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

Hematological parameters and free eythrocyte protoporphyrin (FEP) on a capillary blood sample were measured in 175 apparently healthy children ranging from 6 months to six years of age. Thirty eight children had hematological parameters descended and/or FEP elevated were asked to return for blood counts, FEP, serum ferritin, serum iron, total iron binding, capacity, transferrin saturation and ALA-D activity, on a venous blood sample. Only 34 children returned. Twenty seven, 15.4%, had iron stores descended or iron deficiency, 18 of them with anemia. FEP had significant correlation coefficients with hematologic parameters (p less than 0.001), serum iron and transferrin saturation (p less than 0.01). On iron deficiency anemia detection, the FEP had a sensibility and specificity of 0.94 and 0.75 respectively.
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PMID:[Usefulness of the determination of free erythrocyte protoporphyrin in relation to other hematologic parameters in iron deficiency]. 227 93

The baseline hematologic status of 27 patients with modest degrees of aluminum overload was examined. In addition, hematologic data were evaluated in 19 of these patients during and after treatment with DFO. Although neither severe anemia nor microcytosis was observed pretreatment, there was a significant correlation between hemoglobin level and degree of aluminum burden as determined by bone surface aluminum staining (r = -0.58; P less than 0.007). Following treatment with DFO, hemoglobin concentration increased dramatically by 1.3 to 4.4 g/dl in eight patients but did not change in the remaining eleven. Responders and nonresponders were similar with regard to the degree of aluminum overload both before and after chelation therapy but differed with regard to baseline levels of erythropoietin (higher in responders) and degree of iron overload (greater in nonresponders). Pretherapy levels of red cell ALA dehydratase were depressed in all patients (32 +/- 4 vs. 56 +/- 5 U/g Hb in normals) but did not correlate with the degree of aluminum overload and did not change with chelation therapy. Pretherapy levels of red cell protoporphyrin were elevated in 15 of 24 patients (62%) and were higher in responders than in nonresponders. Following DFO therapy, levels fell by 25 to 50% in 7 of 8 patients with elevated pretherapy values, despite the tendency in several patients to develop iron deficiency with treatment.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Aluminum induced anemia: pathogenesis and treatment in patients on chronic hemodialysis. 261 92

A procedure is described for preparing a fraction highly enriched for chicken blood delta-aminolevulinate synthase (ALA-S) using animals recovering from acetylphenylhydrazine-induced anemia. 1. Blood cells collected from chickens recovering from anemia were disrupted by nitrogen cavitation, and the mitochondrial fraction was prepared from the cell homogenates. ALA-S was released then from mitochondria by sonication and isolated by a procedure involving gel filtration chromatography on Sephadex G-150, fractionation with ammonium sulfate, ion exchange chromatography on DEAE-Sephacel, and preparative isoelectric focusing. 2. Electrophoretic analyses under denaturing conditions indicated that the final ALA-S preparation was particularly enriched from a 62,200 Da polypeptide. The enzyme eluted from Sephadex G-200 with an equivalent molecular weight of 115,000; this suggested that active ALA-S was a dimer. 3. ALA-S was most active in the pH range of 7.0-8.0, with an apparent KM of 13 microM for succinyl-CoA and of 4.0 mM for glycine. The activity was inhibited 50% by 30 microM hemin.
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PMID:Properties of chicken erythrocyte delta-aminolevulinate synthase. 319 10

Lead often affects the erythrocyte membrane. The relationship between the changes in erythrocyte membrane and the anemia caused by lead is still unclear. Initially, the effect of lead injected intraperitoneally on the electrophoretic mobility of rat erythrocytes was investigated in order to study the relationship between them. As indices of lead exposure, hemoglobin (Hb) levels, hematocrits (Ht), delta-aminolevulinic acid dehydratase (ALA-D) activities and blood lead (blood Pb) levels in the injected rats were also examined. Exposure to lead significantly decreased the mobility of rat erythrocytes. The changes in mobility seemed to be less sensitive than those in ALA-D activity, however, the decreases in mobility were simultaneous with or prior to those in Hb level and Ht. The decreases in mobility were evident to some extent below a blood Pb level of 100 micrograms/100 ml and generally present at a level of 100 micrograms/100 ml and over. In the rats exposed to lead a significant negative correlation was found between the mobilities and the logarithms of blood Pb level.
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PMID:Effect of lead on electrophoretic mobility of rat erythrocytes. 375 Mar 26

delta-Aminolevulinic acid dehydratase (ALA-D), respectively porphobilinogen synthase, EC 4.2.1.24) activity can be lowered by toxic, metabolic and hereditary factors. A 30-year-old painter was suffering from lead poisoning with an acute abdominal-neurologic syndrome and anemia. Blood lead was measured at 414 micrograms/l. Urinary ALA and coproporphyrin excretion as well as erythrocyte protoporphyrin had increased extremely, whereas ALA-D activity in erythrocytes had decreased extremely to 8% of controls. Excretion parameters, protoporphyrin, hemoglobin and lead returned to normal after treatment, but four years later ALA-D activity still remained diminished (30% of controls). An inherited enzyme deficiency was assumed and found in the mother, analogous to the subnormal ALA-D activity in heterozygotes of four other families. The inherited enzyme deficiency sensitized the patient to lead exposure and intoxication, which is a toxogenetic disease in this case.
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PMID:Lead poisoning in inherited delta-aminolevulinic acid dehydratase deficiency. 642 16

