G6PD缺陷新生儿高胆红素血症光疗时氧自由基变化探讨

目的探讨G6PD缺陷新生儿光疗时氧自由基变化。方法将G6PD缺陷光疗新生儿随机分维生素E干预组与对照组,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。结果各组30例,光疗前干预组与对照组各指标无显著差异(P>0.05),两组比正常新生儿组MDA、ROS高,HB、SOD低(P<0.01或0.05)。对照组光疗24hSOD、HB降低,MDA增高。光疗24、36、48h干预组比对照组SOD、HB高,MDA、ROS低、光疗指数小(P<0.01或0.05)。结论G6PD缺陷新生儿光疗时,氧自由基致红细胞脂质过氧化损伤加重,溶血加剧,维生素E有保护作用。     

6432例住院分娩活产儿G-6-PD缺陷症筛查状况

目的:了解6 432例住院分娩活产新生儿红细胞葡萄糖-6-磷酸脱氢酶(G-6-PD)缺陷症的筛查状况。方法:采用G6PD/6PGD比值法进行检测,ODG6PD/OD6GPD比值﹤1.0为G-6-PD缺陷症。结果:住院分娩活产儿G-6-PD筛查率为85.17%(5 478/6 432),男性G-6-PD缺陷症发生率为8.98%(253/2 818),高于女性〔1.80%(48/2 660)〕,差异有统计学意义(χ2=145.173,P<0.05)。结论:提高新生儿G-6-PD缺陷症的筛查率,及早发现G-6-PD缺陷症可避免核黄疸和药源性溶血的发生,提高人口素质。     

重组人类促红细胞生成素治疗新生儿高胆红素血症后贫血临床研究

目的 :探讨使用重组人类促红细胞生成素 (rh Epo)治疗新生儿溶血性高胆红素血症后贫血的可行性、有效性及安全性。方法 :对生后 2～ 5 d临床诊断足月新生儿 G- 6 PD缺陷症或 ABO溶血高胆红素血症随机分为两组。行黄疸治疗待肉眼黄疸消退后 ,治疗组 18例皮下注射 rh Epo2 0 0μ /kg/次 ,3d1次 ,共 6次。结果 :开始使用 rh Epo后第8～ 9w,治疗组与对照组 Hb、Hct、Ret等比较已呈显著差异 (P<0 .0 0 0 1)。在治疗期间血清铁蛋白尚在正常值范围。结论 :早期使用 rh Epo积极干预新生儿溶血性高胆红素血症后贫血 ,是可行、安全及有效的。同时进行正常的母乳喂养可不需额外添加铁剂。     

蓝光治疗对新生儿红细胞葡萄糖-6-磷酸脱氢酶缺乏症所致高胆红素血症患儿溶血反应的影响

目的研究蓝光治疗对新生儿红细胞葡萄糖-6-磷酸脱氢酶(G-6-PD)缺乏症所致高胆红素血症患儿溶血反应的影响。方法选择2013年2~12月,广东省肇庆市妇幼保健院收治的59例G-6-PD缺乏症患儿为研究对象。根据入院顺序号随机将其分为G-6-PD缺乏接受蓝光治疗+综合治疗组(研究组,n=34)和G-6-PD缺乏仅接受综合治疗,未接受蓝光治疗组(对照组,n=25)。研究组与对照组患儿的性别构成比、日龄、喂养方式及分娩方式等比较,差异均无统计学意义(P>0.05)。本研究遵循的程序符合广东省肇庆市妇幼保健院人体试验委员会制定的伦理学标准,得到该委员会批准,分组征得受试对象监护人的知情同意,并与其签署临床研究知情同意书。分别比较两组患儿治疗前、后及两组患儿治疗48h后红细胞计数、血红蛋白(Hb)水平及其变化情况。结果研究组和对照组患儿治疗前出生体质量、总胆红素(TBIL)、直接胆红素(DBIL)、间接胆红素(IBIL)、红细胞计数、Hb水平比较,差异均无统计学意义(P>0.05)。研究组及对照组患儿接受治疗后,TBIL、DBIL、IBIL、红细胞计数、Hb水平均较治疗前降低,并且差异均有统计学意义(P<0.05)。此外,研究组患儿接受蓝光治疗48h后红细胞计数及Hb水平明显低于对照组,并且差异均有统计学意义(P<0.05)。结论蓝光治疗虽是治疗G-6-PD缺乏所致新生儿高胆红素血症的有效方法,但同时亦可加重G-6-PD缺乏所致新生儿高胆红素血症患儿溶血反应。     

