An excessive accumulation of iron in the body due to a greater than normal absorption of iron from the gastrointestinal tract or from parenteral injection. This may arise from idiopathic hemochromatosis, excessive iron intake, chronic alcoholism, certain types of refractory anemia, or transfusional hemosiderosis. (From Churchill's Illustrated Medical Dictionary, 1989)
A metallic element with atomic symbol Fe, atomic number 26, and atomic weight 55.85. It is an essential constituent of HEMOGLOBINS; CYTOCHROMES; and IRON-BINDING PROTEINS. It plays a role in cellular redox reactions and in the transport of OXYGEN.
Organic chemicals that form two or more coordination links with an iron ion. Once coordination has occurred, the complex formed is called a chelate. The iron-binding porphyrin group of hemoglobin is an example of a metal chelate found in biological systems.
A disorder of iron metabolism characterized by a triad of HEMOSIDEROSIS; LIVER CIRRHOSIS; and DIABETES MELLITUS. It is caused by massive iron deposits in parenchymal cells that may develop after a prolonged increase of iron absorption. (Jablonski's Dictionary of Syndromes & Eponymic Diseases, 2d ed)
Iron-containing proteins that are widely distributed in animals, plants, and microorganisms. Their major function is to store IRON in a nontoxic bioavailable form. Each ferritin molecule consists of ferric iron in a hollow protein shell (APOFERRITINS) made of 24 subunits of various sequences depending on the species and tissue types.
Therapy of heavy metal poisoning using agents which sequester the metal from organs or tissues and bind it firmly within the ring structure of a new compound which can be eliminated from the body.
A disorder characterized by reduced synthesis of the beta chains of hemoglobin. There is retardation of hemoglobin A synthesis in the heterozygous form (thalassemia minor), which is asymptomatic, while in the homozygous form (thalassemia major, Cooley's anemia, Mediterranean anemia, erythroblastic anemia), which can result in severe complications and even death, hemoglobin A synthesis is absent.
Forms of hepcidin, a cationic amphipathic peptide synthesized in the liver as a prepropeptide which is first processed into prohepcidin and then into the biologically active hepcidin forms, including in human the 20-, 22-, and 25-amino acid residue peptide forms. Hepcidin acts as a homeostatic regulators of iron metabolism and also possesses antimicrobial activity.
Natural product isolated from Streptomyces pilosus. It forms iron complexes and is used as a chelating agent, particularly in the mesylate form.
Conditions in which there is a generalized increase in the iron stores of body tissues, particularly of liver and the MONONUCLEAR PHAGOCYTE SYSTEM, without demonstrable tissue damage. The name refers to the presence of stainable iron in the tissue in the form of hemosiderin.
An iron-binding beta1-globulin that is synthesized in the LIVER and secreted into the blood. It plays a central role in the transport of IRON throughout the circulation. A variety of transferrin isoforms exist in humans, including some that are considered markers for specific disease states.
The techniques used to draw blood from a vein for diagnostic purposes or for treatment of certain blood disorders such as erythrocytosis, hemochromatosis, polycythemia vera, and porphyria cutanea tarda.
Iron or iron compounds used in foods or as food. Dietary iron is important in oxygen transport and the synthesis of the iron-porphyrin proteins hemoglobin, myoglobin, cytochromes, and cytochrome oxidase. Insufficient amounts of dietary iron can lead to iron-deficiency anemia.
A complex of ferric oxyhydroxide with dextrans of 5000 to 7000 daltons in a viscous solution containing 50 mg/ml of iron. It is supplied as a parenteral preparation and is used as a hematinic. (Goodman and Gilman's The Pharmacological Basis of Therapeutics, 8th ed, p1292)
Disorders in the processing of iron in the body: its absorption, transport, storage, and utilization. (From Mosby's Medical, Nursing, & Allied Health Dictionary, 4th ed)
A form of pneumoconiosis resulting from inhalation of iron in the mining dust or welding fumes.
Small cationic peptides that are an important component, in most species, of early innate and induced defenses against invading microbes. In animals they are found on mucosal surfaces, within phagocytic granules, and on the surface of the body. They are also found in insects and plants. Among others, this group includes the DEFENSINS, protegrins, tachyplesins, and thionins. They displace DIVALENT CATIONS from phosphate groups of MEMBRANE LIPIDS leading to disruption of the membrane.
Hemosiderin is an iron-containing pigment that originates from the breakdown of hemoglobin and accumulates in tissues, primarily in macrophages, as a result of various pathological conditions such as hemorrhage, inflammation, or certain storage diseases.
A group of hereditary hemolytic anemias in which there is decreased synthesis of one or more hemoglobin polypeptide chains. There are several genetic types with clinical pictures ranging from barely detectable hematologic abnormality to severe and fatal anemia.
The introduction of whole blood or blood component directly into the blood stream. (Dorland, 27th ed)
Pyridine derivatives with one or more keto groups on the ring.
Membrane glycoproteins consisting of an alpha subunit and a BETA 2-MICROGLOBULIN beta subunit. In humans, highly polymorphic genes on CHROMOSOME 6 encode the alpha subunits of class I antigens and play an important role in determining the serological specificity of the surface antigen. Class I antigens are found on most nucleated cells and are generally detected by their reactivity with alloantisera. These antigens are recognized during GRAFT REJECTION and restrict cell-mediated lysis of virus-infected cells.
Complex of iron atoms chelated with carbonyl ions.
A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances.
Organic and inorganic compounds that contain iron as an integral part of the molecule.
Membrane proteins whose primary function is to facilitate the transport of positively charged molecules (cations) across a biological membrane.
Membrane glycoproteins found in high concentrations on iron-utilizing cells. They specifically bind iron-bearing transferrin, are endocytosed with its ligand and then returned to the cell surface where transferrin without its iron is released.
Unstable isotopes of iron that decay or disintegrate emitting radiation. Fe atoms with atomic weights 52, 53, 55, and 59-61 are radioactive iron isotopes.
Derivatives of BENZOIC ACID. Included under this heading are a broad variety of acid forms, salts, esters, and amides that contain the carboxybenzene structure.
Inorganic or organic compounds containing trivalent iron.
Proteins which are found in membranes including cellular and intracellular membranes. They consist of two types, peripheral and integral proteins. They include most membrane-associated enzymes, antigenic proteins, transport proteins, and drug, hormone, and lectin receptors.
Anemia characterized by decreased or absent iron stores, low serum iron concentration, low transferrin saturation, and low hemoglobin concentration or hematocrit value. The erythrocytes are hypochromic and microcytic and the iron binding capacity is increased.
Puncture of a vein to draw blood for therapeutic purposes. Bloodletting therapy has been used in Talmudic and Indian medicine since the medieval time, and was still practiced widely in the 18th and 19th centuries. Its modern counterpart is PHLEBOTOMY.
Stable iron atoms that have the same atomic number as the element iron, but differ in atomic weight. Fe-54, 57, and 58 are stable iron isotopes.
Low-molecular-weight compounds produced by microorganisms that aid in the transport and sequestration of ferric iron. (The Encyclopedia of Molecular Biology, 1994)
The transfer of erythrocytes from a donor to a recipient or reinfusion to the donor.
Ceruloplasmin is a blue copper-containing protein primarily synthesized in the liver, functioning as a ferroxidase enzyme involved in iron homeostasis and contributing to copper transportation in the body.
Inorganic or organic compounds that contain divalent iron.
Proteins that specifically bind to IRON.
Anemia characterized by the presence of erythroblasts containing excessive deposits of iron in the marrow.
Anemia characterized by a decrease in the ratio of the weight of hemoglobin to the volume of the erythrocyte, i.e., the mean corpuscular hemoglobin concentration is less than normal. The individual cells contain less hemoglobin than they could have under optimal conditions. Hypochromic anemia may be caused by iron deficiency from a low iron intake, diminished iron absorption, or excessive iron loss. It can also be caused by infections or other diseases, therapeutic drugs, lead poisoning, and other conditions. (Stedman, 25th ed; from Miale, Laboratory Medicine: Hematology, 6th ed, p393)
The protein components of ferritins. Apoferritins are shell-like structures containing nanocavities and ferroxidase activities. Apoferritin shells are composed of 24 subunits, heteropolymers in vertebrates and homopolymers in bacteria. In vertebrates, there are two types of subunits, light chain and heavy chain. The heavy chain contains the ferroxidase activity.
A reduction in the number of circulating ERYTHROCYTES or in the quantity of HEMOGLOBIN.
An individual in which both alleles at a given locus are identical.
Triazoles are a class of antifungal drugs that contain a triazole ring in their chemical structure and work by inhibiting the synthesis of ergosterol, an essential component of fungal cell membranes, thereby disrupting the integrity and function of the membrane.
The muscle tissue of the HEART. It is composed of striated, involuntary muscle cells (MYOCYTES, CARDIAC) connected to form the contractile pump to generate blood flow.
Proteins that regulate cellular and organismal iron homeostasis. They play an important biological role by maintaining iron levels that are adequate for metabolic need, but below the toxicity threshold.
Pathological processes of the LIVER.
The oxygen-carrying proteins of ERYTHROCYTES. They are found in all vertebrates and some invertebrates. The number of globin subunits in the hemoglobin quaternary structure differs between species. Structures range from monomeric to a variety of multimeric arrangements.
The processes whereby the internal environment of an organism tends to remain balanced and stable.
An autosomal dominant or acquired porphyria due to a deficiency of UROPORPHYRINOGEN DECARBOXYLASE in the LIVER. It is characterized by photosensitivity and cutaneous lesions with little or no neurologic symptoms. Type I is the acquired form and is strongly associated with liver diseases and hepatic toxicities caused by alcohol or estrogenic steroids. Type II is the familial form.
Enlargement of the HEART, usually indicated by a cardiothoracic ratio above 0.50. Heart enlargement may involve the right, the left, or both HEART VENTRICLES or HEART ATRIA. Cardiomegaly is a nonspecific symptom seen in patients with chronic systolic heart failure (HEART FAILURE) or several forms of CARDIOMYOPATHIES.
A disease characterized by chronic hemolytic anemia, episodic painful crises, and pathologic involvement of many organs. It is the clinical expression of homozygosity for hemoglobin S.
Liver disease in which the normal microcirculation, the gross vascular anatomy, and the hepatic architecture have been variably destroyed and altered with fibrous septa surrounding regenerated or regenerating parenchymal nodules.
A multifunctional iron-sulfur protein that is both an iron regulatory protein and cytoplasmic form of aconitate hydratase. It binds to iron regulatory elements found on mRNAs involved in iron metabolism and regulates their translation. Its RNA binding ability and its aconitate hydrolase activity are dependent upon availability of IRON.
Clonal hematopoietic stem cell disorders characterized by dysplasia in one or more hematopoietic cell lineages. They predominantly affect patients over 60, are considered preleukemic conditions, and have high probability of transformation into ACUTE MYELOID LEUKEMIA.
A group of diseases in which the dominant feature is the involvement of the CARDIAC MUSCLE itself. Cardiomyopathies are classified according to their predominant pathophysiological features (DILATED CARDIOMYOPATHY; HYPERTROPHIC CARDIOMYOPATHY; RESTRICTIVE CARDIOMYOPATHY) or their etiological/pathological factors (CARDIOMYOPATHY, ALCOHOLIC; ENDOCARDIAL FIBROELASTOSIS).
The production of red blood cells (ERYTHROCYTES). In humans, erythrocytes are produced by the YOLK SAC in the first trimester; by the liver in the second trimester; by the BONE MARROW in the third trimester and after birth. In normal individuals, the erythrocyte count in the peripheral blood remains relatively constant implying a balance between the rate of erythrocyte production and rate of destruction.
Uptake of substances through the lining of the INTESTINES.
Any detectable and heritable change in the genetic material that causes a change in the GENOTYPE and which is transmitted to daughter cells and to succeeding generations.
Naturally occurring or experimentally induced animal diseases with pathological processes sufficiently similar to those of human diseases. They are used as study models for human diseases.
A disturbance in the prooxidant-antioxidant balance in favor of the former, leading to potential damage. Indicators of oxidative stress include damaged DNA bases, protein oxidation products, and lipid peroxidation products (Sies, Oxidative Stress, 1991, pxv-xvi).
Inorganic salts of the hypothetical acid ferrocyanic acid (H4Fe(CN)6).
An alcoholic beverage usually made from malted cereal grain (as barley), flavored with hops, and brewed by slow fermentation.
Mononuclear cells with pronounced phagocytic ability that are distributed extensively in lymphoid and other organs. It includes MACROPHAGES and their precursors; PHAGOCYTES; KUPFFER CELLS; HISTIOCYTES; DENDRITIC CELLS; LANGERHANS CELLS; and MICROGLIA. The term mononuclear phagocyte system has replaced the former reticuloendothelial system, which also included less active phagocytic cells such as fibroblasts and endothelial cells. (From Illustrated Dictionary of Immunology, 2d ed.)
Antigens determined by leukocyte loci found on chromosome 6, the major histocompatibility loci in humans. They are polypeptides or glycoproteins found on most nucleated cells and platelets, determine tissue types for transplantation, and are associated with certain diseases.
Non-invasive method of demonstrating internal anatomy based on the principle that atomic nuclei in a strong magnetic field absorb pulses of radiofrequency energy and emit them as radiowaves which can be reconstructed into computerized images. The concept includes proton spin tomographic techniques.
A multifunctional iron-sulfur protein that is both an iron regulatory protein and cytoplasmic form of aconitate hydratase. It binds to iron regulatory elements found on mRNAs involved in iron metabolism and regulates their translation. Its rate of degradation is increased in the presence of IRON.
The shortest and widest portion of the SMALL INTESTINE adjacent to the PYLORUS of the STOMACH. It is named for having the length equal to about the width of 12 fingers.
"Ethyl ethers, also known as diethyl ether, is a colorless, highly volatile, and flammable liquid that belongs to the class of organic compounds called ethers, used as an anesthetic in medicine."
Inbred C57BL mice are a strain of laboratory mice that have been produced by many generations of brother-sister matings, resulting in a high degree of genetic uniformity and homozygosity, making them widely used for biomedical research, including studies on genetics, immunology, cancer, and neuroscience.
Removal and pathologic examination of specimens in the form of small pieces of tissue from the living body.
The color-furnishing portion of hemoglobin. It is found free in tissues and as the prosthetic group in many hemeproteins.
Chemicals that bind to and remove ions from solutions. Many chelating agents function through the formation of COORDINATION COMPLEXES with METALS.
A genus of the family CEBIDAE, subfamily CEBINAE, consisting of four species which are divided into two groups, the tufted and untufted. C. apella has tufts of hair over the eyes and sides of the head. The remaining species are without tufts - C. capucinus, C. nigrivultatus, and C. albifrons. Cebus inhabits the forests of Central and South America.
Inborn errors of metal metabolism refer to genetic disorders resulting from mutations in genes encoding proteins involved in the transportation, storage, or utilization of essential metals, leading to imbalances that can cause toxicity or deficiency and subsequent impairment of normal physiological processes.
The genetic constitution of the individual, comprising the ALLELES present at each GENETIC LOCUS.
A bone morphogenetic protein that is a potent inducer of BONE formation. It plays additional roles in regulating CELL DIFFERENTIATION of non-osteoblastic cell types and epithelial-mesenchymal interactions.
Lipid infiltration of the hepatic parenchymal cells resulting in a yellow-colored liver. The abnormal lipid accumulation is usually in the form of TRIGLYCERIDES, either as a single large droplet or multiple small droplets. Fatty liver is caused by an imbalance in the metabolism of FATTY ACIDS.
Strains of mice in which certain GENES of their GENOMES have been disrupted, or "knocked-out". To produce knockouts, using RECOMBINANT DNA technology, the normal DNA sequence of the gene being studied is altered to prevent synthesis of a normal gene product. Cloned cells in which this DNA alteration is successful are then injected into mouse EMBRYOS to produce chimeric mice. The chimeric mice are then bred to yield a strain in which all the cells of the mouse contain the disrupted gene. Knockout mice are used as EXPERIMENTAL ANIMAL MODELS for diseases (DISEASE MODELS, ANIMAL) and to clarify the functions of the genes.
An autosomal recessive disease, usually of childhood onset, characterized pathologically by degeneration of the spinocerebellar tracts, posterior columns, and to a lesser extent the corticospinal tracts. Clinical manifestations include GAIT ATAXIA, pes cavus, speech impairment, lateral curvature of spine, rhythmic head tremor, kyphoscoliosis, congestive heart failure (secondary to a cardiomyopathy), and lower extremity weakness. Most forms of this condition are associated with a mutation in a gene on chromosome 9, at band q13, which codes for the mitochondrial protein frataxin. (From Adams et al., Principles of Neurology, 6th ed, p1081; N Engl J Med 1996 Oct 17;335(16):1169-75) The severity of Friedreich ataxia associated with expansion of GAA repeats in the first intron of the frataxin gene correlates with the number of trinucleotide repeats. (From Durr et al, N Engl J Med 1996 Oct 17;335(16):1169-75)

Iron depletion by phlebotomy with recombinant erythropoietin prior to allogeneic transplantation to prevent liver toxicity. (1/690)

Iron overload may induce liver toxicity after hematopoietic stem cell transplantation (HSCT), but it is not known if iron depletion prior to HSCT can reduce the risk of severe toxicity in this setting. We used subcutaneous recombinant erythropoietin (EPO) (25 UI/kg) three times a week and phlebotomy once a week, to prevent liver toxicity in a patient with advanced acute leukemia and liver disease due to severe iron overload, previous drug toxicity and hepatitis C viral infection. Over the 9 months prior to allogeneic HSCT, 34 phlebotomies were carried out. Serum ferritin dropped from 2964 to 239 microg/l and the ALT dropped to near normal values. At allogeneic HSCT no liver toxicity was observed, suggesting that iron depletion in the pretransplant period may contribute to reducing transplant-related toxicity in selected cases.  (+info)

Iron overload in porphyria cutanea tarda. (2/690)

BACKGROUND AND OBJECTIVE: Porphyria cutanea tarda (PCT) is a disorder of porphyrin metabolism associated with decreased activity of uroporphyrinogen decarboxylase (URO-D) in the liver. The relevance of iron in the pathogenesis of PCT is well established: iron overload is one of the factors that trigger the clinical manifestations of the disease and iron depletion remains the cornerstone of therapy for PCT. A role for genetic hemochromatosis in the pathogenesis of iron overload in PCT has been hypothesized in the past but only after the recent identification of the genetic defect causing hemochromatosis has the nature of this association been partially elucidated. This review will outline current concepts of the pathophysiology of iron overload in PCT as well as recent contributions to the molecular epidemiology of hemochromatosis defects in PCT. EVIDENCE AND INFORMATION SOURCES: The authors of the present review have a long-standing interest in the pathogenesis, etiology and epidemiology of iron overload syndromes. Evidence from journal articles covered by the Science Citation Index(R) and Medline(R) has been reviewed and collated with personal data and experience. STATE OF THE ART AND PERPECTIVES: Mild to moderate iron overload plays a key role in the pathogenesis of PCT. The recent identification of genetic mutations of the hemochromatosis gene (HFE) in the majority of patients with PCT confirms previous hypotheses on the association between PCT and hemochromatosis, allows a step forward in the understanding of the pathophysiology of the disturbance of iron metabolism in the liver of PCT patients, and provides an easily detectable genetic marker which could have a useful clinical application. Besides the epidemiological relevance of the association between PCT and hemochromatosis, however, it remains to be fully understood how iron overload, and in particular the cellular modifications of the iron status secondary to hemochromatosis mutations, affect the activity of URO-D, and how the altered iron metabolism interacts with the other two common triggers for PCT and etiological agents for the associated liver disease: alcohol and hepatitis viruses. The availability of a genetic marker for hemochromatosis will allow some of these issues to be addressed by studying aspects of porphyrins and iron metabolism in liver samples obtained from patients with PCT, liver disease of different etiology and different HFE genotypes, and by in vitro studies on genotyped cells and tissues.  (+info)

Heterozygotes for HFE mutations have no increased risk of advanced alcoholic liver disease. (3/690)