A retrospective study was carried out in a rural area of north-eastern Brazil, to search for interactions between biological and chemical agents present in the environment as causes of anaemia. A lead smelter situated in a hookworm endemic area provided the opportunity to look at the effect of this double exposure in relation to laboratory defined anaemia, by comparing the odds ratios in different subgroups of 109 "cases" and 107 "controls". The subgroup with both hookworm infection and chronic lead poisoning--measured by the concentration of delta-ALA excreted in urine--had an odds ratio of developing anaemia five times greater than individuals with neither. Although a synergistic effect could not be demonstrated, the additive effect of the two conditions placed this group in a higher risk category.
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PMID:Lead poisoning and hookworm infection as multiple factors in anaemia. 662 88

Sideroblastic Anaemias are characterised by a) chronic hypochromic anemia, b) ringed sideroblasts in the bone marrow, c) an increase in total body iron, d) ineffective erythropoiesis and e) often abnormal concentrations of F.E.P. A classification of Sideroblastic Anaemia is given and the pathophysiology of Sideroblastic Anaemia is discussed. A series of seven paediatric cases with Sideroblastic Anaemia is presented and the results of studies of the iron, vitamin B6 and porphyrin metabolism are discussed. In two cases arguments for an ALA-synthetase deficiency are given. All five males were diagnosed as hereditary X-linked Sideroblastic Anaemia, one female as I.R.S.A. and the other female, who showed the features of the X-linked type, as congenital Sideroblastic Anaemia.
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PMID:Sideroblastic anaemia. A review of seven paediatric cases. 709 32

The amount of ALA-D in human erythrocytes was determined directly by radioimmunoassay or calculated from the restored activity assayed in the presence of zinc and dithiothreitol, and a good correlation was observed between the RIA-based and the restored activity-based amounts. The RIA-based amount of ALA-D in the blood of 10 normal individuals (blood lead levels of 5.6 +/- 2.3 micrograms/ml: mean +/- SD) and 19 lead-exposed workers (blood lead levels of 41.2 +/- 10.2 micrograms/100 ml) was 54.1 +/- 11.8 microgram/ml blood and 92.3 +/- 20.6 micrograms/ml blood, respectively, indicating an apparent increase of the enzyme amount in lead-exposed workers. A significant increase in the amount of erythrocyte ALA-D calculated from the restored activity in lead-exposed workers was observed even in the low blood lead level of 10-20 microgram/100 ml, resulting in the range of blood lead level 20-40 microgram/100 ml. No significant difference was observed in hematocrit and hemoglobin content between lead-exposed and non-exposed groups. These observations suggested that the increase of erythrocyte ALA-D in lead exposure was not due to anemia, which might result in the increase of young erythrocytes in peripheral blood. This increase in the amount of ALA-D in human erythrocytes might be a result of the function to overcome the inhibition of the enzyme in bone marrow cells during lead exposure, and these findings may throw light on the danger to human health of low-level lead toxicity.
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PMID:Increase in the amount of erythrocyte delta-aminolevulinic acid dehydratase in workers with moderate lead exposure. 712 53

Experimental sideroblastic anaemia was produced in normal and in iron loaded guinea pigs by intraperitoneal (i.p.) administration of isoniazid and cycloserine. Subsequently, the activities of delta-aminolaevulinic acid synthase (ALA-S) and of haem synthase in peripheral red blood cells were measured and in particular the relationships of enzyme activities to the iron status were examined. The ALA-S activity showed a similar decrease in all animals with sideroblastic anaemia. The haem synthase activity was increased probably due to secondary induction, but it was significantly less increased in animals with the highest values for iron status. This finding indicates that mitochondrial iron accumulation may have limited the compensatory increase of haem synthase activity. It is likely that also in human sideroblastic anaemia mitochondrial iron overload may have a secondary limiting effect on the haem synthase activity in erythroid cells.
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PMID:The activities of delta-aminolaevulinic acid synthase and haem synthase in experimental sideroblastic anaemia. Effect of mitochondrial iron excess on the enzyme activity in peripheral red blood cells. 731 98

The activities of 5 enzymes of the haem biosynthetic pathway and the protoporphyrin concentrations have been measured in peripheral red blood cells of 23 patients having a preleukaemic syndrome with refractory sideroblastic anaemia. A decreased delta-aminolaevulinic acid synthase (ALA-S) activity, an increased uroporphyrinogen I synthase activity and an increased red cell protoporphyrin concentration were consistent findings. Patients with abnormal leucocyte and/or platelet counts in the peripheral blood as well as patients with an excess of blast cells in the bone marrow had the lowest ALA-S activities. A further decrease in ALA-S activity was observed in 3 patients after leukaemic change in the disease. Patients having cytogenetic abnormalities showed no unique enzyme abnormalities. These results indicate that enzymatic disturbances of haem synthesis cannot be used as prognostic indicator of leukaemic transformation in refractory sideroblastic anaemia, but a very low ALA-S activity appears to accompany the development of a leukaemia in such patients.
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PMID:Haem biosynthesis in refractory sideroblastic anaemia associated with the preleukaemic syndrome. 733 57


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