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Query: UMLS:C0002871 (anemia)
 52,094 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Oxidant and free radical-generating system were used to promote oxidative damage in erythrocytes. Among the oxidants used, phenylhydrazine represents one of the most investigated intracellular free radical-generating probes, which in the presence of haemoglobin auto-oxidises and give rise to hydroxyl radical, a marker for cellular damage. Erythrocyte, as a single cell, is a good model to be used for studying the haemolytic mechanism of anaemia. Our present investigations reveal increased lipid peroxidation of erythrocyte using phenylhydrazine as well as other oxygen-generating systems (hydrogen peroxide, iron with hydrogen peroxide). It has further been observed that not only lipid peroxidation, phenylhydrazine causes significant elevation in methemoglobin formation, catalase activity and turbidity, in the above system, which are the typical characteristics of haemolytic anaemia. However, exogenous administration of green tea leaf extract and ascorbic acid as natural antioxidants and free radical scavengers were shown to protect separately increased lipid peroxidation caused by phenylhydrazine, though the degree of protection is more in case of green tea leaf extract than ascorbic acid. Results suggest that oxidative damage in vivo due to haemolytic disease may be checked to some extent by using natural antioxidants.
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PMID:Oxidant induced injury of erythrocyte-role of green tea leaf and ascorbic acid. 1613 2

A 48-year-old man had a 30-year history of hemolytic anemia of undetermined cause. Spherocytes were not observed, osmotic fragility was normal, and red cell enzyme activities were normal. His brother and daughter also had hemolytic anemia. The brother had previously undergone splenectomy, and the anemia had been ameliorated. In the proband and daughter, no abnormal hemoglobin was apparent in the results of isoelectric focusing and DEAE anion-exchange high-performance liquid chromatography analyses. On evaluation with the isopropanol test, unstable hemoglobin was not observed in the proband but was detected in the daughter. There was also a decreased ratio of 3 globin/3 globin chain production. Analysis of the 32 gene demonstrated the presence of a mutation (alpha43 [CE1] Phe --> Leu), hemoglobin Hirosaki.
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PMID:A family with hemoglobin Hirosaki. 1614 43

We describe a new hyper-unstable beta chain variant (codons 137-139, -6 bp) in a 2-year-old Bulgarian boy. The abnormal hemoglobin (Hb) is associated with severe hemolytic anemia as a consequence of its hyper instability. The child was admitted to the Pediatric Clinic (Faculty of Medicine, Stara Zagora, Bulgaria) at the age of 2 months. Because of anemia (Hb 6.9 g/dL) and high serum iron level (58 microM/L) the child was transfused. However, a month later his Hb level had dropped to 7.5 g/dL, and since then he has been on a regular monthly blood transfusion regimen. Hemoglobin analysis of a blood sample collected 2 months after the last transfusion at the age of 2 years, revealed no abnormalities except for the presence of inclusion bodies after incubation of peripheral blood with brilliant cresyl blue. Sequencing of the beta-globin gene revealed heterozygosity for a 6 bp deletion (-TGGCTA) at codons 137 [the second and third base pair (bp)], 138 and 139 (the first bp), forming a new codon at position 137 (GAT). This event eliminates three amino acids (Val-Ala-Asn) and introduces a new residue (Asp). It creates a new restriction site for HphI. The parents and his dizygotic twin brother had no history of hemolysis. The paternity of the child was confirmed by DNA analysis.
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PMID:Hb Stara Zagora: a new hyper-unstable hemoglobin causing severe hemolytic anemia. 1637 Apr 84