BACKGROUND: Iron overload is common in the livers of alcoholics and may play a role in disease pathogenesis. An MHC like gene, HFE, has recently been identified that is mutated in most patients with hereditary haemochromatosis (C282Y in 90% and H63D in 45% of the remainder). AIM: To examine the hypothesis that these mutations determine hepatic iron status in alcoholics and play a role in pre-disposition to advanced alcoholic liver disease. METHODS: The HFE gene was genotyped in 257 patients with alcoholic liver disease and 117 locally matched healthy volunteers. In addition, iron staining was scored (0-4) on biopsy specimens from fibrotic/cirrhotic patients with and without HFE mutations matched for age and sex. RESULTS: Some 15.7% of fibrotic/cirrhotic patients were C282Y heterozygotes compared with 13.7% of controls (p = 0.77). One control and three patients were C282Y homozygotes. Of chromosomes without the C282Y mutation, 68/442 (15.4%) of patients' chromosomes carried the H63D mutation compared with 36/216 (16.6%) of control chromosomes (p = 0.91). Significant (> grade 1) hepatocyte iron staining was seen in 6/23 C282Y heterozygotes and 4/26 H63D heterozygotes compared with 4/23 controls. CONCLUSIONS: Possession of a single copy of either of the two HFE mutations influences neither liver iron content nor the risk of fibrotic disease in alcoholics.  (+info)

Iron overload upregulates haem oxygenase 1 in the lung more rapidly than in other tissues. (4/690)

Haem oxygenase-1 is upregulated by numerous insults, including oxidative stress, and under such circumstances it is considered to be a protective stratagem. We have measured the haem oxygenase-1 expression in heart, lung and liver tissues of control and iron-overloaded rats. Lung tissue from iron-overloaded rats displayed a significant increase in the haem oxygenase-1 protein but no changes in haem oxygenase-1 mRNA. Conversely, heart tissue showed a significant increase in haem oxygenase-1 mRNA but no changes in haem oxygenase-1 protein. We conclude that during oxidative stress caused by iron overload, lung tissue responds with a rapid upregulation of haem oxygenase-1 levels.  (+info)

Immunological analysis of beta-thalassemic mouse intestinal proteins reveals up-regulation of sucrase-isomaltase in response to iron overload. (5/690)

Maintenance of iron homeostasis must balance the demand for iron due to heme synthesis, which is driven by hematopoiesis, and the restricted intestinal uptake of iron, which otherwise limits absorption of this toxic element. The consequences of perturbed iron homeostasis are witnessed in inherited forms of beta-thalassemia in which erythroid hyperplasia results in enhanced intestinal iron absorption despite tissue iron overload. To gain a better understanding of intestinal factors that are induced when iron homeostasis is disrupted, a panel of monoclonal antibodies that recognize intestinal microvillous membrane proteins of the beta-thalassemic Hbbd(th3)/Hbbd(th3) mouse was established. The monoclonal antibodies were screened by differential Western blotting against normal and beta-thalassemic mouse intestine to identify antigens modulated in the disease state. Here we report the initial characterization of one immunoreactive species that is up-regulated in beta-thalassemic mouse intestine and the tentative identification of this antigen as sucrase-isomaltase. Studies in Caco-2 cells revealed the rather unexpected finding that expression of this intestinal hydrolase is increased in response to iron toxicity.  (+info)

Respiratory function in patients with thalassaemia and iron overload. (6/690)

Iron deposition in the respiratory system has been proposed as a potential cause of the ventilatory restrictive impairment seen in patients with thalassaemia major (TM) and iron overload. In this study, magnetic resonance imaging (MRI) measurements of the liver (T2 relaxation time) were used as a surrogate index of total body iron burden and the extent to which these measurements correlated with total lung capacity (TLC) in patients with TM was examined. Twenty-one patients (aged 25+/-5 yrs) with TM participated in the study. Standard pulmonary function tests were undertaken and the T2 relaxation time of the liver was measured in all patients. Ventilatory restrictive impairment (mean TLC 74+/-11 (SD)% predicted) was the most common abnormality found in 71% of TM patients. There was no correlation between TLC (% pred) and T2 relaxation time (r=0.06, p=0.78). T2 relaxation time correlated weakly with average serum ferritin levels (r=-0.56, p=0.008). In conclusion, the data do not support the notion that the restrictive impairment in patients with thalassaemia major and iron overload is related to iron deposition in the respiratory system.  (+info)

Iron loading and disease surveillance. (7/690)

Iron is an oxidant as well as a nutrient for invading microbial and neoplastic cells. Excessive iron in specific tissues and cells (iron loading) promotes development of infection, neoplasia, cardiomyopathy, arthropathy, and various endocrine and possibly neurodegenerative disorders. To contain and detoxify the metal, hosts have evolved an iron withholding defense system, but the system can be compromised by numerous factors. An array of behavioral, medical, and immunologic methods are in place or in development to strengthen iron withholding. Routine screening for iron loading could provide valuable information in epidemiologic, diagnostic, prophylactic, and therapeutic studies of emerging infectious diseases.  (+info)

Iron overload in urban Africans in the 1990s. (8/690)

BACKGROUND: In a previously described model, heterozygotes for an African iron loading locus develop iron overload only when dietary iron is high, but homozygotes may do so with normal dietary iron. If an iron loading gene is common, then homozygotes with iron overload will be found even in an urban population where traditional beer, the source of iron, is uncommon. AIMS: To determine whether iron overload and the C282Y mutation characteristic of hereditary haemochromatosis are readily identifiable in an urban African population. METHODS: Histological assessment, hepatocellular iron grading, and dry weight non-haem iron concentration were determined in post mortem tissue from liver, spleen, heart, lungs, and skin. DNA of subjects with elevated hepatic iron indexes was analysed for the C282Y mutation. Iron concentrations in other tissues were compared. RESULTS: A moderate increase (>30 micromol/g) in hepatic iron concentrations was found in 31 subjects (23%; 95% confidence interval 15.9 to 30.1%), and they were considerably elevated (>180 micromol/g) in seven subjects (5.2%; 95% confidence interval 1.5 to 8.9%). Appreciably elevated hepatic iron concentrations were associated with heavy iron deposition in both hepatocytes and macrophages, and either portal fibrosis or cirrhosis. All were negative for the C282Y mutation. Very high concentrations were uncommon in subjects dying in hospital. Concentrations of iron in spleen, heart, lung, and skin were significantly higher in subjects with elevated hepatic iron. CONCLUSIONS: Iron overload is readily identified among urban Africans and is associated with hepatic damage and iron loading of several tissues. The condition is unrelated to the genetic mutation found in hereditary haemochromatosis.  (+info)

Iron overload is a condition characterized by an excessive accumulation of iron in the body's tissues and organs, particularly in the liver, heart, and pancreas. This occurs when the body absorbs more iron than it can use or eliminate, leading to iron levels that are higher than normal.

Iron overload can result from various factors, including hereditary hemochromatosis, a genetic disorder that affects how the body absorbs iron from food; frequent blood transfusions, which can cause iron buildup in people with certain chronic diseases such as sickle cell anemia or thalassemia; and excessive consumption of iron supplements or iron-rich foods.

Symptoms of iron overload may include fatigue, joint pain, abdominal discomfort, irregular heartbeat, and liver dysfunction. If left untreated, it can lead to serious complications such as cirrhosis, liver failure, diabetes, heart problems, and even certain types of cancer. Treatment typically involves regular phlebotomy (removal of blood) to reduce iron levels in the body, along with dietary modifications and monitoring by a healthcare professional.

In the context of medicine, iron is an essential micromineral and key component of various proteins and enzymes. It plays a crucial role in oxygen transport, DNA synthesis, and energy production within the body. Iron exists in two main forms: heme and non-heme. Heme iron is derived from hemoglobin and myoglobin in animal products, while non-heme iron comes from plant sources and supplements.

The recommended daily allowance (RDA) for iron varies depending on age, sex, and life stage:

* For men aged 19-50 years, the RDA is 8 mg/day
* For women aged 19-50 years, the RDA is 18 mg/day
* During pregnancy, the RDA increases to 27 mg/day
* During lactation, the RDA for breastfeeding mothers is 9 mg/day

Iron deficiency can lead to anemia, characterized by fatigue, weakness, and shortness of breath. Excessive iron intake may result in iron overload, causing damage to organs such as the liver and heart. Balanced iron levels are essential for maintaining optimal health.

Iron chelating agents are medications that bind to iron in the body, forming a stable complex that can then be excreted from the body. These agents are primarily used to treat iron overload, a condition that can occur due to frequent blood transfusions or certain genetic disorders such as hemochromatosis. By reducing the amount of iron in the body, these medications can help prevent or reduce damage to organs such as the heart and liver. Examples of iron chelating agents include deferoxamine, deferasirox, and deferiprone.

Hemochromatosis is a medical condition characterized by excessive absorption and accumulation of iron in the body, resulting in damage to various organs. It's often referred to as "iron overload" disorder. There are two main types: primary (hereditary) and secondary (acquired). Primary hemochromatosis is caused by genetic mutations that lead to increased intestinal iron absorption, while secondary hemochromatosis can be the result of various conditions such as multiple blood transfusions, chronic liver disease, or certain types of anemia.

In both cases, the excess iron gets stored in body tissues, particularly in the liver, heart, and pancreas, which can cause organ damage and lead to complications like cirrhosis, liver failure, diabetes, heart problems, and skin discoloration. Early diagnosis and treatment through regular phlebotomy (blood removal) or chelation therapy can help manage the condition and prevent severe complications.

Ferritin is a protein in iron-metabolizing cells that stores iron in a water-soluble form. It is found inside the cells (intracellular) and is released into the bloodstream when the cells break down or die. Measuring the level of ferritin in the blood can help determine the amount of iron stored in the body. High levels of ferritin may indicate hemochromatosis, inflammation, liver disease, or other conditions. Low levels of ferritin may indicate anemia, iron deficiency, or other conditions.

Chelation therapy is a medical treatment that involves the use of chelating agents to remove heavy metals and minerals from the body. A chelating agent is a molecule that bonds with the metal ions, forming a stable, water-soluble complex that can be excreted through urine or stool.

The most common chelating agent used in medical settings is ethylene diamine tetraacetic acid (EDTA), which is administered intravenously. EDTA binds with metals such as lead, mercury, iron, and calcium, and helps to eliminate them from the body.

Chelation therapy is primarily used to treat heavy metal poisoning, such as lead or mercury toxicity. It may also be used in some cases to treat cardiovascular disease, although its effectiveness for this use is still a matter of debate and controversy.

It's important to note that chelation therapy should only be administered under the supervision of a qualified healthcare professional, as improper use can lead to serious side effects and complications.

Beta-thalassemia is a genetic blood disorder that affects the production of hemoglobin, a protein in red blood cells that carries oxygen throughout the body. Specifically, beta-thalassemia is caused by mutations in the beta-globin gene, which leads to reduced or absent production of the beta-globin component of hemoglobin.

There are two main types of beta-thalassemia:

1. Beta-thalassemia major (also known as Cooley's anemia): This is a severe form of the disorder that typically becomes apparent in early childhood. It is characterized by a significant reduction or absence of beta-globin production, leading to anemia, enlarged spleen and liver, jaundice, and growth retardation.
2. Beta-thalassemia intermedia: This is a milder form of the disorder that may not become apparent until later in childhood or even adulthood. It is characterized by a variable reduction in beta-globin production, leading to mild to moderate anemia and other symptoms that can range from nonexistent to severe.

Treatment for beta-thalassemia depends on the severity of the disorder and may include blood transfusions, iron chelation therapy, and/or bone marrow transplantation. In some cases, genetic counseling and prenatal diagnosis may also be recommended for families with a history of the disorder.

Hepcidin is a peptide hormone primarily produced in the liver that plays a crucial role in regulating iron homeostasis within the body. It acts by inhibiting the absorption of dietary iron in the intestines and the release of iron from storage sites, such as macrophages, into the bloodstream. By reducing the amount of iron available for use, hepcidin helps prevent excessive iron accumulation in tissues, which can be harmful and contribute to the development of various diseases, including iron overload disorders and certain types of anemia. The production of hepcidin is regulated by several factors, including iron levels, inflammation, and erythropoiesis (the production of red blood cells).

Deferoxamine is a medication used to treat iron overload, which can occur due to various reasons such as frequent blood transfusions or excessive iron intake. It works by binding to excess iron in the body and promoting its excretion through urine. This helps to prevent damage to organs such as the heart and liver that can be caused by high levels of iron.

Deferoxamine is an injectable medication that is typically administered intravenously or subcutaneously, depending on the specific regimen prescribed by a healthcare professional. It may also be used in combination with other medications to manage iron overload more effectively.

It's important to note that deferoxamine should only be used under the guidance of a medical professional, as improper use or dosing can lead to serious side effects or complications.

Hemosiderosis is a medical condition characterized by the abnormal accumulation of hemosiderin, an iron-containing protein, in various organs and tissues of the body. Hemosiderin is derived from the breakdown of hemoglobin, which is the oxygen-carrying protein in red blood cells. When there is excessive breakdown of red blood cells or impaired clearance of hemosiderin, it can lead to its accumulation in organs such as the liver, spleen, and lungs.

Hemosiderosis can be classified into two types: primary and secondary. Primary hemosiderosis is a rare condition that is caused by genetic disorders affecting red blood cells, while secondary hemosiderosis is more common and is associated with various conditions that cause excessive breakdown of red blood cells or chronic inflammation. These conditions include hemolytic anemias, repeated blood transfusions, liver diseases, infections, and certain autoimmune disorders.

The accumulation of hemosiderin can lead to tissue damage and organ dysfunction, particularly in the lungs, where it can cause pulmonary fibrosis, and in the heart, where it can lead to heart failure. Hemosiderosis is typically diagnosed through a combination of medical history, physical examination, and laboratory tests, including blood tests and imaging studies such as chest X-rays or MRI scans. Treatment of hemosiderosis depends on the underlying cause and may include medications, blood transfusions, or supportive care to manage symptoms and prevent complications.

Transferrin is a glycoprotein that plays a crucial role in the transport and homeostasis of iron in the body. It's produced mainly in the liver and has the ability to bind two ferric (Fe3+) ions in its N-lobe and C-lobe, thus creating transferrin saturation.

This protein is essential for delivering iron to cells while preventing the harmful effects of free iron, which can catalyze the formation of reactive oxygen species through Fenton reactions. Transferrin interacts with specific transferrin receptors on the surface of cells, particularly in erythroid precursors and brain endothelial cells, to facilitate iron uptake via receptor-mediated endocytosis.

In addition to its role in iron transport, transferrin also has antimicrobial properties due to its ability to sequester free iron, making it less available for bacterial growth and survival. Transferrin levels can be used as a clinical marker of iron status, with decreased levels indicating iron deficiency anemia and increased levels potentially signaling inflammation or liver disease.

Phlebotomy is a medical term that refers to the process of making an incision in a vein, usually in the arm, in order to draw blood. It is also commonly known as venipuncture. This procedure is performed by healthcare professionals for various purposes such as diagnostic testing, blood donation, or therapeutic treatments like phlebotomy for patients with hemochromatosis (a condition where the body absorbs too much iron from food).

The person who performs this procedure is called a phlebotomist. They must be trained in the proper techniques to ensure that the process is safe and relatively pain-free for the patient, and that the blood sample is suitable for laboratory testing.

Dietary iron is a vital nutrient that plays a crucial role in the production of hemoglobin, a protein in red blood cells responsible for carrying oxygen throughout the body. It is also essential for various other bodily functions, including energy production and immune function.

There are two forms of dietary iron: heme and non-heme. Heme iron is found in animal products such as meat, poultry, and fish, while non-heme iron is found in plant-based foods such as beans, lentils, tofu, spinach, and fortified cereals.

The recommended daily intake of dietary iron varies depending on age, sex, and other factors. For example, adult men typically require 8 milligrams (mg) per day, while adult women need 18 mg per day. Pregnant women may require up to 27 mg per day, while breastfeeding women need around 9-10 mg per day.

It is important to note that the absorption of non-heme iron from plant-based foods can be enhanced by consuming them with vitamin C-rich foods or drinks, such as citrus fruits, strawberries, and bell peppers. On the other hand, certain substances such as tannins (found in tea and coffee) and phytates (found in whole grains and legumes) can inhibit the absorption of non-heme iron.

Iron-dextran complex is a parenteral preparation used as an iron supplement to treat or prevent iron deficiency anemia in patients who cannot take oral iron or do not respond well to oral iron therapy. The complex is formed by combining iron salts with dextran, a type of polysaccharide derived from cornstarch, which acts as a carrier and helps increase the solubility and stability of the iron.

The iron-dextran complex is available in various forms, including injectable solutions and intravenous (IV) infusions. It works by releasing iron ions slowly into the body, where they can be taken up by red blood cell precursors in the bone marrow and used to synthesize hemoglobin, a protein that carries oxygen in the blood.

It is important to note that iron-dextran complex can cause anaphylactic reactions in some individuals, so it should be administered with caution and under medical supervision. Patients should be monitored for signs of allergic reactions during and after administration, and appropriate measures should be taken if necessary.

Iron metabolism disorders are a group of medical conditions that affect the body's ability to absorb, transport, store, or utilize iron properly. Iron is an essential nutrient that plays a crucial role in various bodily functions, including oxygen transportation and energy production. However, imbalances in iron levels can lead to several health issues.

There are two main types of iron metabolism disorders:

1. Iron deficiency anemia (IDA): This condition occurs when the body lacks adequate iron to produce sufficient amounts of hemoglobin, a protein in red blood cells responsible for carrying oxygen throughout the body. Causes of IDA may include inadequate dietary iron intake, blood loss, or impaired iron absorption due to conditions like celiac disease or inflammatory bowel disease.
2. Hemochromatosis: This is a genetic disorder characterized by excessive absorption and accumulation of iron in various organs, including the liver, heart, and pancreas. Over time, this excess iron can lead to organ damage and diseases such as cirrhosis, heart failure, diabetes, and arthritis. Hemochromatosis is typically caused by mutations in the HFE gene, which regulates iron absorption in the intestines.

Other iron metabolism disorders include:

* Anemia of chronic disease (ACD): A type of anemia that occurs in individuals with chronic inflammation or infection, where iron is not efficiently used for hemoglobin production due to altered regulation.
* Sideroblastic anemias: These are rare disorders characterized by the abnormal formation of ringed sideroblasts (immature red blood cells containing iron-laden mitochondria) in the bone marrow, leading to anemia and other symptoms.
* Iron-refractory iron deficiency anemia (IRIDA): A rare inherited disorder caused by mutations in the TMPRSS6 gene, resulting in impaired regulation of hepcidin, a hormone that controls iron absorption and distribution in the body. This leads to both iron deficiency and iron overload.

Proper diagnosis and management of iron metabolism disorders are essential to prevent complications and maintain overall health. Treatment options may include dietary modifications, iron supplementation, phlebotomy (bloodletting), or chelation therapy, depending on the specific disorder and its severity.

Siderosis is a medical condition characterized by the abnormal accumulation of iron in various tissues and organs, most commonly in the lungs. This occurs due to the repeated inhalation of iron-containing dusts or fumes, which can result from certain industrial processes such as welding, mining, or smelting.

In the lungs, this iron deposit can lead to inflammation and fibrosis, potentially causing symptoms like coughing, shortness of breath, and decreased lung function. It is important to note that siderosis itself is not contagious or cancerous, but there may be an increased risk for lung cancer in individuals with severe and prolonged exposure to iron-containing particles.

While siderosis is generally non-reversible, the progression of symptoms can often be managed through medical interventions and environmental modifications to reduce further exposure to iron-containing dusts or fumes.

Antimicrobial cationic peptides (ACPs) are a group of small, naturally occurring peptides that possess broad-spectrum antimicrobial activity against various microorganisms, including bacteria, fungi, viruses, and parasites. They are called "cationic" because they contain positively charged amino acid residues (such as lysine and arginine), which allow them to interact with and disrupt the negatively charged membranes of microbial cells.

