Haemochromatosis

2011 ◽  
pp. 1684-1694
Author(s):  
K.J. Allen
Keyword(s):  
Hepatocellular Carcinoma ◽  
Life Expectancy ◽  
Iron Metabolism ◽  
Cardiac Failure ◽  
Iron Absorption ◽  
Iron Storage ◽  
Hereditary Haemochromatosis ◽  
Iron Stores ◽  
Excess Iron ◽  
Progressive Accumulation

Hereditary haemochromatosis is an inherited iron storage disorder in which altered iron metabolism leads to an increase in intestinal iron absorption. This results in a progressive accumulation of body iron stores particularly in the liver, heart, pancreas, and pituitary. The excess iron deposited in tissues may result in cirrhosis, diabetes, cardiac failure and arrhythmias, hypogonadism, arthritis, hepatocellular carcinoma, and a shortened life expectancy.

scholarly journals Iron age: novel targets for iron overload

Hematology ◽  
2014 ◽  
Vol 2014 (1) ◽  
pp. 216-221 ◽  
Author(s):  
Carla Casu ◽  
Stefano Rivella
Keyword(s):  
Iron Overload ◽  
Iron Age ◽  
Iron Metabolism ◽  
Iron Absorption ◽  
Hereditary Hemochromatosis ◽  
Therapeutic Approaches ◽  
Hepcidin Expression ◽  
Beneficial Effects ◽  
Excess Iron ◽  
Major Factors

Abstract Excess iron deposition in vital organs is the main cause of morbidity and mortality in patients affected by β-thalassemia and hereditary hemochromatosis. In both disorders, inappropriately low levels of the liver hormone hepcidin are responsible for the increased iron absorption, leading to toxic iron accumulation in many organs. Several studies have shown that targeting iron absorption could be beneficial in reducing or preventing iron overload in these 2 disorders, with promising preclinical data. New approaches target Tmprss6, the main suppressor of hepcidin expression, or use minihepcidins, small peptide hepcidin agonists. Additional strategies in β-thalassemia are showing beneficial effects in ameliorating ineffective erythropoiesis and anemia. Due to the suppressive nature of the erythropoiesis on hepcidin expression, these approaches are also showing beneficial effects on iron metabolism. The goal of this review is to discuss the major factors controlling iron metabolism and erythropoiesis and to discuss potential novel therapeutic approaches to reduce or prevent iron overload in these 2 disorders and ameliorate anemia in β-thalassemia.

scholarly journals Editorial Review: The Control of Iron Balance by the Intestinal Mucosa

Blood ◽  
1963 ◽  
Vol 22 (4) ◽  
pp. 441-449 ◽  
Author(s):  
WILLIAM H. CROSBY
Keyword(s):  
Epithelial Cells ◽  
Binding Capacity ◽  
The Body ◽  
Dietary Iron ◽  
Iron Storage ◽  
Iron Stores ◽  
Excess Iron ◽  
Internal Surfaces ◽  
Editorial Review ◽  
Iron Binding Capacity

Abstract In this hypothetical model of intestinal function the ferritin apparatus of the absorptive epithelial cells provides the most important mechanism for day-to-day control of iron balance. Iron incorporated into epithelial ferritin cannot be released and therefore is lost when the cell is lost at the end of the life cycle. Information concerning the state of the body’s iron stores is conveyed to the intestine, perhaps by the concentration of plasma iron or iron-binding capacity. When the iron stores are replete the newly forming epithelial cells are constructed to contain a functional ferritin apparatus capable of synthesizing apoferritin and incorporating iron into ferritin, thus preventing its entering the body. However, this mechanism is not completely adequate to intercept all unneeded iron. When too much iron has bypassed the ferritin apparatus it may be intercepted by tissue macrophages in the villus and lost when these macrophages work their way into the lumen; or it may be loaded into newly forming villous epithelial cells thereby saturating a part of their ferritin capacity. Cells thus loaded would develop increased resistance to the incursion of dietary iron; they would also carry the ferritin iron away with them at the end of their life span. In iron deficiency the absorptive cells are constructed with a relative lack of ferritin apparatus so that dietary iron can pass freely into the cell and thence freely into the body. In iron storage disease other populations of cells are used for excretion of iron. In general these are the glandular epithelial cells and tissue macrophages. Both varieties of cells are able to accept and store relatively large amounts of nonfunctional iron. They are deciduous cells which are shed from the internal surfaces of the body, and when they go their iron goes with them. The capacity of the excretory mechanism is not great, perhaps about 5 mg. per day. When the rate of introduction of iron into the body exceeds this capacity, siderosis of parenchymal tissues develops due to accumulation of excess iron.

