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Query: UMLS:C0039730 (thalassemia)
 10,305 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Glutathione peroxidase (GSHPx) activity was found to be greatly elevated in members of a family with alpha-thalassemia. Eleven other families with proven alpha-thalassemia were investigated, and all but one subject with hemoglobin H disease had increased red cell GSHPx. Most persons with alpha-thalassemia trait also had increased activity of red cell GSHPx. In contrast, only very modest increases in glutathione peroxidase activity were observed in subjects with various forms of beta-thalassemia.
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PMID:Increased glutathione peroxidase activity in alpha-thalassemia. 90 38

The mass concentrations of whole blood reduced glutathione and catalytic activity concentrations of the enzymes, glucose-6-phosphate dehydrogenase (EC 1.1.1.49), glutathione reductase (EC 1.6.4.2) and glutathione peroxidase (EC 1.11.9) were analysed in 25 cases of homozygous beta-thalassaemia, 20 cases of heterozygous beta-thalassaemia and 10 controls. The results showed a significant elevation of reduced glutathione and enzymes of the pentose phosphate pathway in homozygous beta-thalassaemia, indicating the existence of an enzyme-regulated glutathione turnover system in the overt state to combat the augmented red cell membrane damage due to auto-oxidant threat. However, in heterozygous beta-thalassaemia, reduced glutathione was increased, but there was no similar elevation of enzymes except for glutathione peroxidase.
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PMID:Enzymes of the pentose phosphate pathway in glutathione-regulated membrane protection in beta-thalassaemia. 144 62

Erythrocyte antioxidant enzymes, superoxide dismutase, catalase and glutathione peroxidase were found to be significantly high in subjects with alpha-thalassaemia and Hb Lepore trait, as a consequence of the increased oxidant stress which is known to exist in these conditions. Among the serum trace elements present in these enzymes, selenium was increased in subjects with Hb Lepore trait and significantly low in those with alpha-thalassaemia trait, while selenium erythrocyte content was significantly increased in alpha-thalassaemic subjects.
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PMID:Antioxidant system and serum trace elements in alpha-thalassaemia and Hb Lepore trait. 365 68

The activities of erythrocyte antioxidative enzymes were measured in two groups of patients with different genotypes of haemoglobin (Hb) H disease: 21 with alpha-thalassaemia 1 or alpha-thalassaemia 2 (alpha-thalassaemia 1/2) and 21 with alpha-thalassaemia 1/Hb Constant Spring (HbCS). They were compared with 21 normal subjects. Both genotypes of Hb H disease had increased activities of erythrocyte superoxide dismutase (SOD), glutathione peroxidase (GSH-Px), and catalase when compared with those of controls. Comparison of the two genotypes showed that subjects with alpha-thalassaemia 1/Hb CS, the more severe disease, had higher SOD and GSH-Px activities but lower catalase activity than those with alpha-thalassaemia 1/2. This indicates that there are compensatory mechanisms in Hb H erythrocytes to cope with increased generation of oxygen free radicals as a result of increased excess beta chain.
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PMID:Comparison of erythrocyte antioxidative enzyme activities between two types of haemoglobin H disease. 380 16

The activity of 18 red blood cell (RBC) enzymes and reduced glutathione (GSH) content were measured in 70 normal subjects, in 50 heterozygous beta-thalassaemia carriers and in 50 non-thalassaemic patients with haemolytic anaemia and high reticulocyte counts. In addition, pyrimidine 5'nucleotidase (P5N) activity was also determined in 34 patients with hypochromic, microcytic, iron deficiency anaemia. beta-Thalassaemia trait was associated with an increase in almost all of the enzyme activities, except for 2,3-bisphosphoglycerate synthetase (BPGS) and glutathione reductase (GR) which were normal and for acetylcholinesterase (AChE) and P5N which were slightly and markedly decreased respectively. The increases in enzyme activities were similar to those observed in patients with non-thalassaemic reticulocytosis except for glyceraldehydephosphate dehydrogenase (GAPD), phosphoglyceratekinase (PGK), pyruvate kinase (PK), glutathione peroxidase (GPX) and adenylate kinase (AK) which were higher than in non-thalassaemic group of patients with increased number of reticulocytes. No correlation was found between the severity of P5N deficiency and the intensity of basophilic stippling which was present in 46 of 50 thalassaemic carriers here studied. In addition, GSH content and UV absorption spectra of deproteinized thalassaemic RBC extracts were also found to be normal. The present findings provide further information on the metabolic status of RBC in beta-thalassaemia trait and suggest a possible molecular explanation for the frequently observed basophilic stippling in this disease.
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PMID:Pyrimidine 5'nucleotidase and several other red cell enzyme activities in beta-thalassaemia trait. 632 Aug 62