ACPs are produced by a wide range of organisms, including humans, animals, and plants, as part of their innate immune response to infection. They play an important role in protecting the host from invading pathogens by directly killing them or inhibiting their growth.

The antimicrobial activity of ACPs is thought to be mediated by their ability to disrupt the membranes of microbial cells, leading to leakage of cellular contents and death. Some ACPs may also have intracellular targets, such as DNA or protein synthesis, that contribute to their antimicrobial activity.

ACPs are being studied for their potential use as therapeutic agents to treat infectious diseases, particularly those caused by drug-resistant bacteria. However, their clinical application is still in the early stages of development due to concerns about their potential toxicity to host cells and the emergence of resistance mechanisms in microbial pathogens.

Hemosiderin is a golden-brown pigment that consists of iron-containing protein complexes called ferritin and ferrikinase. It is insoluble in water and forms as a result of the breakdown of hemoglobin in the reticuloendothelial system, primarily in macrophages. Hemosiderin deposits can be found in various tissues and organs, such as the spleen, liver, and brain, under conditions of increased red blood cell destruction or impaired iron metabolism. These deposits are often associated with diseases such as hemochromatosis, thalassemia, and chronic inflammation.

Thalassemia is a group of inherited genetic disorders that affect the production of hemoglobin, a protein in red blood cells responsible for carrying oxygen throughout the body. The disorder results in less efficient or abnormal hemoglobin, which can lead to anemia, an insufficient supply of oxygen-rich red blood cells.

There are two main types of Thalassemia: alpha and beta. Alpha thalassemia occurs when there is a problem with the alpha globin chain production, while beta thalassemia results from issues in beta globin chain synthesis. These disorders can range from mild to severe, depending on the number of genes affected and their specific mutations.

Severe forms of Thalassemia may require regular blood transfusions, iron chelation therapy, or even a bone marrow transplant to manage symptoms and prevent complications.

A blood transfusion is a medical procedure in which blood or its components are transferred from one individual (donor) to another (recipient) through a vein. The donated blood can be fresh whole blood, packed red blood cells, platelets, plasma, or cryoprecipitate, depending on the recipient's needs. Blood transfusions are performed to replace lost blood due to severe bleeding, treat anemia, support patients undergoing major surgeries, or manage various medical conditions such as hemophilia, thalassemia, and leukemia. The donated blood must be carefully cross-matched with the recipient's blood type to minimize the risk of transfusion reactions.

Pyridones are a class of organic compounds that contain a pyridone ring, which is a heterocyclic ring consisting of a six-membered ring with five carbon atoms and one nitrogen atom, with one oxygen atom attached to the nitrogen atom by a double bond. Pyridones can be found in various natural sources, including plants and microorganisms, and they also have important applications in the pharmaceutical industry as building blocks for drug design and synthesis. Some drugs that contain pyridone rings include antihistamines, anti-inflammatory agents, and antiviral agents.

Histocompatibility antigens, class I are proteins found on the surface of most cells in the body. They play a critical role in the immune system's ability to differentiate between "self" and "non-self." These antigens are composed of three polypeptides - two heavy chains and one light chain - and are encoded by genes in the major histocompatibility complex (MHC) on chromosome 6 in humans.

Class I MHC molecules present peptide fragments from inside the cell to CD8+ T cells, also known as cytotoxic T cells. This presentation allows the immune system to detect and destroy cells that have been infected by viruses or other intracellular pathogens, or that have become cancerous.

There are three main types of class I MHC molecules in humans: HLA-A, HLA-B, and HLA-C. The term "HLA" stands for human leukocyte antigen, which reflects the original identification of these proteins on white blood cells (leukocytes). The genes encoding these molecules are highly polymorphic, meaning there are many different variants in the population, and matching HLA types is essential for successful organ transplantation to minimize the risk of rejection.

Iron carbonyl compounds are a group of chemical compounds that contain iron and carbon monoxide (CO) molecules. The most common iron carbonyl compound is Iron pentacarbonyl (Fe(CO)5), which is a colorless liquid with a faint, sweet odor. It is used as a reducing agent and a catalyst in various chemical reactions. Other iron carbonyl compounds include diiron decacarbonyl (Fe2(CO)10), triiron dodecacarbonyl (Fe3(CO)12), and tetracarbonylferrate(II) ion [Fe(CO)4]2-. These compounds are typically prepared by the direct reaction of iron with carbon monoxide under high pressure. They are sensitive to oxygen, moisture, and light, and must be handled carefully to prevent degradation.

The liver is a large, solid organ located in the upper right portion of the abdomen, beneath the diaphragm and above the stomach. It plays a vital role in several bodily functions, including:

1. Metabolism: The liver helps to metabolize carbohydrates, fats, and proteins from the food we eat into energy and nutrients that our bodies can use.
2. Detoxification: The liver detoxifies harmful substances in the body by breaking them down into less toxic forms or excreting them through bile.
3. Synthesis: The liver synthesizes important proteins, such as albumin and clotting factors, that are necessary for proper bodily function.
4. Storage: The liver stores glucose, vitamins, and minerals that can be released when the body needs them.
5. Bile production: The liver produces bile, a digestive juice that helps to break down fats in the small intestine.
6. Immune function: The liver plays a role in the immune system by filtering out bacteria and other harmful substances from the blood.

Overall, the liver is an essential organ that plays a critical role in maintaining overall health and well-being.

Iron compounds refer to chemical substances that contain iron (Fe) combined with other elements. Iron is an essential mineral for the human body, playing a crucial role in various bodily functions such as oxygen transport, DNA synthesis, and energy production.

There are several types of iron compounds, including:

1. Inorganic iron salts: These are commonly used in dietary supplements and fortified foods to treat or prevent iron deficiency anemia. Examples include ferrous sulfate, ferrous gluconate, and ferric iron.
2. Heme iron: This is the form of iron found in animal products such as meat, poultry, and fish. It is more easily absorbed by the body compared to non-heme iron from plant sources.
3. Non-heme iron: This is the form of iron found in plant-based foods such as grains, legumes, fruits, and vegetables. It is not as well-absorbed as heme iron but can be enhanced by consuming it with vitamin C or other organic acids.

It's important to note that excessive intake of iron compounds can lead to iron toxicity, which can cause serious health problems. Therefore, it's essential to follow recommended dosages and consult a healthcare professional before taking any iron supplements.

Cation transport proteins are a type of membrane protein that facilitate the movement of cations (positively charged ions) across biological membranes. These proteins play a crucial role in maintaining ion balance and electrical excitability within cells, as well as in various physiological processes such as nutrient uptake, waste elimination, and signal transduction.

There are several types of cation transport proteins, including:

1. Ion channels: These are specialized protein structures that form a pore or channel through the membrane, allowing ions to pass through rapidly and selectively. They can be either voltage-gated or ligand-gated, meaning they open in response to changes in electrical potential or binding of specific molecules, respectively.

2. Ion pumps: These are active transport proteins that use energy from ATP hydrolysis to move ions against their electrochemical gradient, effectively pumping them from one side of the membrane to the other. Examples include the sodium-potassium pump (Na+/K+-ATPase) and calcium pumps (Ca2+ ATPase).

3. Ion exchangers: These are antiporter proteins that facilitate the exchange of one ion for another across the membrane, maintaining electroneutrality. For example, the sodium-proton exchanger (NHE) moves a proton into the cell in exchange for a sodium ion being moved out.

4. Symporters: These are cotransporter proteins that move two or more ions together in the same direction, often coupled with the transport of a solute molecule. An example is the sodium-glucose cotransporter (SGLT), which facilitates glucose uptake into cells by coupling its movement with that of sodium ions.

Collectively, cation transport proteins help maintain ion homeostasis and contribute to various cellular functions, including electrical signaling, enzyme regulation, and metabolic processes. Dysfunction in these proteins can lead to a range of diseases, such as neurological disorders, cardiovascular disease, and kidney dysfunction.

Transferrin receptors are membrane-bound proteins found on the surface of many cell types, including red and white blood cells, as well as various tissues such as the liver, brain, and placenta. These receptors play a crucial role in iron homeostasis by regulating the uptake of transferrin, an iron-binding protein, into the cells.

Transferrin binds to two ferric ions (Fe3+) in the bloodstream, forming a complex known as holo-transferrin. This complex then interacts with the transferrin receptors on the cell surface, leading to endocytosis of the transferrin-receptor complex into the cell. Once inside the cell, the acidic environment within the endosome causes the release of iron ions from the transferrin molecule, which can then be transported into the cytoplasm for use in various metabolic processes.

After releasing the iron, the apo-transferrin (iron-free transferrin) is recycled back to the cell surface and released back into the bloodstream, where it can bind to more ferric ions and repeat the cycle. This process helps maintain appropriate iron levels within the body and ensures that cells have access to the iron they need for essential functions such as DNA synthesis, energy production, and oxygen transport.

In summary, transferrin receptors are membrane-bound proteins responsible for recognizing and facilitating the uptake of transferrin-bound iron into cells, playing a critical role in maintaining iron homeostasis within the body.

"Iron radioisotopes" refer to specific forms of the element iron that have unstable nuclei and emit radiation. These isotopes are often used in medical imaging and treatment procedures due to their ability to be detected by specialized equipment. Common iron radioisotopes include Iron-52, Iron-55, Iron-59, and Iron-60. They can be used as tracers to study the distribution, metabolism, or excretion of iron in the body, or for targeted radiation therapy in conditions such as cancer.

Benzoates are the salts and esters of benzoic acid. They are widely used as preservatives in foods, cosmetics, and pharmaceuticals to prevent the growth of microorganisms. The chemical formula for benzoic acid is C6H5COOH, and when it is combined with a base (like sodium or potassium), it forms a benzoate salt (e.g., sodium benzoate or potassium benzoate). When benzoic acid reacts with an alcohol, it forms a benzoate ester (e.g., methyl benzoate or ethyl benzoate).

Benzoates are generally considered safe for use in food and cosmetics in small quantities. However, some people may have allergies or sensitivities to benzoates, which can cause reactions such as hives, itching, or asthma symptoms. In addition, there is ongoing research into the potential health effects of consuming high levels of benzoates over time, particularly in relation to gut health and the development of certain diseases.

In a medical context, benzoates may also be used as a treatment for certain conditions. For example, sodium benzoate is sometimes given to people with elevated levels of ammonia in their blood (hyperammonemia) to help reduce those levels and prevent brain damage. This is because benzoates can bind with excess ammonia in the body and convert it into a form that can be excreted in urine.

Ferric compounds are inorganic compounds that contain the iron(III) cation, Fe3+. Iron(III) is a transition metal and can form stable compounds with various anions. Ferric compounds are often colored due to the d-d transitions of the iron ion. Examples of ferric compounds include ferric chloride (FeCl3), ferric sulfate (Fe2(SO4)3), and ferric oxide (Fe2O3). Ferric compounds have a variety of uses, including as catalysts, in dye production, and in medical applications.

Membrane proteins are a type of protein that are embedded in the lipid bilayer of biological membranes, such as the plasma membrane of cells or the inner membrane of mitochondria. These proteins play crucial roles in various cellular processes, including:

1. Cell-cell recognition and signaling
2. Transport of molecules across the membrane (selective permeability)
3. Enzymatic reactions at the membrane surface
4. Energy transduction and conversion
5. Mechanosensation and signal transduction

Membrane proteins can be classified into two main categories: integral membrane proteins, which are permanently associated with the lipid bilayer, and peripheral membrane proteins, which are temporarily or loosely attached to the membrane surface. Integral membrane proteins can further be divided into three subcategories based on their topology:

1. Transmembrane proteins, which span the entire width of the lipid bilayer with one or more alpha-helices or beta-barrels.
2. Lipid-anchored proteins, which are covalently attached to lipids in the membrane via a glycosylphosphatidylinositol (GPI) anchor or other lipid modifications.
3. Monotopic proteins, which are partially embedded in the membrane and have one or more domains exposed to either side of the bilayer.

Membrane proteins are essential for maintaining cellular homeostasis and are targets for various therapeutic interventions, including drug development and gene therapy. However, their structural complexity and hydrophobicity make them challenging to study using traditional biochemical methods, requiring specialized techniques such as X-ray crystallography, nuclear magnetic resonance (NMR) spectroscopy, and single-particle cryo-electron microscopy (cryo-EM).

Iron-deficiency anemia is a condition characterized by a decrease in the total amount of hemoglobin or red blood cells in the blood, caused by insufficient iron levels in the body. Hemoglobin is a protein in red blood cells that carries oxygen from the lungs to the rest of the body. When iron levels are low, the body cannot produce enough hemoglobin, leading to the production of smaller and fewer red blood cells, known as microcytic hypochromic anemia.

Iron is essential for the production of hemoglobin, and a deficiency in iron can result from inadequate dietary intake, chronic blood loss, or impaired absorption. In addition to fatigue and weakness, symptoms of iron-deficiency anemia may include shortness of breath, headaches, dizziness, pale skin, and brittle nails. Treatment typically involves iron supplementation and addressing the underlying cause of the iron deficiency.

Bloodletting is a medical procedure that was commonly used in the past to balance the four humors of the body, which were believed to be blood, phlegm, black bile, and yellow bile. The procedure involved withdrawing blood from a patient through various methods such as venesection (making an incision in a vein), leeches, or cupping.

The theory behind bloodletting was that if one humor became overabundant, it could cause disease or illness. By removing some of the excess humor, practitioners believed they could restore balance and promote healing. Bloodletting was used to treat a wide variety of conditions, including fever, inflammation, and pain.

While bloodletting is no longer practiced in modern medicine, it was once a common treatment for many different ailments. The practice dates back to ancient times and was used by various cultures throughout history, including the Greeks, Romans, Egyptians, and Chinese. However, its effectiveness as a medical treatment has been called into question, and it is now considered an outdated and potentially harmful procedure.

I must clarify that "Iron Isotopes" is not a medical term, but rather a scientific concept from the field of physics and chemistry. However, I can certainly provide a general explanation of isotopes and then focus on iron isotopes specifically.

An isotope is a variant of a chemical element that has the same number of protons (and thus the same atomic number) but a different number of neutrons within its nucleus. This results in variations of the atomic mass of isotopes of the same element. Some isotopes are stable, while others are unstable and will decay over time into other elements or isotopes, a process called radioactive decay.

Iron (Fe) has four naturally occurring stable isotopes: Fe-54, Fe-56, Fe-57, and Fe-58. These iron isotopes have different numbers of neutrons in their nuclei, resulting in slightly different atomic masses. The most abundant iron isotope is Fe-56, which contains 26 protons and 30 neutrons in its nucleus.

In the context of human health, iron is an essential nutrient that plays a crucial role in various biological processes, such as oxygen transport and energy production. However, the concept of iron isotopes does not have a direct medical relevance, but it can be useful in scientific research related to fields like geochemistry, environmental science, or nuclear physics.

Siderophores are low-molecular-weight organic compounds that are secreted by microorganisms, such as bacteria and fungi, to chelate and solubilize iron from their environment. They are able to bind ferric iron (Fe3+) with very high affinity and form a siderophore-iron complex, which can then be taken up by the microorganism through specific transport systems. This allows them to acquire iron even in environments where it is present at very low concentrations or in forms that are not readily available for uptake. Siderophores play an important role in the survival and virulence of many pathogenic microorganisms, as they help them to obtain the iron they need to grow and multiply.

An erythrocyte transfusion, also known as a red blood cell (RBC) transfusion, is the process of transferring compatible red blood cells from a donor to a recipient. This procedure is typically performed to increase the recipient's oxygen-carrying capacity, usually in situations where there is significant blood loss, anemia, or impaired red blood cell production.

During the transfusion, the donor's red blood cells are collected, typed, and tested for compatibility with the recipient's blood to minimize the risk of a transfusion reaction. Once compatible units are identified, they are infused into the recipient's circulation through a sterile intravenous (IV) line. The recipient's body will eventually eliminate the donated red blood cells within 100-120 days as part of its normal turnover process.

Erythrocyte transfusions can be lifesaving in various clinical scenarios, such as trauma, surgery, severe anemia due to chronic diseases, and hematologic disorders. However, they should only be used when necessary, as there are potential risks associated with the procedure, including allergic reactions, transmission of infectious diseases, transfusion-related acute lung injury (TRALI), and iron overload in cases of multiple transfusions.

Ceruloplasmin is a protein found in blood plasma that binds and transports copper ions. It plays a crucial role in copper metabolism, including the oxidation of ferrous iron to ferric iron, which is necessary for the incorporation of iron into transferrin, another protein responsible for transporting iron throughout the body. Ceruloplasmin also acts as an antioxidant by scavenging free radicals and has been implicated in neurodegenerative disorders like Alzheimer's disease and Wilson's disease, a genetic disorder characterized by abnormal copper accumulation in various organs.

Ferrous compounds are inorganic substances that contain iron (Fe) in its +2 oxidation state. The term "ferrous" is derived from the Latin word "ferrum," which means iron. Ferrous compounds are often used in medicine, particularly in the treatment of iron-deficiency anemia due to their ability to provide bioavailable iron to the body.

Examples of ferrous compounds include ferrous sulfate, ferrous gluconate, and ferrous fumarate. These compounds are commonly found in dietary supplements and multivitamins. Ferrous sulfate is one of the most commonly used forms of iron supplementation, as it has a high iron content and is relatively inexpensive.

It's important to note that ferrous compounds can be toxic in large doses, so they should be taken under the guidance of a healthcare professional. Overdose can lead to symptoms such as nausea, vomiting, diarrhea, abdominal pain, and potentially fatal consequences if left untreated.

Iron-binding proteins, also known as transferrins, are a type of protein responsible for the transport and storage of iron in the body. They play a crucial role in maintaining iron homeostasis by binding free iron ions and preventing them from participating in harmful chemical reactions that can produce reactive oxygen species (ROS) and cause cellular damage.

Transferrin is the primary iron-binding protein found in blood plasma, while lactoferrin is found in various exocrine secretions such as milk, tears, and saliva. Both transferrin and lactoferrin have a similar structure, consisting of two lobes that can bind one ferric ion (Fe3+) each. When iron is bound to these proteins, they are called holo-transferrin or holo-lactoferrin; when they are unbound, they are referred to as apo-transferrin or apo-lactoferrin.

Iron-binding proteins have a high affinity for iron and can regulate the amount of free iron available in the body. They help prevent iron overload, which can lead to oxidative stress and cellular damage, as well as iron deficiency, which can result in anemia and other health problems.

In summary, iron-binding proteins are essential for maintaining iron homeostasis by transporting and storing iron ions, preventing them from causing harm to the body's cells.

Sideroblastic anemia is a type of anemia characterized by the presence of ringed sideroblasts in the bone marrow. Ringed sideroblasts are red blood cell precursors that have an abnormal amount of iron accumulated in their mitochondria, which forms a ring around the nucleus. This results in the production of abnormal hemoglobin and impaired oxygen transport.

Sideroblastic anemia can be classified as congenital or acquired. Congenital sideroblastic anemias are caused by genetic defects that affect heme synthesis or mitochondrial function, while acquired sideroblastic anemias are associated with various conditions such as myelodysplastic syndromes, chronic alcoholism, lead toxicity, and certain medications.

Symptoms of sideroblastic anemia may include fatigue, weakness, shortness of breath, and pallor. Diagnosis is typically made through a bone marrow aspiration and biopsy, which can identify the presence of ringed sideroblasts. Treatment depends on the underlying cause but may include iron chelation therapy, vitamin B6 supplementation, or blood transfusions.

Hypochromic anemia is a type of anemia characterized by the presence of red blood cells that have lower than normal levels of hemoglobin and appear paler in color than normal. Hemoglobin is a protein in red blood cells that carries oxygen from the lungs to the rest of the body. In hypochromic anemia, there may be a decrease in the production or increased destruction of red blood cells, leading to a reduced number of red blood cells and insufficient oxygen supply to the tissues.

Hypochromic anemia can result from various underlying medical conditions, including iron deficiency, thalassemia, chronic inflammation, lead poisoning, and certain infections or chronic diseases. Treatment for hypochromic anemia depends on the underlying cause and may include iron supplements, dietary changes, medications, or blood transfusions.

