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

Serum ferritin, liver iron stores, and liver histology were studied in 38 children with thalassaemia major who were being treated by regular blood transfusions. There was no correlation between serum ferritin levels and either the number of transfusions or the amount of iron deposited in the liver. However, for a given level of iron stores, ferritin levels were higher in patients with chronic hepatitis (including chronic aggressive and chronic persistent forms) than in those with hepatic siderosis only. We conclude that serum ferritin reflects tissue iron deposits in regularly transfused thalassaemic patients, only in the absence of hepatitis.
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PMID:Serum ferritin, liver iron stores, and liver histology in children with thalassaemia. 737 16

The distribution of stainable iron stores was investigated in various organs of 50 hemodialysis patients with chronic renal disease. Massive iron deposits were found in the liver and spleen. Among 18 patients with severe hepatosplenic siderosis, iron deposits were abundant in the adrenal glands, lymph nodes, and lungs and were sparse in the heart, kidneys, and pancreas. There was an absence or scarcity of stainable iron in bone marrow of 19 pats. In five of these marrow-iron-depleted patients, serum concentrations of ferritin were high. In long-term hemodialysis patients, a variety of factors make massive iron overloads of various organs a likely occurrence, severe hepatosplenic siderosis may occur in marrow-iron-depleted patients, and serum ferritin levels in this setting may not always accurately reflect the status of marrow iron store.
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PMID:Hemosiderosis in hemodialysis patients. An autopsy study of 50 cases. 739 25

Homozygote hypotransferrinaemic mice (hpx/hpx) have cytopathological features similar to those of human congenital atransferrinaemia, genetic haemochromatosis, and neonatal haemochromatosis. These conditions all have in common high levels of cytotoxic non-transferrin-bound serum iron. This study describes the ultrastructural features of iron overload in liver, pancreas, heart, and small intestine of 2- and 12-month-old hypotransferrinaemic mice. Electron microscopic studies of unstained sections showed early parenchymal cell siderosis, with accumulation of numerous ferritin particles and clusters in the cytosol, as well as ferritin and haemosiderin in lysosomes (siderosomes). In the 12-month-old animals, iron was also found in Kupffer cells and macrophages in other tissues. In addition, there were conspicuous iron-containing compounds in the bile canaliculi, and marked iron deposition in the pancreas and heart. Laser microprobe mass analysis (LAMMA) enabled localization and relative quantitation of iron deposition in subcellular compartments providing in situ documentation of iron accumulation in siderosomes and contributed in assessing total cytosolic iron in various cell types. Moreover, it demonstrated the importance and magnitude of the biliary route for iron excretion in these animals.
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PMID:The hypotransferrinaemic mouse: ultrastructural and laser microprobe analysis observations. 747 84

Secondary heart failure induced by organ siderosis is the main cause of death in patients affected by thalassemia major. At present it cannot be predicted whether heart siderosis is correlated with iron overload and little is known about the real cardiac histological pattern of post transfusional hemochromatosis in patients with thalassemia major and intermedia. The study aim was to evaluate cardiac iron overload by non invasive and invasive techniques. Fifteen thalassemic patients were investigated and endomyocardial biopsy performed in ten revealed different grades of endomyocardial iron overload with histochemical positivity. Non invasive techniques are not able to furnish an exact picture of the cardiac hemochromatosis. There was a significant correlation between serum ferritin and myocardial iron grade. Patients with elevated ferritin levels and poor compliance to chelating therapy are at high risk of severe heart hemochromatosis. It was seen that endomyocardial biopsy is a useful tool in studying myocardial iron.
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PMID:Cardiac iron overload in thalassemic patients: an endomyocardial biopsy study. 754 32