新生儿脐血筛查对诊断G-6-PD缺陷的应用价值

目的 :通过比较新生儿脐血筛查 G- 6 - PD缺陷与幼儿血检查 G- 6 - PD缺陷 ,了解本地区 G- 6 - PD缺陷的发生率及新生儿脐血筛查对诊断 G- 6 - PD缺陷的应用价值。方法 :对我院产科出生的 115 74例全部活产婴作脐血 G- 6 - PD筛查 ,分别于 3～ 5岁对脐血检测 G- 6 - PD缺陷者 ( 32 0例 )和 G- 6 - PD正常者 ( 2 15例 )复查 G- 6 - PD活性。结果 :115 74例中 ,脐血检测 G- 6 - PD缺陷 6 4 1例 ,缺陷率为 5 .5 % ;男性缺陷率为 7.9% ,女性为 3.0 % ,两者有高度显著性差异 ( P<0 .0 0 1) ,对缺陷者复查幼儿血检测 G- 6 - PD活性 ,两者符合率高达 96 .7%。结论 :G- 6 - PD缺陷在本地区常见 ,脐血筛查可作为诊断 G- 6 - PD缺陷的有力依据 ,对降低其发病率有重要意义     

脐血溶血三项对新生儿ABO溶血病早期诊断和治疗的意义

目的:探讨脐血溶血三项在新生儿ABO溶血病早期诊断和治疗中的作用。方法:根据新生儿ABO溶血病的确诊时间及是否给予人免疫球蛋白治疗分组,将脐血溶血三项确诊者分为A组(给予静脉滴注大剂量人免疫球蛋白)和B组(未给予静脉滴注大剂量人免疫球蛋白),C组(对照组)为黄疸以溶血三项确诊者,并予静脉滴注大剂量人免疫球蛋白。观察各组的入院日龄、入院胆红素(TB)值、TB峰值、蓝光光疗时间、平均住院时间及血红蛋白(Hb)低值的差异。结果:A组与B组比较其光疗时间、平均住院时间及Hb低值差异均有统计学意义(P0.05);A组与C组比较其入院日龄、入院TB值、TB峰值、光疗时间、平均住院时间及Hb低值差异均有统计学意义(P0.05)。结论:监测脐血溶血三项有助于早期确诊新生儿ABO溶血病,对脐血溶血三项阳性患儿早期静脉应用大剂量人免疫球蛋白可有效降低胆红素水平和贫血程度,缩短光疗时间及平均住院日。     

综合护理干预在丙种球蛋白联合光疗治疗新生儿ABO溶血中的应用效果

目的探究综合护理干预在新生儿ABO溶血应用丙种球蛋白联合光疗治疗的效果。方法选取2019年9月至2020年9月蚌埠市第三人民医院收治的82例新生儿ABO溶血患儿作为研究对象,按照随机数字表法将其分为对照组与观察组,每组各41例。两组均采用丙种球蛋白联合光疗治疗,对照组采用常规护理干预,观察组采用综合护理干预,比较两组的临床效果、血清总胆红素、血红蛋白、网织红细胞、不良反应。结果观察组的黄疸消退时间、住院时间及光疗时间短于对照组;两组护理后的血清总胆红素、网织红细胞低于护理前,血红蛋白高于护理前,且观察组护理后的血清总胆红素、网织红细胞低于对照组,血红蛋白高于对照组,观察组的不良反应总发生率低于对照组,差异均有统计学意义(P<0.05)。结论在新生儿ABO溶血提供丙种球蛋白联合光疗治疗的基础上,配合综合护理干预,能够有效改善患儿临床效果,具有临床推广价值。     