Apoferritins are the protein shells or apoproteins of ferritin molecules that are devoid of iron. Ferritin is a protein in cells that stores iron and releases it in a form that can be used by the body. Apoferritin can bind with iron ions to form ferritin. It has a hollow, spherical structure and is often used as a model for studying protein folding and assembly.

Anemia is a medical condition characterized by a lower than normal number of red blood cells or lower than normal levels of hemoglobin in the blood. Hemoglobin is an important protein in red blood cells that carries oxygen from the lungs to the rest of the body. Anemia can cause fatigue, weakness, shortness of breath, and a pale complexion because the body's tissues are not getting enough oxygen.

Anemia can be caused by various factors, including nutritional deficiencies (such as iron, vitamin B12, or folate deficiency), blood loss, chronic diseases (such as kidney disease or rheumatoid arthritis), inherited genetic disorders (such as sickle cell anemia or thalassemia), and certain medications.

There are different types of anemia, classified based on the underlying cause, size and shape of red blood cells, and the level of hemoglobin in the blood. Treatment for anemia depends on the underlying cause and may include dietary changes, supplements, medication, or blood transfusions.

A homozygote is an individual who has inherited the same allele (version of a gene) from both parents and therefore possesses two identical copies of that allele at a specific genetic locus. This can result in either having two dominant alleles (homozygous dominant) or two recessive alleles (homozygous recessive). In contrast, a heterozygote has inherited different alleles from each parent for a particular gene.

The term "homozygote" is used in genetics to describe the genetic makeup of an individual at a specific locus on their chromosomes. Homozygosity can play a significant role in determining an individual's phenotype (observable traits), as having two identical alleles can strengthen the expression of certain characteristics compared to having just one dominant and one recessive allele.

Triazoles are a class of antifungal medications that have broad-spectrum activity against various fungi, including yeasts, molds, and dermatophytes. They work by inhibiting the synthesis of ergosterol, an essential component of fungal cell membranes, leading to increased permeability and disruption of fungal growth. Triazoles are commonly used in both systemic and topical formulations for the treatment of various fungal infections, such as candidiasis, aspergillosis, cryptococcosis, and dermatophytoses. Some examples of triazole antifungals include fluconazole, itraconazole, voriconazole, and posaconazole.

The myocardium is the middle layer of the heart wall, composed of specialized cardiac muscle cells that are responsible for pumping blood throughout the body. It forms the thickest part of the heart wall and is divided into two sections: the left ventricle, which pumps oxygenated blood to the rest of the body, and the right ventricle, which pumps deoxygenated blood to the lungs.

The myocardium contains several types of cells, including cardiac muscle fibers, connective tissue, nerves, and blood vessels. The muscle fibers are arranged in a highly organized pattern that allows them to contract in a coordinated manner, generating the force necessary to pump blood through the heart and circulatory system.

Damage to the myocardium can occur due to various factors such as ischemia (reduced blood flow), infection, inflammation, or genetic disorders. This damage can lead to several cardiac conditions, including heart failure, arrhythmias, and cardiomyopathy.

Iron-regulatory proteins (IRPs) are specialized RNA-binding proteins that play a crucial role in the post-transcriptional regulation of iron homeostasis in mammalian cells. They are named as such because they regulate the expression of genes involved in iron metabolism, primarily by binding to specific cis-acting elements known as iron-responsive elements (IREs) located within the untranslated regions (UTRs) of target mRNAs.

There are two main IRPs: IRP1 and IRP2. Both proteins contain an N-terminal RNA-binding domain that recognizes and binds to IREs, as well as a C-terminal region involved in protein-protein interactions and other regulatory functions. Under conditions of iron deficiency or oxidative stress, IRPs become activated and bind to IREs, leading to changes in mRNA stability, translation, or both.

IRP1 can exist in two distinct conformational states: an active RNA-binding form (when iron levels are low) and an inactive aconitase form (when iron levels are sufficient). In contrast, IRP2 is primarily regulated by protein degradation, with its stability being modulated by the presence or absence of iron.

By binding to IREs within mRNAs encoding proteins involved in iron uptake, storage, and utilization, IRPs help maintain cellular iron homeostasis through a variety of mechanisms, including:

1. Promoting translation of transferrin receptor 1 (TfR1) mRNA to increase iron import when iron levels are low.
2. Inhibiting translation of ferritin heavy chain and light chain mRNAs to reduce iron storage when iron levels are low.
3. Stabilizing the mRNA encoding divalent metal transporter 1 (DMT1) to enhance iron uptake under conditions of iron deficiency.
4. Promoting degradation of transferrin receptor 2 (TfR2) and ferroportin mRNAs to limit iron import and export, respectively, when iron levels are high.

Overall, the regulation of iron metabolism by IRPs is crucial for maintaining proper cellular function and preventing the accumulation of toxic free radicals generated by iron-catalyzed reactions.

Liver diseases refer to a wide range of conditions that affect the normal functioning of the liver. The liver is a vital organ responsible for various critical functions such as detoxification, protein synthesis, and production of biochemicals necessary for digestion.

Liver diseases can be categorized into acute and chronic forms. Acute liver disease comes on rapidly and can be caused by factors like viral infections (hepatitis A, B, C, D, E), drug-induced liver injury, or exposure to toxic substances. Chronic liver disease develops slowly over time, often due to long-term exposure to harmful agents or inherent disorders of the liver.

Common examples of liver diseases include hepatitis, cirrhosis (scarring of the liver tissue), fatty liver disease, alcoholic liver disease, autoimmune liver diseases, genetic/hereditary liver disorders (like Wilson's disease and hemochromatosis), and liver cancers. Symptoms may vary widely depending on the type and stage of the disease but could include jaundice, abdominal pain, fatigue, loss of appetite, nausea, and weight loss.

Early diagnosis and treatment are essential to prevent progression and potential complications associated with liver diseases.

Hemoglobin (Hb or Hgb) is the main oxygen-carrying protein in the red blood cells, which are responsible for delivering oxygen throughout the body. It is a complex molecule made up of four globin proteins and four heme groups. Each heme group contains an iron atom that binds to one molecule of oxygen. Hemoglobin plays a crucial role in the transport of oxygen from the lungs to the body's tissues, and also helps to carry carbon dioxide back to the lungs for exhalation.

There are several types of hemoglobin present in the human body, including:

* Hemoglobin A (HbA): This is the most common type of hemoglobin, making up about 95-98% of total hemoglobin in adults. It consists of two alpha and two beta globin chains.
* Hemoglobin A2 (HbA2): This makes up about 1.5-3.5% of total hemoglobin in adults. It consists of two alpha and two delta globin chains.
* Hemoglobin F (HbF): This is the main type of hemoglobin present in fetal life, but it persists at low levels in adults. It consists of two alpha and two gamma globin chains.
* Hemoglobin S (HbS): This is an abnormal form of hemoglobin that can cause sickle cell disease when it occurs in the homozygous state (i.e., both copies of the gene are affected). It results from a single amino acid substitution in the beta globin chain.
* Hemoglobin C (HbC): This is another abnormal form of hemoglobin that can cause mild to moderate hemolytic anemia when it occurs in the homozygous state. It results from a different single amino acid substitution in the beta globin chain than HbS.

Abnormal forms of hemoglobin, such as HbS and HbC, can lead to various clinical disorders, including sickle cell disease, thalassemia, and other hemoglobinopathies.

Homeostasis is a fundamental concept in the field of medicine and physiology, referring to the body's ability to maintain a stable internal environment, despite changes in external conditions. It is the process by which biological systems regulate their internal environment to remain in a state of dynamic equilibrium. This is achieved through various feedback mechanisms that involve sensors, control centers, and effectors, working together to detect, interpret, and respond to disturbances in the system.

For example, the body maintains homeostasis through mechanisms such as temperature regulation (through sweating or shivering), fluid balance (through kidney function and thirst), and blood glucose levels (through insulin and glucagon secretion). When homeostasis is disrupted, it can lead to disease or dysfunction in the body.

In summary, homeostasis is the maintenance of a stable internal environment within biological systems, through various regulatory mechanisms that respond to changes in external conditions.

Porphyria Cutanea Tarda (PCT) is a type of porphyria, a group of rare genetic disorders that affect the production of heme, a component in hemoglobin. PCT is primarily an acquired disorder, although it can have a hereditary component as well.

In PCT, there is a dysfunction in the enzyme uroporphyrinogen decarboxylase (UROD), which leads to the accumulation of porphyrins and porphyrin precursors in the skin. This buildup causes the characteristic symptoms of PCT, which include:

* Blisters, particularly on sun-exposed areas such as the hands and face
* Fragile, thin skin that tears easily
* Scarring
* Hypertrichosis (abnormal hair growth)
* Changes in skin color, including redness, increased pigmentation, or loss of pigment

PCT is typically triggered by factors such as alcohol consumption, estrogen use, hepatitis C infection, and exposure to certain chemicals. Treatment often involves addressing these triggers, along with the use of phlebotomy (removal of blood) or low-dose hydroxychloroquine to reduce porphyrin levels in the body.

It's important to note that PCT is a complex disorder and its diagnosis and management should be done by healthcare professionals with experience in managing porphyrias.

Cardiomegaly is a medical term that refers to an enlarged heart. It can be caused by various conditions such as high blood pressure, heart valve problems, cardiomyopathy, or fluid accumulation around the heart (pericardial effusion). Cardiomegaly can be detected through imaging tests like chest X-rays or echocardiograms. Depending on the underlying cause, treatment options may include medications, lifestyle changes, or in some cases, surgery. It is important to consult with a healthcare professional for proper diagnosis and treatment.

Sickle cell anemia is a genetic disorder that affects the hemoglobin in red blood cells. Hemoglobin is responsible for carrying oxygen throughout the body. In sickle cell anemia, the hemoglobin is abnormal and causes the red blood cells to take on a sickle shape, rather than the normal disc shape. These sickled cells are stiff and sticky, and they can block blood vessels, causing tissue damage and pain. They also die more quickly than normal red blood cells, leading to anemia.

People with sickle cell anemia often experience fatigue, chronic pain, and jaundice. They may also have a higher risk of infections and complications such as stroke, acute chest syndrome, and priapism. The disease is inherited from both parents, who must both be carriers of the sickle cell gene. It primarily affects people of African descent, but it can also affect people from other ethnic backgrounds.

There is no cure for sickle cell anemia, but treatments such as blood transfusions, medications to manage pain and prevent complications, and bone marrow transplantation can help improve quality of life for affected individuals. Regular medical care and monitoring are essential for managing the disease effectively.

Liver cirrhosis is a chronic, progressive disease characterized by the replacement of normal liver tissue with scarred (fibrotic) tissue, leading to loss of function. The scarring is caused by long-term damage from various sources such as hepatitis, alcohol abuse, nonalcoholic fatty liver disease, and other causes. As the disease advances, it can lead to complications like portal hypertension, fluid accumulation in the abdomen (ascites), impaired brain function (hepatic encephalopathy), and increased risk of liver cancer. It is generally irreversible, but early detection and treatment of underlying causes may help slow down its progression.

Iron Regulatory Protein 1 (IRP1) is a protein that plays a crucial role in the post-transcriptional regulation of iron homeostasis in cells. It is involved in the detection of cellular iron levels and responds by modulating the translation and stability of messenger RNAs (mRNAs) that encode proteins essential for iron metabolism.

IRP1 can bind to specific sequences called Iron Responsive Elements (IREs) present in the untranslated regions of mRNAs. When cellular iron levels are low, IRP1 binds to IREs and inhibits the translation of mRNAs encoding proteins responsible for iron uptake and storage, while stabilizing mRNAs that encode proteins involved in iron mobilization. Conversely, when iron levels are high, IRP1 dissociates from IREs, allowing for the normal translation of these mRNAs and maintaining iron homeostasis within the cell.

It is important to note that IRP1 has dual functions: it can act as an Iron Regulatory Protein (IRP) when iron levels are low, and as a cytosolic aconitase (an enzyme in the citric acid cycle) when iron levels are sufficient. This ability to switch between these two roles is facilitated by the presence of a [4Fe-4S] cluster, which is sensitive to cellular iron levels. When iron is abundant, the [4Fe-4S] cluster assembles, converting IRP1 into its cytosolic aconitase form; when iron is scarce, the cluster disassembles, enabling IRP1 to bind IREs and regulate iron metabolism-related gene expression.

Myelodysplastic syndromes (MDS) are a group of diverse bone marrow disorders characterized by dysplasia (abnormal development or maturation) of one or more types of blood cells or by ineffective hematopoiesis, resulting in cytopenias (lower than normal levels of one or more types of blood cells). MDS can be classified into various subtypes based on the number and type of cytopenias, the degree of dysplasia, the presence of ring sideroblasts, and cytogenetic abnormalities.

The condition primarily affects older adults, with a median age at diagnosis of around 70 years. MDS can evolve into acute myeloid leukemia (AML) in approximately 30-40% of cases. The pathophysiology of MDS involves genetic mutations and chromosomal abnormalities that lead to impaired differentiation and increased apoptosis of hematopoietic stem and progenitor cells, ultimately resulting in cytopenias and an increased risk of developing AML.

The diagnosis of MDS typically requires a bone marrow aspiration and biopsy, along with cytogenetic and molecular analyses to identify specific genetic mutations and chromosomal abnormalities. Treatment options for MDS depend on the subtype, severity of cytopenias, and individual patient factors. These may include supportive care measures, such as transfusions and growth factor therapy, or more aggressive treatments, such as chemotherapy and stem cell transplantation.

Cardiomyopathies are a group of diseases that affect the heart muscle, leading to mechanical and/or electrical dysfunction. The American Heart Association (AHA) defines cardiomyopathies as "a heterogeneous group of diseases of the myocardium associated with mechanical and/or electrical dysfunction that usually (but not always) exhibit inappropriate ventricular hypertrophy or dilatation and frequently lead to heart failure."

There are several types of cardiomyopathies, including:

1. Dilated cardiomyopathy (DCM): This is the most common type of cardiomyopathy, characterized by an enlarged left ventricle and impaired systolic function, leading to heart failure.
2. Hypertrophic cardiomyopathy (HCM): In this type, there is abnormal thickening of the heart muscle, particularly in the septum between the two ventricles, which can obstruct blood flow and increase the risk of arrhythmias.
3. Restrictive cardiomyopathy (RCM): This is a rare form of cardiomyopathy characterized by stiffness of the heart muscle, impaired relaxation, and diastolic dysfunction, leading to reduced filling of the ventricles and heart failure.
4. Arrhythmogenic right ventricular cardiomyopathy (ARVC): In this type, there is replacement of the normal heart muscle with fatty or fibrous tissue, primarily affecting the right ventricle, which can lead to arrhythmias and sudden cardiac death.
5. Unclassified cardiomyopathies: These are conditions that do not fit into any of the above categories but still significantly affect the heart muscle and function.

Cardiomyopathies can be caused by genetic factors, acquired conditions (e.g., infections, toxins, or autoimmune disorders), or a combination of both. The diagnosis typically involves a comprehensive evaluation, including medical history, physical examination, electrocardiogram (ECG), echocardiography, cardiac magnetic resonance imaging (MRI), and sometimes genetic testing. Treatment depends on the type and severity of the condition but may include medications, lifestyle modifications, implantable devices, or even heart transplantation in severe cases.

Erythropoiesis is the process of forming and developing red blood cells (erythrocytes) in the body. It occurs in the bone marrow and is regulated by the hormone erythropoietin (EPO), which is produced by the kidneys. Erythropoiesis involves the differentiation and maturation of immature red blood cell precursors called erythroblasts into mature red blood cells, which are responsible for carrying oxygen to the body's tissues. Disorders that affect erythropoiesis can lead to anemia or other blood-related conditions.

Intestinal absorption refers to the process by which the small intestine absorbs water, nutrients, and electrolytes from food into the bloodstream. This is a critical part of the digestive process, allowing the body to utilize the nutrients it needs and eliminate waste products. The inner wall of the small intestine contains tiny finger-like projections called villi, which increase the surface area for absorption. Nutrients are absorbed into the bloodstream through the walls of the capillaries in these villi, and then transported to other parts of the body for use or storage.

A mutation is a permanent change in the DNA sequence of an organism's genome. Mutations can occur spontaneously or be caused by environmental factors such as exposure to radiation, chemicals, or viruses. They may have various effects on the organism, ranging from benign to harmful, depending on where they occur and whether they alter the function of essential proteins. In some cases, mutations can increase an individual's susceptibility to certain diseases or disorders, while in others, they may confer a survival advantage. Mutations are the driving force behind evolution, as they introduce new genetic variability into populations, which can then be acted upon by natural selection.

Animal disease models are specialized animals, typically rodents such as mice or rats, that have been genetically engineered or exposed to certain conditions to develop symptoms and physiological changes similar to those seen in human diseases. These models are used in medical research to study the pathophysiology of diseases, identify potential therapeutic targets, test drug efficacy and safety, and understand disease mechanisms.

The genetic modifications can include knockout or knock-in mutations, transgenic expression of specific genes, or RNA interference techniques. The animals may also be exposed to environmental factors such as chemicals, radiation, or infectious agents to induce the disease state.

Examples of animal disease models include:

1. Mouse models of cancer: Genetically engineered mice that develop various types of tumors, allowing researchers to study cancer initiation, progression, and metastasis.
2. Alzheimer's disease models: Transgenic mice expressing mutant human genes associated with Alzheimer's disease, which exhibit amyloid plaque formation and cognitive decline.
3. Diabetes models: Obese and diabetic mouse strains like the NOD (non-obese diabetic) or db/db mice, used to study the development of type 1 and type 2 diabetes, respectively.
4. Cardiovascular disease models: Atherosclerosis-prone mice, such as ApoE-deficient or LDLR-deficient mice, that develop plaque buildup in their arteries when fed a high-fat diet.
5. Inflammatory bowel disease models: Mice with genetic mutations affecting intestinal barrier function and immune response, such as IL-10 knockout or SAMP1/YitFc mice, which develop colitis.

Animal disease models are essential tools in preclinical research, but it is important to recognize their limitations. Differences between species can affect the translatability of results from animal studies to human patients. Therefore, researchers must carefully consider the choice of model and interpret findings cautiously when applying them to human diseases.

Oxidative stress is defined as an imbalance between the production of reactive oxygen species (free radicals) and the body's ability to detoxify them or repair the damage they cause. This imbalance can lead to cellular damage, oxidation of proteins, lipids, and DNA, disruption of cellular functions, and activation of inflammatory responses. Prolonged or excessive oxidative stress has been linked to various health conditions, including cancer, cardiovascular diseases, neurodegenerative disorders, and aging-related diseases.

Ferrocyanides are salts or complex ions containing the ferrocyanide ion (Fe(CN)2-4). The ferrocyanide ion is a stable, soluble, and brightly colored complex that contains iron in the +2 oxidation state coordinated to four cyanide ligands. Ferrocyanides are commonly used in various industrial applications such as water treatment, chemical synthesis, and photography due to their stability and reactivity. However, they can be toxic if ingested or inhaled in large quantities, so proper handling and disposal procedures should be followed.

Beer is a fermented alcoholic beverage typically made from malted barley, hops, water, and yeast. The brewing process involves steeping the malt in water to create a sugary solution called wort, which is then boiled with hops for flavor and preservation. After cooling, the wort is fermented with yeast, which converts the sugar into alcohol and carbon dioxide. There are many varieties of beer, including ales, lagers, stouts, and porters, that differ in their ingredients, brewing methods, and flavor profiles. The alcohol content of beer generally ranges from 3% to 12% ABV (alcohol by volume).

The Mononuclear Phagocyte System (MPS) is a network of specialized immune cells distributed throughout the body, primarily consisting of monocytes, macrophages, and dendritic cells. These cells share a common bone marrow-derived precursor and play crucial roles in innate and adaptive immunity. They are involved in various functions such as:

1. Phagocytosis: engulfing and destroying foreign particles, microbes, and cellular debris.
2. Antigen presentation: processing and presenting antigens to T-cells to initiate an adaptive immune response.
3. Cytokine production: releasing pro- and anti-inflammatory cytokines to regulate immune responses and maintain tissue homeostasis.
4. Immune regulation: modulating the activity of other immune cells, including T-cells, B-cells, and natural killer (NK) cells.