A survey is given of methods involving decalcification and paraffin embedding of iliac crest biopsy for osteological and haematological diagnostic procedures. In order to avoid shrinkage, loss of antigens, and fading of ferritin iron and enzymes, a fixative has been designed that is composed of an aqueous solution of calcium acetate (10(-1) M), glutaraldehyde (0.5%), and formaldehyde (1%; CGF). CGF-fixated specimens are decalcified in an aqueous solution of 10% di-sodium ethylene-diaminotetraacetate (EDTA) neutralized by tris[hydroxy]methylaminomethane and embedded in paraffin. Tissue prepared in this manner allows histochemical detection of naphthol AS-D chloroacetate esterase in the neutrophilic cell line and in tissue mast cells, tartrate-resistant acid phosphatase in hairy cells and certain other low malignant B-cell lymphomas, in Gaucher cells, and in osteoclasts, and a specific platelet esterase in megakaryocytes and leukaemic megakaryoblasts. A broad panel of antigens is well preserved. Beside haemosiderin, cytosolic ferritin can be detected by Perls' reaction in acute phase-stimulated macrophages. Emphasis is placed on the diagnostic impact of plasma cell siderosis and lysosomal sideroblastocytosis in haemochromatosis and in alcoholism respectively. A technique is presented to discriminate mineralized and non-mineralized bone even after decalcification.
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PMID:[Histological processing of iliac crest biopsies based on decalcification and paraffin embedding with reference to osteolytic and hematologic diagnosis]. 788 10

Female Wistar rats with slight iron deficiency anemia were kept on a diet containing 0.5% trimethylhexanoyl (TMH)-ferrocene for up to 79 weeks. In the state of iron deficiency, the heart was free of light-microscopically detectable iron. After 7 weeks of the TMH-ferrocene diet, the first iron-positive granules appeared in perivascular macrophages. Further oral administration caused a progression of iron deposition in these cells, visible in the form of a granular staining but also as a diffuse iron staining of the cytoplasm. Accordingly, at the electron-microscopical level, the iron was stored partly as free ferritin molecules in the cytosol, and partly in lysosomes in the form of ferritin and/or hemosiderin. After 11 weeks, further iron-positive cells with relatively small dark-blue granules were found in the vicinity of capillaries, which could be identified as fibrocytes by means of electron microscopy. In addition, slight iron deposition occurred in the endothelial cells of the cardiac capillaries, likewise mainly in the form of small, uniform siderosomes. The myocytes showed no product of Perls' Prussian blue reaction during the whole period of investigation. From the 11th week onwards, discrete ferritin molecules were detected electron microscopically within lysosomes of these cells. Their amount increased slowly with progression of the TMH-ferrocene feeding period. Free ferritin molecules could be observed in the cytosol of fibrocytes, endothelial cells and myocytes in only very slight concentrations, whilst they were more plentiful in macrophages. In hereditary hemochromatosis and posttransfusional siderosis, the iron is found predominantly in myocytes and appears to cause cell damage, whilst this is not the case in experimental iron overload in rats.
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PMID:Pattern of iron storage in the rat heart following iron overloading with trimethylhexanoyl-ferrocene. 797 87

Superficial hemosiderosis (SH) of the CNS is a rare disease caused by repeated subarachnoid hemorrhage, with progressive superficial siderosis of the CNS. We report a patient with SH whose clinical picture was marked by progressive gait ataxia, hearing loss, dysarthria, and recurrent episodes of hemifacial spasm. Iron and ferritin levels in the CSF were significantly higher than in a control group of patients. Six month's treatment with the iron-chelating agent trientine dihydrochloride led to clinical improvement, with a concomitant reduction of CSF iron level. We suggest that, in addition to magnetic resonance imaging findings, CSF levels of iron and ferritin should be used as diagnostic criteria for SH, as well as to estimate the efficacy of iron chelation treatment.
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PMID:Superficial hemosiderosis of the central nervous system. 855 30

