去氢表雄酮及G6PD基因反义寡核苷酸对Raji细胞的某些影响

目的:观察去氢表雄酮（DHEA）及G6PD基因的全硫代修饰反义寡核苷酸对体外培养Raji细胞的抑制作用。 方法:分别用不同浓度DHEA及G6PD反义寡核苷酸作用于体外培养Raji细胞，观察细胞存活、生长情况，同时采用逆转录-聚合酶链反应检测Raji细胞G6PD基因mRNA表达水平，测定G6PD活性。 结果:DHEA及G6PD反义寡核苷酸不影响Raji细胞存活，50 μmol/L、500μmol/L浓度的DHEA作用72 h小时及10.0 μmol/L反义寡核苷酸作用48 h后均明显抑制Raji细胞生长（P＜0.01）；当DHEA≥5.0 μmol/L时，作用Raji细胞72 h，则G6PD活性明显低于未用DHEA作用的对照组（P＜0.01），但不影响G6PD基因mRNA表达；10.0 μmol/L反义寡核苷酸作用Raji细胞48 h后，G6PD基因mRNA表达水平低于未用反义寡核苷酸作用的对照组，G6PD活性亦明显低于对照组（P＜0.01）。 结论:一定浓度的DHEA及G6PD反义寡核苷酸均能抑制Raji细胞G6PD活性并不同程度影响细胞生长，但其抑制机制不同。     

MCS＋血细胞分离机在治疗性红细胞采集术中的应用

目的探讨MCS＋血细胞分离机应用于治疗性红细胞采集（TE）时的操作要点和临床观察指标。方法采用治疗性自体红细胞采集（TAE）程序，选择随机等量补充补偿液，不加添加剂模式。比较采集前后的红细胞（RBC）、红细胞比积（HCT）、血红蛋白（HGB）、B超等结果，观察治疗效果；分析比较机器显示的预计采后HCT值与实际采后的外周血HCT的差别。结果6例病人采集后外周血血常规（血Rt）示RBC、HCT、HGB均明显下降，与采集前比较差异有统计学意义（P〈0．01），原有脾肿大的患者B超均明显缩小；机器显示的预计采后HCT均比实际采后外周血HCT低，差异有统计学意义（P〈0．01）。结论应用MCS＋血细胞分离机进行他是安全有效的。     

吩嗪甲酸硫酯对葡萄糖-6-磷酸脱氢酶缺乏的人红细胞某些生化指标的影响

为探讨葡萄糖－6－磷酸脱氢酶（C6PD）缺乏的人红细胞在在作用下的变化情况。通过测定在作用前后G6PD缺乏人红细胞变形能力，高铁血红蛋白生成，过氧化氢酶活力，还原型谷胱甘肽（GSH），还原型三磷酸吡啶核苷（NADPH）的浓度，并与正常红细胞比较。结果显示G6PD缺乏红细胞在氧自由基作用下变形能力，过氧化氢酶活力，GSH及NADPH水平与正常红细胞相比显著下降（P＜0．01），而高铁血红蛋白生成显著增加（P＜0．01），从而得出G6PD缺乏红细胞氧化易感性增强导致细胞损伤，变形能力下降是其易发溶血的主要原因的结论。     

脊柱结核患者血常规参数的变化及其临床意义

目的 研究脊柱结核患者血常规中各项参数的变化及其临床意义。方法 选取宁夏医科大学总医院2017年1月~2018年6月收治的脊柱结核患者70例为研究组,对照组为同期该院体检中心体检的健康人员70例。统计两组血常规中的参数值,比较两组之间的差异。结果 与对照组相比,研究组的白细胞总数（WBC）无明显变化（P＞0.05）,中性粒细胞计数（NEU）、单核细胞计数（MXD）增加,淋巴细胞计数（LYM）减少,单核细胞/淋巴细胞比例（M/L）增加（P＜0.05）;研究组存在轻度贫血,表现为红细胞计数（RBC）、血红蛋白含量（HGB）、平均红细胞HGB含量（MCH）、平均红细胞HGB浓度（MCHC）、红细胞压积（HCT）下降,红细胞分布宽度（RDW）增加（P＜0.05）;研究组的血小板计数（PLT）和血小板压积（PCT）明显增加（P＜0.05）。结论 与健康体检人员相比,脊柱结核患者的血常规中某些参数值存在异常,可能为脊柱结核的早期诊断提供依据。     

