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)

A patient with a biochemically "new" type of congenital erythropoietic porphyria has been studied under various therapeutic trials. Splenectomy had no demonstrable effect on porphyrin excretion or clinical picture. Vitamin E caused a moderate fall in porphyrin excretion, however, there was no significant improvement in light tolerance and tendency to hemolysis. Beta-carotene reduced skin photosensitivity appreciably, while total porphyrin excretion remained unchanged and the tendency to develop hemolytic anemia showed only slight improvement. Red cell transfusion caused a rapid, dramatic fall in prophyrin excretion (in 4-5 days) and a transient increase in light tolerance, while the distribution of the different porphyrins excreted remained unchanged. These observations indicate that all or nearly the abnormal porphyrins excreted are of erythropoietic origin, and that the overwhelming part of the porphyrins originate from an abnormal population of shortlived red cells. Findings on fluorescence microscopy of blood and bone marrow support this view. Meticulous protection against light of the shorter wavelengths caused a similar rise in hemoglobin level as produced by red cell transfusion, however, in this instance the total excretion of porphyrins did not fall. It is suggested that the inhibitory effect of transfusion on erythropoiesis (and thereby porphyrin excretion) might be due partly to a depression of erythropoietin formation, partly to the presence of an erythropoiesis inhibiting factor (chalone) in the transfused red cells.
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PMID:The effect of various therapeutic trials on the prophyrin excretion in a case of congenital erythropoietic prophyria. 113 Jan 87

Vitamin E (alpha-toxopherol) is widely used, at different dosages and schedules and in different formulations, in neonatal intensive care units to prevent intraventricular hemorrhage, retrolental fibroplasia, bronchopulmonary dysplasia and hemolytic anemia of preterm infants. As part of a wider project to assess the effect of vitamin E, the present study was designed to determine whether the only intramuscular formulation available today in Italy and in other European countries (olive oil solution), and widely used in all Italian neonatal intensive care units according to a standard schedule, achieves and maintains suggested therapeutic levels, compared to the colloidal aqueous solution used in clinical trials in England and Canada, which contained the acetate ester of alpha-tocopherol and as used in the USA, which contained the free tocopherol, but is not yet available in Italy. Forty-four babies, of less than 32 weeks' gestation, 670-1,800 g birth weight, were randomly allocated to one of the two formulations. We gave 20 mg/kg of vitamin E intramuscularly on 3 consecutive days starting within 8 h of birth (day 0), 24 and 48 h later (days 1 and 2). Blood, plasma, red blood cells after transfusions and milk were sampled up to the sixth day of life. Clinical data were collected up to discharge from the neonatal intensive care unit. Plasma concentrations of vitamin E (free tocopherol) averaged 1.1 at 24 h and 3.3 mg/dl at 72 h after the colloidal aqueous solution, and were about 6 times lower after the oil solution. Plasma vitamin E levels did not rise above baseline after injection of the olive oil preparation. The acetate ester of vitamin E was measured only after use of the colloidal aqueous preparation, and the highest plasma concentration averaged 1.01 mg/dl 72 h after injection. These findings have far-reaching implications related to the current clinical practice in Italy of prophylaxis with intramuscular vitamin E, using the scantily bioavailable olive oil formulation currently on the market.
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PMID:Absorption of intramuscular vitamin E in premature babies. Italian Collaborative Group on Preterm Delivery. 187 47

alpha-Tocopherol (vitamin E) is widely prescribed in neonatal intensive care units, in large doses and by different schedules, for the prevention of retrolental fibroplasia, intraventricular haemorrhage, bronchopulmonary dysplasia, and haemolytic anaemia. Since the efficacy of the drug in premature newborns seems related to early administration, the physicochemical characteristics of the drug itself and available formulations limit the major therapeutic aim of promptly raising levels of vitamin E in premature babies during the early hours of life. It has thus been suggested that vitamin E be given to the mother before delivery to produce higher drug concentrations in the newborn. To see whether this would work, the tissue distribution and transplacental transfer of vitamin E were studied in six pregnant rabbits at steady-state after an i.v. bolus + infusion to give a mean venous blood concentration of about 325 mumol l-1 of alpha-tocopherol acetate, corresponding to about 30 mumol l-1 of alpha-tocopherol. Endogenous levels were measured in six control pregnant rabbits. In treated animals alpha-tocopherol was increased in liver, spleen, placenta, lung, mammary gland, blood, and bile but not in brain, heart, fat, muscle or adrenals probably because distribution into these tissues is very slow. Vitamin E levels in the placenta of treated mothers were 15 times those of control rabbits, but the vitamin was not detectable in amniotic fluid and only very low levels were found in fetal blood. These findings do not indicate any advantage of giving mothers alpha-tocopherol acetate before delivery.
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PMID:Placental transfer and tissue distribution of vitamin E in pregnant rabbits. 227 44

