表达量为0.57±0.19,父系组为0.74±0.21,母系组为0.67±0.21,患儿组与父系组比较t=-3.18,(P<0.01);与母系组比较t=-2.54,(P<0.05)。结论：G-6-PD缺乏症患者的G-6-PD基因发生突变后其G-6-PDmRNA表达量发生了改变,提示该病的发生与在转录水平上发生变化有关,在G-6-PD缺乏症的发病过程中起到一定的作用。     

Acute intravascular haemolysis following exchange transfusion with G-6-PD deficient blood

A neonate with hyperbilirubinaemia who developed massive intravascular haemolysis following exchange transfusion with glucose-6-phosphate dehydrogenase deficient blood is described. It is recommended that in areas endemic for this enzyme deficiency the donor blood should be screened before being used for exchange transfusion.     

β-Thalassemia trait and hyperbilirubinemia in G-6-PD deficient newborn infants

Hb A₂ was determined in 50 subjects with erythrocyte G-6-PD deficiency who presented with hyperbilirubinemia in the neonatal period and in 100 non-hyperbilirubinemic G-6-PD deficient newborn infants, at the age of 12 months or more. Six subjects in the first group and 13 in the second were found to be carriers of the -thalassemia trait. Statistical analysis of the data did not show any significant difference between the two groups. It seems that the -thalassemia trait does not provide any protection against neonatal hyperbilirubinemia associated with G-6-PD deficiency.     

丙种球蛋白辅助治疗红细胞G-6-PD缺陷所致新生儿黄疸疗效观察

目的 观察由红细胞葡萄糖-6-磷酸脱氢酶缺陷(G-6-PD)所致新生儿黄疸治疗时,静脉注射丙种球蛋白(IVIG)的退黄效果。方法 31例G-6-PD缺陷新生儿黄疸中12例对照组用常规治疗,19例治疗组除常规治疗外加用IVIG每日0.4～1.0 g/kg。用经皮胆红素测定仪监测治疗疗效。结果 在治疗24和48 h后与治疗前对比,治疗组的退黄效果和对照组比较差异有显著性意义(P<0.001)。结论 加用IVIG治疗因G-6-PD缺陷致新生儿黄疸有效。