SEARCH · PubMed Health

Results for “SIDEROSIS”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 667 records · Page 37Linked to original sources

PubMed2006

Alpha 1-antitrypsin mutations in NAFLD: high prevalence and association with altered iron metabolism but not with liver damage.

Hyperferritinemia, a common feature of nonalcoholic fatty liver disease (NAFLD), has been associated with steatohepatitis and fibrosis. Heterozygosity for alpha 1-antitrypsin (AAT) mutations is a cofactor of liver damage, and AAT influences inflammation and iron metabolism. This study evaluated the prevalence of the common AAT PiS/PiZ mutants in 353 patients with NAFLD, 195 of whom had hyperferritinemia, versus 114 matched controls and their influence on iron metabolism and the severity of liver damage in the 212 patients submitted to biopsy. PiS and PiZ alleles were searched for by restriction analysis. Thirty-eight patients (10.8%) carried non-MM genotypes versus 4/114 (3.5%) controls (P = .02). Patients carrying AAT mutations had higher ferritin (573 [454-966] vs. 348 [201-648]; P = .001) with similar transferrin saturation. The difference was more evident in males (P < .0001) and significant in patients not carrying HFE genotypes associated with iron overload (P = .015). The prevalence of non-MM genotypes was higher in patients with hyperferritinemia than in those without (28/195, 14% vs. 10/158, 6%, P = .016), and AAT mutations were associated with higher prevalence of sinusoidal siderosis (17/27, 63% vs. 70/180, 39%; P = .02), and sinusoidal/total iron score (46.3 +/- 38% vs. 25.1 +/- 35%, P = .01). Although ferritin was independently associated with fibrosis (P = .047), AAT mutations favoring sinusoidal iron deposition did not affect liver damage. In conclusion, AAT mutations are associated with hyperferritinemia and sinusoidal iron accumulation, but not with more severe liver damage in NAFLD.

Alleles↗

PubMed1976

Iron storage in macrophages and endothelial cells. Histochemistry, ultrastructure, and clinical significance.

1 hour after i. v. infusion of colloidal iron in iron deficient subjects uniform phagosomal iron granules were observed in macrophages and endothelial cells of several organs. 7 to 10 days later transformation into ferritin coould be visualized in macrophages only. Now, these cells showed diffuse iron staining of the cytoplasm due to dispersed ferritin molecules. Polymorphous lysosomes contained densely packed particles from still unchanged ferric hydroxide to paracristalline ferritin. The macrophageal iron was mobilizable in few days to several weeks. The univorm lysosomal iron granules of endothelial cells disappeared after 1 to 2 years. Endothelial iron siderosis without previous i. v. iron application was a frequent finding in pernicious anaemia and iron overload of diverse origin.

Anemia, Hypochromic↗

PubMedDate not supplied

Procollagen-type III peptide serum concentrations in alcoholic and non-alcoholic liver disease.

The type III procollagen aminopeptide (sPIIIP) serum levels were measured in 197 patients with liver disease and were correlated with morphological and serological alterations and with alcohol drinking habits. The sPIIIP levels resulted significantly increased in 51% of 43 patients with untreated chronic active hepatitis (CAH), in 61% of 36 patients with CAH plus cirrhosis, in 69% of 26 patients with inactive cirrhosis, in 4 out of 8 patients with alcoholic steatosis and fibrillogenesis, but remained unchanged in 38 cases of alcoholic steatosis plus siderosis and in 13 cases of chronic persistent hepatitis. A correlation between sPIIIP levels and the histological pattern of fibrosis could not be demonstrated in a single type of fibrotic liver disease and no differences were found between alcoholic and non-alcoholic patients. We agree upon the opinion that high sPIIIP levels may identify liver fibrogenic activity, but this test needs further technical improvements before it could be widely used in the clinical practice.

Adult↗

PubMed2002

[In memory of the 160(th) birthday and the 80(th) anniversary of the death of Heinrich Irenäus Quincke, as well as of his description of angioedema 120 years ago].