1. Mean concentration of serum alpha-tocopherol (Vitamin E) in 28 free-living black rhinoceroses sedated during translocation in Zimbabwe was 1.92 (SD, 0.43) mg/l. 2. Alpha-tocopherol was not detectable (less than 0.15 mg/l) in five captive black rhinoceroses held at London Zoo. 3. Circulating levels of all-trans retinol (Vitamin A) were not different between the two groups. 4. The low level of alpha-tocopherol in captive rhinoceroses suggests a risk of acute haemolytic anaemia.
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PMID:Serum alpha-tocopherol, all-trans retinol, total lipids and cholesterol in the black rhinoceros (Diceros bicornis). 290 45

The unique association of idiopathic diffuse membranous nephropathy and micro-angiopathic haemolytic anaemia and thrombocytopenia is described. A 7 year old Vietnamese boy with a 1-month history of anaemia resistant to oral iron supplements presented with acute onset of nephrotic syndrome. Investigations revealed a micro-angiopathic haemolytic anaemia and thrombocytopenia. There was no associated oliguria or uraemia. Diffuse membranous nephropathy was diagnosed by renal biopsy. Apart from a fourfold rise in enterovirus titres, no underlying cause for the haematological or glomerular abnormalities was found. There was an apparent, partial haematological response to fresh frozen plasma infusions, but not to Vitamin E.
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PMID:Micro-angiopathic haemolysis, thrombocytopenia and nephrotic syndrome associated with membranous nephropathy in a Vietnamese boy. 322

The mean plasma level of alpha-tocopherol (vitamin E) measured in 31 free-ranging black rhinoceros (Diceros bicornis) was significantly higher (P less than 0.001) than that in 11 captive animals (means +/- SE = 0.77 +/- 0.05 and 0.18 +/- 0.03 micrograms/ml, respectively). Vitamin E status may influence the health of captive black rhinoceros; in particular, it may be linked to hemolytic anemia commonly observed in these animals in captivity.
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PMID:Vitamin E in captive and wild black rhinoceros (Diceros bicornis). 341 12

Plasma vitamin E levels and the ascorbate cyanide test were assessed in 15 chronic hemodialysis (HD) patients (group A) exposed to persistently elevated chloramine levels in the dialysis water. The vitamin E levels in these patients, who had evidence of oxidant induced hemolytic anemia, were compared to 15 chronic HD patients exposed to low chloramine levels (group B) and 17 controls (group C). Vitamin E levels were found to be significantly lower in group A than in either groups B or C (p less than 0.001). Within group A, levels were lower in those patients who had a positive ascorbate cyanide test (p less than 0.05). Upon removal of chloramines from the dialysis water, vitamin E levels in group A were no longer significantly different from those found in groups B or C.
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PMID:Plasma vitamin E levels in a chronically hemolyzing group of dialysis patients. 395 8

Vitamin E refers to a family of fat-soluble phenolic compounds called tocopherols, which have been established as essential nutrients in vertebrates. In animals the deficiency state has resulted in diminished reproductive capabilities, muscular dystrophy, exudative diathesis, megaloblastosis, gastrointestinal and pulmonary degeneration, and nephrosis. In humans with low vitamin E levels a subclinical diminished erythrocyte life-span has been demonstrated by hydrogen peroxide hemolysis test. This effect may have clinical significance among premature infants. The metabolic function of vitamin E appears to be as a scavenger of lipid peroxides and free oxygen radicals which enter into chain reactions to cause breakdown of lipids. Normal levels of the vitamin serve to prevent this cellular oxidative breakdown. Laboratory measurement of vitamin E is chromatographic, with HPLC presently used in both research and clinical applications. The association between vitamin E levels and hemolytic anemia in humans is currently under investigation.
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PMID:Vitamin E and autoxidation. 634 59

Certain owl monkeys (AOT) develop spontaneous hemolytic anemia that responds to vitamin E. The anemia is associated with red blood cell lipid peroxidation and altered red blood cell membrane lipid composition. To investigate these changes, plasma lipid and lipoprotein profiles were characterized in anemic, anemia-susceptible, and anemia-resistant AOT. The plasma vitamin E and vitamin A concentrations were assessed as an index of fat absorption and the effect of corn oil supplementation and vitamin E-selenium injection were measured. Anemia-susceptible AOT had depressed plasma levels of vitamin E and A and an altered lipoprotein metabolism characterized by elevated ratios of low/high density lipoprotein cholesterol and free to esterified cholesterol in these lipoproteins. Vitamin E-selenium injection in anemia-susceptible AOT increased the plasma vitamin E, and vitamin E and corn oil supplements reduced the high density lipoprotein free to esterified cholesterol ratio. The data suggest that the AOT suffer from fat malabsorption and that the consequences (including tocopherol deficiency) result in altered cholesterol metabolism.
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PMID:Altered lipoprotein metabolism in spontaneous vitamin E deficiency of owl monkeys. 641 97

The immunosuppressant cyclosporine induces acute hemolysis (hemolytic uremic syndrome) in some patients after transplantation, possibly because of its effects on the vascular endothelium. We report a case of Coombs-negative, non-immune severe hemolytic anemia during cyclosporine therapy after allogeneic bone marrow transplantation for severe aplastic anemia. Uremia was not observed. Vitamin E was effective in treating the intravascular hemolysis.
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PMID:Acute hemolysis during cyclosporine therapy successfully treated with vitamin E. 758 Nov 57


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