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.     

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.     

VitB_2、VitE联合应用防治G-6PD缺陷高胆新生儿光疗时溶血

目的探讨VitB2、VitE联合防治光疗致G-6PD缺陷新生儿溶血加重疗效。方法将G-6PD缺陷需光疗治疗的新生儿随机分为VitB2、VitE干预组和对照组,测定比较总胆红素(TB)、血红蛋白(Hb)及光疗指数。结果光疗后对照组Hb下降比干预组显著,光疗指数大(均p<0·01)。结论光疗致G-6PD缺陷患儿溶血加重,VitB2、VitE干预有效。     

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。     

Haemolytic effect of trimethoprim:Sulphamethoxazole in G-6-PD deficiency

Observations are reported on the survival of ⁵¹Cr-labelled glucose-6-phosphate dehydrogenase (G-6-PD) deficient cells transfused into normal recipients before and after the administration to the recipient of trimethoprim and sulphamethoxazole, alone or in combination, in varying doses. Trimethoprim did not reduce the $\text{T}\text{1}\text{2}$ of the transfused deficient red cells. In 4 of 6 instances sulphamethoxazole did so but only in the somewhat high dose of 90 mg./kg./day, the $\text{T}\text{case1}\text{2}$ being unaffected by smaller doses.However it cannot be concluded from the foregoing that the haemolysis of G-6-PD deficient red cells, provoked by sulphamethoxazole is dose dependent, for severe intravascular haemolysis has been encountered in 2 G-6-PD deficient patients with trivial infections within 48 hours of starting to take 30 mg./kg./day of sulphamethoxazole. It is concluded that the drug should be administered to those of ethnic groups in which this deficiency is common only when the deficiency has been excluded.     

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。     

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.     

Low glucose-6-phosphate dehydrogenase (G-6-PD) activity in red blood cells and susceptibility to copper-induced oxidative damage

The effects of copper on selected characteristics of blood from normal and G-6-PD deficient humans and sheep of the Dorset Strain (which also have comparable red cell G-6-PD activity units as G-6-PD deficient humans) were compared. Both the G-6-PD deficient humans and sheep were found to be markedly more sensitive to several indicators of oxidant stress as measured by increases in methemoglobin levels and decreases in the activity of red cell acetylcholinesterase. These findings indicate that susceptibility to copper-induced oxidative stress is associated with the presence of low red cell G-6-PD activity.     

'Idiopathic' jaundice in Sardinian full-term newborn infants: a multivariate study

Summary. 'Idiopathic' hyperbilirubinaemia in the first 4 days of life was studied in 431 unselected healthy full-term (gestational age ≥37 weeks) singleton Sardinian infants with birthweight ≥2500 g. All infants were free from malformations or any disease requiring treatment other than jaundice, they were ABO and Rh compatible with their mothers and were not G6PD deficient. The serum bilirubin level was > 11.9 mg/dl (204 μmol/1) and > 14.9 mg/dl (256 μmol/1) in 37.1% and 15.3% of the study subjects. The vast majority of the infants (94%) were breast fed; no difference in the incidence of jaundice was found between breast-fed and bottle-fed infants. A logistic regression analysis indicated that high alpha-fetoprotein concentrations in cord blood, history of neonatal jaundice in previous full-term siblings, delayed first meconium passage and weight loss were associated with jaundice, defined as a serum bilirubin level > 11.9 mg/dl. These results suggest that the high rate of neonatal hyperbilirubinaemia in Sardinia is mostly related to constitutional and possibly hereditary factors.     

Adequacy of maternal pyridoxine supplementation during pregnancy in relation to the vitamin B6 status and growth of neonates at birth

To evaluate the adequacy of maternal pyridoxine supplementation during pregnancy for both maternal and neonatal status at birth, vitamin B6 status, assessed by plasma pyridoxal phosphate (PLP), pyridoxal (PL) and total aldehyde vitamer (PLP + PL) concentrations, and the growth of neonates, including weight, length, head and chest circumferences, were examined for 209 neonates whose mothers were supplemented with 0, 1, 2 or 3 mg pyridoxine.HCl (PN.HCl)/d during pregnancy. Maternal PN.HCl supplementations were positively correlated to both maternal (r = 0.62) and cord (r = 0.78) plasma PLP concentrations (p < 0.0001). Mothers supplemented with 2 or 3 mg/d PN.HCl had significantly higher plasma concentrations of PLP and total B6 aldehyde vitamer in maternal and cord blood compared with those receiving 0 or 1 mg PN.HCl/d. A growth benefit for neonates whose mothers had maternal and cord plasma PLP concentrations > or = 40 nM was revealed by the maternal supplementation of 2 mg PN.HCl/d during pregnancy. Thus, in healthy pregnant women, according to our study, a daily supplement of 2 mg PN.HCl provides the adequacy of maternal and neonatal vitamin B6 status and the satisfactory growth of neonates at birth.     

