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Query: UNIPROT:P02794 (ferritin)
17,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Serum ferritin spectrotypes from patients heterozygous for beta-thalassaemia were determined after agarose isoelectric focusing followed by radio-immunofixation with anti-ferritin antibody. Multivariate analysis demonstrated a specific spectrotype for heterozygous beta-thalassaemia. This spectrotype was shown to be different from those in hereditary spherocytosis and idiopathic haemochromatosis. Statistical discrimination reached 100% of well-classified patients between these pathological conditions.
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PMID:Serum ferritin spectrotypes in patients with heterozygous beta-thalassaemia. 204 59

The basic ferritin level in red cells was examined in 60 healthy volunteers and in 110 patients with various diseases of the red cell series. From samples of heparinized whole blood first leucocytes were removed and then they were haemolyzed. After subsequent centrifuging in the supernatant the basic ferritin content was assessed by means of an Amersham Ferritin RIA kit (Amersham, Great Britain). Normal values in healthy volunteers varied between 3.2 and 30.2 ag/ery. In patients with iron deficiency the values were significantly reduced, in patients with pernicious anaemia, beta-thalassemia minor, hereditary spherocytosis and glucose-6-phosphate dehydrogenase deficiency or pyruvate kinase deficiency the levels of red cell ferritin were significantly elevated, as compared with the control group. The red cell ferritin level depends on the iron supply to cells of the red cell series and the amount needed for erythropoiesis. A reduced supply in sideropenia leads to a reduced level of red cell ferritin, in haemolytic anaemia its level depends on the generally elevated iron supply in the organism and possibly impaired haemoglobin synthesis, on the one hand, and concurrent elevated iron requirements in case of hyperplasia of red cell formation, on the other hand. Assessment of red cell ferritin is important not only for accurate estimation of the amount of iron available in the cell for haeme formation but also to elucidate the pathogenesis of some changes of iron metabolism in diseases of the red cell series.
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PMID:[The importance of determination of ferritin levels in erythrocytes]. 224 33

Seventy-three patients with hereditary spherocytosis (HS) (58 nonsplenectomized, 15 splenectomized) were studied to evaluate iron status and the adequacy of iron availability for erythropoiesis. Splenectomized patients, who had hemoglobin levels in the normal or upper normal range, had higher levels of serum iron, transferrin saturation, and serum ferritin than normal matched controls and normal zinc protoporphyrin (ZnPP) levels. On the contrary, nonsplenectomized patients presenting with mild to severe anemia had higher red cell ZnPP concentrations than both splenectomized subjects and matched normal controls. ZnPP in nonsplenectomized patients correlated inversely with Hb concentration, mean corpuscular volume (MCV), mean red cell hemoglobin concentration (MCHC), transferrin saturation, and serum iron, and directly with reticulocyte count. At multiple regression analysis only Hb concentration was a significant explanatory variable for high ZnPP. The authors conclude that a number of nonsplenectomized HS patients have relative iron deficiency primarily because of expansion of erythropoiesis caused by anemia.
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PMID:Relative iron deficiency in hereditary spherocytosis. 273 20

Measurements of erythropoiesis and iron balance were made in eight normal and 32 anemic subjects. The latter consisted of 12 individuals with ineffective erythropoiesis (beta-thalassemia/hemoglobin E), 13 subjects with ineffective erythropoiesis and hemolytic anemia (hemoglobin H), and seven subjects with hemolytic anemia (hereditary spherocytosis). A consistent relationship within each group existed between the degree of erythropoiesis and radioiron absorption. Although the effect of erythropoiesis on iron absorption was of similar magnitude in the two thalassemia groups, the effect in hereditary spherocytosis was much less. There was agreement between absorption and ferritin or magnetic susceptibility (SQUID) measurements of iron stores in thalassemia, but in hereditary spherocytosis a discrepancy existed between absorption and ferritin. It is concluded that, although increased erythropoiesis is associated with increased iron absorption, some additional factor associated with red cell breakdown is more directly responsible for the positive iron balance in thalassemia.
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PMID:The effect of erythroid hyperplasia on iron balance. 335 91

Serum ferritin was measured in 61 patients with autosomal dominant hereditary spherocytosis (HS), 44 splenectomized and 17 with intact spleens. In the majority (78%) the serum ferritin concentration was not elevated. Thirteen (22%) had midly elevated levels, including five splenectomized females (range 181--236 microgram/l), and eight males, two with intact spleens (range 236--436 microgram/l). The serum ferritin was not raised in 15 of the 17 non-splenectomized individuals. This group included one female who had been venesected for iron overload and further investigations have shown that both the genes for HS and haemochromatosis are present in her family. These results demonstrate that iron stores are usually normal in HS, and that prevention of iron overload alone is not an indication for splenectomy. The rare report of a patient with HS and severe iron overload may perhaps be explained by the fact that the gene frequency for haemochromatosis is common in the population.
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PMID:Serum ferritin in patients with hereditary spherocytosis. 727 24

