Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “G6PD”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 397 records · Page 22Linked to original sources
PubMed1991

A freeze-dried method for preparation of G6PD reagent tubes.

Mixed reagents for the Glucose-6-phosphate dehydrogenase (G6PD) deficiency fluorescent screening test were freeze-dried in plastic tubes. The reagents were then reconstituted with distilled water and the test was performed in the usual way. Initial testing with the freeze-dried mixed reagents gave consistent positive reaction to 12 normal blood samples and negative reaction to 9 G6PD deficient blood samples. This will enable a laboratory with freeze-drying facilities to prepare reagent tubes in bulk. As these tubes can be kept at 4 degrees C and do not require to be stored at -20 degrees C, a major laboratory can prepare these tubes and supply small laboratories for screening purposes.

Fluorescence↗
PubMed1991

[Hemolytic anemia associated with minor salmonellosis in an HIV positive, G6PD deficient Congolese woman].

Haemolytic anaemia in G6PD-deficient patients with thyphoid fever is well known, but there is only one case-report associated with non-typhic salmonella fever. We report here a case observed in a black african young woman whose HIV infection has been discovered on this occasion. Because of the high prevalence of HIV infection, salmonellosis and G6PD deficiency in sub-saharian Africa, an increasing number of such haemolytic anaemias should be expected in this geographic area.

Adult↗
PubMed1989

Change of deformability and Heinz body formation in G6PD-deficient erythrocytes treated with 5-hydroxy-6-desmethylprimaquine.

Glucose-6-phosphate deficient human erythrocytes were incubated with low concentrations of 5-hydroxy-6-desmethylprimaquine, a metabolite of primaquine in animals, for up to 18 hours under sterile conditions. These erythrocytes became less deformable than untreated erythrocytes. This decrease in deformability was closely associated with the extent and time course of formation of Heinz bodies in the G6PD-deficient erythrocytes. These results support the hypothesis that the in vivo formation of low concentrations of 5H6DPQ by metabolism of primaquine could be the cause of Heinz body formation and the hemolytic anemia seen when primaquine is administered to G6PD-deficient individuals.

Antimalarials↗
PubMed1988

[The distribution of 4 genes (alpha GAL, PGK-1, HPRT and G6PD) on the X chromosome of the American mink (Mustela vison)].

The segregation of X-linked markers (alpha GAL, PGK-1, HPRT and G6PD) was analysed in hybrids between gamma ray-irradiated mink fibroblasts and Chinese hamster cells, or between mink cells and mouse hepatoma cells. Based on the segregation data and the data of cytogenetics analysis of a few hybrids, the order of the mink genes was deduced as alpha GAL--PGK-1--HPRT--G6PD--qter. This order differs from that reported for human and murine genes, in spite of the very obvious similarity between G-banding of the mink and human X chromosomes. Therefore, at least one reversion is responsible for the differences observed for the human and mink X chromosomes.

Animals↗
PubMed1986

[Screening for hemoglobinopathies and G6PD deficiencies in Morocco].

Screening for abnormal hemoglobins and G6PD deficiency was conducted in adult and new born Maroccans from Casablanca. The results presented deal with HbS and HbC traits, alpha, gamma, and delta mutations, the presence of detectable amount of Hb Bart's and the G6PD deficiency frequency.

Adult↗
PubMed1983

Oxidant damage of normal and glucose 6-phosphate dehydrogenase (G6PD)-deficient red blood cells is enhanced by iron-EDTA complex.

A combination of divicine (an aglycone from the fava bean beta-glucoside vicine) and ascorbate results in a marked production of ethylene from methional, as a probable indication of OH radical formation. Addition of iron-EDTA to this oxidising system enhances the ethylene production significantly. The enhancing effect of iron-EDTA is also observed when both normal and Glucose 6-phosphate dehydrogenase (G6PD)-deficient red cells are exposed to the divicine-ascorbate system. Moreover, iron-EDTA magnifies other consequences of oxidant damage afforded by divicine-ascorbate or by ascorbate alone on the target red cells, such as depletion of reduced glutathione, formation of methemoglobin, stimulation of hexose monophosphate shunt activity and lipid peroxidation. Although the biochemical changes induced by this oxidative system are not remarkably different in normal and in G6PD-deficient red cells, the extra-damaging effect of chelated iron might be important in the mechanism of hemolysis.