重庆地区G6PD缺乏症的产前筛查结果分析

目的建立适合于G6PD缺乏症的产前筛查方法 ,了解G6PD缺乏症的发病情况。方法应用荧光斑点法（FST）和G6PD/6PGD比值法检测1500对孕妇夫妇G6PD活性。结果 FST检出男性G6PD缺乏阳性64例,阳性率4.27%;女性59例,阳性率3.93%。男女之间G6PD缺乏程度差异非常显著（χ^2=107.62,P〈0.01）。比值法检出男性G6PD缺乏阳性64例,阳性率4.27%;女性49例,阳性率3.77%,男女G6PD缺乏者之间的酶活性差异非常显著（χ^2=97.70,P〈0.01）。两种方法的符合率男性为100%,女性为83.1%,总符合率为91.9%,FST和比值法的检出率无差异（P〉0.05）。结论 FST法具有高的敏感性、特异性和准确性,方法简便、快捷、费用低廉,同时利用比值法进行确诊,结合两法的结果综合判断,可减少假阳性及假阴性,有利于早期诊断和防治G6PD缺乏症所致的急性溶血。     

羊水红细胞计数在产前诊断的应用

目的探讨中孕期羊膜腔穿刺后羊水中红细胞计数的结果与羊水细胞（染色体制备）培养成功率的关系。方法测定中孕期羊膜腔穿刺后羊水中红细胞计数的结果 ,根据羊水的物理性状,红细胞计数测定值与培养成功率将28例羊膜腔穿刺病例分为组Ⅰ组Ⅱ组Ⅲ组,并对各组数值进行统计学分析。结果Ⅲ组的RBC计数值显著高于其他各组（P〈0.01）。Ⅰ组中占分布最高百分比的RBC计数值〈0.2×10^12/L,0.2±10^12/L〈Ⅱ组〈0.9×10^12/L,Ⅲ组〉1.0×10^12/L。结论中孕期羊膜腔穿刺术血性羊水中红细胞计数测定有利于羊水细胞（染色体制备）培养成功率的估计。当RBC计数〉1.0×10^12/L时,血性羊水细胞培养失败的可能性大;RBC计数〈0.9-1.0×10^12/L时,血性羊水细胞培养成功的可能性较大。     

低剂量γ射线辐照对人红细胞免疫功能和血清抗氧化酶活性的影响

目的：观察低剂量γ射线对人红细胞免疫功能和血清抗氧化酶活性的影响。方法：10份人全血，采用γ射线照射，照射剂量为1Gy，并分别于照射前及照射后1h，2h检测红细胞C3b受体花环（RBC—C30bRR）、红细胞免疫复合花环（RBC—ICR）及肿瘤红细胞花环率（RTRR）的变化情况及血清中超氧化物歧化酶（SOD），谷胱甘肽过氧化物酶（GSH—Px），谷胱甘肽还原酶（GR）及过氧化氢酶（CAT）的活性。结果：人红细胞经γ射线照射后，RBC—C3R及RTRR较照射前明显升高（P〈0．05），而RBC—ICR无明显变化（P〉0．05）。经γ射线照射1h后，SOD，GSH—Px，GR及CAT活性与照射前比较明显升高（P〈0.1），照射后2h，除SOD外，所有指标与照射后1h比较进一步升高（P〈0．05）。结论：人红细胞在体外经低剂量γ射线照射后，可明显增强红细胞的免疫功能和明显提高血清抗氧化酶活性，即可诱导红细胞免疫和抗氧化酶的兴奋效应。     

镰状细胞病合并G6PD缺陷症五例临床分析

目的总结5例血红蛋白S病（HbS）各类实验数据,为临床提供实验诊断,预防新生儿的严重并发症。方法血常规检测,全自动血红蛋白电泳,G6PD/6PGD直接比值法,G6PD/6PGD全自动生化仪日立7600检测G6PD缺陷症。结果检测5例HbS患者,发现合并a地贫1例,4例合并G6PD缺陷症。结论 Hb电泳区带定量是诊断HbS的重要方法。镰变试验是鉴别HbS与HbD的确诊试验。G6PD缺陷症在非洲裔HbS患者中有较高的发生率,如果同时合并2种遗传病会加重贫血症状。     