The effect of clofibrate on hyperbilirubinemia of term neonates

Clofibrate is a glucuronosyl transferase inducer that has been proposed to increase the elimination of bilirubin in neonates with hyperbilirubinemia. This study was conducted to determine the therapeutic effect of clofibrate in term neonates with non-hemolytic jaundice. This study was conducted on 52 newborns with pathologic unconjugated jaundice in Qazvin children hospital. Newborns divided randomly in two groups. Case group treated with clofibrate and intensive phototherapy, while control group treated only with intensive phototherapy. Serum bilirubin level was measured before and 6, 12, 24 and 48 hours after treatment. Results were compared and analyzed. The mean serum level of bilirubin before treatment in the case and control groups were 20.78 ± 2.38 and 20.52 ± 2.44 mg/dl, respectively (P=0.69). The mean serum level of bilirubin in 6, 12, 24 and 48 hours after treatment in the case group were 18.20 ± 2.20, 14.70 ± 2.06, 10.72 ± 2.40 and 8.90 ± 0.83 mg/dl , respectively. These values in control group were 18.26 ± 2.42, 15.36 ± 2.59, 12.29 ± 2.28 and 10.23 ± 1.50 mg/dl, respectively. There was significant difference between two groups regarding mean serum level of bilirubin 24 hours (P=0.019) and 48 hours after treatment (P=0.005). In conclusion, clofibrate was effective in reducing neonatal jaundice and its effect appeared 24 hours after treatment.     

妊娠期糖尿病与新生儿黄疸的相关性研究

目的为探索新生儿黄疸高危因素,了解妊娠期糖尿病与新生儿黄疸的相关性。方法选择2008年1月～2010年6月某院妊娠期糖尿病母亲所分娩的50例足月新生儿,依据母亲妊娠期血糖水平,随机抽样将其中25例分为A组,余者25例分为B组,对照组25例为C组,为同期剖宫产的正常足月新生儿。结果 A组出生24h后胆红素测定值[(4.3±1.8)mg/dl]、72h后胆红素测定值[(9.2±1.7)mg/dl]及峰值[(12.3±2.1)mg/dl]比B组24h后[(2.9±1.7)mg/dl]、72h后胆红素测定值[(7.3±1.5)mg/dl]及峰值[(10.1±1.7)mg/dl]、C组24h后[(2.7±1.6)mg/dl]、72h后胆红素测定值[(7.0±1.4)mg/dl]及峰值[(9.3±1.4)mg/dl]显著高(P﹤0.05)。A组需光疗的患儿构成比(20.0%)比B组(0.0%)、C组(0.0%)显著高(P﹤0.05)。结论妊娠期母体血糖水平较高患儿,黄疸症状较为严重。妊娠期有效控制孕妇血糖水平,能有效改善新生儿黄疸情况。     

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.     

Adding zinc to prenatal iron and folate supplements improves maternal and neonatal zinc status in a Peruvian population.

BACKGROUND: Maternal zinc deficiency during pregnancy may be widespread among women in developing countries, but few data are available on whether prenatal zinc supplementation improves maternal and neonatal zinc status. OBJECTIVE: We studied whether maternal zinc supplementation improved the zinc status of mothers and neonates participating in a supplementation trial in a shantytown in Lima, Peru. DESIGN: Beginning at gestation week 10-24, 1295 mothers were randomly assigned to receive prenatal supplements containing 60 mg Fe and 250 microg folate, with or without 15 mg Zn. Venous blood and urine samples were collected at enrollment, at gestation week 28-30, and at gestation week 37-38. At birth, a sample of cord vein blood was collected. We measured serum zinc concentrations in 538 women, urinary zinc concentrations in 521 women, and cord zinc concentrations in 252 neonates. RESULTS: At 28-30 and 37-38 wk, mothers receiving zinc supplements had higher serum zinc concentrations than mothers who did not receive zinc (8.8 +/- 1.9 compared with 8.4 +/- 1.5 micromol/L and 8.6 +/- 1.5 compared with 8.3 +/- 1.4 micromol/L, respectively). Urinary zinc concentrations were also higher in mothers who received supplemental zinc (P < 0.05). After adjustment for covariates and confounding factors, neonates of mothers receiving zinc supplements had higher cord zinc concentrations than neonates of mothers who did not receive zinc (12.7 +/- 2.3 compared with 12.1 +/- 2.1 micromol/L). Despite supplementation, maternal and neonatal zinc concentrations remained lower than values reported for well-nourished populations. CONCLUSION: Adding zinc to prenatal iron and folate tablets improved maternal and neonatal zinc status, but higher doses of zinc are likely needed to further improve maternal and neonatal zinc status in this population.     

