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Query: UMLS:C0002878 (hemolytic anemia)
 7,530 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Kinetic and electrophoretic properties of 230--300 fold purified preparations of glucose-6-phosphate dehydrogenase (G6PD) from red cells of donors and patients with acute drug hemolytic anemia due to G6PD deficiency were studied. A new abnormal variant of G6PD isolated from red cell of a patient with acute drug hemolytic anemia, which was not described in literature, has been discovered. The abnormal enzyme differs from the normal by decreased Michaelis constant for glucose-6-phosphate and nicotinamide adenine dinucleotide phosphate (NADP), by increased utilization of analogues of substrates--2-deoxy-glucose-6-phosphate and particularly deamino-NADP, by low thermal stability, by the character of pH-dependence, by the appearance of a single band of G6PD activity in polyacrylamide gel electrophoresis.
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PMID:[Detection of a new anomalous variant of glucose-6-phosphate dehydrogenase in human erythrocytes]. 2 88

Glutathione reductase plays an important role in protecting hemoglobin, red cell enzymes, and biological cell membranes against oxidative damage by increasing the level of reduced glutathone (GSSGR) in the process of aerobic glycolysis. The enzyme deficiency may result in mild to moderately severe hemolytic anemia upon exposure to certain drugs or chemicals. However, hereditary deficiency of the enzyme is extremely rare. Recent studies on glutathione reductase in the red cell have shown more insight in the understanding of red cell metabolism and interactions with other enzymes, especially glucose-6-phosphate dehydrogenase (G-6-PD). Glutathione reducatase in serum may be a source of error in any clinical laboratory test in which an enzyme activity is determined indirectly by measuring the change in reduced nicotinamide-adenine dinucleotide (NADH) or reduced nicotinamide adenine dinucleotide phosphate (NADPH) absorbance. Glutathione reductase levels are reduced in banked blood when citrate-phosphate-dextrose (CPD) is used as a preservative. Reviewed is the role of glutathione reductase in the metabolism of the red cell and its clinical implication and usefulness.
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PMID:Glutathione reductase in the red blood cells. 62 27

The percentage of nicotinamide adenine dinucleotide (NAD) in the oxidized form [NAD+/(NAD+ and NADH); i.e. the NAD+/NADT ratio] is increased in the red cell (RBC) in sickle cell disease. We tested the hypothesis that the increased NAD+/NADT ratio was a determinant of the increased 2,3-diphosphoglycerate (DPG) content of the SCD RBC. Using normal subjects and individuals with sickle cell disease or autoimmune haemolytic anaemia (AIHA), we observed an inverse relationship between the packed cell volume (PCV) and the RBC DPG concentration (r = -0.69) and a direct relationship between the RBC NAD+/NADT ratio and the DPG concentration (r = 0.74). When the effect of the PCV on DPG was removed using analysis of covariance [DPGady(PCV)], the NAD+/NADT ratio had a significant relationship with the DPGadj(PCV) (r = 0.50, P less than 0.001). In in vitro incubation studies, increasing the NAD+/NADT ratio significantly increased the DPG content of both normal and AIHA RBC. Conversely, decreasing the NAD+/NADT ratio decreased the DPG content of normal, AIHA and SCD RBC. Thus, the increased DPG content in the SCD RBC appears to be due, in part, to the increased NAD+/NADT ratio and is not purely a physiologic response to decreased oxygen carrying capacity.
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PMID:Relationship between the nicotinamide adenine dinucleotide redox potential and the 2,3-diphosphoglycerate content in the erythrocyte in sickle cell disease. 275 69

The human erythrocyte generates high-energy adenosine triphosphate by anaerobic glycolysis and cycles oxidized and reduced nicotinamide adenine dinucleotide phosphate by the aerobic pentose phosphate shunt pathway. Certain enzymopathies of the pentose phosphate shunt are associated with hemolysis resulting from oxidative denaturation of hemoglobin. Glucose-6-phosphate dehydrogenase deficiency, an X-chromosome-linked disorder, is the prototype of these diseases and is genetically and clinically polymorphic. Six enzymopathies of anaerobic glycolysis cause hemolytic anemia; lactate dehydrogenase deficiency does not. In 2,3-diphosphoglycerate mutase deficiency, 2,3-diphosphoglycerate is greatly reduced and asymptomatic polycythemia is noted. Pyrimidine-5'-nucleotidase deficiency, an enzymopathy of nucleotide metabolism, is characterized by intracellular accumulations of pyrimidine-containing nucleotides, marked basophilic stippling on the stained blood film, splenomegaly, and hemolysis. Lead inhibits the nucleotidase and an identical syndrome occurs during severe lead poisoning. Hemolysis also accompanies an unusual enzymopathy characterized by a 40- to 70-fold increase (not decrease) in adenosine deaminase activity.
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PMID:Hemolytic anemias and erythrocyte enzymopathies. 299 Feb 76