抗氧化剂治疗G6PD缺乏孕妇对降低新生儿黄疸的探讨

采用G6PD/6PGD比值法筛查了2682例孕妇,发现303例有不同程度的G6PD缺乏。对其中195例自妊娠30周起服用较大剂量的抗氧化剂VitE和VitC进行诱导性治疗。与同期108例未接受治疗者对照。结果:两组新生儿黄疸及高胆红素血症发生率均有显著性差异,治疗组无1例核黄疸发生。说明用抗氧化剂治疗孕妇G6PD缺陷症,对预防孕妇溶血及降低新生儿黄疸具有显著的效果。     

96例新生儿G-6PD缺陷所致高间接胆红素血症临床诱因分析及治疗探讨

我国南方地区红细胞葡萄糖6-磷酸脱氢酶缺陷病(简称G-6PD缺陷)是常见病,也是引起新生儿高间接胆红素血症(简称高胆)主要病因。本文对我院近三年收治的96例新生儿G-6PD缺陷所致高胆进行临床分析及治疗探讨。     

双重病因对新生儿高胆红素血症发生率影响探讨

目的:探讨存在葡萄糖6磷酸脱氢酶(G6PD)缺乏和新生儿溶血病(HDN)双重病因对新生儿高胆红素血症发生率的影响。方法:按检验操作规程对足月健康新生儿脐血进行G6PD和HDN血型血清学检验,对存在G6PD缺乏和HDN病因的新生儿调查其高胆红素血症发生率。结果:①G6PD缺乏组、HDN病因组、G6PD缺乏与HDN双重病因组的患儿高胆红素血症发生率均极显著高于正常对照组的新生儿(P<0.01);②存在G6PD缺乏和HDN双重病因的患儿高胆红素血症发生率极显著高于G6PD缺乏组和HDN病因组的患儿(P<0.01)。结论:存在G6PD缺乏和HDN双重病因的患儿红细胞溶血破坏的几率增高,其发生高胆红素血症的可能性更大。     

新生儿红细胞葡萄糖-6-磷酸脱氢酶缺乏筛查结果分析

目的:了解广东省肇庆市端州区新生儿脐血红细胞葡萄糖-6-磷酸脱氢酶(G-6-PD)缺乏的发生率。方法:2 236例活产新生儿出生后即取脐血,采用定量法测定红细胞G-6-PD/6-PGD的比值,低于1.0者为G-6-PD缺乏。结果:G-6-PD缺乏的患儿152例,总发生率为6.79%。其中男129例,发生率为10.34%;女23例,发生率为2.32%,男性发生率明显高于女性(2χ=90.03,P<0.001)。结论:脐血G-6-PD活性筛查,能比较准确地检测出G-6-PD缺乏患儿,指导临床对其并发症进行早期干预,避免智力低下等后遗症的发生,提高人口素质。     

11574例新生儿脐血G-6-PD筛查结果分析

目的了解本地区G-6-PD缺陷的发生率及新生儿脐血筛查对诊断G-6-PD缺陷的应用价值。方法对产科出生的11574例全部活产婴作脐血G-6-PD筛查,分别于3~5岁对脐血检测G-6-PD缺陷者(320例)和G-6-PD正常者(215例)复查G-6-PD活性。结果11574例中,脐血检测G-6-PD缺陷641例,缺陷率为5.5%;男性缺陷率为7.9%,女性为3.0%,二者比较差异有统计学意义(P<0.001),对缺陷者复查幼儿血检测G-6-PD活性,二者符合率高达96.7%。结论G-6-PD缺陷在本地区常见,脐血筛查可作为诊断G-6-PD缺陷的有力依据,对降低其发病率有重要意义。     

Association between glucose-6-phosphate dehydrogenase deficiency and neonatal jaundice: interaction with multiple risk factors.