Chronic hemodialysis (HD) patients manifest anemia and atherosclerosis with associated oxidative stress. We explored whether intravenous infusion of vitamin C (VC) and/or use of vitamin E (VE)-coated dialysis membrane could palliate HD-evoked oxidative stress. Eighty patients undergoing chronic HD were enrolled and randomly assigned into four groups: HD with intravenous VC (n=20), HD with VE-coated dialyzer (n=20), HD with both (n=20), and HD with neither (n=20). We evaluated oxidative stress in blood and plasma, erythrocyte methemoglobin/ferricyanide reductase (red blood cells (RBC)-MFR) activity, plasma methemoglobin, and pro-inflammatory cytokines in these patients. All patients showed marked increases (14-fold) in blood reactive oxygen species (ROS) after HD. The types of ROS were mostly hydrogen peroxide, and in lesser amounts, O2*- and HOCl. HD resulted in decreased plasma VC, total antioxidant status, and RBC-MFR activity and increased plasma and erythrocyte levels of phosphatidylcholine hydroperoxide (PCOOH) and methemoglobin. Intravenous VC significantly palliated HD-induced oxidative stress, plasma and RBC levels of PCOOH, and plasma methemoglobin levels and preserved RBC-MFR activity. The VE-coated dialyzer effectively prevented RBCs from oxidative stress, although it showed a partial effect on the reduction of total ROS activity in whole blood. In conclusion, intravenous VC plus a VE-coated dialyzer is effective in palliating HD-evoked oxidative stress, as indicated by hemolysis and lipid peroxidation, and by overexpression of proinflammation cytokines in HD patients. Using VE-coated dialyzer per se is, however, effective in reducing lipid peroxidation and oxidative damage to RBCs.
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PMID:Effects of vitamin C infusion and vitamin E-coated membrane on hemodialysis-induced oxidative stress. 1639 51

Sickle cell disease is a genetic autosomal recessive disease of hemoglobin. The disease results from a mutation of the sixth codon of the beta-globin gene, which induces the synthesis of an abnormal hemoglobin called hemoglobin S (HbS). The polymerisation of deoxy HbS molecules causes a chronic hemolytic anemia and vaso-occlusive phenomenons. The disease affects mainly people from West Indies and Sub saharian Africa. Due to recent movements of these populations over the past years, sickle cell disease has spread across all continents. Painful crises, severe infections such as septicemia, meningitis, osteomyelytis, acute anemia episodes, and severe vaso-occlusive events, mainly neurological, are the most frequent complications affecting children. Recent progresses in the care of patients have deeply modified the prognosis. The mean life expectancy of patients is now above 40 years. The conventional treatment includes antibiotics and immunizations, analgesics, and blood transfusion. The effects of chronic blood transfusion, hydroxyurea and bone marrow transplantation are the subject of current comparative evaluations.
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PMID:[Current developments on sickle cell disease]. 1645 28

We present a family of North European extraction referred for a refractory non iron depleted microcytic anemia. The proband, a 36 year-old male, presented with chronic borderline anemia and microcytic hypochromic parameters. No abnormal hemoglobin (Hb) fractions were observed on high performance liquid chromatography (HPLC) or on alkaline electrophoresis. Gap-polymerase chain reaction (gap-PCR) excluded the seven common alpha-thalassemia (thal) deletion defects. However, the beta/alpha-globin chain synthesis ratio measured in vitro was unbalanced, indicating a reduced expression of the alpha-globin genes. Direct sequencing of the alpha-globin genes revealed heterozygosity for a T --> A transversion at the IVS-II-2 position of the alpha2 gene. This is the first IVS-II splice donor site mutation described on the alpha2-globin gene.
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PMID:Alpha-thalassemia phenotype induced by the new IVS-II-2 (T --> A) splice donor site mutation on the alpha2-globin gene. 1654 Apr 8

