Brilliant cresyl blue screening test for demonstrating glucose-6-phosphate dehydrogenase deficiency in red cells

1963 ◽  
Vol 8 (1) ◽  
pp. 158-160 ◽  
Author(s):  
R.E. Bernstein
Keyword(s):  
Screening Test ◽  
Dehydrogenase Deficiency ◽  
Red Cells ◽  
Brilliant Cresyl Blue ◽  
Cresyl Blue ◽  
Glucose 6 Phosphate Dehydrogenase

Erythrocytary Glucose-6-Phosphate Dehydrogenase Deficiency – Study on its distribution in the province of Ferrara and its relation to malaria and thalassemia

1969 ◽  
Vol 18 (3) ◽  
pp. 271-284 ◽  
Author(s):  
E. Gandini ◽  
C. Menini ◽  
A. De Filippis ◽  
G. Dell'Acqua
Keyword(s):  
Gene Frequency ◽  
Dehydrogenase Deficiency ◽  
Brilliant Cresyl Blue ◽  
Cresyl Blue ◽  
The Past ◽  
Blue Discoloration ◽  
Thalassemia Trait ◽  
The One ◽  
Glucose 6 Phosphate Dehydrogenase

SUMMARYThe distribution of G6PD-D in the Ferrara country has been studied by means of the Brilliant Cresyl Blue discoloration test: 2437 males have been tested in 11 localities.The highest gene frequency of G6PD-D observed is consistent with the one expected after 25-30 generations of malaria selection, assuming an increased fitness of the heterozygotes, as it has been shown in computer simulated evolutionary trends for sex-linked genes. The thalassemia trait has also been shown to be very frequent in the same localities; its frequency is correlated with G6PD-D gene frequency and with the incidence of malaria in the past (1900).G6PD-D frequency is not significantly correlated with malaria. Such a result is due to the significantly lower gene frequency of G6PD-D observed in the localities where the percentage of population affected by malaria was higher (40-60%). On the other hand, G6PD-D is frequently significantly correlated with malaria in the remaining localities where the incidence of the latter ranged from 0 to 35% of the affected population.The mechanisms possibly involved in the determination of this distribution of G6PD-D are discussed.

Glucose-6-phosphate dehydrogenase deficiency in red cells

1961 ◽  
Vol 134 (3) ◽  
pp. 260-267 ◽  
Author(s):  
I. S. Bailey ◽  
W. C. J. Loecker ◽  
T. A. J. Prankerd
Keyword(s):  
Dehydrogenase Deficiency ◽  
Red Cells ◽  
Glucose 6 Phosphate Dehydrogenase

CONGENITAL NONSPHEROCYTIC HEMOLYTIC ANEMIA ASSOCIATED WITH ERYTHROCYTE GLUCOSE-6-PHOSPHATE DEHYDROGENASE DEFICIENCY IN A NEGRO FAMILY

PEDIATRICS ◽  
1966 ◽  
Vol 37 (4) ◽  
pp. 624-629
Author(s):  
Aaron Grossman ◽  
K. Ramanathan ◽  
Parvin Justice ◽  
Joan Gordon ◽  
Nasrollah T. Shahidi ◽  
...  
Keyword(s):  
Enzyme Activity ◽  
Hemolytic Anemia ◽  
Gel Electrophoresis ◽  
Dehydrogenase Deficiency ◽  
Red Cells ◽  
Starch Gel Electrophoresis ◽  
Negro Family ◽  
Starch Gel ◽  
Normal Enzyme ◽  
Glucose 6 Phosphate Dehydrogenase