This nonconcurrent cohort study was carried out to evaluate the association of neonatal jaundice with glucose-6-phosphate dehydrogenase (G-6-PD) deficiency and its interactions with other risk factors. The G-6-PD enzyme activity of 12,379 neonates was screened by a semi-quantitative fluorometric assay and double-checked by a quantitative method to identify a G-6-PD deficient cohort of 333 neonates. Matched with these on birth date, sex and delivery hospital were a G-6-PD normal cohort of 653 neonates. Neonatal jaundice was defined by a peak serum bilirubin (PSB) level of > or = 15 mg/dl. A significant association between G-6-PD deficiency and neonatal jaundice was observed in male but not female neonates. There was an inverse dose-response relation between G-6-PD activity and neonatal jaundice among male neonates. Both hypoxia/asphyxia and maternal hepatitis B surface antigen (HBsAg) carrier status were associated with an increased risk of neonatal jaundice among G-6-PD deficient but not G-6-PD normal male neonates. Based on multiple regression analyses, an additively synergistic effect on PSB level and severe jaundice (PSB > or = 20 mg/dl) was observed for G-6-PD deficiency and maternal HBsAg carrier status.     

Glucose-6-phosphate dehydrogenase deficiency, neonatal tetanus and neonatal jaundice in Nigeria: Clinical observation

In a study of 92 cases of neonatal tetanus (NNT), it was found that 23 (25%) had associated neonatal jaundice (NNJ) but in none of them was NNJ severe enough to cause kernicterus. Glucose-6-Phosphate dehydrogenase (G-6-PD) deficiency was less common in babies with NNT than expected in general population (p 0.02). These babies with NNT were as exposed to icterogenic agents as jaundiced babies previously reported from the same institution. The lower incidence of G-6-PD deficiency in babies with NNT was probably due to the fact that G-6-PD deficient babies on exposure to icterogenic agents develop severe NNJ early and may die of kernicterus. Antibiotic therapy in jaundiced babies may prevent NNT in some of the G-6-PD deficient babies who are more prone to severe NNJ. More of the G-6-PD normal babies therefore remained at home till they develop NNT.

It is speculated that steps taken to prevent NNJ in the G-6-PD deficient babies by avoiding application of potentially infected icterogenic agents (i.e. menthol containing dusting powder or balms) to the cord will also prevent NNT in some of the G-6-PD normal babies.     

Genetico-epidemiological and molecular investigation of G-6-PD deficiency in a Brazilian community

This paper reports on a study of the G-6-PD deficiency in Bragan?a Paulista, S?o Paulo State, Brazil. A total of 4,621 male blood donors were investigated over a 36-month period. Of these, 80 had the G-6-PD deficiency. Molecular analysis was performed on 70 unrelated G-6-PD deficients through DNA amplification followed by digestion with restriction enzymes and single strand conformation polymorphism analysis (SSCP). In 98.6%, the G-6-PD A- (202 G<--A) mutation was observed through digestion of exon 4 with Nla III. The presence of an uncommon mutation in exon 9 was also observed through SSCP. No case of the Mediterranean variant was observed. These results indicate that the A- (202G<--A) variant, almost exclusive, was introduced into the community not only by individuals of African origin, but also by European immigrants, mainly Italian, Spanish, and Portuguese. The Italian contribution in terms of the G-6-PD Mediterranean variant was smaller than its contribution to beta thalassemia, probably due to the Northern Italian origin of these immigrants.     

An evaluation of the dorset sheep as a predictive animal model for the response of glucose-6-phosphate dehydrogenase-deficient human erythrocytes to a proposed systemic toxic ozone intermediate, methyl oleate ozonide

Erythrocytes of both glucose-6-phosphate dehydrogenase (G-6-PD)-deficient humans and Dorset sheep, an animal model with an erythrocyte G-6-PD deficiency, responded in a dose-dependent manner to the oxidant stress of methyl oleate ozonide (MOO) as measured by decreases in G-6-PD activity, increases in methemoglobin (METHB) levels, and decreases in GSH. However, the human G-6-PD-deficient erythrocytes were considerably more sensitive to the formation of METHB than the sheep erythrocytes while the reverse was the case for the GSH parameter. The results suggest a qualitative difference in the response of sheep erythrocytes and human G-6-PD-deficient erythrocytes to MOO that seriously questions the value of the sheep erythrocyte as a quantitatively accurate predictive model.     