Some biochemical changes in lenses of riboflavin deficient rats.

In order to better understand the role of riboflavin in the lens, we investigated the lenses of rats fed on a riboflavin deficient diet for 7 weeks to determine changes in the inactivation of cortisol, the glucose-6-phosphate dehydrogenase (G-6-PD) activity and the histidine or 5-hydroxytryptophan decarboxylase activity. The cortisol-binding capacity and G-6-PD activity in the lenses of rats fed on a riboflavin deficient diet were found to be decreased as compared with a control. Histidine decarboxylase activity was found twice as high in the riboflavin deficient rats as in the normal lenses however, 5-hydroxytryptophan decarboxylase was somewhat decreased. These findings show that catabolical changes in the metabolism of rat lenses are induced comprehensively by feeding on a riboflavin deficient diet in the same manner as changes are seen in the cataractous lens.     

High prevalence of vitamin D insufficiency in black and white pregnant women residing in the northern United States and their neonates

In utero or early-life vitamin D deficiency is associated with skeletal problems, type 1 diabetes, and schizophrenia, but the prevalence of vitamin D deficiency in U.S. pregnant women is unexplored. We sought to assess vitamin D status of pregnant women and their neonates residing in Pittsburgh by race and season. Serum 25-hydroxyvitamin D (25(OH)D) was measured at 4-21 wk gestation and predelivery in 200 white and 200 black pregnant women and in cord blood of their neonates. Over 90% of women used prenatal vitamins. Women and neonates were classified as vitamin D deficient [25(OH)D<37.5 nmol/L], insufficient [25(OH)D 37.5-80 nmol/L], or sufficient [25(OH)D>80 nmol/L]. At delivery, vitamin D deficiency and insufficiency occurred in 29.2% and 54.1% of black women and 45.6% and 46.8% black neonates, respectively. Five percent and 42.1% of white women and 9.7% and 56.4% of white neonates were vitamin D deficient and insufficient, respectively. Results were similar at <22 wk gestation. After adjustment for prepregnancy BMI and periconceptional multivitamin use, black women had a smaller mean increase in maternal 25(OH)D compared with white women from winter to summer (16.0+/-3.3 nmol/L vs. 23.2+/-3.7 nmol/L) and from spring to summer (13.2+/-3.0 nmol/L vs. 27.6+/-4.7 nmol/L) (P<0.01). These results suggest that black and white pregnant women and neonates residing in the northern US are at high risk of vitamin D insufficiency, even when mothers are compliant with prenatal vitamins. Higher-dose supplementation is needed to improve maternal and neonatal vitamin D nutriture.     

驻马店市新生儿黄疸发生情况及其影响因素

目的 探讨新生儿黄疸发生情况、病例特征及其影响因素.方法 对驻马店市某医院2018年7月至2020年6月新生儿及其母亲资料进行分析,采用描述流行病学方法对新生儿黄疸发生情况进行描述,并采用单、多因素分析方法对新生儿黄疸发生的影响因素进行分析.结果 共收集有效新生儿及其母亲信息l 359对,新生儿日龄2～24d,平均日龄...     

Hepatitis D infectivity profile among hepatitis B infected hospitalised patients in Calcutta.

450 hospitalised cases of hepatic and non hepatic disorders and 100 normal individuals were examined for serum Hepatitis B Surface antigen and Delta Virus antigen by ELISA to find out its association with different clinical disorders. 105 patients (23.3%) and 2 control (2%) were positive for HBsAG. 60 cases with jaundice (26%) were HBsAg positive. 65% of HBsAg positive jaundiced patients had serum bilirubin level more than 2 mg per dl with a mean SGPT level of 488 iu/L. Only two cases were positive for HDV antigen among 60 HBsAg positive jaundice patients indicating a lower rate of prevalence of infection (3.3%). 62 (59%) out of 105 HBsAg positive cases did not show any history of blood transfusion or surgical interference indicating a positive HBV transmission through needle prick during investigative procedures.     

新生儿葡萄糖-6-磷酸脱氢酶缺乏症的筛查现状

葡萄糖-6-磷酸脱氢酶（G6PD）缺乏症是一种最常见的酶功能缺陷而致的溶血性疾病,具有遗传性,世界范围内约4亿人受累.新生儿G6PD缺乏症的主要临床表现为各因素触发的新生儿黄疸、急性溶血,而核黄疸是新生儿G6PD缺乏症患儿致死的重要因素.国内外文献报道,在新生儿G6PD缺乏症筛查的基础上,予以综合防治,其诱发的部分疾病可得到很好的控制.目前,一些国家已经建立完善的新生儿G6PD缺乏症筛查体系,但部分高发地区尚未开展该项工作.作者拟就新生儿G6PD缺乏症筛查的现状、必要性、可行性、存在问题及解决路径,作一综述.