新生儿脐带血与静脉血G6PD定量结果的比较

目的探讨新生儿脐带血与静脉血G6PD定量结果的相关性。方法留取2010年07月在我院产科出生的新生儿脐带血112例,（其中男婴65例,女婴47例）,同步抽取静脉血,用日立7180全自动生化仪测定HB及G6PD活性,以Ug/HB单位结果进行比较。结果 112例脐带血G6PD活性为10.08±3.27 U/gHB,（其中男婴为10.10±3.33U/gHB,女婴为10.05±3.23U/gHB）;112例静脉血G6PD活性为8.75±2.96 U/gHB,（其中男婴为8.72±3.03U/gHB,女婴为8.80±2.89U/gHB）。男婴与女婴G6PD结果无统计学意义（P〉0.05）,脐带血与静脉血G6PD结果有统计学意义U=3.19,（P〈0.01）。脐带血G6PD活性与静脉血G6PD活性具有高度正相关（r=0.974）。结论新生儿脐带血G6PD定量结果稍高于静脉血,但两者具有高度正相关,且脐带血取材方便、快捷、无创伤,更适宜在南方高发区开展G6PD缺乏症的新生儿早期筛查和早期诊断。     

4560例新生儿脐带血G6PD定量检测报告

目的通过新生儿脐带血葡萄糖6磷酸脱氢酶（G6PD）活性检测,探讨脐带血G6PD检测在新生儿黄疸鉴别诊断及早期防治中的临床意义。方法4560例新生儿出生后即取脐带血,采用生化仪器进行定量检测,低于参考值为缺乏。结果G6PD缺乏452例,缺乏率为9.91%,其中男11.99%（308/2568）,女7.23%（144/1992）,男性缺乏率大于女性缺乏率（P〈0.01）。结论采用新生儿脐带血检测G6PD活性,取材方便、简单,能有效早期检测G6PD缺乏患儿,脐带血G6PD活性检测在新生儿黄疸鉴别诊断及早期治疗中有重要临床价值,应用新生儿脐血检测G6PD活性值得推广。     

Human genetic factors related to susceptibility to mild malaria in Gabon

Several human genetic factors, including red blood cell polymorphisms (ABO blood group, sickle-cell trait, G6PD deficiency) as well as point mutations in the mannose binding protein (MBP) and in the promoter regions of both the TNF-alpha and NOS2 genes, influence the severity of disease due to infection with Plasmodium falciparum. We assessed their impact on mild P. falciparum malaria, as part of a longitudinal investigation of clinical, parasitological and immunological parameters in a cohort of 300 Gabonese schoolchildren. We found the following frequencies: blood group O (0.54), sickle-cell trait (0.23), G6PD deficiency (0.09), MBP gene mutations (0.34), TNF-alpha promoter mutations (at positions -238: 0.17 and -308: 0.22) and NOS2 promoter mutation (0.18). Blood group O or hemoglobin AA were associated with protection against higher parasitemia. Girls with normal G6PD enzyme activity were protected against clinical malaria attacks. In addition, we demonstrated for the first time that the mutation at position -238 of the gene coding for the promoter region of TNF-alpha was positively correlated with the level of the antibody response specific for epitopes of the antigens MSA-2 and RAP-1 of P. falciparum.     

The activity of erythrocyte enzymes in rats subjected to running exercises

Summary The studies were carried out on male Wistar rats subjected to running within an electric rotating drum. The animals were divided into four experimental groups, differing one from another as to the duration of training. Each training session lasted 30 days. In the first group the daily run lasted 3 min, in the second group 5 min; in the third group, a 1 min run on the first day, and one min longer on each successive day; in the fourth group a 2 min run on the first day and for two min longer on each successive day.The determinations made prior to and after training included the peripheral blood erythrocyte (Er) and reticulocyte (Ret.) count, the hemoglobin concentration (Hb) and packed cell volume (PCV) and, determined by spectrophotometric methods, the activity of pyruvate kinase (PK), glucose-6-phosphate dehydrogenase (G6PD) and glutathione reductase (GR). Training induced an improvement of all enzymatic activities. The heavier the physical exertion, the more intensive was the enzymatic activity of red blood cells, due to the intensification of bone marrow erythropoetic activity under physical exertion and the appearance of young red cells in peripheral blood. All the experimental groups revealed a drop in erythrocyte count (Er), hemoglobin concentration (Hb), and hematocrit values (PCV), as well as an increase in the reticulocytes count (Ret) and in the activity of all the enzymes investigated. In the fourth group anemia was detected: prolonged endurance training decreased the RBC by 24.2%, Hb by 31.1%, PCV by 26.2% and increased the reticulocyte count by 881.6%. Pronounced loading with physical effort leads to shifts in the glucose utilization ratio along particular erythrocyte metabolic pathways. This change in enzymatic activities may prove to be one of the causes of faster elimination of old RBCs.     