scholarly journals Distinct Effects of Escherichia coli,Pseudomonas aeruginosa and Staphylococcus aureus Cell Wall Component-Induced Inflammation on the Iron Metabolism of THP-1 Cells

2021 ◽  
Vol 22 (3) ◽  
pp. 1497
Author(s):  
Edina Pandur ◽  
Kitti Tamási ◽  
Ramóna Pap ◽  
Gergely Jánosa ◽  
Katalin Sipos
Keyword(s):  
Cell Wall ◽  
Inflammatory Cytokines ◽  
Iron Metabolism ◽  
Inflammatory Cytokine ◽  
Cytokine Production ◽  
Storage Proteins ◽  
Iron Storage ◽  
E Coli ◽  
Iron Storage Proteins ◽  
Pro Inflammatory Cytokines

Macrophages are essential immune cells of the innate immune system. They participate in the development and regulation of inflammation. Macrophages play a fundamental role in fighting against bacterial infections by phagocytosis of bacteria, and they also have a specific role in immunomodulation by secreting pro-inflammatory cytokines. In bacterial infection, macrophages decrease the serum iron concentration by removing iron from the blood, acting as one of the most important regulatory cells of iron homeostasis. We examined whether the Gram-positive and Gram-negative cell wall components from various bacterial strains affect the cytokine production and iron transport, storage and utilization of THP-1 monocytes in different ways. We found that S. aureus lipoteichoic acid (LTA) was less effective in activating pro-inflammatory cytokine expression that may related to its effect on fractalkine production. LTA-treated cells increased iron uptake through divalent metal transporter-1, but did not elevate the expression of cytosolic and mitochondrial iron storage proteins, suggesting that the cells maintained iron efflux via the ferroportin iron exporter. E. coli and P. aeruginosa lipopolysaccharides (LPSs) acted similarly on THP-1 cells, but the rates of the alterations of the examined proteins were different. E. coli LPS was more effective in increasing the pro-inflammatory cytokine production, meanwhile it caused less dramatic alterations in iron metabolism. P. aeruginosa LPS-treated cells produced a smaller amount of pro-inflammatory cytokines, but caused remarkable elevation of both cytosolic and mitochondrial iron storage proteins and intracellular iron content compared to E. coli LPS. These results prove that LPS molecules from different bacterial sources alter diverse molecular mechanisms in macrophages that prepossess the outcome of the bacterial infection.

Effect of ascorbic acid on apparent iron absorption by women with low iron stores

1994 ◽  
Vol 59 (6) ◽  
pp. 1381-1385 ◽  
Author(s):  
J R Hunt ◽  
S K Gallagher ◽  
L K Johnson
Keyword(s):  
Ascorbic Acid ◽  
Iron Absorption ◽  
Iron Stores

Iron absorption of ferric glycinate is controlled by iron stores

1998 ◽  
Vol 18 (1) ◽  
pp. 3-9 ◽  
Author(s):  
Fernando Pizarro ◽  
Raúl Uicich ◽  
Manuel Olivares ◽  
Carlos Almeida ◽  
MaráL. Díaz ◽  
...  
Keyword(s):  
Iron Absorption ◽  
Iron Stores
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