Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002878 (hemolytic anemia)
 7,530 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Ninety adult Indian typhoid and paratyphoid fever (enteric fever, EF) patients and 91 controls were tested for glucose-6-phosphate dehydrogenase (G6PD) deficiency using the fluorescent spot test (FST) and the quantitative methaemoglobin reduction test ( QMRT ). There was a threefold higher incidence of G6PD deficiency in North Indian EF patients (10.6%) than in controls (3.6%) (P = 0.15) which may be attributable to the greater morbidity of the G6PD-deficient EF patients; six of nine had haemolytic anaemia. A transient depression of mean erythrocyte G6PD activity was observed in a subgroup of 49 non-deficient EF patients in whom the spectrophotometric G6PD assay was done. It did not appear to be related to reticulocyte count, chloramphenicol therapy, or differences in leucocyte contamination of the haemolysate used for the G6PD assay. If this depression of G6PD activity occurs in deficient patients as well, it may help to explain the haemolysis seen in them during EF. Of the three tests used, the QMRT and the spectrophotometric assay clearly identified G6PD deficiency in males during haemolysis, whereas the FST was unreliable in this situation.
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PMID:Depression of erythrocyte glucose-6-phosphate dehydrogenase (G6PD) activity in enteric fever. 643 56

Red cell glucose-6-phosphate dehydrogenase (G6PD) deficiency is an X-chromosomal-linked abnormality often associated with hemolytic anemia. The G6PD variants obtained from 2 unrelated males, one associated with enzyme deficiency and history of hemolytic jaundice, and the other associated with enzyme deficiency but no hemolytic problems, were examined. Although the 2 subjects have no known consanguinity, the two enzymes could not be distinguished from each other in respect to their electrophoretic mobilities and kinetic properties, both exhibiting slower than normal anodal electrophoretic mobility, lower Km for G6P and NADP and higher rate of utilization of 2-deoxy-G6P and deamino-NADP. An unique double-banded pattern was observed in starch gel electrophoresis at pH 7.0 and pH 8.6. The variant is distinguished from all reported Gd variants, and it is designated Gd(-) Santamaria.
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PMID:A glucose-6-phosphate dehydrogenase variant, Gd(-) Santamaria found in Costa Rica. 643 30

In a 36-month-old child a severe hemolytic anemia occurred after the administration of a pyrazolonic drug. Erythrocyte glucose-6-phosphate dehydrogenase activity was absent in the patient. Intermediate enzymatic levels were demonstrated in the mother and in the maternal grand-mother with a corresponding erythrocyte mosaicism at the methemoglobin elution test. Some cells showed Heinz bodies and peculiar changes in the morphology in the initial phase of the crisis. There is no mention in the literature of a hemolytic action of this drug.
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PMID:Acute hemolytic anemia induced by a pyrazolonic drug in a child with glucose-6-phosphate dehydrogenase deficiency. 643 97

Light and scanning electron microscopic studies of blood from five patients with drug-induced oxidant hemolysis are presented. None of the patients had a previous history of hemolytic disease and laboratory studies indicated no evidence of either glucose-6-phosphate dehydrogenase (G6PD) deficiency or unstable hemoglobinopathy. Although the red cell deformities in our patients overlapped to some extent with those reported in patients with microangiopathic hemolytic anemia (MAHA) and in patients with G6PD deficiency undergoing oxidant hemolysis, striking differences were also observed. Cell fragments, commonly found in patients with MAHA, and eccentrocytes, frequently found in patients with G6PD deficiency undergoing oxidant hemolysis, were seldom found in blood samples from the five patients in this study. Bite cells were extremely common in our patients. They are rare in patients with either of the above disorders. An awareness of the morphologic abnormalities detailed in this report may help characterize the nature of a hemolytic process so that appropriate therapy can be initiated.
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PMID:Heinz-body anemia: "bite cell" variant--a light and electron microscopic study. 661 84

A new glucose-6-phosphate dehydrogenase (G6PD) variant associated with chronic nonspherocytic hemolytic anemia was discovered. It was found in a 2-year-old male who had a hemolytic crisis after an upper respiratory tract infection. The enzyme activity of the variant was 8.4% of that of the normal enzyme. The enzymatic characteristics were slower than normal anodal electrophoretic mobility, low Km G6P, normal Km NADP, increased utilization of substrate analogues, high Ki NADPH, decreased heat stability, and an alkaline pH optimum. From these results, this was considered to be a new variant and was designated G6PD Sendagi.
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PMID:G6PD Sendagi: a new glucose-6-phosphate dehydrogenase variant associated with congenital hemolytic anemia. 665 37

A study of the haemolytic anaemia observed in protein-energy malnutrition (PEM) in Kivu disclosed the following results. The in vitro resistance to oxidative aggressions of PEM patients' erythrocytes was decreased: when incubated with acetylphenylhydrazine, a higher percentage of the cells showed Heinz bodies, as compared with erythrocytes of local controls. Normal or increased activities were found for certain erythrocyte enzymes involved in the detoxification of activated oxygen: glucose-6-phosphate dehydrogenase, 6-phosphogluconate dehydrogenase and glutathione reductase. The level of reduced glutathione was not decreased. Reduced activities were observed for two enzymes containing trace elements: glutathione peroxidase and superoxide dismutase. It is suggested that the shortened erythrocyte lifespan observed in PEM patients corresponds to an oxidative process which results from the decrease of both enzyme activities. The hypothesis that depletion of trace elements could be responsible for the decreased activity of those enzymes is discussed.
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PMID:Oxidative haemolysis in protein malnutrition. 679 56