In the year 2002 we celebrate the 160(th) birthday and the 80(th) anniversary of the death of Heinrich Irenäus Quincke, former head of the department of internal medicine at the University of Kiel from 1878 to 1908. Moreover we remember the description of the angioedema by Quincke in the "Monatsheften für Praktische Dermatologie" 120 years ago. Because his name is so tightly linked with angioedema, Quincke's other discoveries and achievements are scarcely recalled today. Some of his other accomplishments include the invention of the lumbar puncture, the initiation of the term siderosis, the study of the resorption of inorganic iron to treat iron-deficiency anemia and his early contributions to pulmonary surgery. Quincke was the first to describe the causative organism of animal favus, which today is known as Trichophyton quinckeanum. Quincke suspected a syphilitic infection as the cause of aortic aneurysms and recommended antisyphilitic therapy. In the beginning of his career in Kiel, Quincke gave lectures not only in internal medicine but also in dermatology and venerology, pediatrics, bacteriology and public health, until chairs were established for these disciplines. Quincke was for four terms Dean of the medical faculty and in 1900 Rector of the University of Kiel.

Angioedema↗

PubMed1985

Molecular size heterogeneity of ferritin in mouse liver.

As much as 4% of the total protein in pure liver ferritin from mice with short-term parenteral iron overload produces a minor band migrating anodally to the major (alpha) band of holoferritin with non-denaturing polyacrylamide gel electrophoresis. The components in this minor band and the alpha band have been isolated to purity by preparative electrophoretic fractionation. The protein in the minor band is ferritin, since it contains ferric iron and fulfills defining criteria at the level of biochemistry, immunology and ultrastructure. Native polyacrylamide electrophoresis with pore-size-gradient gels shows that the ferritin molecules in the minor band have a slightly smaller diameter than the holoferritin in the alpha band. Isoelectric focusing reveals that the smaller ferritin has an identical number and range of charge isomers (pI 4.9-5.3) as the larger ferritin, but the relative amount of each size class within some isoferritin bands differs. The smaller ferritin molecules are structurally intact and are made from polypeptide subunits with Mr 18 000; the larger ferritin molecules have subunits with Mr 22 000. The minor species of hepatic ferritin thus has a smaller molecular size because it is made mainly from smaller subunits. No minor electrophoretic band can be detected in liver ferritin obtained from mice with normal iron levels. These results demonstrate that siderosis induces the formation of molecular size polymorphism (macroheterogeneity) in mouse liver ferritin. The new smaller hepatic ferritin could serve to redistribute excess iron into the main storage organs during the early response to iron overload, since it appears to be identical to one of the two types of serum ferritin molecules present in these siderotic mice.

Animals↗

PubMed1980

Clinical trial of desferrioxamine entrapped in red cell ghosts.

Ten patients with excess body iron stores of different levels and with different diagnoses were given desferrioxamine inside osmotically lysed and resealed red blood cells (red cell ghosts). Red cell ghost entrapped desferrioxamine was given intravenously and, ten days later, an equal dose of desferrioxamine was administered via slow subcutaneous infusion over 10 h. Differences were apparent for these single dose challenges with respect to the pattern of urine iron excretion and the efficiency of iron chelation. The red cell ghost method consistently improved the efficiency of chelation, especially in patients with modestly increased body iron stores. This method of treatment may therefore prove to be of particular value in the patient with early siderosis who is embarking on a programme of regular transfusion for refractory anaemia.

Adult↗

PubMed1979

Iron negative foci and nodules in safrole-exposed mouse liver made siderotic by iron-dextran injection.

A procedure for the production of mouse hepatic siderosis is described which results in extensive iron deposition in all lobular zones. Mice exposed to safrole for 24 weeks displayed basophilic and acidophilic foci which did not accumulate iron. 36 weeks of dietary safrole exposure resulted in nodular lesions comprised of basophilic and hyalinized cells. The nodules displayed decreased or negative reactions for iron in hepatic parenchymal cells when the surrounding liver was siderotic.

Animals↗

PubMed1987

Siderosomal ferritin. The missing link between ferritin and haemosiderin?