Red cell ferritin (rFt) values were measured in 18 patients with iron deficiency anemia (IDA), 7 patients with hereditary spherocytosis (HS), 8 patients with megaloblastic anemia (MA), and 6 patients with polycythemia vera (PV). Furthermore, rFt was analyzed by column isoelectric focusing (IEF). The rFt values (Mean +/- SD) for healthy males and females are 14.3 +/- 10.3 and 7.5 +/- 3.6 ag/cell (ag = 10(-18)g), respectively, and a significant difference was noted between them by Student's t-test (p < 0.01). The mean rFt values in IDA, HS, MA, and PV patients were 1.9, 115.5, 198.3, and 3.3 ag/cell, respectively. The rFt values in IDA and PV patients were significantly lower than normal. However, there was no significant difference between levels presented in the two disease states. The rFt levels in HS and MA were significantly higher than those in healthy subjects. The isoelectric point (pI) of rFt determined by IEF in healthy subjects ranged from 5.1 to 5.7. The pI ranges for IDA patients showed a slight basic shift compared with those of normal subjects, and so did the ranges for PV patients. Marked increase in acidic red cell ferritin was detected by IEF in patients with IDA after iron therapy. The pI ranges for HS patients were diverse, and were considered to depend on the severity or clinical stage of the disease. The rFt of MA patients showed pI ranges similar to healthy subjects and the ranges differed little after Vitamin B12 therapy, irrespective of a drastic decrease in rFt concentration.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Red cell ferritin in non-leukaemic various erythropoietic disorders]. 792 80

Hereditary spherocytosis is the most common inherent, autosomal dominant hemolytic anemia. Mild splenomegaly, venostasis and common decrease of while pulp is characteristic for hereditary spherocytosis. Cords are filled with spherocytes, sinuses can be empty or squeezed. Both sinuses and veins include ghost erythrocytes that lost haemoglobin. They are seen light-microscopically in differential interferent contrast. Macrophages are numerous, sinus lining cells are hypertrophic. Erythrophagocytosis is hardly seen by electron microscope. Not all red blood cells are spherocytes. Spherocytes are seen well in electronmicroscopy. We did not observed transition of erythrocytes through sinus walls. In certain circumstances lots of ferritin is seen both intra and extracytoplasmically. Iron accumulation in cords can result in their fibrosis.
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PMID:[The spleen in hereditary spherocytosis]. 956 Aug 87

Circulating serum transferrin receptor level was measured using mouse monoclonal antibody against transferrin receptor (Orion Diagnostica, Finland) in 126 patients with various disorders of erythropoiesis and the results were compared to those obtained form control group consisted of 30 healthy volunteers with normal iron stores. Serum transferrin receptor level was significantly elevated in patients with iron deficiency and in all patients with hyperplastic erythropoiesis (hereditary spherocytosis, immune hemolytic anemia, beta thalassemia, myelodysplasia). Measurement of circulating serum transferrin receptor level was a sensitive indicator of iron depletion as well as a helpful parameter in differential diagnosis between iron deficiency and anemia of chronic disease where circulating transferrin receptor level was not elevated. Index transferrin receptor/ferritin calculated as a ratio of circulating serum transferrin receptor level to log serum ferritin level was a more sensitive parameter than measurement of serum transferrin receptor not only for determination of patients with anemia of chronic disease, but also for discrimination of patients with elevated serum transferrin receptor level due to true iron deficiency from those with high serum transferrin receptor level caused by relative iron deficiency in hyperplastic erythropoiesis.
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PMID:[Clinical importance of determining levels of circulating transferrin receptors in blood]. 1104 46

Infections with parvovirus B 19 can cause aplastic crises with a rapid decline of hemoglobin levels in patients with hereditary spherocytosis. Usually, the symptoms and signs of the actual infection are mild. We here report on an eight year old girl with hereditary spherocytosis who was admitted to hospital with high temperature, headache, impaired consciousness and a profound anemia (Hb 2.9 mmol/l). Since she also developed low leukocyte and platelet counts a hematological malignancy was suspected. The bone marrow aspirate showed only 1 % erythroblasts and macrophages with active hemophagocytosis. The serum ferritin was 1381,4 ng/ml. Both, serology and PCR revealed an active infection with parvovirus B 19. Coagulation analysis suggested a low degree of disseminated intravasal coagulation (low fibrinogen, high D-dimers). We diagnosed a parvovirus B 19 associated hemophagocytic syndrome. With only symptomatic treatment the patient's condition and laboratory findings improved during the course of a few days. In accordance with other reported cases, the prognosis of parvovirus B 19 associated hemophagocytic syndrome seems to be better than in hemophagocytic syndrome of other origin.
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PMID:[Parvovirus B 19 associated hemophagocytic syndrome in a patient with hereditary sperocytosis]. 1452 May 89

Modern double beam laser technique allows screening for hereditary spherocytosis in the course of routine hematology. An incidence of 1:150 men and 1:800 women has been determined. The anomaly is symptomless in the majority of the cases. This explains the discrepancy between our values and the incidence of 1:5,000 reported in the literature. The diagnosis of hereditary spherocytosis should be reported to the physician and the patient, as it may be wayleading in case of unexpected, unspecific complications such as anemia, jaundice, cholelithiasis, liver cell damage and iron overload. Regular monitoring of plasma ferritin and glucose is recommended.
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PMID:Hereditary spherocytosis is more frequent than expected: what to tell the patient? 1604 49


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