The MPS is essential for maintaining tissue integrity, fighting infections, and orchestrating immune responses. Its components are found in various tissues, including the liver (Kupffer cells), spleen, lymph nodes, bone marrow, and connective tissues.

HLA (Human Leukocyte Antigen) antigens are a group of proteins found on the surface of cells in our body. They play a crucial role in the immune system's ability to differentiate between "self" and "non-self." HLA antigens are encoded by a group of genes located on chromosome 6, known as the major histocompatibility complex (MHC).

There are three types of HLA antigens: HLA class I, HLA class II, and HLA class III. HLA class I antigens are found on the surface of almost all cells in the body and help the immune system recognize and destroy virus-infected or cancerous cells. They consist of three components: HLA-A, HLA-B, and HLA-C.

HLA class II antigens are primarily found on the surface of immune cells, such as macrophages, B cells, and dendritic cells. They assist in the presentation of foreign particles (like bacteria and viruses) to CD4+ T cells, which then activate other parts of the immune system. HLA class II antigens include HLA-DP, HLA-DQ, and HLA-DR.

HLA class III antigens consist of various molecules involved in immune responses, such as cytokines and complement components. They are not directly related to antigen presentation.

The genetic diversity of HLA antigens is extensive, with thousands of variations or alleles. This diversity allows for a better ability to recognize and respond to a wide range of pathogens. However, this variation can also lead to compatibility issues in organ transplantation, as the recipient's immune system may recognize the donor's HLA antigens as foreign and attack the transplanted organ.

Medical Definition:

Magnetic Resonance Imaging (MRI) is a non-invasive diagnostic imaging technique that uses a strong magnetic field and radio waves to create detailed cross-sectional or three-dimensional images of the internal structures of the body. The patient lies within a large, cylindrical magnet, and the scanner detects changes in the direction of the magnetic field caused by protons in the body. These changes are then converted into detailed images that help medical professionals to diagnose and monitor various medical conditions, such as tumors, injuries, or diseases affecting the brain, spinal cord, heart, blood vessels, joints, and other internal organs. MRI does not use radiation like computed tomography (CT) scans.

Iron Regulatory Protein 2 (IRP2) is a regulatory protein involved in the post-transcriptional control of iron homeostasis. It binds to specific sequences called Iron Responsive Elements (IREs) found in the untranslated regions of mRNAs encoding proteins involved in iron metabolism, such as ferritin and transferrin receptor.

When cellular iron levels are low, IRP2 binds to the IREs and prevents the degradation of iron-related mRNAs, leading to increased synthesis of iron uptake proteins and decreased synthesis of iron storage proteins. Conversely, when iron levels are high, IRP2 is degraded, allowing for the normal turnover and translation of these mRNAs.

IRP2 plays a crucial role in maintaining appropriate intracellular iron concentrations and protecting cells from iron-induced oxidative stress. Dysregulation of IRP2 has been implicated in various diseases, including anemia, neurodegenerative disorders, and cancer.

The duodenum is the first part of the small intestine, immediately following the stomach. It is a C-shaped structure that is about 10-12 inches long and is responsible for continuing the digestion process that begins in the stomach. The duodenum receives partially digested food from the stomach through the pyloric valve and mixes it with digestive enzymes and bile produced by the pancreas and liver, respectively. These enzymes help break down proteins, fats, and carbohydrates into smaller molecules, allowing for efficient absorption in the remaining sections of the small intestine.

Ethyl ether, also known as diethyl ether or simply ether, is a type of organic compound that is classified as a simple ether. It is a colorless and highly volatile liquid with a characteristic odor that is often described as sweet or fruity. In medical contexts, ethyl ether has been historically used as an anesthetic agent due to its ability to produce unconsciousness and insensitivity to pain when inhaled. However, its use as an anesthetic has largely been replaced by safer and more effective alternatives due to its flammability, explosiveness, and potential for causing serious adverse effects such as heart problems and liver damage.

Ethyl ether is a simple ether consisting of two ethyl groups (-C2H5) linked to an oxygen atom (O), with the molecular formula C4H10O. It is produced by the reaction of ethanol with sulfuric acid, followed by distillation to separate the resulting ethyl ether from other products.

In addition to its historical use as an anesthetic, ethyl ether has been used in various industrial and laboratory applications, such as a solvent for fats, oils, resins, and waxes, and as a starting material for the synthesis of other chemicals. However, due to its flammability and potential for causing harm, it is important to handle ethyl ether with care and follow appropriate safety precautions when using it.

C57BL/6 (C57 Black 6) is an inbred strain of laboratory mouse that is widely used in biomedical research. The term "inbred" refers to a strain of animals where matings have been carried out between siblings or other closely related individuals for many generations, resulting in a population that is highly homozygous at most genetic loci.

The C57BL/6 strain was established in 1920 by crossing a female mouse from the dilute brown (DBA) strain with a male mouse from the black strain. The resulting offspring were then interbred for many generations to create the inbred C57BL/6 strain.

C57BL/6 mice are known for their robust health, longevity, and ease of handling, making them a popular choice for researchers. They have been used in a wide range of biomedical research areas, including studies of cancer, immunology, neuroscience, cardiovascular disease, and metabolism.

One of the most notable features of the C57BL/6 strain is its sensitivity to certain genetic modifications, such as the introduction of mutations that lead to obesity or impaired glucose tolerance. This has made it a valuable tool for studying the genetic basis of complex diseases and traits.

Overall, the C57BL/6 inbred mouse strain is an important model organism in biomedical research, providing a valuable resource for understanding the genetic and molecular mechanisms underlying human health and disease.

A biopsy is a medical procedure in which a small sample of tissue is taken from the body to be examined under a microscope for the presence of disease. This can help doctors diagnose and monitor various medical conditions, such as cancer, infections, or autoimmune disorders. The type of biopsy performed will depend on the location and nature of the suspected condition. Some common types of biopsies include:

1. Incisional biopsy: In this procedure, a surgeon removes a piece of tissue from an abnormal area using a scalpel or other surgical instrument. This type of biopsy is often used when the lesion is too large to be removed entirely during the initial biopsy.

2. Excisional biopsy: An excisional biopsy involves removing the entire abnormal area, along with a margin of healthy tissue surrounding it. This technique is typically employed for smaller lesions or when cancer is suspected.

3. Needle biopsy: A needle biopsy uses a thin, hollow needle to extract cells or fluid from the body. There are two main types of needle biopsies: fine-needle aspiration (FNA) and core needle biopsy. FNA extracts loose cells, while a core needle biopsy removes a small piece of tissue.

4. Punch biopsy: In a punch biopsy, a round, sharp tool is used to remove a small cylindrical sample of skin tissue. This type of biopsy is often used for evaluating rashes or other skin abnormalities.

5. Shave biopsy: During a shave biopsy, a thin slice of tissue is removed from the surface of the skin using a sharp razor-like instrument. This technique is typically used for superficial lesions or growths on the skin.

After the biopsy sample has been collected, it is sent to a laboratory where a pathologist will examine the tissue under a microscope and provide a diagnosis based on their findings. The results of the biopsy can help guide further treatment decisions and determine the best course of action for managing the patient's condition.

Heme is not a medical term per se, but it is a term used in the field of medicine and biology. Heme is a prosthetic group found in hemoproteins, which are proteins that contain a heme iron complex. This complex plays a crucial role in various biological processes, including oxygen transport (in hemoglobin), electron transfer (in cytochromes), and chemical catalysis (in peroxidases and catalases).

The heme group consists of an organic component called a porphyrin ring, which binds to a central iron atom. The iron atom can bind or release electrons, making it essential for redox reactions in the body. Heme is also vital for the formation of hemoglobin and myoglobin, proteins responsible for oxygen transport and storage in the blood and muscles, respectively.

In summary, heme is a complex organic-inorganic structure that plays a critical role in several biological processes, particularly in electron transfer and oxygen transport.

Chelating agents are substances that can bind and form stable complexes with certain metal ions, preventing them from participating in chemical reactions. In medicine, chelating agents are used to remove toxic or excessive amounts of metal ions from the body. For example, ethylenediaminetetraacetic acid (EDTA) is a commonly used chelating agent that can bind with heavy metals such as lead and mercury, helping to eliminate them from the body and reduce their toxic effects. Other chelating agents include dimercaprol (BAL), penicillamine, and deferoxamine. These agents are used to treat metal poisoning, including lead poisoning, iron overload, and copper toxicity.

"Cebus" is a genus of New World monkeys, also known as capuchin monkeys. They are small to medium-sized primates that are native to Central and South America. Capuchin monkeys are named after the Order of Friars Minor Capuchin, because of their similarity in color to the robes worn by the friars.

Capuchin monkeys are highly intelligent and social animals, living in groups of up to 30 individuals. They have a diverse diet that includes fruits, nuts, seeds, insects, and small vertebrates. Capuchin monkeys are known for their problem-solving abilities and have been observed using tools in the wild.

There are several species of capuchin monkeys, including the white-fronted capuchin (Cebus albifrons), the tufted capuchin (Cebus apella), and the weeper capuchin (Cebus olivaceus). They vary in size, coloration, and behavior, but all share the characteristic cap of hair on their heads that gives them their name.

Inborn errors of metal metabolism refer to genetic disorders that affect the way the body processes and handles certain metallic elements. These disorders can result in an accumulation or deficiency of specific metals, leading to various clinical manifestations. Examples of such conditions include:

1. Wilson's disease: An autosomal recessive disorder caused by a mutation in the ATP7B gene, which results in abnormal copper metabolism and accumulation in various organs, particularly the liver and brain.
2. Menkes disease: An X-linked recessive disorder caused by a mutation in the ATP7A gene, leading to impaired copper transport and deficiency, affecting the brain, bones, and connective tissue.
3. Hemochromatosis: An autosomal recessive disorder characterized by excessive iron absorption and deposition in various organs, causing damage to the liver, heart, and pancreas.
4. Acrodermatitis enteropathica: A rare autosomal recessive disorder caused by a mutation in the SLC39A4 gene, resulting in zinc deficiency and affecting the skin, gastrointestinal system, and immune function.
5. Disturbances in manganese metabolism: Rare genetic disorders that can lead to either manganese accumulation or deficiency, causing neurological symptoms.

These conditions often require specialized medical management, including dietary modifications, chelation therapy, and/or supplementation to maintain appropriate metal homeostasis and prevent organ damage.

Genotype, in genetics, refers to the complete heritable genetic makeup of an individual organism, including all of its genes. It is the set of instructions contained in an organism's DNA for the development and function of that organism. The genotype is the basis for an individual's inherited traits, and it can be contrasted with an individual's phenotype, which refers to the observable physical or biochemical characteristics of an organism that result from the expression of its genes in combination with environmental influences.

It is important to note that an individual's genotype is not necessarily identical to their genetic sequence. Some genes have multiple forms called alleles, and an individual may inherit different alleles for a given gene from each parent. The combination of alleles that an individual inherits for a particular gene is known as their genotype for that gene.

Understanding an individual's genotype can provide important information about their susceptibility to certain diseases, their response to drugs and other treatments, and their risk of passing on inherited genetic disorders to their offspring.

Bone Morphogenetic Protein 6 (BMP-6) is a member of the transforming growth factor-beta (TGF-β) superfamily of proteins. It plays crucial roles in bone and cartilage formation, as well as in the regulation of iron metabolism. BMP-6 stimulates the differentiation of mesenchymal stem cells into osteoblasts, which are bone-forming cells, and contributes to the maintenance of bone homeostasis. Additionally, BMP-6 is involved in the process of hepcidin regulation, a hormone that controls iron absorption and recycling in the body. Dysregulation of BMP-6 has been implicated in various diseases, including skeletal disorders and iron metabolism-related conditions.

Fatty liver, also known as hepatic steatosis, is a medical condition characterized by the abnormal accumulation of fat in the liver. The liver's primary function is to process nutrients, filter blood, and fight infections, among other tasks. When excess fat builds up in the liver cells, it can impair liver function and lead to inflammation, scarring, and even liver failure if left untreated.

Fatty liver can be caused by various factors, including alcohol consumption, obesity, nonalcoholic fatty liver disease (NAFLD), viral hepatitis, and certain medications or medical conditions. NAFLD is the most common cause of fatty liver in the United States and other developed countries, affecting up to 25% of the population.

Symptoms of fatty liver may include fatigue, weakness, weight loss, loss of appetite, nausea, abdominal pain or discomfort, and jaundice (yellowing of the skin and eyes). However, many people with fatty liver do not experience any symptoms, making it essential to diagnose and manage the condition through regular check-ups and blood tests.

Treatment for fatty liver depends on the underlying cause. Lifestyle changes such as weight loss, exercise, and dietary modifications are often recommended for people with NAFLD or alcohol-related fatty liver disease. Medications may also be prescribed to manage related conditions such as diabetes, high cholesterol, or metabolic syndrome. In severe cases of liver damage, a liver transplant may be necessary.

A "knockout" mouse is a genetically engineered mouse in which one or more genes have been deleted or "knocked out" using molecular biology techniques. This allows researchers to study the function of specific genes and their role in various biological processes, as well as potential associations with human diseases. The mice are generated by introducing targeted DNA modifications into embryonic stem cells, which are then used to create a live animal. Knockout mice have been widely used in biomedical research to investigate gene function, disease mechanisms, and potential therapeutic targets.

Friedreich Ataxia is a genetic disorder that affects the nervous system and causes issues with movement. It is characterized by progressive damage to the nerves (neurons) in the spinal cord and peripheral nerves, which can lead to problems with muscle coordination, gait, speech, and hearing. The condition is also associated with heart disorders, diabetes, and vision impairment.

Friedreich Ataxia is caused by a mutation in the FXN gene, which provides instructions for making a protein called frataxin. This protein plays a role in the production of energy within cells, particularly in the mitochondria. The mutation in the FXN gene leads to reduced levels of frataxin, which can cause nerve damage and other symptoms associated with Friedreich Ataxia.

The condition typically begins in childhood or early adulthood and progresses over time, often leading to significant disability. There is currently no cure for Friedreich Ataxia, but treatments are available to help manage the symptoms and improve quality of life.