A minor electrophoretically fast component was found in ferritin from iron-loaded rat liver in addition to a major electrophoretically slow ferritin similar to that observed in control rats. The electrophoretically fast ferritin showed immunological identity with the slow component, but on electrophoresis in SDS it gave a peptide of 17.3 kDa, in contrast with the electrophoretically slow ferritin, which gave a major band corresponding to the L-subunit (20.7 kDa). Thus the electrophoretically fast ferritin resembles that reported by Massover [(1985) Biochim. Biophys. Acta 829, 377-386] in livers of mice with short-term parenteral iron overload. The electrophoretically fast ferritin had a lower iron content (2000 Fe atoms/molecule) than the electrophoretically slow ferritin (3000 Fe atoms/molecule). Removal and re-incorporation of iron was possible without effect on the electrophoretic mobility of either ferritin species. On subcellular fractionation the electrophoretically fast ferritin was enriched in pellet fractions and was the sole soluble ferritin isolated from iron-laden secondary lysosomes (siderosomes). The amount and relative proportion of the electrophoretically fast species increased with iron loading. Haemosiderin isolated from siderosomes was found to contain a peptide reactive to anti-ferritin serum and corresponding to the 17.3 kDa peptide of the electrophoretically fast ferritin species. Unlike the electrophoretically slow ferritin, the electrophoretically fast ferritin did not become significantly radioactive in a 1 h biosynthetic labelling experiment. We conclude that the minor ferritin is not, as has been suggested for mouse liver ferritin, 'a completely new species of smaller holoferritin that represents a shift in the ferritin phenotype' in response to siderosis, but a precursor of haemosiderin, in agreement with the proposal by Richter [(1984) Lab. Invest. 50, 26-35] concerning siderosomal ferritin.

Animals↗

PubMed1977

Effects of hexachlorobenzene feeding and iron overload on enzymes of haem biosynthesis and cytochrome P 450 in rat liver.

1. The effect of hexachlorobenzene feeding on liver delta-aminolaevulinate synthase, uroporphyrinogen decarboxylase and cytochrome P 450 was studied at various time-intervals in siderotic and non-siderotic rats. 2 In the non-siderotic group hexachlorobenzene feeding led to a progress decrease in liver uroporphyrinogen decarboxylase activity, accompanied by a progressive increase in delta-aminolaevulinate synthase activity. Cytochrome P 450 concentrations were above normal throughout but fell toward the end of the experiment. 3. Similar but more marked changes were found in the siderotic animals. The fall in uroporphyrinogen decarboxylase activity occurred earlier and was significantly greater in these animals, whereas the increase in delta-aminolaevulinate synthase activity was consistently larger. Liver cytochrome P 450 concentration also rose but to a lesser extent than that in the non-siderotic rats. 4. Hexachlobenzene-induced porphyria would seem to be attributable to inhibition or inactivation of hepatic uroporphyrinogen decarboxylase. Hepatic siderosis has a synergistic effect with hexachlorobenzene on this enzyme and may exert additional effects by promoting cytochrome P 450 turnover.

5-Aminolevulinate Synthetase↗

PubMedDate not supplied

[Skeletal changes in thalassemia].

Display of this hereditary blood disease (haemoglobinopathy) which is endemic in the Mediterranean area and rarely appearing in our area, characterized by early blood cell destruction and increasing transfusion siderosis of internal and endocrine organs, secondary osteoporosis, epiphyseal growth disturbances and spontaneous fractures by means of two typical cases.

Adolescent↗

PubMedDate not supplied

Arc Welders' pneumoconiosis: application of advanced scanning electron microscopy.

Study of lung tissue from necropsy of a 58-year-old arc welder with arc welders' pneumoconiosis, confirmed by history, chest radiography, and pathology, demonstrates the versatility and usefulness of new techniques in scanning electron microscopy (SEM). Secondary electron imaging, the most familiar SEM mode, showed heavy cellular infiltrates in alveoli, the interstitium, and within the interstices of loose whorled fibrotic nodules. Backscattered electron imaging, in which contrast is proportional to elemental atomic number, revealed intracellular metal particles not otherwise visible. Microprobe analysis, energy-dispersive x-ray spectrometry, mapped elemeental iron over the particle image and identified traces of silicon in the whorled nodules. Arc welders' pneumoconiosis appears to be more than a benign siderosis resulting from particulate iron deposition. Simultaneous exposure to other components of welding fumes may alter the pathologic picture, inducing a more complicated fibrotic reaction. The more recently developed advanced techniques of SEM are well suited to the study of pneumoconioses and other problems of heterogenous tissue and mixed chemical systems.