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

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is a well-characterized X-linked inherited disorder in humans but has not been reported in horses. We describe a persistent hemolytic anemia and hyperbilirubinemia due to a severe G6PD deficiency in an American Saddlebred colt. Other abnormalities in the colt's erythrocytes as compared with those of healthy horses (n = 22-35) included increased activities of hexokinase and pyruvate kinase, decreased concentrations of reduced glutathione and reduced nicotinamide adenine dinucleotide phosphate (NADP), and increased concentration of oxidized NADP. Morphologic abnormalities included eccentrocytosis, pyknocytosis, anisocytosis, macrocytosis, and increased number of Howell-Jolly bodies. Scanning and transmission electron microscopic examinations revealed that eccentrocytes had contracted to spherical regions and thin collapsed regions. Eccentrocytes were more electron dense than were normal erythrocytes when examined by transmission electron microscopy. When exposed to acetylphenylhydrazine, erythrocytes from the G6PD-deficient colt produced more and smaller Heinz bodies than did erythrocytes from normal horses. Abnormalities in the colt's dam included presence of eccentrocytes and pyknocytes; her average erythrocyte G6PD activity was slightly below the range of reference values.
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PMID:Equine glucose-6-phosphate dehydrogenase deficiency. 780 29

The most important products of the hexose monophosphate pathway is reduced nicotinamide adenine dinucleotide phosphate (NADPH). Reduced glutathione (GSH) maintained by the reduction of oxidized glutathione (GSSG) using NADPH as a cofactor, is a major reducing agent in the red cell and the ultimate source of protection against oxidative attack. In the syndromes associated with dysfunction of the hexose monophosphate pathway and glutathione synthesis and metabolism, oxidative denaturation of hemoglobin is the major contributor to the hemolytic process. Glucose-6-phosphate dehydrogenase (G6PD) plays a key role in the generation of NADPH. G6PD deficiency is the most common metabolic disorder, and it is associated with chronic hemolytic anemia and/or drug- or infection-induced acute hemolytic attack. It is estimated that 400 million people are affected worldwide. The mutations responsible for 101 variants have been determined. Some of them have polymorphic frequencies in different populations. Most variants are produced by one or two nucleotide substitutions. Molecular studies have disclosed that most of the class 1 G6PD variants associated with chronic hemolysis have the mutations surrounding the site of dimer formation.
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PMID:[Hemolytic anemia due to the dysfunction of the protection against oxidative attack]. 889 May 80

Ingestion of strong oxidant substances may result in acquired methemoglobinemia, a clinical condition in which the oxidized blood hemoglobin is incapable of delivering oxygen to the tissues, and the patient becomes cyanotic. Traditional first-line therapy consists of infusion of methylene blue, whose action depends on the availability of reduced nicotinamide adenine nucleotide phosphate (NADPH) within the red blood cell (RBC). Some patients, particularly those who are deficient in glucose-6-phosphate dehydrogenase (G6PD), will not benefit from methylene blue. In these patients, and in some patients who have ingested very strong oxidants, methylene blue may also precipitate Heinz body hemolytic anemia. We present a case of severe, acquired methemoglobinemia in a 26-month-old, 9.8-kg boy with G6PD deficiency. He was cyanotic, in respiratory failure, intubated in a pediatric intensive care unit. In typical fashion, he did not respond to methylene blue. Manual exchange of two whole blood volumes, performed over 4 1/2 hr, also failed to resolve his severe methemoglobinemia. An automated RBC exchange (1.3 RBC volume), lowered his methemoglobin content from 31.8% to 7% in a single 40-min procedure. Thereafter his methemoglobin level continued to decrease rapidly and spontaneously. He was discharged home 2 days later, with 0.4% methemoglobin. To our knowledge, this is the first report to demonstrate the (potentially superior) effectiveness of automated RBC exchange for treatment of patients with high-risk acquired methemoglobinemia, that is, those with G6PD deficiency or who have ingested strong oxidants.
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PMID:Treatment of high-risk, refractory acquired methemoglobinemia with automated red blood cell exchange. 959 Apr 95