This paper describes a Negro family with congenital nonspherocytic hemolytic anemia associated with glucose-6-phosphate dehydrogenase deficiency. All four affected males in this family showed a hemolytic anemia characterized by low hemoglobin, reticulocytosis, and jaundice. There was no detectable G-6-P D in the red cells and about a tenth of normal enzyme activity in the white cells. By starch gel electrophoresis, the G-6-P D was present as a single band which migrated at the rate of 104% of normal. Physico-chemical studies revealed a marked increase of the Michaelis constant for both G-6-P and TPN, a marked lability of the enzyme upon heating at 40°C and 48°C, and a single narrow peak at pH 9.0. Most of these features were similar to those seen in the Oklahoma I variant. Four of the females in the family were studied (two sisters, the mother, and a maternal aunt of the propositus); all showed a lesser degree of anemia and reticulocytosis but no jaundice, except in the mother. There was a decrease of haptoglobins and both the mother and one sister showed a decrease of erythrocyte survival time as measured by chromium-51. The female members had between 11 and 26% of normal G-6-P D activity in the red cells and between 35 and 63% of normal enzyme activity in the white cells. Starch gel electrophoresis in the mother and aunt showed a single band which migrated at 110% of normal with a trail at the position of the affected males. The presence of a milder degree of hemolysis in the heterozygous carriers of the gene for G-6-P D deficiency associated with congenital nonspherocytic hemolytic anemia provides further support for the Lyon hypothesis in man.

scholarly journals Glucose-6-Phosphate Dehydrogenase Deficiency in Greece

Blood ◽  
1961 ◽  
Vol 18 (1) ◽  
pp. 34-47 ◽  
Author(s):  
LEDA ZANNOS-MARIOLEA ◽  
CHRISTOS KATTAMIS
Keyword(s):  
Individual Differences ◽  
Dehydrogenase Deficiency ◽  
Normal Limit ◽  
Red Cells ◽  
Female Patients ◽  
Male Patients ◽  
Stability Method ◽  
Fava Beans ◽  
Glucose 6 Phosphate Dehydrogenase ◽  
Genetic Hypothesis

Abstract The glutathione stability of red cells was estimated in 40 patients during acute hemolysis induced by fava beans. There were wide individual differences but in all cases except one (Case 18) the post-incubation GSH fell to levels below 40 mg. per cent packed RBC which is the lower normal limit. The GSH stability on 44 mothers and 37 fathers gave results consistent with the genetic hypothesis that in male patients the mother is the carrier of the biochemical defect, while in female patients both parents are carriers, since, as a rule, only female homozygotes suffer from hemolytic episodes. However, in only 77.7 per cent of the mothers could the biochemical defect be proved by this method. The Motulsky test was performed in 30 of the 40 patients. It gave abnormal decolorization times in 25 or 83 per cent of the cases. This test is therefore valuable for diagnosing "sensitivity" during a hemolytic episode; it is, nevertheless, less sensitive than the GSH stability method. The Motulsky test was also performed on 31 mothers, 18 fathers and 8 siblings. It proved to be unreliable in the detection of female heterozygotes. G-6-PD deficiency is widely disseminated in Greece; it is, however, not evenly distributed throughout the country. The highest frequency of G-6-PD deficiency found so far in males was about 3 per cent; the lowest was 0.7 per cent.

scholarly journals Haemolytic potential of three chemotherapeutic agents and aspirin in glucose-6-phosphate dehydrogenase deficiency

1999 ◽  
Vol 5 (3) ◽  
pp. 457-464
Author(s):  
N. A. J. Ali ◽  
L. M. Al Naama ◽  
L. O. Khalid
Keyword(s):  
High Frequency ◽  
Dehydrogenase Deficiency ◽  
Local Population ◽  
Chemotherapeutic Agents ◽  
Red Cells ◽  
New Drugs ◽  
Local Variant ◽  
Glucose 6 Phosphate Dehydrogenase ◽  
Significant Concentration

The potential haemolytic effect of three chemotherapeutic drugs and aspirin was tested in vitro by gluthathione stability tests. Blood was collected from the local population of Basra, Iraq where previous studies had found a high frequency of glucose-6-phosphate dehydrogenase [G6PD] deficiency. Primaquine, chloramphenicol and sulfanilamide caused significant concentration-dependent reductions of glutathione levels in G6PD-deficient red cells when compared to normal red cells. Acetylsalicylic acid had no effect on glutathione level. The G6PD-deficient erythrocytes behaved as previously reported, probably due to similar patterns in the distribution of its variants. Studies on each local variant are warranted and new drugs should be tested for haemolytic potential prior to their introduction in areas where the deficiency is common

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