Ascorbic Acid↗
PubMed1982

Hemolytic anemia in hereditary pyrimidine 5'-nucleotidase deficiency: nucleotide inhibition of G6PD and the pentose phosphate shunt.

We evaluated the erythrocytes of two patients with hereditary pyrimidine 5'-nucleotidase deficiency. Significant findings included an increased reduced glutathione content, increased incubated Heinz body formation, a positive ascorbate cyanide test, and decreased intraerythrocytic pH. The pentose phosphate shunt activity of the patients' red cells as measured by the release of 14CO2 from 14C-1-glucose was decreased compared to high reticulocyte controls. Glucose-6-phosphate dehydrogenase (G6PD) activity in hemolysates from control erythrocytes was inhibited 43% by 5.5 mM cytidine 5'-triphosphate (CTP) and 50% by 5.5 mM in uridine 5'-triphosphate (UTP) at pH 7.1. CTP was a competitive inhibitor for G6P (Ki = 1.7 mM) and a noncompetitive inhibitor for NADP+ (Ki = 7.8 mM). Glutathione peroxidase, glutathione reductase, and 6-phosphogluconate dehydrogenase were not affected by these compounds. Pentose phosphate shunt activity in control red cell hemolysate at pH 7.1 was inhibited to a similar degree by 5.5 mM CTP or UTP. Since the intracellular concentrations of G6P and NADP+ are below their KmS for G6PD, these data suggest that high concentrations of pyrimidine 5'-nucleotides depress pentose phosphate shunt activity in pyrimidin 5'-nucleotidase deficiency. Thus, this impairment of the pentose phosphate pathway appears to contribute to the pathogenesis of hemolysis in pyrimidine 5'-nucleotidase deficiency hemolytic anemia.

5'-Nucleotidase↗
PubMedDate not supplied

[G6PD deficiency in a western area of Genoa. Genetic study of 4 new variants].

Investigations for G6PD deficiency have been carried out among 900 subjects at the Paediatric Departments of the hospitals in Sestri Ponente and Pegli, in the western part of Genoa. 24 G6PD deficient males, 15 heterozygous and 1 homozygous females have been detected. Four variants, which have been encountered during the population survey, are reported. All of them, upon full characterization, were found to be unique new variants.

Adult↗
PubMed1981

Effect of divicine and isouramil on red cell metabolism in normal and G6PD-deficient (Mediterranean variant) subjects. Possible role in the genesis of favism.

Fava beans contain high amounts (up to 6.7 g/100 g dry weight) vicine and convicine. Their active aglycones divicine and isouramil have equivalent metabolic effects. They rapidly oxidize GSH to GSSG in normal and G6PD-deficient red cells. No regeneration of GSH occurs in deficient cells. The stoichiometry of the divicine oxidation of GSH is 1:1. Ascorbic acid is quickly oxidized by isouramil in both normal and deficient cells but regenerates only in normal cells. Isouramil oxidizes NADH at a much lesser extent than NADPH. Glycolysis is activated at the glyceraldehyde 3-phosphate dehydrogenase step. Divicine strongly stimulates hexone monophosphate shunt only in normal red cells. Divicine alone or associated with ascorbic acid has almost no effect in deficient red cells. Malonyl dialdehyde production is slight and virtually the same in normal and deficient cells treated with 5 mM isouramil. Large polypeptide aggregates are formed after 12 and 24 hours incubation with 1 mM divicine in deficient cells only. Divicine (0.25 mM) markedly decreases the filterability of deficient cells. The results are consistent with a causal role of divicine/isouramil in the genesis of the hemolytic crisis occurring in G6PD-deficient subjects after fava bean ingestion.

Ascorbic Acid↗
PubMed1980

Viral hepatitis with extreme hyperbilirubinemia, massive hemolysis and encephalopathy in a patient with a new G6PD variant.

Extreme hyperbilirubinemia, massive hemolysis and hepatic encephalopathy were reported in a G6PD-deficient patient with viral hepatitis. Investigation of the G6PD properties revealed that this patient's enzyme represented a hitherto undescribed variant (Bnei Brak), characterized by heat stability and a unique triphasic pH-related enzyme activity curve. It is possible that the marked hemolytic process in this patient was enhanced by a further decrease in the activity of the already deficient enzyme during acidosis.

Adolescent↗
PubMed1995

Prevalence of G6PD deficiency among recruits in the Singapore Armed Forces.