Once iron overload has been established, HFE gene mutation genetic testing for hereditary causes of iron overload is indicated ... Human iron metabolism Iron deficiency Hsu CC, Senussi NH, Fertrin KY, Kowdley KV (June 2022). "Iron overload disorders". ... Source for mesenchymal versus parenchymal iron overload Deugnier Y, Turlin B (2007). "Pathology of hepatic iron overload". ... The human diet contains iron in two forms: heme iron and non-heme iron. Heme iron is usually found in red meat, whereas non- ...
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"Iron Overload. Medical information about Iron Overload , Patient". Patient. Retrieved 2016-01-29. Heimpel, Hermann; ... This is true for ferritin levels and iron levels in the organs as well, it is important for patients to go regularly for ... It is more important to check iron levels in the organs through MRI scans, however, than to simply get regular blood tests to ... Iron Storage Disease) , NCBDDD , CDC". www.cdc.gov. Retrieved 2016-01-29. "Gene Therapy is 'Becoming a Clinical Reality'". " ...
Iron overload (hemochromatosis). Diet: Malnutrition, particularly vitamin A and E deficiencies, can worsen alcohol-induced ...
PDF version Smith RS (August 1965). "Iron Deficiency and Iron Overload". Archives of Disease in Childhood. 40 (212): 343-363. ... Pancreatin reduces the absorption of iron from food in the duodenum during digestion. Some contact lens-cleaning solutions ...
In the case of iron overload, which may occur in individuals with β-thalessemia who have received blood transfusions, the ... Iron: Effect of Overload and Deficiency". In Astrid Sigel, Helmut Sigel and Roland K. O. Sigel (ed.). Interrelations between ... ISBN 978-0-87983-730-3. Iron-Selective Chelators With Therapeutic Potential in Hider, Robert C.; Kong, Xiaole (2013). "Chapter ...
Crisponi, Guido; Nurchi, Valeria M.; Lachowicz, Joanna (2019). "Chapter 3. Iron Chelation for Iron Overload in Thalassemia". In ... Hider, Robert C.; Kong, Xiaole (2013). "Chapter 8. Iron: Effect of Overload and Deficiency". In Astrid Sigel, Helmut Sigel and ... Since the 1970s, iron chelation therapy has been used as an alternative to regular phlebotomy to treat excess iron stores in ... Only the three-year-old girl had been found to have an elevated blood lead level and resulting low iron levels and anemia, ...
... and iron, in Hider, Robert C.; Kong, Xiaole (2013). "Chapter 8. Iron: Effect of Overload and Deficiency". In ...
Certain mutations in the HFE gene cause hemochromatosis (an iron overload disorder). People who have these mutations are also ... Additional risk factors include alcohol use disorder, excess iron (from iron supplements as well as cooking on cast iron ... A borderline iron deficiency has been found to have a protective effect by limiting heme synthesis. In the absence of iron, ... Sampietro, M; Fiorelli, G; Fargion, S (1999). "Iron overload in porphyria cutanea tarda". Haematologica. 84 (3): 248-53. PMID ...
This involves delivering iron chelating agents such as deferoxamine, deferiprone or deferasirox. If iron overload has caused ... Lu JP, Hayashi K (1994). "Selective iron deposition in pancreatic islet B cells of transfusional iron-overloaded autopsy cases ... Hider, Robert C.; Kong, Xiaole (2013). "Chapter 8. Iron: Effect of Overload and Deficiency". In Astrid Sigel, Helmut Sigel and ... Shander, A.; Cappellini, M. D.; Goodnough, L. T. (2009). "Iron overload and toxicity: the hidden risk of multiple blood ...
The iron overload aspect of the disorder means that the iron accumulates in the liver and can cause liver impairment in ... Hypochromic anemia may be caused by vitamin B6 deficiency from a low iron intake, diminished iron absorption, or excessive iron ... Hypochromic anemia occurs in patients with hypochromic microcytic anemia with iron overload. The condition is autosomal ... "hypochromic microcytic anemia with iron overload". Genetics Home Reference. Retrieved 2016-10-29. Hershko, Chaim; Camaschella, ...
A quick response to iron poisoning can significantly improve clinical outcomes. Overnutrition Iron overload Manoguerra, Anthony ... Any excess iron is stored as ferritin in the liver. In the event of iron overdose, iron stores become oversaturated and the ... Iron poisoning in General Practice Notebook Iron Poisoning at WebMD Iron Poisoning Merck Manual (Articles with short ... When being transported throughout the body, iron is bound to an iron transporting protein called transferrin to prevent iron ...
Iron overload: People with thalassemia can get an overload of iron in their bodies, either from the disease itself or from ... Complications may include iron overload from the transfusions with resulting heart or liver disease, infections, and ... Cianciulli P (October 2008). "Treatment of iron overload in thalassemia". Pediatr Endocrinol Rev. 6 (Suppl 1): 208-13. PMID ... Without adequate iron chelation therapy, almost all patients with beta-thalassemia accumulate potentially fatal iron levels. ...
... elevated plasma iron and transferrin saturation levels, and iron overload, mainly in hepatocytes. Mice have also been created ... Iron-related traits of Hfe−/− mice, including increased iron absorption and hepatic iron loading, are inherited in an autosomal ... Ombiga J, Adams LA, Tang K, Trinder D, Olynyk JK (November 2005). "Screening for HFE and iron overload". Seminars in Liver ... The black rhinoceros (Diceros bicornis) can develop iron overload. To determine whether the HFE gene of black rhinoceroses has ...
... which can be problematic in cases of iron overload. The ferritin storage iron pool is much larger than the labile iron pool, ... If systemic iron overload is corrected, over time the hemosiderin is slowly resorbed by the macrophages. Human iron homeostasis ... The ubiquity of Iron in life has led to the Iron-sulfur world hypothesis that Iron was a central component of the environment ... Iron can also be oxidized by marine microbes under conditions that are high in iron and low in oxygen. Iron can enter marine ...
... even more iron can enter the bloodstream and cause a potentially deadly syndrome of iron overload. Large amounts of free iron ... However, some of the intracellular iron is bound to low-affinity complexes, and is termed labile iron or "free" iron. Iron in ... which can be problematic in cases of iron overload. The ferritin storage iron pool is much larger than the labile iron pool, ... If systemic iron overload is corrected, over time the hemosiderin is slowly resorbed by the macrophages. Human iron homeostasis ...
Concerns from repeated blood transfusions include iron overload. This iron overload may require chelation therapy. The global ... Iron deficiency due to inadequate dietary iron intake is rare in men and postmenopausal women. The diagnosis of iron deficiency ... parenteral iron preparations can be used. Parenteral iron can improve iron stores rapidly and is also effective for treating ... this decreases the amount of iron absorbed. Vitamin C aids in the body's ability to absorb iron, so taking oral iron ...
S. "Iron overload in ß2 microglobulin deficient mice". Immunol. Lett. 39: 105-111. 1994. Santos, M., Schilham, MW, Rademakers, ... In the 1980s she focused on the study of hereditary hemochromatosis, an iron overload genetic disease. Maria de Sousa was born ... An interest in the non-immunological functions of lymphocytes, such as iron metabolism led her to study hemochromatosis, which ... "An outsider's perspective-ecotaxis revisited: an integrative review of cancer environment, iron and immune system cells". ...
Systemic iron overload results, and liver iron deposition is primarily in the hepatocytes. Diagnosis is based upon ... Iron in the bloodstream is then bound by transferrin, which carries the iron to target cells. Iron is stored in cells and blood ... Hemochromatosis type 4 is a hereditary iron overload disorder that affects ferroportin, an iron transport protein needed to ... In normal iron regulation, iron is absorbed in the intestine, and ferroportin transports iron from the cells of the intestinal ...
1 August 1999). "Iron overload in urban Africans in the 1990s". Gut. 45 (2): 278-283. doi:10.1136/GUT.45.2.278. ISSN 0017-5749 ... "Serum transferrin receptors are decreased in the presence of iron overload". Clinical Chemistry. 44 (1): 40-44. ISSN 0009-9147 ... His areas of interest include colorectal cancer and African iron excess as well as gastroenterological and hepatological ... 17 August 2001). "Association of pulmonary tuberculosis with increased dietary iron". The Journal of Infectious Diseases. 184 ( ...
... is an oral iron chelator. Its main use is to reduce chronic iron overload in patients who are receiving long-term blood ... "FDA Approves First Oral Drug for Chronic Iron Overload" (Press release). United States Food and Drug Administration. November 9 ... Choudhry VP, Naithani R (August 2007). "Current status of iron overload and chelation with deferasirox". Indian Journal of ... for children six years and older for chronic iron overload from repeated blood transfusions. It is on the World Health ...
In the absence of regular iron chelation therapy, the iron loading rates vary. Monitoring of transfusion iron overload is ... Source for mesenchymal versus parenchymal iron overload Deugnier Y, Turlin B (2007). "Pathology of hepatic iron overload". ... Iron overload occurs when iron intake is increased over a sustained period of time due to regular transfusion of whole blood ... Brown induration Iron overload Siderosis Lu JP, Hayashi K (1995). "Transferrin receptor distribution and iron deposition in the ...
2008-01-17). "Iron-Overload-Related Disease in HFE Hereditary Hemochromatosis" (PDF). New England Journal of Medicine. 358 (3 ... excess absorption of iron. These variants all reflect a failure in a metabolic pathway associated with iron metabolism, however ... Compound heterozygotes are often observed only through subclinical symptoms such as excess iron. Disease is rarely observed in ... "Compound heterozygous hemochromatosis genotype predicts increased iron and erythrocyte indices in women". Clinical Chemistry. ...
Franchini M (2006). "Hereditary iron overload: update on pathophysiology, diagnosis, and treatment". Am. J. Hematol. 81 (3): ... and HFE mutational analysis in non-C282Y iron overload: identification of a novel TfR2 mutation". Blood. 100 (3): 1075-7. doi: ... This protein is involved in the uptake of transferrin-bound iron into cells by endocytosis, although its role is minor compared ... Deicher R, Hörl WH (2006). "New insights into the regulation of iron homeostasis". Eur. J. Clin. Invest. 36 (5): 301-9. doi: ...
Kelley et al Iron overload is rare in patients homozygous for the H63D mutation Can J Gastroenterol Hepatol 2014 Preprint: ... Iron overload primarily affects nerve cells in the substantia nigra and basal ganglia. Here, a slowly progressive degeneration ... This allows free iron of non-transferrin bound type (NTBI, labile iron pool) can enter various parenchymal tissues and trigger ... Franchini M (2006). "Hereditary iron overload: Update on pathophysiology, diagnosis, and treatment". Am. J. Hematol. 81 (3): ...
2008-01-17). "Iron-Overload-Related Disease in HFE Hereditary Hemochromatosis" (PDF). New England Journal of Medicine. 358 (3 ... Individuals who are homozygotes for the C282Y allele of the HFE gene are at risk for developing lethal concentrations of iron, ... For hereditary hemochromatosis, a disease caused by excess intestinal iron absorption, the degree of penetrance has been a ... deeming that a homozygote is affected with the disease if they simply have elevated blood iron levels, but no physiological ...
... is used to treat iron overload from transfusions. Therapeutic phlebotomy can be used to manage iron overload. Sideroblastic ... ISBN 978-0-07-145410-0. Peto, T. E. A., Pippard, M. J., Weatherall, D. J. Iron overload in mild sideroblastic anaemias" Lancet ... Some patients may have bronze-colored skin due to an overload of iron. Patients with syndromic hereditary sideroblastic anemia ... Serum iron, percentage saturation and ferritin are increased. The total iron-binding capacity of the cells is normal to ...
"Nonabsorbable Iron Binding Polymers Prevent Dietary Iron Absorption for the Treatment of Iron Overload". ACS Macro Letters. 6 ( ... iron overload) or Wilson's disease (copper overload), by chelating the metal ions in GIT and thus limiting its biological ... Hamilton, Jasmine L; Kizhakkedathu, Jayachandran N (2015). "Polymeric nanocarriers for the treatment of systemic iron overload ... Chelating polymers (ion-exchange resins) were proposed for maintenance therapy of pathologies accompanied by iron accumulation ...
This overload of iron brings with it many biochemical complications.[citation needed] Two key substances involved in iron ... This iron overload may be treated with chelation therapy. Deferoxamine, deferiprone and deferasirox are the three most widely ... For more severe forms, treatment may consist in blood transfusion; chelation therapy to reverse iron overload, using drugs such ... It binds iron, decreasing the toxic reactions catalysed by the unbound metal, and it also decreases the uptake of iron by ...
Once iron overload has been established, HFE gene mutation genetic testing for hereditary causes of iron overload is indicated ... Human iron metabolism Iron deficiency Hsu CC, Senussi NH, Fertrin KY, Kowdley KV (June 2022). "Iron overload disorders". ... Source for mesenchymal versus parenchymal iron overload Deugnier Y, Turlin B (2007). "Pathology of hepatic iron overload". ... The human diet contains iron in two forms: heme iron and non-heme iron. Heme iron is usually found in red meat, whereas non- ...
African iron overload is a condition that involves absorption of too much iron from the diet. Explore symptoms, inheritance, ... When too much iron is absorbed, the resulting iron overload can eventually damage tissues and organs. Iron overload in the ... African iron overload is a condition that involves absorption of too much iron from the diet. The excess iron is stored in the ... Iron overload in immune cells may affect their ability to fight infections. African iron overload is associated with an ...
Iron homeostasis thus relies on the amount that is absorbed from the small intestine. ... The human body has no active mechanism for the excretion of iron. ... When the plasma iron-binding protein transferrin is oversaturated, as in transfusion-induced iron overload, the excess iron ... encoded search term (Transfusion-Induced Iron Overload) and Transfusion-Induced Iron Overload What to Read Next on Medscape ...
... frequent transfusions can result in iron overload, which can cause widespread damage to organs throughout the body if left ... Iron overload can be managed through close monitoring of iron levels, iron chelation therapy, and the use of hematopoietic ... High ferritin levels could indicate iron overload.. More than just blood work may be necessary to verify iron overload and its ... contributing to iron overload. The number of blood transfusions that may lead to iron overload can vary from person to person. ...
Iron overload is a concern for patients who require chronic transfusions as a result of inherited or acquired anemias, ... Iron chelation therapy (ICT) is the primary treatment for iron overload in these patients. The ICT deferasirox, which has been ... Iron overload is a concern for patients who require chronic transfusions as a result of inherited or acquired anemias, ...
Iron deficiency and overload in relation to nutrition ... a hereditary iron overload disease. Iron overload is an ... Nutritional iron intake in the Netherlands has been reviewed with respect to both iron deficiency and iron overload. In general ... Iron supplementation or fortification in functional foods should be avoided and discouraged until the risks of iron overload ... Iron overload is, on the contrary, more common than generally thought. About 10% of the general population is heterozygote for ...
Iron is the midpoint between FUSION and FISSION. ... Iron Sulfate Overload By Atom Bergstrom Atoms Blog IRON is ... Iron is the midpoint between FUSION and FISSION.. The explosive power of the H-BOMB comes from the FUSION of lighter atoms into ... The explosive power of the A-BOMB comes from the FISSION of heavier atoms into lighter ones down to iron-56. ... Paradoxically, GRAMS of iron sulfate have been used in therapy for cancer pain. ...
19 dogs have been subjected to massive parenteral iron loading using intravenous iron-dextran and intramuscular iron-sorbitol. ... The large doses of iron employed (up to 5·8 g/kg) were well tolerated by the surviving animals, and after 35 to 47 months five ... the dog which has not yet developed cirrhosis received the smallest iron load. The liver pathology in many ways resembles that ... of human haemochromatosis, and may provide an experimental model for the study of chronic iron-induced liver injury. ...
Hypochromic microcytic anemia with iron overload is a condition that impairs the normal transport of iron in cells. Iron is an ... Microcytic anemia and hepatic iron overload. Microcytic anemia with liver iron overload. AHMIO1. ... In hypochromic microcytic anemia with iron overload, the iron that is not used by red blood cells accumulates in the liver, ... The lack of involvement of other tissues in hypochromic microcytic anemia with iron overload is likely because these tissues ...
... po BID pre-dosed followed by exposure to iron-containing water and same regime was repeated for 14 days relative to control. ... Prevention of iron overload in beta2-microglobulin deficient male mouse assessed as reduction in total liver iron accumulation ...
Here, an injectable nanoparticle encapsulated core-shell hydrogel was fabricated for simultaneous iron overload clearance and ... Iron overload occurs in many hemorrhagic injuries due to hemolysis and hemoglobin degradation, which not only mediates local ... Iron overload occurs in many hemorrhagic injuries due to hemolysis and hemoglobin degradation, which not only mediates local ... Nanoparticle encapsulated core-shell hydrogel for on-site BMSCs delivery protects from iron overload and enhances functional ...
... only one treatment for iron overload , The Coast News Group ... Arthritis caused by iron overload will not benefit from ... Unfortunately, only one treatment for iron overload. by dr..gott. October 21, 2010. 1259 ... It is the most common form of iron-overload disease. There are several forms, including primary/hereditary, secondary, juvenile ... She has a problem with producing too much iron in her blood. The only treatment that we are aware of is to have blood drawn off ...
Iron, and Transferrin saturations. Iron deficiency is investigated with endoscopy and sero.. ... Screening for Iron deficiency or overload is done by checking Serum Ferritin, ... Baseline Iron studies are commonly conducted by the doctors and nurses for either Iron deficiency or Iron overload. A lot of ... Checking Serum Iron and Transferrin were not useful in Iron overload. Serum Ferritin was more useful in screening for Iron ...
The primary goal of iron chelation therapy is to prevent the accumulation of iron reaching harmful levels by matching iron ... with iron excreted by iron chelation. Over 30 years of experience with deferoxamine has shown iron chelation to be an effective ... Concepts and goals in the management of transfusional iron overload.. John B Porter. American Journal of Hematology 2007 ... and only a small fraction of body iron is chelatable at any moment. Once iron has been deposited in organs other than the liver ...
Iron represents a micronutrient for cellular metabolism and aerobic respiration, but cellular iron overload produces toxic ... Iron represents a micronutrient for cellular metabolism and aerobic respiration, but cellular iron overload produces toxic ... Could Iron Overload Impair the Migratory Ability of Neurons? Evidence from a Cell-Based Model. L. Steffani;M. Ruscica;F. Di ... Could Iron Overload Impair the Migratory Ability of Neurons? Evidence from a Cell-Based Model / L. Steffani, M. Ruscica, F. Di ...
Iron homeostasis thus relies on the amount that is absorbed from the small intestine. ... The human body has no active mechanism for the excretion of iron. ... Iron status in iron deficiency and overload. NTBI = non-transferrin-bound iron; TIBC = total iron-binding capacity. ... encoded search term (Transfusion-Induced Iron Overload) and Transfusion-Induced Iron Overload What to Read Next on Medscape ...
Thus, people rarely have too much iron in their body. Causes of excess iron in the body (iron overload) include the following: ... People lose small amounts of iron every day, and even a healthy diet contains only a small amount of iron. ... so the body usually tightly controls iron absorption from food and recycles the iron from red blood cells. ...
Iron overload Iron is an essential mineral to almost all life forms, including humans. It is the essential part of the ... Too little iron leads to feelings of weakness, reduced immunity and anemia, all conditions that can become dangerous if not... ...
We propose that anemia, secondary to the impaired macrophage iron release, plays a major role in hepatic iron overload through ... We propose that anemia, secondary to the impaired macrophage iron release, plays a major role in hepatic iron overload through ... iron overload, and neurodegeneration, caused by the absence of ceruloplasmin (Cp), a multicopper oxidase important for iron ... iron overload, and neurodegeneration, caused by the absence of ceruloplasmin (Cp), a multicopper oxidase important for iron ...
Article Chelation Disease-Free Survival Erythrocyte Transfusion Female Humans Iron Chelating Agents Iron Overload Male ... Iron loading and disease surveillance. Cite CITE. Title : Iron loading and disease surveillance. Personal Author(s) : Weinberg ... The impact of chelation therapy on survival in transfusional iron overload: a meta-analysis of myelodysplastic syndrome. ... The impact of chelation therapy on survival in transfusional iron overload: a meta-analysis of myelodysplastic syndrome ...
Transfusion-Induced iron overload - evidence and research - oneSCDvoice ... Day-to-day iron requirements, as iron is needed by virtually all body cells and especially erythrocytes, are met by recycling ... Iron chelation therapy is used to prevent the accumulation of iron to harmful levels. Liver and cardiac transplantation should ... During normal physiology, the amount of iron absorbed from the small intestine (1-2 mg/d) is balanced by the iron lost through ...
Iron overload. Iron overload is a serious, but not an unusual, complication in pyruvate kinase deficiency, even in patients not ... The loss of protection against iron absorption in patients with iron overload who have chronic hemolysis has been shown to be ... The dangers of iron overload in pyruvate kinase deficiency. Br J Haematol. 2003 Mar. 120(6):1090-1. [QxMD MEDLINE Link]. ... Patients with iron overload not related to chronic hemolysis, such as in hemochromatosis, are usually protected from absorbing ...
Dietary iron overload disease. Synonyms: African iron overload , Bantu siderosis. A rare disorder described in sub-Saharan ... Dietary iron overload disease?. Our RARE Concierge Services Guides are available to assist you by providing information, ... Dietary iron overload disease. Get in touch with RARE Concierge.. Contact RARE Concierge ... African populations and characterized by iron overload due to excess dietary iron intake and possibly genetic factors leading ...
... known as Inherited Iron Overload Disorder, the most common genetic disorder in Australia, affecting 1 in 200 people. ... Haemochromatosis is a genetic iron overload condition. It can be very serious if untreated or undiagnosed.. The good news is ...
Potential Iron Overload? Kat3v0 I am 30 weeks and 5 days pregnant and my doctor prescribed me an iron supplement (325mg once ... I am 30 weeks and 5 days pregnant and my doctor prescribed me an iron supplement (325mg once daily) about 2 weeks ago because ...
Potential Iron Overload? Kat3v0 I am 30 weeks and 5 days pregnant and my doctor prescribed me an iron supplement (325mg once ... I am 30 weeks and 5 days pregnant and my doctor prescribed me an iron supplement (325mg once daily) about 2 weeks ago because ...