Pentose phosphate shunt activity was studied by the release of 14CO2 from 14C-1-glucose and 14C-2-glucose in the red cells of five patients with pyruvate kinase deficiency and found to be significantly decreased after new methylene blue stimulation when compared to high reticulocyte controls. Incubated Heinz body formation was increased and the ascorbate cyanide test was positive in blood from these patients. The activity of glucose-6-phosphate dehydrogenase (G6PD) as well as that of 6-phosphogluconate dehydrogenase (6PGD) was inhibited to 20% of baseline in normal red cell haemolysate by 4 mM 2,3-diphosphoglycerate at pH 7.1. 2,3-Diphosphoglycerate was a competitive inhibitor with 6-phosphogluconate (Ki=1.05 mM) and a noncompetitive inhibitor with NADP (Ki=3.3 mM) for 6PGD. Since the intracellular concentrations of glucose-6-phosphate, 6-phosphogluconate and NADP are below their Kms for G6PD and 6PGD, the kinetic data suggest that increased concentrations of 2,3-diphosphoglycerate in pyruvate kinase deficient red cells are sufficiently high to suppress pentose phosphate shunt activity. This suppression may be an additional factor contributing to the haemolytic anaemia of pyruvate kinase deficiency, particularly during periods of infection or metabolic stress.
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PMID:Inhibition of the pentose phosphate shunt by 2,3-diphosphoglycerate in erythrocyte pyruvate kinase deficiency. 686 May 90

The red cells' antioxidant defense mechanisms were compared between individuals with sickle cell disease and those with hemolytic anemia and reticulocytosis. In sickle cell disease, there was a significant increase in incubated Heinz body formation (p less than .001), a decrease in reduced glutathione concentration (p less than .01), an increase in glucose-6-phosphate dehydrogenase activity (p less than .01), and a decrease in glutathione reductase activity (p less than .005). The patients with sickle cell disease hd an absolute increase in the activity of the pentose shunt in the intact red cell after methylene blue stimulation (p less than .05) and in red cell hemolysates (p less than .0250. Heinz body formation (r = .75) and pentose shunt activity in red cell hemolysates (r = .83) were strongly related to the degree of reticulocytosis. Although there was a correlation between the pentose shunt activity in the stimulated red cell and in red cell hemolysates for the patients with hemolytic anemia (r = .58), stimulated shunt activity did not increase as the hemolysate shunt activity increased for the patients with sickle cell disease. There were very strong relationships between the ATP concentration and the reticulocyte count (r = .80) and the hemolysate pentose shunt activity (r = .77) in sickle cel disease. These data suggest that in spite of an absolute increase in stimulated pentose shunt activity, there Is a relative suppression of stimulated shunt activity in the youngest sickle erythrocytes. This may be related, in part, to the inhibitory effects of high concentrations of ATP on the activity of glucose-6-phosphate dehydrogenase.
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PMID:Impaired pentose phosphate shunt function in sickle cell disease: a potential mechanism for increased Heinz body formation and membrane lipid peroxidation. 688 Nov 34

The clinical spectrum of hemolytic anemia as a consequence of oxidant stress in black children deficient in erythrocyte glucose-6-phosphate dehydrogenase (G-6-PD) has not been well described in the pediatric literature. During a 3 1/2-year period 14 hospitalized black G-6-PD-deficient children with moderate to severe hemolytic reactions were studied. The vast majority (13/14) were boys and were less than 3 years of age. Nine of the patients required blood transfusion. Eleven of the 14 episodes occurred with infection (five bacterial, six viral); those children with viral syndromes tended to have more severe hemolysis. Naphthalene was responsible for three episodes, but oxidant drugs were implicated in no instances. Findings on the blood smears of most subjects included irregular dense misshapen erythrocytes with asymmetrical distribution of hemoglobin and an adjacent membrane-bound clear zone ("eccentrocytes"). It is concluded that hemolytic reactions in the black G-6-PD-deficient child may be severe, are most commonly associated with infection, and are frequently characterized by distinctive erythrocyte morphology.
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PMID:Severe hemolytic anemia in black children with glucose-6-phosphate dehydrogenase deficiency. 711 Aug 9

A glucose-6-phosphate dehydrogenase (G6PD) variant was studied in a mulatto patient with chronic nonspherocytic hemolytic anemia. This variant has reduced activity, increased thermolability, a reduced Michaelis constant for glucose-6-phosphate, slightly increased electrophoretic mobility, a biphasic pH activity profile, high 2-deoxyglucose-6-phosphate utilization, normal diamino nicotinamide adenine dinucleotide phosphate utilization and a peak of elution profile after G6PD B. The electrophoretic, kinetic, and chromatographic properties of this erythrocyte G6PD variant allow the conclusion that G6PD Varadero is probably a new variant.
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PMID:G6PD Varadero. A new variant of glucose-6-phosphate dehydrogenase associated with congenital nonspherocytic hemolytic anemia. 712 3


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