Electron Probe Microanalysis↗

PubMed1977

Characterization of serum ferritin in iron overload: possible identity to natural apoferritin.

Serum ferritins from a patient with haemochromatosis and from a patient with transfusional siderosis were compared with tissue isoferritins on the basis of their iron content, isoferritin spectrum and immunological properties. Both serum ferritins had a low iron content and corresponded to only the most basic isoferritins in liver. The serum ferritins were very similar to the natural apoferritin from liver in all respects.

Apoferritins↗

PubMed1993

Iron status in red cell pyruvate kinase deficiency: study of Italian cases.

We investigated the iron status of 33 pyruvate kinase (PK) deficient patients, most of the cases reported in Italy. Serum ferritin (SF) was higher than the upper limit of the range of matched controls in 15/25 (60%) non-transfused patients (median 228 micrograms/l, range 58-3160 v 43, 22-310). Liver siderosis and fibrosis were found in 8/9, and cirrhosis in two who died at age 39 and 42 of complications of iron overload. SF was independent of age, sex, or severity of haemolysis. The prevalence of HLA-A3 antigen in PK deficient patients was not significantly different from that of our healthy population (29.6% v 23%). The HLA-A3 positive, non-transfused patients had significantly higher SF values than the HLA-A3 negative ones (median 675 micrograms/l, range 340-3160 v 145, 58-400). A pedigree study of six high SF-probands indicated that iron overload has a multifactorial pathogenesis. In particular, the association of PK deficiency-induced haemolysis, splenectomy and an additional factor (heterozygosity for idiopathic haemochromatosis, ineffective erythropoiesis) leads to severe iron accumulation. We suggest that monitoring iron status would be useful in PK deficient patients, particularly in splenectomized and HLA-A3 positive ones, to identify those at risk of iron overload and prevent the clinical consequences of iron accumulation.

Adolescent↗

PubMed1995

Pathology of the liver in leukaemia and lymphoma. A study of 110 autopsies.

Histopathological findings in the liver in a series of autopsies on 110 patients suffering from leukaemia or lymphoma were reviewed. No treatment had been given to 23 patients, 50 had received chemotherapy alone, 23 chemo- and radiotherapy and 14 underwent bone marrow transplantation. The 23 untreated patients showed neoplastic infiltration in 10 (44%) and fibrosis in 16 cases (70%). The 14 bone marrow recipients showed mainly acute necrosis, zonal in six (43%) and focal in one (7%), and cholestasis in five (36%), together with marked siderosis in 11 (79%). Patients receiving chemotherapy or chemo-/radiotherapy showed similar changes consisting of neoplastic infiltration in 14 (28%) and in five (22%) cases respectively, and fibrosis in 31 (62%) and 17 (74%) cases respectively. Steatosis was seen in 70% of cases in the chemo-/radiotherapy group and in 50% of cases in the other groups. Infections were seen in all groups, particularly in the chemotherapy group (four fungal and two bacterial including one tuberculosis) and in the chemo-/radiotherapy group (one fungal and one bacterial). Cytomegalovirus infection was seen only in the bone marrow transplant group.

Adolescent↗

PubMed1993

Prevention of heart disease by subcutaneous desferrioxamine in patients with thalassaemia major.