The mechanism of hemolysis induced by onion poisoning in dogs was studied. Six adult, clinically normal Pekingese dogs were fed cooked onions at 30 g/kg body weight/day for 2 days. Blood samples were collected on days 1, 3, 5, 8, 12, 18 and 24 after onion administration, and urine was collected the day after bleeding. Red blood cell counts, hemoglobin and hematocrit were decreased from day 1, and significantly so on day 5 (P < 0.01), contrary to the results of white blood cell counts. So the plasma bilirubin levels and urobilinogen were increased on day 3 (P < 0.01) and day 4 (P < 0.01), respectively. The Heinz body counts were increased dramatically from day 1 (P < 0.01), peaking on day 3 (P < 0.01). Reticulocyte counts were increased from day 1 and the highest value was on day 8 (P < 0.01). Besides anemia, the following erythrocyte parameters were altered: erythrocyte glucose-6-phosphate dehydrogenase was reduced from day 1 and reached the lowest value on day 5 (P < 0.01); the reduced form of nicotinamide adenine dinucleotide phosphate was decreased on day 1 (P < 0.01); reduced glutathione was decreased from day 1 (P < 0.01) and the lowest value was on day 3 (P < 0.01); glutathione-peroxidase was increased on day 1, but decreased significantly on day 3 (P < 0.01); hydrogen peroxide was increased on day 1 (P < 0.01), then went down on days 3-12 (the undermost value on day 5, P < 0.01); catalase was risen dramatically on day 5 (the peak value, P < 0.01); malondialdehyde (MDA) was increased on days 1-8, (P < 0.01), and reached the peak value on day 5 (P < 0.01). Deformity of the erythrocyte membrane was decreased on days 1-12 and fluorescence polarization (rho) and microviscosity (eta) of the erythrocyte membrane were increased on days 1-12 (P < 0.01). There were positive correlations between MDA and rho as well as eta, with correlation coefficients of 0.908 and 0.922, respectively (P < 0.01), but there was a negative correlation between MDA and deformity index, with a correlation coefficient of -0.887 (P < 0.05). This study confirmed that onion poisoning in dogs causes hemolytic anemia.
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PMID:An experimental study of hemolysis induced by onion (Allium cepa) poisoning in dogs. 1830 6

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is a typical X-linked enzymopathy causing severe haemolytic anaemia in males, and mild to moderate anaemia in homozygous females. Haemolysis due to G6PD deficiency in patients with type 1 diabetes mellitus (T1DM) has been principally reported in males, but is uncommon. During the last 10 years 2 girls with an unknown incomplete G-6-PD deficiency showed haemolysis during the treatment of DKA at the onset of T1DM. We speculate that the patients here described showed haemolytic anaemia as a phenotypic expression of the lyonization process and/or an uncommon penetrance of the defective gene. Haemolysis occurred when blood glucose levels were returning to normal values. In normal red blood cells, G6PD provides a source of reducing power for maintaining sulphydryl groups (SH) and facilitating the detoxification of free radicals and peroxides. During insulin i.v. infusion the copious glucose available due to the hyperglycaemia progressively decreased and affected the old red blood cells to generate nicotinamide adenine dinucleotide (NADPH), a crucial source for energy-dependent functions. This NADPH loss could have enhanced the rate of all factors such as methaemoglobin generation, Heinz body formation, and lipid peroxidation, which occur in G6PD deficient cells in response to both endogenous and exogenous oxidants. The direct consequence of this phenomenon is an increased erytrocyte oxidant sensitivity and a loss of sulphydryl group availability causing premature red blood cell destruction.
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PMID:Haemolysis during diabetic ketoacidosis treatment in two girls with incomplete glucose-6-phosphate dehydrogenase deficiency. 1970 24


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