Between 1986 and 1990, 107,397 National Servicemen between the ages of 17 to 18 years were screened on enlistment for Glucose-6-Phosphate Dehydrogenase (G6PD) enzyme activity by fluorescent spot test. The overall prevalence rate was 1.6% with ethnic differences as follows: 1.62% for Chinese, 1.80% for Malay, 0.76% for Indian and 0.68% for other races. The majority had no prior knowledge of their G6PD deficiency status (89.9%), or of any significant haemolytic episode (93.3%). The rest gave a history of neonatal jaundice, non-neonatal jaundice, anaemia and haemoglobinuria. Maloprim, fava beans, sulphur drugs and aspirin were cited as triggering factors.

Adolescent↗
PubMed1993

Acute haemolytic episodes & fava bean consumption in G6PD deficient Iraqis.

The relation between fava bean ingestion and the occurrence of a haemolytic episode was studied in 102 glucose-6-phosphate dehydrogenate (G6PD) deficient Iraqi patients. None of the patients (mean age 12.8 yr) had a documented similar illness earlier, although all of them gave history of reported regular fava bean ingestion in the past. Further, none of the three patients who were rechallenged (2-3 months later) by the beans developed any clinical or laboratory evidence of haemolysis. The incidence of the haemolytic episodes was found to peak in April, while the fava bean season extends from February to June. This study thus does not support a causal relation between the bean ingestion and the haemolytic episodes in G6PD deficient Iraqis. Possibly, some other factor such as a viral infection may be involved.

Adolescent↗
PubMed1993

[Establishment of linkage and the order of the genes GALA, G6PD, HPRT and PGK on the X-chromosome in two species of voles of the genus Microtus].

Localization of genes GALA, G6PD, HPRT and PGK on X-chromosome of Microtus subarvalis has been proved. Using the radiation hybrid mapping technique of Goss and Harris, the order of these genes for two species M. subarvalis and M. Kirgisorum was established. Statistical methods (program package RHMAP) result in the only gene order PKG--HPRT--G6PD--GALA for M. subarvalis. The same order was found to be the most probable for M. kirgisorum. Relative distances between these genes in two species appeared to be practically equal. A conservatism of a linear order of the X-linked genes in various mammalian taxons is discussed.

Animals↗
PubMed1977

Comparison of GdA and GdB activities in Nigerians. A study of the variation of the G6PD activity.

We report a comparison between G6PD enzyme activities in lysates from GdA and GdB Nigerian healthy males. We confirm the previously reported higher mean activity of G6PD B. An analysis of the enzyme activity variation has been performed: the relative amount of variation found to be associated with this polymorphism is about 8%. A comparison has been made with other genetic polymorphisms.

Child↗
PubMedDate not supplied

[Use of the NSAID ketoprofen lysine salt in glucose-6-phosphate dehydrogenase (G6PD) deficiency in inflammatory disease in children].

Nineteen young females, aged 4 months-13 years, suffering from intermediate erythrocyte G6PD deficiency plus 14 hemizygote males, aged 6 months and 16 years, and one dizigote and completely deficient female were treated with ketoprofen lysine salt for upper and lower respiratory tract diseases. The drug was administered rectally at the dose of 30 or 60 mg twice or thrice a day according to the weight of the patients, i.e. around 2,5 mg ketoprofen lysine salt pro kg. Concomitant drugs were present in 6 cases, i.e. antibiotics and or inhaled corticosteroids. Fever disappeared within two days in 80% of cases. No systemic and/or local adverse event or haemolysis was evident both clinically and at laboratory analysis. Ketoprofen lysine salt, when administered for a short time period, is effective and safe also in G6PD deficient patients.

Adolescent↗
PubMed1976

Hair root versus red cell individual phenotype in Sardinian heterozygotes for G6PD deficiency (Mediterranean type).

G6PD activity was assayed in 20 Sardinian heterozygotes for G6PD deficiency and related to that of LDH and MDH. One of these heterozygotes showed a deficient phenotype in all her follicles, while the remaining 19 had different proportions of deficient, intermediate, and normal follicles. This is in accordance with a previous estimate. Because of the broad fiducial limits at the 5% level and because of some developmental considerations, this value cannot be interpreted as indicative of the number of primordial cells for scalp epidermis at the time of X-chromosome inactivation, as previously stated. The assay of single hair follicles is, however, a very valuable tool for establishing the role of cell selection in the same or in a different tissue, like peripheral blood.

Erythrocytes↗