... we will explore iron chelation therapy, its purpose, methods, and the conditions it is commonly used to treat... ... Iron is an essential mineral for the body, but excessive iron accumulation can lead to a condition called iron overload. This ... Iron chelation therapy is a medical treatment used to manage iron overload in the body, a condition that can have serious ... Iron chelations therapy plays a vital role in managing iron overload and preventing the serious health complications it can ...
... I. DE DOMENICO;D. MCVEY WARD;MUSCI, Giovanni;J. KAPLAN 2006-01-01. Abstract. ... Iron overload disease due to mutations in ferroportin has a dominant inheritance and a variable clinical phenotype, such that ... Iron overload disease due to mutations in ferroportin has a dominant inheritance and a variable clinical phenotype, such that ... some patients show early Küpffer cell iron loading and low transferrin saturation, while others show hepatocyte iron loading ...
Bennett JM, MDS Foundations Working Group on Transfusional Iron Overload. Consensus statement on iron overload in ... deferasirox for iron chelation of iron overloaded patients with MDS.. Dental amalgam is a widely used restorative material ... Evolution of iron overload in patients with low-risk myelodysplastic syndrome: Iron chelation therapy and organ complications. ... Treatment of acute iron intoxication and chronic iron overload due to transfusion-dependent anemias ...
What are your thoughts on overload training? Let us know in the comments below and be sure to follow Generation Iron on ... Overload training is great for building strength and putting on muscle mass as well. Overload training forces your body to get ... Overload training is a great method to get some gains without just completing the typical three sets of ten reps basic program ... If youre one of those hit the gym three times a week kind of guys then youre going to have to change things up for overload ...
  • The most important causes are hereditary haemochromatosis (HH or HHC), a genetic disorder, and transfusional iron overload, which can result from repeated blood transfusions. (wikipedia.org)
  • Concepts and goals in the management of transfusional iron overload. (qxmd.com)
  • Title : The impact of chelation therapy on survival in transfusional iron overload: a meta-analysis of myelodysplastic syndrome Personal Author(s) : Mainous, Arch G.;Tanner, Rebecca J.;Hulihan, Mary M.;Amaya, Mirna;Coates, Thomas D. (cdc.gov)
  • For the treatment of transfusional iron overload in patients with chronic anemia. (nih.gov)
  • Carlos Chiattone The efficacy and safety of a 2-year treatment with deferasirox was evaluated in 31 patients with sickle cell anemia and transfusional iron overload. (karger.com)
  • Iron overload or haemochromatosis (also spelled hemochromatosis in American English) indicates increased total accumulation of iron in the body from any cause and resulting organ damage. (wikipedia.org)
  • Risk factors for the development of arthritis in those with hemochromatosis include elevated iron levels (ferritin greater than 1000 or transferrin saturation greater than 50%) for an extended period of time, increasing age and concurrent advanced liver fibrosis. (wikipedia.org)
  • Currently, hemochromatosis (without further specification) is mostly defined as iron overload with a hereditary or primary cause, or originating from a metabolic disorder. (wikipedia.org)
  • However, the term is currently also used more broadly to refer to any form of iron overload, thus requiring specification of the cause, for example, hereditary hemochromatosis. (wikipedia.org)
  • Non-HFE hereditary hemochromatosis involves mutations in genes coding for the iron regulatory proteins hemojuvelin, transferrin receptor-2 and ferroportin. (wikipedia.org)
  • Hereditary hemochromatosis is characterized by an accelerated rate of intestinal iron absorption and progressive iron deposition in various tissues. (wikipedia.org)
  • Hemosiderosis is iron overload that does not cause tissue damage, while hemochromatosis does. (wikipedia.org)
  • Hemosiderosis is arbitrarily differentiated from hemochromatosis by the reversible nature of the iron accumulation in the reticuloendothelial system. (wikipedia.org)
  • This pattern differs from that seen in a similar iron overload disorder called hereditary hemochromatosis , in which the excess iron accumulates primarily in the hepatocytes. (medlineplus.gov)
  • Frequent blood transfusions may come with risks associated with hemochromatosis, also known as iron overload. (healthline.com)
  • About 10% of the general population is heterozygote for the C282Y mutation, which can result in hemochromatosis, a hereditary iron overload disease. (rivm.nl)
  • Primary or hereditary hemochromatosis is generally caused by a defect of the gene HFE, which regulates the amount of iron absorbed from food. (thecoastnews.com)
  • Those with one copy of the defect C282Y become carriers who don't develop hemochromatosis but may have higher-than-normal iron levels throughout life. (thecoastnews.com)
  • For individuals with chronic iron overload conditions, such as thalassemia, sickle cell disease, or hereditary hemochromatosis, iron chelations therapy may be a long-term or lifelong treatment to maintain iron levels within a safe range. (realtimes.info)
  • Patients with mutant ferroportin proteins that do not localize to the cell surface show typical ferroportin disease with low transferrin saturation and early Küpffer cell iron loading, while patients with mutant proteins unable to respond to hepcidin show high transferrin saturation and early hepatocyte iron loading similar to classic hereditary hemochromatosis. (unimol.it)
  • Therapeutic phlebotomy is used to remove excess iron and maintain low normal body iron stores in patients with hemochromatosis. (aetna.com)
  • Hepatic iron stores and markers of iron overload in alcoholics and patients with idiopathic hemochromatosis. (ox.ac.uk)
  • Liver iron concentrations were determined in 60 alcoholics with liver disease of varying severity, 15 patients with untreated idiopathic hemochromatosis, and 16 control subjects with biliary tract disease. (ox.ac.uk)
  • Liver iron concentrations of greater than 140 micrograms/100 were found in 17 alcoholics (29%) and in all 15 patients with idiopathic hemochromatosis. (ox.ac.uk)
  • Liver iron concentrations greater than 1000 micrograms/100 mg were found in all patients with idiopathic hemochromatosis but in none of the alcoholics. (ox.ac.uk)
  • Serum ferritin concentrations reflected iron stores in patients with hemochromatosis and in alcoholics with minimal liver disease. (ox.ac.uk)
  • Serum iron concentration and percentage saturation of total iron-binding capacity were of little value in assessing iron status in either alcoholics or patients with hemochromatosis. (ox.ac.uk)
  • Measurement of the liver iron concentration clearly differentiates between alcoholics with significant siderosis and patients with idiopathic hemochromatosis. (ox.ac.uk)
  • Hemochromatosis are inherited disorders that can cause iron overload. (familymedexamprep.com)
  • Hereditary hemochromatosis (HH) is characterized by progressive iron deposition and tissue injury secondary to inappropriate intestinal iron absorption. (scitechnol.com)
  • Ferroportin disease (hemochromatosis type 4) is a recently recognized disorder of human iron metabolism, characterized by iron deposition in macrophages, including Kupffer cells. (intrinsiclifesciences.com)
  • utations in the human gene encoding ferroportin 1 (FPN1/IREG-1/MTP-1/SLC40A1) are associated with an unusual iron overload syndrome, recently named hemochromatosis type 4 or ferroportin disease. (intrinsiclifesciences.com)
  • Deferoxamine mesylate is not indicated for the treatment of primary hemochromatosis (since phlebotomy is the method of choice for removing excess iron in this disorder). (nih.gov)
  • Hereditary HFE hemochromatosis accounts for most cases of excess body iron that result from increased iron absorption. (medscape.com)
  • [ 2 ] Homozygous C282Y hemochromatosis is the cause of 85%-90% of cases of iron overload. (medscape.com)
  • Other genetic mutations involved in iron homeostasis, which account for most of the remaining patients with inherited disorders of iron overload, include juvenile hemochromatosis (type 2), transferrin receptor 2 disease (type 3 hemochromatosis), and ferroportin disease (type 4 hemochromatosis). (medscape.com)
  • Type 1 hemochromatosis results from reduced hepcidin expression and increased iron absorption, exceeding the capacity of transferrin to transport iron. (medscape.com)
  • The need for regular blood transfusions, as well as underlying processes in MDS, can result in iron overload. (healthline.com)
  • The number of blood transfusions that may lead to iron overload can vary from person to person. (healthline.com)
  • The exact number of blood transfusions that lead to significant iron overload varies with the patient's underlying disease and the duration of their transfusion dependence. (medscape.com)
  • Iron overload often occurs in individuals who require frequent blood transfusions, such as those with certain types of anemia, like thalassemia or sickle cell disease. (realtimes.info)
  • If you or a loved one are facing iron overload due to a medical condition that requires frequent blood transfusions, it's essential to work closely with a healthcare provider who can determine the appropriate iron chelation therapy and provide ongoing care and monitoring to ensure the best possible outcome. (realtimes.info)
  • Aim:Beta-thalassemia major requires regular blood transfusions throughout life, which in turn leads to iron accumulation in the body. (istinye.edu.tr)
  • Background Routine blood transfusions and increased intestinal iron absorption lead to iron accumulation in various organs, especially the liver. (ui.ac.id)
  • Either we do not treat anaemia and the patient suffers from chronic tiredness and fatigue , or we do treat it through blood transfusions , leading to iron overload , which is a quite harmful consequence. (bvsalud.org)
  • Without genetic predisposition, secondary iron overload can develop in patients who have undergone multiple blood transfusions, those with ineffective erythropoiesis coupled with oral iron supplementation, or patients in end-stage liver disease. (medscape.com)
  • When too much iron is absorbed, the resulting iron overload can eventually damage tissues and organs. (medlineplus.gov)
  • Ferroportin then transports iron from the small intestine into the bloodstream, and the iron is carried by the blood to the tissues and organs of the body. (medlineplus.gov)
  • If left untreated, excess iron can cause widespread damage to organs throughout the body. (healthline.com)
  • Excess iron deposits frequently affect the liver, heart, and endocrine organs, but multiple organs can be affected simultaneously. (healthline.com)
  • Tissue samples through biopsy and magnetic resonance imaging (MRI) scans can help your doctor verify iron overload and any damage to specific organs. (healthline.com)
  • Once iron has been deposited in organs other than the liver, for example the heart, removal by chelation is slow and inefficient. (qxmd.com)
  • Iron represents a micronutrient for cellular metabolism and aerobic respiration, but cellular iron overload produces toxic build-up in many organs (including brain) via free radical formation. (unimi.it)
  • This excess iron can be toxic and damaging to organs and tissues, potentially causing severe health problems. (realtimes.info)
  • On day 30 animals were sacrificed and organs were preserved to evaluate iron levels. (org.pk)
  • Transfusion-dependent patients with thalassemia major(TM) develop iron overload which leads to damage of the liver, heart, and endocrine organs and related morbidity and mortality ( 1 1. (scielo.br)
  • Because human beings do not possess effective mechanisms to get rid of iron, chronic transfusion leads to iron overload responsible of lesions in parenchymal organs and or in dysfunctions of endocrine glands. (haema-journal.gr)
  • Iron chelation removes toxic iron and blocks its entry into tissues and organs to prevent complications of iron overload. (ferriprox.com)
  • In patients with most, but not all, ferroportin mutations, retention of iron in macrophages of the liver and other organs may protect against damage to parenchymal cells. (intrinsiclifesciences.com)
  • Impaired iron export from macro-phages in patients with mutations in the FPN1 gene has been proposed as the explanation for the accumulation of iron that occurs in organs containing abundant macro-phages such as the liver, spleen, and bone marrow. (intrinsiclifesciences.com)
  • To date, T2-star magnetic resonance imaging (T2*MRI) is a valuable tool to evaluate iron level in organs. (ui.ac.id)
  • To date, T2-star magnetic resonance imaging (T2*MRI) is a valuable tool to evaluate iron level in organs.Objective To assess the degree of liver iron overload among children with thalassemia major (TM) and its possible correlations with hepatic function laboratory values.Methods This cross-sectional study was conducted in Cipto Mangunkusumo Hospital. (ui.ac.id)
  • Despite chelation therapy , diffuse iron deposition was occurring in multiple organs, markedly in the heart and liver . (bvsalud.org)
  • As a result of multiple transfusions over the years, various organs become severely overloaded with iron, and, in order to avoid death in adolescence, regular subcutaneous infusion of an iron-chelating agent is essential. (who.int)
  • [ 4 ] In patients who receive numerous transfusions-notably those with thalassemia major , sickle cell disease , myelodysplastic syndrome , aplastic anemia , hemolytic anemia , and refractory sideroblastic anemias , who may become transfusion dependent-the excess iron from the transfused erythrocytes gradually accumulates in various tissues, causing morbidity and mortality. (medscape.com)
  • In contrast, in thalassemia major, transfusions decrease erythropoietic drive and increase the iron load, resulting in relatively higher hepcidin levels. (medscape.com)
  • Efficacy of deferoxamine in preventing complications of iron overload in patients with thalassemia major. (scielo.br)
  • Can biomarkers predict myocardial iron overload in children with thalassemia major? (istinye.edu.tr)
  • Herein, the value of growth differentiation factor-15, galectin-3, and N-terminal pro-B-type natriuretic peptide in predicting cardiac iron accumulation is investigated in asymptomatic children with beta-thalassemia major. (istinye.edu.tr)
  • Conclusion:All three biomarkers investigated in this study failed to predict myocardial iron accumulation in asymptomatic children with beta-thalassemia major. (istinye.edu.tr)
  • Objective To assess the degree of liver iron overload among children with thalassemia major (TM) and its possible correlations with hepatic function laboratory values. (ui.ac.id)
  • The excess iron also leads to a faster-than-normal breakdown of vitamin C in the body, so affected individuals are at increased risk of vitamin C deficiency problems such as scurvy. (medlineplus.gov)
  • Thus, iron-deficiency states exhibit reduced hepcidin and iron-excess states have high levels of hepcidin to maintain the amount of iron secreted into the circulation. (medscape.com)
  • Nutritional iron intake in the Netherlands has been reviewed with respect to both iron deficiency and iron overload. (rivm.nl)
  • The prevalence of iron deficiency is probably much lower than currently assumed. (rivm.nl)
  • The general overestimation of iron deficiency is caused both by the incorrect comparison of the recommended daily intake with iron intake data and by the use of insufficiently specific diagnostic tests to determine real iron deficiency. (rivm.nl)
  • An analysis of the cost and clinical effectiveness of the laboratory tests for Iron studies including deficiency (Anaemia) and overload (Haemochromatosis): The district general perspective. (alliedacademies.org)
  • Background: Screening for Iron deficiency or overload is done by checking Serum Ferritin, Iron, and Transferrin saturations. (alliedacademies.org)
  • Iron deficiency is investigated with endoscopy and serology for coeliac disease and Iron overload (Hereditary Haemochromatosis) by C282Y genetic test. (alliedacademies.org)
  • Aims and Objectives: To validate the cost effectiveness of tests recommended for making the diagnosis of Iron deficiency or overload. (alliedacademies.org)
  • Serum Ferritin was more useful in screening for Iron overload but less helpful in Iron deficiency. (alliedacademies.org)
  • Transferrin saturation was twice more sensitive than Iron level in indicating Iron deficiency in our data. (alliedacademies.org)
  • Baseline Iron studies are commonly conducted by the doctors and nurses for either Iron deficiency or Iron overload. (alliedacademies.org)
  • doing the basic screening tests, and then tests that are subsequently indicated according to findings of Iron deficiency or an overload. (alliedacademies.org)
  • In patients with Iron deficiency, if there is no other systemic cause for Iron deficiency, then usually gastroscopy and colonoscopy are undertaken. (alliedacademies.org)
  • Common causes of Iron deficiency include non- gastrointestinal reasons like menorrhagia in women, silent haematuria etc. (alliedacademies.org)
  • Ferrlecit is an iron replacement product for treatment of iron deficiency anemia in adult patients and in pediatric patients age 6 years and older with chronic kidney disease receiving hemodialysis who are receiving supplemental epoetin therapy. (nih.gov)
  • Ferrlecit treatment may be repeated if iron deficiency reoccurs. (nih.gov)
  • The recommended dosage of Ferrlecit for the repletion treatment of iron deficiency in hemodialysis patients is 10 mL of Ferrlecit (125 mg of elemental iron). (nih.gov)
  • Growth hormone deficiency can occur despite effective chelation therapy, due to iron deposition in the pituitary gland. (challengetdt.com)
  • Iron deficiency is the most common known form of nutritional deficiency. (cdc.gov)
  • In children, iron deficiency causes developmental delays and behavioral disturbances, and in pregnant women, it increases the risk for a preterm delivery and delivering a low-birthweight baby. (cdc.gov)
  • In the past three decades, increased iron intake among infants has resulted in a decline in childhood iron-deficiency anemia in the United States. (cdc.gov)
  • As a consequence, the use of screening tests for anemia has become a less efficient means of detecting iron deficiency in some populations. (cdc.gov)
  • For women of childbearing age, iron deficiency has remained prevalent. (cdc.gov)
  • To address the changing epidemiology of iron deficiency in the United States, CDC staff in consultation with experts developed new recommendations for use by primary health-care providers to prevent, detect, and treat iron deficiency. (cdc.gov)
  • These recommendations update the 1989 'CDC Criteria for Anemia in Children and Childbearing-Aged Women' (MMWR 1989;38(22):400-4) and are the first comprehensive CDC recommendations to prevent and control iron deficiency. (cdc.gov)
  • In the United States, the prevalence of iron-deficiency anemia among children declined during the 1970s in association with increased iron intake during infancy (1-3). (cdc.gov)
  • Because of this decline, the value of anemia as a predictor of iron deficiency has also declined, thus decreasing the effectiveness of routine anemia screening among children. (cdc.gov)
  • These findings, plus increased knowledge about screening for iron status, raised questions about the necessity and effectiveness of existing U.S. programs to prevent and control iron deficiency. (cdc.gov)
  • CDC requested the Institute of Medicine to convene an expert committee to develop recommendations for preventing, detecting, and treating iron-deficiency anemia among U.S. children and U.S. women of childbearing age. (cdc.gov)
  • Preventing and controlling iron deficiency are also addressed in Nutrition and Your Health: Dietary Guidelines for Americans (14). (cdc.gov)
  • When iron levels are low (as in iron deficiency), hepcidin promotes iron absorption from the small intestine. (medscape.com)
  • Beta thalassaemia syndromes are the other tissues such as cardiac or renal aly, clinical heart failure, and chelation most common inherited haemoglobi- tissue have different mechanisms and therapy with a method other than using nopathies caused by a genetic deficiency kinetics of iron uptake, storage and desferrioxamine. (who.int)
  • In African iron overload, excess iron typically accumulates primarily in certain immune cells called reticuloendothelial cells. (medlineplus.gov)
  • Ferritin stores and releases iron in cells, and cells produce more ferritin in response to excess amounts of iron. (medlineplus.gov)
  • Men seem to be affected more often than women, possibly due to some combination of differences in dietary iron consumption and women's shedding of excess iron through blood loss in menstruation and childbirth. (medlineplus.gov)
  • When the plasma iron-binding protein transferrin is oversaturated, as in transfusion-induced iron overload, the excess iron circulates as relatively free non-transferrin-bound iron (NTBI). (medscape.com)
  • A rare disorder described in sub-Saharan African populations and characterized by iron overload due to excess dietary iron intake and possibly genetic factors leading to hepatic portal fibrosis and micronodular cirrhosis. (globalgenes.org)
  • Iron chelations therapy works by using chelating agents that bind to excess iron in the body, forming a compound that can be excreted through urine or feces. (realtimes.info)
  • By removing excess iron, this therapy helps prevent iron-related complications. (realtimes.info)
  • Macrophages release iron excess in the plasma where iron is bound to transferrin. (haema-journal.gr)
  • The radiological findings indicated the presence of excess iron in bone marrow and spleen. (intrinsiclifesciences.com)
  • Ghazaiean M, Aliasgharian A, Karami H, Darvishi-Khezri H. Ebselen: a promising therapy protecting cardiomyocytes from excess iron in iron-overloaded thalassemia patients . (hematologyadvisor.com)
  • African iron overload is a condition that involves absorption of too much iron from the diet. (medlineplus.gov)
  • This protein is involved in the process of iron absorption in the body. (medlineplus.gov)
  • [ 1 ] Normally, the amount of iron absorbed from the small intestine is balanced by the iron lost through sweat, menstruation, shedding of hair and skin cells, and rapid turnover and excretion of enterocytes, with daily absorption and excretion of iron both being about 1 mg in a healthy individual. (medscape.com)
  • In some disorders, such as β-thalassemia, excessive intestinal absorption also adds to the transfusion-induced iron overload. (medscape.com)
  • In the presence of higher hepcidin levels, dietary iron absorption is moderated and macrophages retain iron, but body iron stores increase due to the inability to excrete iron in transfused red blood cells. (medscape.com)
  • In the duodenum, a shortage of DMT1 protein decreases iron absorption. (checkorphan.org)
  • To compensate, cells increase production of functional DMT1 protein, which increases iron absorption. (checkorphan.org)
  • We propose that anemia, secondary to the impaired macrophage iron release, plays a major role in hepatic iron overload through increased absorption mediated by the erythroid regulator. (unito.it)
  • These conditions can lead to increased iron absorption and accumulation, making iron chelation therapy a crucial treatment option. (realtimes.info)
  • 2. Try to correct iron hyper absorption which is associated with low levels of hepcidin. (haema-journal.gr)
  • Figures 2 and 3 illustrate the role of these two proteins in iron absorption. (haema-journal.gr)
  • Role of erythroferrone and hepcidin in iron absorption in normal situation. (haema-journal.gr)
  • Role of erythroferrone and hepcidin in iron absorption in hemolysis and ineffective erythropoiesis. (haema-journal.gr)
