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At least 19 recordsLinked to original sources
PubMed1978

Ferritin in bone marrow and serum in iron deficiency and iron overload.

Nonheme iron and ferritin in the bone marrow and serum ferritin was investigated in patients with iron deficiency anaemia or iron overload. As controls served patients without any disturbance of the iron metabolism. There is a precise correlation between the nonheme iron and ferritin in the bone marrow of patients with and without disturbance of iron metabolism. A correlation was also found between the ferritin in the bone marrow and the serum. Nonheme iron and ferritin in the bone marrow and serum ferritin was decreased in patients with iron deficiency anaemia. Conversely, the same parameters were increased in patients with iron overload.

Anemia, Hypochromic↗
PubMed1979

Iron overload despite partial gastrectomy.

Iron overload was found in 3 patients who had undergone partial gastrectomy: a 61-year-old woman developed iron overload because she may have had idiopathic haemochromatosis and had also been given parenteral iron; in a 62-year-old man with thalassaemia minor, iron overload may have developed because of increased oral iron ingestion, low serum folate, increased, albeit ineffective, erythropoiesis and sideroblastic anaemia; a 74-year-old man with thalassaemia minor developed iron overload without exogenous therapy and died from a hepatoma. These cases illustrate that partial gastrectomy fails to protect patients from developing iron overload, particularly if given uncontrolled iron therapy.

Aged↗
PubMed1979

Induction of liver cell haem oxygenase in iron-overloaded rats.

Rats were chronically iron-overloaded by intraperitonel injections of iron-dextran. Electron microscopy revealed that the excess iron was deposited in ferritin-like particles packed in lysosomes and scattered in hepatic cytoplasm. No mitochondrial iron deposition or damage was seen. Furthermore, mitochondrial preparations from chronically iron-overloaded animals were found to be contaminated with lysosomes, which could explain previously reported increases in mitochondrial iron by chemical analysis. Mitochondrial function, as measured by cytochromes a-a3, b and c concentrations as well as activity of the rate-limiting enzyme of haem synthesis, delta-aminolaevulinate synthetase, was not diminished by chronic iron-overloading. Microsomal haem was decreased by 30% at the time that haem oxygenase, the rate-limiting enzyme of haem degradation, was increased approx. 3-fold. Animals were given a single intraperitoneal injection of iron-dextran and the activities of delta-aminolaevulinate synthetase and haem oxygenase were measured over 24 h. delta-Aminolaevulinate synthetase activity increased approx. 2-fold in these acutely iron-overloaded rat livers, but at a time after the increase in haem oxygenase. These results suggest that an early consequence of excess iron in liver is acceleration of the rate of haem degradation, possible by haem oxygenase.

5-Aminolevulinate Synthetase↗
PubMed1978

Deferoxamine-chelatable iron in hemochromatosis and other disorders of iron overload.

Deferoxamine-chelatable iron was measured in 103 patients with known or suspected iron overload. All of 34 patients with untreated hemochromatosis had distinctly elevated values for deferoxamine-chelatable iron. The mean value in these cases was significantly greater than that in patients with cirrhosis, who had little or no stainable hepatic iron. In 15 patients with hemochromatosis who were tested sequentially during the course of phlebotomy therapy, deferoxamine-chelatable iron proved a reliable index of the degree of reduction of storage iron. In 22 additional patients with partially treated hemochromatosis and 14 with iron overload accompanying chronic anemia, this test correlated well with the magnitude of iron deposits in liver or bone marrow. In patients with unexplained elevations of serum iron, normal or only slightly elevated deferoxamine-chelatable iron correctly indicated that storage (hepatic) iron was not excessive. The test was more reliable than determination of serum iron or transferrin saturation as an indicator of increased storage iron. Elevated values could not be attributed to disturbed liver function. Determination of deferoxamine-chelatable iron is a safe, practical, and useful procedure for identifying persons with increased iron stores and for assessing the effect of phlebotomy therapy.

Adult↗
PubMed1978

Diagnostic efficacy of tests for the detection of iron overload in chronic liver disease.

The value of tests for the detection of body iron overload was investigated in 8 patients with clinically manifest primary hemochromatosis, 12 patients with cirrhosis and iron overload and 20 patients with liver disease and low or normal iron stores. Iron overload was defined as the presence of stainable iron in more than 50% of hepatocytes in a liver biopsy specimen. The percentages of patients with a true-positive (abnormal) or true-negative (normal) result were: serum iron concentration 65%, transferin saturation 85%, serum ferritin concentration 78%, serum ferritin:serum glutamic oxaloacetic transaminase (SGOT) index 78%, percent iron absorption 58%, percent iron absorption in relation to serum ferritin concentration 80% and percent iron absorption in relation to serum ferritin:SGOT index 93%. The calculated predictive value of a normal test result for the exclusion of iron overload in patients with liver disease, a group with an assumed prevalence of iron overload of 10%, was 98% to 99% for transferrin saturation and serum ferritin concentration used alone and 100% for these measures used together; the predictive value of an abnormal result for the diagnosis of iron overload was less than 50% for all of the above measures used alone or in combination. Hence, in patients with an increased serum ferritin concentration or transferrin saturation, or both, determination of the hepatocellular iron content of a specimen from a percutaneous liver biopsy is required for the diagnosis of iron overload.