云南省新平地区傣族儿童地中海贫血与G6PD缺乏症现况调查

目的了解云南省新平县傣族儿童地中海贫血和G6PD缺乏症的现状。方法地中海贫血检测:对调查者用日本特康血细胞分析仪进行血细胞分析,PH8.6缓冲液醋酸纤维薄膜做电泳分析;G6PD缺乏症检测:采用改良葡萄糖6-磷酸脱氢酶(G6PD)测定比值法(手工操作法)进行检测。结果β一地贫检出率6.5%,α-地贫检出率8.7%,G6PD缺乏症检出率8.3%。结论地贫和G6PD缺乏症在云南省新平县傣族儿童中属高发,地贫和G6PD缺乏症的人群地理分布与历史上疟疾流行的地理分布存在着一定的相关性。该调查为云南省新平县傣族进行遗传咨询及进行地贫和G6PD缺乏症的预防提供了有价值的基础资料。     

Expression and characterization of glucose-6-phosphate dehydrogenase of Plasmodium falciparum

Glucose-6-phosphate dehydrogenase (G6PD) from Plasmodium falciparum has been detected previously in cultures of parasites grown in G6PD-deficient red blood cells. Using polyacrylamide gel electrophoresis, a semi-quantitative assay has been developed to compare the level of the parasite enzyme activity in G6PD normal and in G6PD-deficient host cells. The results do not support the previous contention that the host cell G6PD-deficiency necessarily affects the level of expression of the parasite enzyme. The plasmodial enzyme was partially purified from extracts of parasites prepared by digitonin lysis of infected red blood cells, and its distinctive biochemical properties are described. P. falciparum G6PD has a KmG6P of 27 microM, a KmNADP of 4.5 microM, and KiNADPH of 4.5 microM, indicating an affinity for all its main ligands much higher than that of normal human red cell G6PD.     

Quantification of G6PD in small and large intestine of rat during aging

Numerous studies have demonstrated a decrease in glucose-6-phosphate dehydrogenase (G6PD) activity during aging in many cell types, including red blood cells, fibroblasts and lens cells. Moreover, the intracellular activity of G6PD has been shown to be regulated by binding to cell organelles. To investigate whether binding of G6PD to cell organelles is related with the decrease in its activity during aging, distribution patterns of G6PD activity and protein were assessed in small (SI) and large (LI) intestine of 3-month-old and 28-month-old rats. Enzyme activity, as measured spectrophotometrically, did not show any significant change with aging in SI or LI. Enzyme histochemistry, performed by subtracting activity staining of 6-phosphogluconate dehydrogenase (6PGD) from that of G6PD, showed a lower net G6PD activity in SI and LI epithelium of old rats in comparison with young rats. G6PD activity did not change significantly with aging in the muscularis externa of SI and LI. Immunoelectron microscopic analysis of G6PD protein allowed us to measure the density of G6PD molecules in cellular compartments, and the fraction of enzyme bound to cell organelles. In SI and LI epithelia, density of G6PD molecules was higher in old rats than in young rats; however, the fraction of enzyme bound to cell organelles also increased with aging. These data suggest that G6PD activity in epithelium of SI and LI decreases with aging due to the accumulation of significant amounts of enzyme bound to cell organelles, a condition which makes it less active than the soluble enzyme.     

Red cell genetic abnormalities in Peninsular Arabs: sickle haemoglobin, G6PD deficiency, and alpha and beta thalassaemia.