Genetic traits in the area of Bodrogk?z]

In 1984 a late malaria endemic area, called Bodrogk?z was studied. This was a reexamination of the population genetic work performed by Walter, Nemeskéri. In six villages of Bodrogk?z 328 persons were tested for AB0, Rh blood groups, haptoglobins, haemoglobin concentration, haematocrit, erythrocyte amount, the MCV, the MCH and the G-6-PD were analyzed. The quantitative determination of HbF and HbA2, red cell osmotic resistance and thalassemia were measured as well. Thalassemia heterozygote carriers and an increased level of HbF were revealed. The frequency of G-6-PD deficiency was 0.39%. In Bodrogk?z the frequencies of AB0, Rh and haptoglobin types were similar in the present and all previous studies. The background of this similarity might be the genetic similarity between two following generations. On the basis of these facts, the Hb0 Arab and partially DNA work we suggested an alternative hypothesis that these mutant genes got into Bodrogk?z by the rather later migration than with ancient Hungarian people during the period of conquest of Hungary.     

Blood pressure and other cardiovascular disease risk factors in black adults with sickle cell trait or glucose-6-phosphate dehydrogenase deficiency

In this study, we examined the relationship of two common genetic markers in black populations, sickle cell trait and glucose-6-phosphate dehydrogenase (G-6-PD) deficiency, to cardiovascular risk factors. The subjects were Nigerian civil servants in Benin City, Nigeria. We measured blood pressure, height, weight, sickle cell hemoglobin, G-6-PD, proteinuria, microalbuminuria and fasting serum cholesterol, high-density lipoprotein cholesterol (HDL), triglycerides, apoprotein (APO) AI, and APO B. Data were collected on age, alcohol consumption, cigarette smoking, job status, and years lived in an urban area. There were 257 males (3 SS hemoglobin, 73 AS, 181 AA) and 69 females (23 AS, 46 AA). In comparing cardiovascular risk factors, males differed only in percent of smokers (31.5 in AS vs. 17.8 in AA, P less than 0.01). Among females, only high-density lipoprotein (HDL) cholesterol differed (61.5 mg/dl in AS vs. 52.4 in AA, P less than 0.01). We hypothesize that females with sickle cell trait are more likely to use oral contraceptives than nontrait females. If so, the high-estrogen oral contraceptives available in Nigeria could elevate HDL. G-6-PD deficiency status among males (52 deficient, 207 nondeficient) and females (1 deficient, 5 carriers, 65 nondeficient) was not related to any of the cardiovascular risk factors. We conclude that sickle cell hemoglobin trait and G-6-PD deficiency are not useful genetic markers for risk factors for cardiovascular disease.     

甲醛对雄性小鼠睾丸中酶活力的影响

目的探讨甲醛对雄性小鼠睾丸中酶活力的影响。方法28只昆明种雄性小鼠随机分为4组,每组7只。设低、中、高剂量组分别以0.2、2.0、20.0mg/kg甲醛腹腔注射染毒,每天1次,连续7d,测定睾丸中乳酸脱氢酶(LDH)、葡萄糖-6-磷酸脱氢酶(G-6-PD)、山梨醇脱氢酶(SDH)活力。同时设对照组,腹腔注射生理盐水。结果小鼠睾丸G-6-PD、SDH活力随染毒剂量增大而降低,低、中、高剂量组SDH活力与对照组比较,差异均有统计学意义(P<0.05或P<0.01),低、中、高剂量组LDH和G-6-PD活力与对照组比较,差异无统计学意义(P>0.05)。结论甲醛对雄性小鼠睾丸中的酶具有不良影响,从而产生一定的生殖毒性。