Adolescent↗
PubMed1978

Studies on the rat liver following iron overload. 1. Fine structural appearance.

Iron overload of the rat liver following parenteral administration of Jectofer (an iron sorbitol citric acid complex) was studied in the electron microscope. Abundant ferritin-like granules were present in parenchymal and Kupffer cells, partly free in the cell sap and partly concentrated in 3 types of membrane-bound organelles, with characteristic appearances. In the parenchymal cells these organelles consisted of lysosome-like structures, apparent autophagic vacuoles, and vacuoles lacking features linking them to specific cytoplasmic elements. Organelle-bound ferritinlike granules in the Kupffer cells were demonstrated in lysosomelike structures, in phagocytic vacuoles, and in tubular and vacuolar elements referred to as "type 1" and "type 2" bodies. No ferritin-like granules were observed in other cell types than parenchymal and Kupffer cells.

Adipose Tissue↗
PubMed1976

Subcutaneous infusion and intramuscular injection of desferrioxamine in patients with transfusional iron overload.

The effects of intramuscular injection and subcutaneous infusion of desferrioxamine (D.F.) on urinary iron excretion were compared in eleven patients with thalassaemia major and one with congenital sideroblastic anaemia who were being maintained on regular blood-transfusions. Total (48-hour) urinary iron excretion ranged from 3-3 to 40-3 mg (mean 16-3 mg) in nine patients who received 750 mg D.F. intramuscularly before transfusion and from 3-9 to 32-3 mg (mean 11-9 mg) in ten patients who received D.F. by the same route after transfusion. In all 9 patients studied before transfusion, continuous subcutaneous infusion of 750 mg D.F. over 24 hours increased iron excretion by 61-5 to 135-8% (mean 101+/-25-4 S.D.%) compared with intramuscular injection of a similar dose. In the 10 patients studied after transfusion, the iron excretion produced by continuous subcutaneous infusion was from 18-9 to 213% (mean 128+/-74-3%) more than that produced by a single intramuscular injection of D.F. When the subcutaneous dose over 24 hours was increased to 1500 mg in six patients, 48-hour iron excretion ranged from 29-2 to 81-2 mg (mean 52-4 mg) and was increased by 80-2--794% (mean 429%) compared with the excretion when 750 mg was given by intramuscular injection. It is concluded that continuous subcutaneous infusion of D.F. produces more iron excretion in patients with iron overload than intramuscular injection. Providing a suitable portable pump can be carried by the patients, continuous subcutaneous infusion of desferrioxamine may prove a valuable means of preventing or treating iron overload in anaemic patients maintained on regular transfusions.

Adolescent↗
PubMed1978

Hepatic sinusoidal cells in iron overload. Ultrastructural observations.

The ultrastructure of sinusoidal cells was studied in 18 liver biopsies from patients with homozygous beta-thalassemia. No conspicuous iron was seen in sinusoidal cells from biopsies obtained before transfusion therapy was initiated, although electron-dense iron was present in some parenchymal cells. Biopsies following the high transfusion regime showed progressive increase in the size and number of iron-containing lysosomes in both parenchymal and sinusoidal cells. This study confirms the view that transfusional iron is largely segregated in reticuloendothelial cells. Examination of stained and unstained sections showed the Kupffer cells and endothelial cells had different types of iron-containing lysosome that were also dissimilar from most iron-containing lysosomes seen in hepatocytes. The described cell-specific morphological features of the lysosomes facilitate the identification of various cells during iron overload. The importance of phagocytic sinusoidal cells during chronic iron overload is stressed.

Adolescent↗
PubMed1979

[Demonstration by iron overloading study and HLA genotyping of recessive transmission of idiopathic haemochromatosis in two pseudodominant pedigrees (author's transl)].

We studied iron overloading and HLA genotype in two families with overt forms of idiopathic haemochromatosis in two successive generations. In each family the spouse of the patient with overt haemochromatosis in the first generation had clinical and laboratory signs of moderate iron overload and a HLA haplotype A3, B14 and A3, B7 respectively--which is frequently associated with the haemochromatosis gene. This specific HLA haplotype had been transmitted to the second generation patient with overt disease, which thus could be considered as having received a haemochromatosis gene from each parent. Although the finding of cases of overt disease in successive generation firstly suggests a dominant transmission the genetical analysis of these families lead to further strong argument in favour of recessive inheritance of idiopathic haemochromatosis.