BACKGROUND: Cardiac siderosis from transfused iron remains the major cause of death in thalassaemia major, despite iron chelation therapy with desferrioxamine. AIMS: Our aim was to determine the nature and extent of cardiac involvement and its relationship to desferrioxamine use in a group with thalassaemia major. METHODS: We reviewed 76 patients with thalassaemia major and performed multiple logistic regression to analyse factors affecting cardiac involvement. Factors studied included: patient sex, age, haemoglobin, serum ferritin, total transfusions, liver iron, duration of desferrioxamine use, electrocardiograms, echocardiograms and compliance to desferrioxamine treatment. RESULTS: Thirty-seven patients developed heart disease. They were older (p < 0.001), began desferrioxamine later (p < 0.001), had more liver iron (p = 0.014), higher serum ferritin levels (p = 0.023) and received more blood (p = 0.018). Compared to those with optimal compliance the odds of developing heart disease were increased 10.7 times in fair compliers (p < 0.001) and 5.1 times in poor compliers (p = 0.016). However, there was no significant difference between those with fair and poor compliance. After multivariate analysis only compliance (p = 0.02) and age at desferrioxamine onset (p = 0.004) remained significant. Compliance was inversely related to liver iron (p < 0.001), serum ferritin (p < 0.001) and age at desferrioxamine commencement (p < 0.001). CONCLUSIONS: We conclude that late commencement of desferrioxamine and noncompliance are associated with greater iron loading and an increased risk of heart disease.

Adolescent↗

PubMed1975

Immunoradiometric assay for ferritin in human serum.

A sensitive, specific and precise immunoradiometric assay for ferritin has been developed. Ferritin was measured in the serum of 160 hospital controls, 101 females (118 +/- 9 mug/l) and 59 males (189 +/- 16 mug/l). This difference was statistically significant. In 28 patients with untreated iron deficiency anaemia, serum ferritin concentration (6.1 +/- 0.7 mug/l) was significantly lower than in the controls, but it was within the normal range in 14 cases of polycythaemia vera treated by repeated phlebotomy. In 4 patients with primary haemachromatosis (2884 +/- 56 mug/l), 25 with secondary iron overload states (5702 +/- 1235 mug/l) and 8 with haemolytic anaemia (1612 +/- 605 mug/l), serum ferritin levels were markedly elevated. In 14 cases of transfusional siderosis there was a highly significant correlation between serum ferritin concentration and units of blood transfused. A circadian rhythm in serum ferritin concentration was observed in 7 healthy subjects.

Anemia, Aplastic↗

PubMed1986

Monocyte ferritin in idiopathic haemochromatosis, thalassaemia and liver disease.

Monocyte ferritin (MF) content was measured in normal subjects and patients with a variety of disorders of iron storage. MF was above the normal range in 4 patients with idiopathic haemochromatosis (IHC). However, in 4 patients with transfusion siderosis (TS), secondary to aplastic anaemia, who had similar elevations in serum ferritin, MF was highly elevated. 10 patients with thalassaemia intermedia and thalassaemia major with no previous history of transfusions, but with elevated serum ferritin, also had significantly elevated MF. Disproportionately low MF in IHC could reflect defective ferritin metabolism in reticuloendothelial cells in this disorder. Finally, in 3 patients with acute rises in serum ferritin caused by acute hepatitis, MF was not increased. This suggests that MF is not directly affected by high circulating levels of serum ferritin raised acutely, but rather reflects iron storage status in conditions not associated with primary disorders of iron metabolism.

Acute Disease↗

PubMed1994

Bone marrow transplantation in thalassemia: modifications of hepatic iron overload and associated lesions after long-term engrafting.

Among 450 thalassemic patients treated in the Hematologic Department, 50 patients who were disease-free 4-6 years after allogeneic bone marrow transplantation were sequentially studied by liver biopsy. The patients received marrow from siblings who were genotypically HLA identical at A, B, C and DR loci. For evaluation of siderosis and associated lesions, each patient underwent liver biopsy before, and again 6 months and yearly for 4 to 6 years after bone marrow transplant. Spontaneous reversibility of liver iron overload, once the need for transfusions ceased when a functioning graft had been established, was observed in the youngest patients, aged 1-8 years, whereas iron excess remained at the end of follow-up in many patients aged 9-15 years. Hypotheses about the mechanism of the iron decrease are discussed. Several cases also obtained improvement of associated pathologies such as hepatitis, probably through modifications in the mechanisms controlling their immunological status.

Adolescent↗