Chronic hemodialysis (HD) patients increase erythrocyte susceptibility to hemolysis and impair cell survival. We explored whether electrolyte-reduced water (ERW) could palliate HD-evoked erythrocyte impairment and anemia. Forty-three patients undergoing chronic HD were enrolled and received ERW administration for 6 month. We evaluated oxidative stress in blood and plasma, erythrocyte methemoglobin (metHb)/ferricyanide reductase activity, plasma metHb, and proinflammatory cytokines in the chronic HD patients without treatment (n=15) or with vitamin C (VC)- (n=15), vitamin E (VE)-coated dialyzer (n=15), or ERW treatment (n=15) during an HD course. The patients showed marked increases (15-fold) in blood reactive oxygen species, mostly H(2)O(2), after HD without any treatment. HD resulted in decreased plasma VC, total antioxidant status, and erythrocyte metHb/ferricyanide reductase activity and increased erythrocyte levels of phosphatidylcholine hydroperoxide (PCOOH) and plasma metHb. Antioxidants treatment significantly palliated single HD course-induced oxidative stress, plasma and RBC PCOOH, and plasma metHb levels, and preserved erythrocyte metHb /ferricyanide reductase activity in an order VC>ERW>VE-coated dialyzer. However, ERW had no side effects of oxalate accumulation easily induced by VC. Six-month ERW treatment increased hematocrit and attenuated proinflammatory cytokines profile in the HD patients. In conclusion, ERW treatment administration is effective in palliating HD-evoked oxidative stress, as indicated by lipid peroxidation, hemolysis, and overexpression of proinflammatory cytokines in HD patients.
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PMID:Electrolyzed-reduced water reduced hemodialysis-induced erythrocyte impairment in end-stage renal disease patients. 1676 Sep 3

9 years old male child presented clinically with thalassaemia intermedia phenotype. Investigations revealed hemolytic anaemia due to an unstable hemoglobin. Parents were found negative for the abnormal hemoglobin, suggesting a spontaneous mutation in the child. This is the third case of unstable hemoglobin to be reported from India. Clinically it is important that unstable hemoglobin should be suspected in a patient with thalassaemia intermedia phenotype even if both parents are haematologically normal.
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PMID:Hemolytic anaemia due to unstable hemoglobin arising from spontaneous mutation--a case report. 1747 73

The objective of this review was to direct attention about methemoglobin as a biomarker which has an important role in the detection of adverse effects of the oxidative stress, misbalanced production of ROS, RNS and RSS. According to our hypothesis, a pregnant woman continuously inhaling environmental toxics as fuel burning products, will traverse three, not two from current thought, distinct stages. The main difference among present three-stage hypothesis and other hypotheses is the assertion that, in the pathogenesis of early and late complicated pregnancy, methemoglobin takes on an important role. Secondly, we also observed the utero-placental changes as "locus manifesting minoris resistentiae" in complicated pregnancy are not the causes but a consequence of increased systemic oxidative stress. Methemoglobin and hemolysis both occur as a result of oxidative stress, but the prevalent difference between them is that methemoglobin is a reversible phenomenon (oxidant-antioxidant balance) whereas hemolysis, which occurs as a result of oxidative stress on the erythrocyte membrane, is an irreversible event. Methemoglobinemia can additionally exacerbate an existing anemia, stimulating hypoxia that may be dangerous for both mother and fetus. Own prospective study of methemoglobin in pregnancy, revealed a significant rise in the level of methemoglobin >1.5 g/L (r=0.72, p<0.01) in the exposure period, which can be explained on the basis of an oxidant-antioxidant imbalance, resulting in methemoglobinemia. Methemoglobinemia and stillbirth recorded throughout exposure period are significantly higher than those recorded in the control period (p=0.0205), and the frequencies of reproductive loss were significantly lower in the control than in the exposure period (p<0.05). Results suggest that methemoglobin as individual indicator of oxidative stress is an early marker of the identification of women with a pregnancy risk. It has the advantage of being applicable some time before ultrasonic examination becomes feasible. Further support for this assumption will require further investigations that may lead to the supposition that increasing level of methemoglobin is related to environmental toxicities complicated pregnancy and IUGR, preeclampsia, and a high percentage of perinatal mortality and morbidity.
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PMID:The role of methemoglobinemia in early and late complicated pregnancy. 1711 81

A complex study of the peripheral erythron component was performed during methemoglobinemias induced by single administration of sodium nitrate and phenylhydrazine in LD50. Administration of methemoglobin-forming agents to rats induced the development of hemolytic anemia. The pathogenesis of this disorder included significant long-term modifications of the erythrocyte membrane. The severity and duration of anemia syndrome depended on chemical structure of xenobiotics, blood methemoglobin level, and the duration of the acute period of methemoglobinemia.
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PMID:Mechanisms of hemolytic anemia during experimental methemoglobinemias. 1741 63


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