Adolescent↗
PubMed1979

Changing patterns of dietary iron overload in black South Africans.

Over the last 17 years there has been a significant reduction in the prevalence and severity of dietary iron overload in urban blacks of Johannesburg. This is attributed to a decrease in the consumption of traditionally brewed beer of high iron content over this period. A 40% reduction was found in hepatic iron concentrations measured in necropsy specimens from 248 male patients who died in 1976 as compared with 220 who died in 1959 and 1960. While hepatic iron concentrations rose with age in both groups there was no evidence of iron accumulation during the period between the two studies. Hepatic iron concentrations measured in 345 female subjects were many fold less than those of males and the 1976 group did not differ significantly from the 1959 to 1960 group. A paradoxical increase in the prevalence of portal fibrosis and cirrhosis was seen and may be due to the effects of increased ingestion of spirits and fortified wine in recent years. Iron overload was significantly greater in males with carcinoma of the esophagus and in those with idiopathic heart failure when compared to subjects who died of other causes. This suggests excessive exposure of these subjects to traditionally brewed beverages and the adulterants present in them.

Adult↗
PubMed1979

Improvement in iron status and liver function in patients with transfusional iron overload with long-term subcutaneous desferrioxamine.

Subcutaneous desferrioxamine (2--4 g over 12 h) was administered 6 nights each week to 34 patients with transfusional iron overloads who continued to receive regular blood-transfusions. All 34 patients showed a fall in serum-ferritin after 5 to 12 months. In some patients serum-ferritin fell almost to normal. Liver function improved in all the patients, serum-aspartate-transaminase levels fell in all 17 patients tested, and liver-iron fell in 5 of 6 patients tested. These studies show that body-iron stores can be substantially reduced, to normal or near normal levels, by long-term subcutaneous desferrioxamine in patients with transfusional iron overload despite the need for continued blood-transfusion. They also show that removal of iron is accompanied by improved organ function.

Adolescent↗
PubMed1978

Serum ferritin concentration: a reliable guide to iron overload in uremic and hemodialyzed patients.

The inter-relationships between serum ferritin, hemoglobin, serum iron and total body iron stores were studied in 20 patients with chronic renal failure treated conservatively and in 20 patients on regular hemodialysis. There was no relationship between serum iron or transferrin and bone marrow iron deposits, but serum ferritin concentration was a good indicator of increased marrow iron stores. All patients with serum ferritin levels above 300 microgram/l had increased iron stores. Serum ferritin assay is a useful non-invasive technique for detecting iron overload in uremic and hemodialyzed patients.

Adolescent↗
PubMed1976

Splenectomy, iron overload and liver cirrhosis in beta-thalassemia major.

In order to evaluate the interrelations of splenectomy, iron overload and cirrhosis, histologic specimens of liver and spleen were examined and correlated in 12 children with beta-thalassemia major. All patients had received blood transfusions since infancy. Correlations seemed to exist between splenic hemosiderosis and splenic weight, and between the latter and the age at time of splenectomy. All liver samples showed varying hemosiderosis, not correlated with the number of transfusions or the children's age. Irregular liver cirrhosis existed in three children, 7, 8, and 14 years after splenectomy. No cirrhosis existed in any of the children where the spleen was in situ. Splenectomy in children with thalassemia may carry the long-term risk of liver cirrhosis.

Adolescent↗
PubMed1977

[Iron overload: effect on specific and nonspecific immunity].

Acute overload with an iron salt in mice enhanced the virulence of a relatively avirulent type of Salmonella typhimurium in a dose-dependent fashion. Iron administration also partly abolished specific immunity. Ferritin iron did not display the virulence-enhancing effect.

Animals↗
PubMed1979

Synthesis and evaluation of the thiosemicarbazone, dithiocarbazonate, and 2'-pyrazinylhydrazone of pyrazinecarboxaldehyde as agents for the treatment of iron overload.

Three prototype tridentate ligands (i.e., pyrazinecarboxaldehyde thiosemicarbazone, sodium pyrazinecarboxaldehyde dithiocarbazonate, and pyrazinecarboxaldehyde 2'-pyrazinylhydrazone) were prepared and evaluated for their relative abilities to remove iron from model systems designed to mimic particular aspects of chronic transfusional iron overload. These compounds were synthesized by condensation of pyrazinecarboxaldehyde with the appropriate substituted hydrazide. Iron-binding properties were determined, and the ability to remove iron from the proteins transferrin and ferritin was ascertained. An in vivo model system employing iron-loaded mice was used to demonstrate that all three compounds were effective at reducing tissue iron levels.

Animals↗