Subjects with thalassemia major frequently have bone disorders of debatable pathogenesis. We attempt here to analyze the relationships between siderosis and thalassemic osteodystrophy by assessing calcium-phosphorus balance, hormone-vitamin homeostasis, osteoblastic-osteoclastic activity parameters, and bone mineral density (BMD) in 30 patients with thalassemia major (16 males, 14 females, age range 17-30 years). We found a significant increase in ferritin (p < 0.001) and significant decreases in serum i-PTH, 25OHD3, 1.25(OH)2D3, osteocalcin, estradiol, testosterone and FT4 (p < 0.001) in both sexes. In all patients a net decrease of bone mineral density was documented (p < 0.001). These results were then submitted to linear regression analysis: positive correlations between BMD and FT3, testosterone, estradiol (p < 0.01), were documented, and an inverse correlation between osteocalcin and ferritin was confirmed. Our findings suggest that thalassemic osteodystrophy is the result of several inhibitory influences on osteoblastic activity and bone apposition (related to hormone deficits and siderosis) which are aggravated further by anemia, chronic hypoxia and red marrow expansion.
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PMID:[Osteodystrophy in thalassemia major]. 816 77

The present investigation evaluated the serum transferrin receptor concentration in subjects with nontransfusional iron overload who were identified in two separate studies on the basis of a serum ferritin level above 400 micrograms/L. Subjects with preclinical hereditary hemochromatosis were evaluated in the first study and those with the African form of iron overload in the second. In the first study, hereditary hemochromatosis was identified in 14 white men on the basis of a persistent elevation in transferrin saturation above 55%. The serum receptor concentration was elevated above the upper cut-off of 8.5 mg/L in two of the subjects, but the mean receptor of 6.1 +/- 1.4 mg/L (mean +/- 2 SE) did not differ significantly from the normal mean for this assay of 5.6 +/- 0.3 mg/L. In the same study, 60 control subjects with secondary iron overload were identified on the basis of a serum ferritin persistently above 400 micrograms/L, with a normal serum C-reactive protein concentration but with a transferrin saturation < 55%. Three of these subjects had an elevated serum receptor concentration but the mean value of 5.5 +/- 0.4 mg/L did not differ from normals nor from subjects with hemochromatosis. In the second study, 49 black Africans with iron overload were divided into those with or without an elevated transferrin saturation. The mean serum receptor concentration of 5.0 +/- 0.8 mg/L and 4.5 +/- 0.4 mg/L, respectively, did not differ statistically. It was concluded that there is no evidence of generalized dysregulation of the transferrin receptor in hemochromatosis or African siderosis.
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PMID:Serum transferrin receptor in hereditary hemochromatosis and African siderosis. 817 99

In advanced cases of superficial siderosis of the human central nervous system, the clinical triad of hearing loss, cerebellar ataxia, and myelopathy permits the diagnosis at the bedside, and magnetic resonance imaging readily confirms the hemosiderin deposits in brainstem, cerebellum, and spinal cord. To study the pathogenesis of this condition and explain the selective vulnerability of the cerebellum, experimental siderosis was induced in rabbits by the repeated intracisternal injection of autologous red blood cells. The earliest cellular response in the cerebellar molecular layer was hyperplasia and hypertrophy of microglia as displayed by immunocytochemistry for ferritin. Microglia also contained iron, but ferritin biosynthesis appeared to proceed without commensurate iron accumulation. This early apoferritin response probably occurred due to the presence of heme, rather than iron, in the cerebrospinal fluid and subpial tissue. Ferritin biosynthesis is accelerated when the ferritin repressor protein is dissociated from ferritin messenger ribonucleic acid. A specific antiserum localized ferritin repressor protein predominantly to astrocytes including Bergmann glia. It is proposed that abundance and proximity of ferritin repressor protein--immunoreactive Bergmann glia and ferritin-containing microglia in the cerebellar molecular layer permit prompt cellular interaction in the conversion of heme to ferritin and ultimately hemosiderin.
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PMID:The pathogenesis of superficial siderosis of the central nervous system. 823 56


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