In homozygous beta-thalassemia low serum level of alpha-tocopherol have been found. The administration of high doses of the vitamin increased the serum level, decreased lipid peroxidation and, in some case, prolonged red blood cell survival; no significant change in transfusion requirement was obtained. Only few data are available about the vitamin E in heterozygous beta-thalassemia. We have studied 131 patients aged 1 to 72 years with thalassemic trait and 218 age-matched controls. Serum level of alpha-tocopherol was statistically lower in the former. We have studied the effect of vitamin E on 10 patients. In each subject the subsequent parameters were determined before and after a three month treatment (vitamin E 400-600 mg/day) glutathione peroxidase, pyruvate kinase and creatine in erythrocytes serum vitamin E and red blood cell count. Hematological values were unchanged in all patients. In half of them biochemical parameters showed reduction of lipid peroxidation and increased erythrocyte survival.
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PMID:Vitamin E in beta-thalassemia. 712 66

Glucose-6-phosphate dehydrogenase (G-6-PD) deficient erythrocytes are particularly sensitive to oxidant stress. In order to evaluate if these cells are protected against oxidant damage, we assayed the antioxidant enzymes superoxide dismutase (SOD), catalase and glutathione peroxidase (GSH-Px) in erythrocytes of G-6-PD deficient (hemizygous and heterozygous) subjects. Normal levels of antioxidant enzymes were found in all subjects examined both with positive and negative histories of haemolytic crisis after fava bean or drug ingestion. In contrast, high levels of catalase and GSH-Px were found in a small group of G-6-PD deficient subjects (hemizygous and heterozygous) with beta-thalassaemia trait, probably by reason of the chronically enhanced oxidant stress which is present in beta-thalassaemia.
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PMID:Erythrocyte superoxide dismutase, catalase and glutathione peroxidase in glucose-6-phosphate dehydrogenase deficiency. 713 87

Whole blood glutathione peroxidase was found significantly increased relative to controls in a group of 21 black patients with sickle cell anaemia. One control group consisted of 15 normal black subjects. A second control group, consisting of 21 black patients with various abnormal haemoglobins including alpha-thalassaemia, also showed a tendency to enhanced glutathione peroxidase activity, confirming previous reports that elevated glutathione peroxidase levels are secondary to a variety of haemolytic conditions, rather than typical of sickle cell anaemia.
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PMID:Glutathione peroxidase activity in whole blood of patients with sickle cell anaemia. 723 85

The activities and subcellular distribution of enzymes implicated in the protection of cells from free-radical mediated damage were determined in liver biopsy specimens from control and iron-overloaded patients. 2. There was a small but insignificant decrease in the activity of glutathione reductase in patients with secondary iron overload due to multiple transfusion therapy for thalassaemia major. 3. The activities of superoxide dismutase, catalase and glutathione peroxidase were similar in both patient groups. 4. Subcellular fractionation studies indicated a major cytosolic localization of these enzymes with a minor mitochondrial component. The relative proportions of the enzymes in the two locations was similar in both control and iron-overloaded patients. 5. Approximately 80% of the hepatic glutathione was present in the reduced form in both patient groups and it is concluded that although free-radical mediated damage might be implicated in the pathogenesis of tissue damage due to iron overload no significant defect in these protective mechanisms can be demonstrated.
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PMID:Activities of some free-radical scavenging enzymes and glutathione concentrations in human and rat liver and their relationship to the pathogenesis of tissue damage in iron overload. 736 62

The 'antioxidant' enzymes superoxide dismutase (SD), catalase and glutathione peroxidase (GSH-Px) were found greatly elevated in red blood cells of subjects with beta-thalassaemia minor and similar to normal values in red blood cells of subjects with beta-thalassaemia major. These findings allows us to speculate that red cells in beta-thalassaemia minor react to the increased oxidant threat with augmented antioxidant enzyme activities. The normal levels of antioxidant enzymes in beta-thalassaemia major seem to be due to the presence of normal red cells owing to multiple transfusions.
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PMID:Erythrocyte superoxide dismutase, catalase and glutathione peroxidase activities in beta-thalassaemia (major and minor). 744 78


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