The frequencies of four major red cell genetic defects, sickle haemoglobin (Hb S), glucose 6 phosphate dehydrogenase deficiency (G6PD), and alpha and beta thalassaemia, have been determined in nearly 5000 subjects from the three major Peninsular Arab States, namely Yemen (North and South), the United Arab Emirates, and Oman. All four defects are common with an overall pattern of alpha thalassaemia greater than G6PD deficiency greater than beta thalassaemia greater than Hb A/S. However, the frequencies of these within each state varies and they are, respectively, Oman: 0.389, 0.328, 0.024, and 0.038; the United Arab Emirates: 0.165, 0.087, 0.017, and 0.019; and Yemen: 0.065, 0.062, 0.0624, and 0.0095. Two, namely alpha thalassaemia and G6PD deficiency, are extremely common, but in spite of this there appears to be a lack of observed clinical disease. For example, Hb H disease and Barts hydrops fetalis were not seen and the oxidative haemolytic syndromes are rare.     

荧光测定法定量测定滤纸片干血斑标本G6PD酶活性的可靠性分析

目的探讨荧光测定法测定滤纸片干血斑标本G6PD酶活性的可靠性。方法随机选取43例门诊孕前咨询者为检测对象,每人采集2ml静脉血抗凝保存同时制备滤纸片干血斑标本。采用G6PD/6PGD比值法测定抗凝血G6PD/6PGD比值,同时采用荧光测定法定量测定滤纸片干血斑标本G6PD酶活性,比较两种方法测定结果。结果荧光测定法检测43份滤纸片干血斑标本,检出1例缺乏（1.57 IU/gHb）;比值法检测43份抗凝血标本,检出1例缺乏（0.76）;两种方法符合率为100%。结论荧光测定法定量测定G6PD酶活性准确性高、简单、快捷、费用低廉,可对滤纸片干血斑标本进行G6PD缺乏症的大规模筛查,适于在G6PD缺乏高发区推广应用。     

Phenotypic and quantitative relationship of red cell acid phosphatase with haemoglobin, haptoglobin, and G6PD phenotypes.

The phenotypic and quantitative relationship of red cell acid phosphatase with haemoglobin, haptoglobin, and G6PD phenotypes was investigated in three populations in the Sudan and one population in Nilgiris, India. No significant consistent association of red cell acid phosphatase phenotypes was observed with these polymorphisms. However, there was a lack of acid phosphatase AB in G6PD deficient subjects from Nilgiris. The relative quantitative expression of red cell acid phosphatase genes PA, PB, and PC was 1.0, 1.2, and 1.3, respectively. The red cell acid phosphatase activity was higher (15%) in the presence of raised haemoglobin A2 and in sickle cell anaemia (21%). Those with Hp2 had 18% higher level of acid phosphatase than those with Hp1. G6PD deficient subjects had a lower level of acid phosphatase activity (20%) than those with normal G6PD activity.     

中山地区54258例育龄人群G6PD筛查结果分析

目的通过大样本的筛查了解中山地区育龄人群G6PD的分布情况。方法以紫外速率法检测54258例育龄人群的静脉血液红细胞中葡萄糖-6-磷酸脱氢酶的活性,将G6PD活性按缺乏、正常和增高进行分组。结果中山地区G6PD酶活性检出率分别为：缺乏组4．33％、正常组94．75％、增高组0．92％,其中缺乏组检出率男性高于女性,增高组检出率男性低于女性。结论中山地区是G6PD缺乏症的高发地区,对育龄人群进行G6PD筛查,并进行优生优育的合理指导,是减少出生缺陷和提高出生人口质量的必要措施。     

Volumetric erythrocyte macrocytosis induced by hydroxyurea

An atypical form of macrocytosis termed volumetric macrocytosis is described. In contrast to the macrocyte associated with megaloblastic anemia and the pseudomacrocyte caused by viscoelastic defects, the volumetric macrocyte is characterized by an increased mean corpuscular volume and a normal cell diameter. The volumetric macrocyte proves to be thicker than the normocytic red blood cell. This large erythrocyte is overhydrated and contains an increased quantity of hemoglobin. The cell has many characteristics in common with the red blood cells of neonates. Volumetric macrocytosis accompanies sustained hydroxyurea therapy and may represent a drug-induced dyserythropoiesis.