  • These factors suppress hepcidin production leading to increased iron absorption from the intestine and to iron release from macrophages. (haema-journal.gr)
  • ABSTRACT A case-control study aimed to determine the prevalence of C282Y, H63D and S65C mutations of the HFE gene in -thalassaemia carriers and investigate their influence on iron absorption. (who.int)
  • Hepcidin, which is produced in the liver and to a smaller extent in adipocytes and macrophages, is a key regulator of iron absorption. (medscape.com)
  • [ 6 ] When iron levels are normal or elevated, hepcidin inhibits intestinal iron absorption. (medscape.com)
  • [ 7 , 8 ] Hepcidin acts by binding to the iron exporter ferroportin, blocking the transport and release of iron, leading to retention of iron within enterocytes and reduced iron absorption. (medscape.com)
  • [ 9 ] Chronic ethanol ingestion also reduces hepcidin transcription, thereby increasing iron absorption. (medscape.com)
  • The increased So it has been suggested that a direct Sample size iron deposition coming from multiple estimation of heart iron overload using life-long transfusions and enhanced heart T2* imaging is more useful in The primary outcome of interest was iron absorption leads to organ dysfunc- evaluating the state of heart iron over- the relation between ferritin and liver tion [3,4]. (who.int)
  • Iron chelation therapy is used to prevent the accumulation of iron to harmful levels. (medscape.com)
  • Iron chelation therapy (ICT) is the primary treatment for iron overload in these patients. (nursingcenter.com)
  • In this review, current concepts and goals of iron chelation therapy for thalassemias, sickle cell disease, and myelodysplastic syndromes are discussed. (qxmd.com)
  • The primary goal of iron chelation therapy is to prevent the accumulation of iron reaching harmful levels by matching iron intake from blood transfusion, with iron excreted by iron chelation. (qxmd.com)
  • Over 30 years of experience with deferoxamine has shown iron chelation to be an effective therapeutic modality. (qxmd.com)
  • However, chelation efficiency is limited because most of the body's iron stores are not directly chelatable, and only a small fraction of body iron is chelatable at any moment. (qxmd.com)
  • Chelation efficiency can be improved by designing regimes where chelators are available 24 hr a day to bind labile iron pools in cells and plasma. (qxmd.com)
  • Once-daily oral administration of deferasirox achieves continuous chelation with trough concentrations sufficient to decrease plasma labile iron species progressively, and achieves an efficiency of chelation not obtainable with deferiprone or deferoxamine monotherapy. (qxmd.com)
  • Labile plasma iron in iron overload: redox activity and susceptibility to chelation. (medscape.com)
  • Iron chelation therapy is a medical treatment used to manage iron overload in the body, a condition that can have serious health implications. (realtimes.info)
  • In this article, we will explore iron chelation therapy, its purpose, methods, and the conditions it is commonly used to treat. (realtimes.info)
  • Like many medical treatments, iron chelation therapy may have side effects, which can include gastrointestinal symptoms, rashes, or allergic reactions. (realtimes.info)
  • These practice guidelines, prepared by the Scientific Committee of Associação Brasileira de Thalassemia (ABRASTA), presents a review of the literature regarding iron overload assessment (by imaging and laboratory exams) and the role of T2* magnetic resonance imaging (MRI) to control iron overload and iron chelation therapy, with evidence-based recommendations for each clinical situation. (scielo.br)
  • Based on this review, the authors propose an iron chelation protocol for patients with thalassemia under regular transfusions. (scielo.br)
  • Improvements in survival have been achieved over the last 40 years due to iron chelation therapy and iron assessment by serum ferritin, and liver/cardiac magnetic resonance imaging (MRI) techniques ( 3 3. (scielo.br)
  • The need to have an iron chelation protocol in Brazil to guide the treatment led to the development of this guideline. (scielo.br)
  • Iron chelation therapy with desferrioxamine (DFO), available since the late 1960s and the most widely used iron chelator, decreased iron overload-related complications and mortality rate of patients with TM ( 4 4. (scielo.br)
  • 4. Sometimes (in case of low-risk MDS anemia), chelation removes the toxic effect of iron on erythropoiesis and transforms transfusion dependent anemia in transfusion-independent. (haema-journal.gr)
  • 5. Chelation of iron by available drugs: the most effective way which applies to all cases. (haema-journal.gr)
  • 1 The introduction of effective iron chelation therapy has significantly improved survival and quality of life for people living with TDT. (challengetdt.com)
  • Despite this level of care and utilizing iron chelation therapy, these patients experienced iron overload. (challengetdt.com)
  • Iron monitoring is key to determining the extent of iron overload and establishing an effective iron chelation regimen in accordance with a patient's individual needs. (challengetdt.com)
  • Medical experts recommend preventing significant iron loading with chelation from the start. (ferriprox.com)
  • Vitamin C (up to 200 mg) increases availability of iron for chelation and may be given as an adjuvant to iron chelation therapy. (nih.gov)
  • A multidisciplinary team is essential to care for DBA patients , since there is a significant emotional burden related to the disease , which might impair an effective chelation therapy and lead to severe consequences due to iron deposition. (bvsalud.org)
  • metabolism: diabetes in people with iron overload occurs as a result of selective iron deposition in islet beta cells in the pancreas leading to functional failure and cell death. (wikipedia.org)
  • skeletal: arthritis, from iron deposition in joints leading to joint pains. (wikipedia.org)
  • Less iron deposition and ventricular enlargement, lower brain water content, and faster neurological recovery were also observed. (physiciansweekly.com)
  • Less than 10% of the subjects had normal liver iron deposition. (ui.ac.id)
  • Deposition of iron within the skin causes inflammation and enhances melanin production by melanocytes. (diagnose-me.com)
  • An autopsy was performed and revealed the macroscopic and microscopic findings of a massive iron deposition in the liver , heart , lungs , spleen , bone marrow , thyroid and adrenal glands . (bvsalud.org)
  • However, older thalassaemics may have to contend with multiple conditions including early osteoporosis, cardiac disease, pulmonary hypertension and diabetes, some of which result from increased iron deposition in the endocrine glands and myocardial cells. (who.int)
  • Liver iron concentrations and urinary hepcidin in beta-thalassemia. (medscape.com)
  • In the absence of an iron chelating agent, patients with beta-thalassemia on regular transfusions present complications of transfusion-related iron overload. (scielo.br)
  • Transferrin-bound iron (TBI) is also taken up by these cells through the hepcidin mechanism, which is increased in such states. (medscape.com)
  • All serum samples received in a sample district laboratory of UK for full one year with requested serum Ferritin, Iron level and Transferrin saturation were analysed in retrospect for cost effectiveness. (alliedacademies.org)
  • Serum Iron and Transferrin saturation were checked in 1770 samples. (alliedacademies.org)
  • Checking Serum Iron and Transferrin were not useful in Iron overload. (alliedacademies.org)
  • These basis tests include serum Iron level, Transferrin level, Transferrin saturation, serum Ferritin as a base line. (alliedacademies.org)
  • Zip14 (Slc39a14) mediates non-transferrin-bound iron uptake into cells. (medscape.com)
  • Iron overload disease due to mutations in ferroportin has a dominant inheritance and a variable clinical phenotype, such that some patients show early Küpffer cell iron loading and low transferrin saturation, while others show hepatocyte iron loading and high transferrin saturation. (unimol.it)
  • A huge amount of iron is released in the plasma which exceeds transferrin's capacity to transport iron and thus we have production of NTBI (non-transferrin bound iron), which is the toxic form of iron to cells via the formation of ROS (reactive oxygen species). (haema-journal.gr)
  • In iron overload, transferrin becomes saturated, and iron that is not bound to transferrin (non-transferrin bound iron, or NTBI) accumulates in the plasma. (challengetdt.com)
  • Additionally, co-administration of ebselen and desferrioxamine can improve cardiac function in mice with thalassemia with an iron burden by decreasing cardiac hemosiderosis, cardiac malondialdehyde, and plasma non-transferrin bound iron. (hematologyadvisor.com)
  • Non-transferrin-bound circulating iron is readily taken up by hepatocytes, where it promotes the formation of free radical oxygen species that cause cell injury. (medscape.com)
  • According to a 2018 review , genetic mutations seen in specific subtypes of MDS may contribute to iron buildup in the body. (healthline.com)
  • Mutations in the SLC11A2 gene cause hypochromic microcytic anemia with iron overload. (checkorphan.org)
  • Anemia and iron overload due to compound heterozygosity for novel ceruloplasmin mutations. (unito.it)
  • Mutations in the gene encoding ferroportin 1, a cellular iron exporter, are responsible for this iron storage disease, inherited as an autosomal dominant trait. (intrinsiclifesciences.com)
  • The iron status in these subjects was studied and correlated with the HFE gene mutations. (who.int)
  • Notre étude a démontré que les mutations du gène HFE sont fréquentes en Égypte chez les porteurs d'une -thalassémie par rapport aux sujets témoins. (who.int)
  • In patients with TDT, lifelong regular red blood cell (RBC) transfusions enable survival but lead to iron overload and treatment-related complications. (challengetdt.com)
  • Iron overload in the liver can lead to chronic liver disease (cirrhosis). (medlineplus.gov)
  • Iron overload is a concern for patients who require chronic transfusions as a result of inherited or acquired anemias, including sickle cell disease, thalassemia, and myelodysplastic syndromes. (nursingcenter.com)
  • Iron overload is an established risk factor for cardiovascular disease. (rivm.nl)
  • Newly diagnosed with Dietary iron overload disease? (globalgenes.org)
  • Although available in many countries, one third of the patients developed signs of iron overload, such as delayed or absent puberty, growth disorders, hypothyroidism, hypogonadism, bone abnormalities, cirrhosis and heart disease (main cause of death in transfusion dependent patients with TM). (scielo.br)
  • In the alcoholics no relationship existed between liver iron concentrations and the amount of alcohol consumed daily, the length of the drinking history, the amount of beverage iron consumed daily, or the severity of the liver disease. (ox.ac.uk)
  • However, in alcoholics with significant liver disease serum ferritin concentrations did not reflect iron stores accurately, although with normal values iron overload is unlikely. (ox.ac.uk)
  • Despite a significant burden of iron, no features of chronic liver disease were found in affected members of the family, including individuals aged up to 80 years. (intrinsiclifesciences.com)
  • In conclusion, the systemic iron burden in ferroportin disease is not a sufficient cause for chronic liver disease. (intrinsiclifesciences.com)
  • Finally, macrophage iron storage in ferroportin disease is associated with elevated serum pro-hepcidin levels. (intrinsiclifesciences.com)
  • diabetes, or iron overload disease, are at higher risk for severe disease and death from eating contaminated raw oysters. (cdc.gov)
  • Aceruloplasminemia is a recessive disorder characterized by anemia, iron overload, and neurodegeneration, caused by the absence of ceruloplasmin (Cp), a multicopper oxidase important for iron export. (unito.it)
  • possibly because the iron that accumulates in the liver, bone marrow, and spleen is less available for production of red blood cells. (medlineplus.gov)
  • Ferroportin also transports iron out of reticuloendothelial cells in the liver, spleen, and bone marrow. (medlineplus.gov)
  • Here, an injectable nanoparticle encapsulated core-shell hydrogel was fabricated for simultaneous iron overload clearance and bone marrow mesenchymal stem cell (BMSC) transplantation following intracerebral hemorrhage (ICH). (physiciansweekly.com)
  • The iron chelator-loaded low-molecular-weight keratin hydrogel with quick degradation property was selected as the outer shell to eliminate iron overload, and BMSCs implantation with high-molecular-weight keratin hydrogel was selected as the inner core. (physiciansweekly.com)
  • 0.05) FBS-induced chemo-migration of GN-11 cells, which was rescued by pre-treatment with 100 μM deferoxamine, a specific iron chelator. (unimi.it)
  • Deferiprone (DFP) was the most commonly used iron chelator. (ui.ac.id)
  • With the introduction of the oral iron chelator deferasirox , ICT has become more widely available and feasible. (karger.com)
  • Some people may develop iron overload after 10 to 15 transfusions , while others may not develop it for many years, after many transfusions. (healthline.com)
  • Intrahepatic Cholangiocarcinoma (ICC) secondary to heterozygous H63D HH in the setting of secondary iron overload is a very rare occurrence. (scitechnol.com)
  • In a 2018-2019 real-world, observational study* of 165 patients with TDT conducted in the UK, a subset of patients exhibited significant iron overload while being managed and monitored in a specialist center. (challengetdt.com)
  • Hypochromic microcytic anemia with iron overload is a condition that impairs the normal transport of iron in cells. (checkorphan.org)
  • In this condition, red blood cells cannot access iron in the blood, so there is a decrease of red blood cell production (anemia) that is apparent at birth. (checkorphan.org)
  • Hypochromic microcytic anemia with iron overload can lead to pale skin (pallor), tiredness (fatigue), and slow growth. (checkorphan.org)
  • In hypochromic microcytic anemia with iron overload, the iron that is not used by red blood cells accumulates in the liver, which can impair its function over time. (checkorphan.org)
  • The lack of involvement of other tissues in hypochromic microcytic anemia with iron overload is likely because these tissues have other ways to transport iron. (checkorphan.org)
  • Too little iron leads to feelings of weakness, reduced immunity and anemia, all conditions that can become dangerous if not. (nutricionizam.com)
  • We describe a 62-year-old white woman with heavy liver iron overload, diabetes, anemia, and neurologic symptoms. (unito.it)
  • Although rare in whites, aceruloplasminemia should be considered in the differential diagnosis of unexplained anemia associated with iron overload, because these features anticipate progressive neurologic symptoms. (unito.it)
  • 3. In case of sickle cell anemia correction of iron overload by erythrocytapheresis. (haema-journal.gr)
  • Learn more about the Cooley's Anemia Foundation guidelines for managing transfusions and monitoring iron overload in your patients with TDT. (challengetdt.com)
  • The differential diagnosis of iron accumulation in macrophages also includes the anemia of inflammation, in which anemia is associated with inappropriate retention of iron in macrophages and hepatocytes despite iron-restricted erythropoiesis. (intrinsiclifesciences.com)
  • Hepcidin, which is expressed in the liver, heart, 16 and kidney, 17 is the key mediator of anemia of inflammation, 18,19 and synthetic hepcidin was shown to interact physically with ferroportin in a cellular overexpression system, causing internalization and degradation, and decreased export of iron. (intrinsiclifesciences.com)
  • CDC emphasizes sound iron nutrition for infants and young children, screening for anemia among women of childbearing age, and the importance of low-dose iron supplementation for pregnant women. (cdc.gov)
  • Stoyanova E, Cloutier G, Felfly H, Lemsaddek W, Ah-Son N, Trudel M. Evidence for a novel mechanism independent of myocardial iron in ß-thalassemia cardiac pathogenesis. (medscape.com)
  • The distribution of NTBI and the pattern of tissue iron uptake determine the pattern of organ damage, with myocardial muscle, endocrine tissue, and hepatocytes taking up NTBI rapidly. (challengetdt.com)
  • Symptomatic cardiac arrhythmias associated with myocardial iron overload pose significant clinical risk in older patients. (challengetdt.com)
  • Currently there are three iron chelating agents available for continuous use in patients with thalassemia on regular transfusions (desferrioxamine, deferiprone, and deferasirox) providing good results in reducing cardiac, hepatic and endocrine toxicity. (scielo.br)
  • Currently, a handful of US Food and Drug Administration (FDA)-approved therapies for MDS are available, including lenalidomide, the erythroid maturation agent luspatercept, hypomethylating agents such as azacitidine and decitabine, and iron chelators such as deferasirox. (medscape.com)
  • The dynamics of iron regulation in the body is multifaceted and is altered in transfusion-induced iron overload. (medscape.com)
  • [ 13 ] In addition, hyperabsoroption of iron from the diet is observed in patients with ineffective erythropoiesis, making them iron loaded even in the absence of blood transfusion. (medscape.com)
  • Transfusion-induced iron overload superimposed on hepatitis C would accelerate the development of cirrhosis. (medscape.com)
  • Transfusion and Iron Overload: Where Are the Risks? (haema-journal.gr)
  • Iron overload is still a problem in transfusion-dependent patients. (haema-journal.gr)
  • Chronic transfusion exceeds loading capacity of iron in macrophages and hepatocytes. (haema-journal.gr)
  • However, transfusion is not the only source of excessive iron input. (haema-journal.gr)
  • We studied 4 adult AA patients who had transfusion-induced iron overload. (karger.com)
  • When treatment combining transfusion and iron-chelating agent is started early and maintained, the patient's quality of life can be very good and the prognosis is optimistic. (who.int)
  • This is an autosomal recessive condition characterized by normal iron-driven erythropoiesis and toxic accumulation of iron in the parenchymal cells of the liver, heart and endocrine glands. (alliedacademies.org)
  • 3,8,10,11 Deletion of this amino acid causes loss of function and when studied in vitro causes accumulation of iron in cultured cells. (intrinsiclifesciences.com)
  • The liver pathology in many ways resembles that of human haemochromatosis, and may provide an experimental model for the study of chronic iron-induced liver injury. (bmj.com)
  • When Iron overload is suspected, genetic tests like C282Y for Hereditary Haemochromatosis are undertaken sometimes accompanied by liver biopsy and measurement of liver iron load. (alliedacademies.org)
  • Commonest iron overload condition seen is Hereditary Haemochromatosis (HH). (alliedacademies.org)
  • Haemochromatosis is a genetic iron overload condition. (haemochromatosis.org.au)
  • The modified iron avidity index: a promising phenotypic predictor in HFE-related haemochromatosis. (medscape.com)
  • Ferritin is a protein involved with iron regulation in the body. (healthline.com)
  • High ferritin levels could indicate iron overload. (healthline.com)
  • Provide an approximate level of iron overload, but not a reliable indicator of total body and liver or cardiac burden, as ferritin is an acute phase reactant and levels may change for a variety of non-iron-related reasons. (challengetdt.com)
  • [ 5 ] Circulating hepcidin reduces iron export into the plasma by binding to the iron export protein ferroportin 1 (FPN1) on the surface of enterocytes, macrophages, and other cells and causing its internalization and degradation. (medscape.com)
  • This patient was found to have iron overload with H63D heterozygous mutation and cirrhosis. (scitechnol.com)
  • We present a case of this patient with H63D heterozygous`mutation who developed iron overload as the first sign of cholangiocarcinoma. (scitechnol.com)
  • [ 4 ] H63D mutation does not result in constitutional iron overload unless it occurs as a compound heterozygote with C282Y (C282Y/H63D). (medscape.com)
  • Some evidence suggests that a genetic predisposition to absorbing too much iron may also be involved. (medlineplus.gov)
  • Researchers conducted a systematic review to further uncover the role of ebselen in relieving cardiomyopathy associated with iron overload in patients with thalassemia. (hematologyadvisor.com)
  • Intriguingly, the properties of ebselen…make it an exciting and promising possibility for adjuvant therapy in patients with thalassemia alongside the standard treatment with iron chelators, particularly in severe cases with cardiomyopathy," the authors of the study concluded. (hematologyadvisor.com)
  • Recent is aangetoond dat met name C282Y heterozygote mannen (vanaf 20 jaar) en vrouwen (vanaf 50 jaar) een sterk verhoogd risico hebben. (rivm.nl)
  • As a second step analysis was made of those who had C282Y genetic test for Iron overload. (alliedacademies.org)
  • RÉSUMÉ Des méthodes non-invasives de haute précision sont nécessaires pour l'évaluation de la concentration en fer dans les organes des patients atteints de thalassémie. (who.int)
  • L'évaluation systématique de la concentration de fer dans le foie et le coeur à l'aide de l'IRM pondérée en T2* semble produire une meilleure évaluation de la présence d'une hémosidérose chez les patients atteints de thalassémie. (who.int)
  • Hershko C. Iron loading and its clinical implications. (medscape.com)
  • In general, the term hemosiderosis is used to indicate the pathological effect of iron accumulation in any given organ, which mainly occurs in the form of the iron-storage complex hemosiderin. (wikipedia.org)
  • Iron overload, organ damage, Cassia Senna. (org.pk)
  • Figure 1 schematically represents organ lesions secondary to iron overload in chronically transfused patients (From Reference 1). (haema-journal.gr)
  • ABSTRACT There is a need for higly accurate non-invasive methods for assessing organ iron content in thalassaemia patients. (who.int)
  • Regulation of systemic iron homeostasis: how the body responds to changes in iron demand. (medscape.com)
  • Adjustments to the treatment plan may be made based on the patient's individual response and iron levels to ensure optimal results. (realtimes.info)
  • A specific portion of NTBI is the chelatable labile plasma iron (LPI), which is not found in healthy individuals. (medscape.com)
  • Your doctor can diagnose iron overload with laboratory tests and diagnostic imaging. (healthline.com)
  • Aetna considers laboratory testing medically necessary for heavy metal poisoning (e.g., arsenic, cadmium, copper, gold, iron, mercury) for members with specific signs and symptoms of heavy metal toxicity and/or a history of likely exposure to heavy metals. (aetna.com)
  • Humans cannot increase the excretion of iron, although some iron is lost through bleeding or when cells of the intestine (enterocytes) are shed at the end of the cells' lifespan. (medlineplus.gov)
  • Iron is an essential mineral to almost all life forms, including humans. (nutricionizam.com)
  • Iron overload and its association with cancer risk in humans: evidence for iron as a carcinogenic metal. (cdc.gov)
  • Iron supplementation or fortification in functional foods should be avoided and discouraged until the risks of iron overload have been more clearly determined, since in the general population iron overload is associated with increased risk of several chronic diseases as well. (rivm.nl)
  • Suppressed red blood cell production, for example, can lower your body's production of peptides that regulate iron uptake and storage within cells. (healthline.com)
  • Diseases of iron metabolism. (medscape.com)
  • [ 10 ] An excellent review of iron metabolism is available for those who desire more information. (medscape.com)
  • [ 12 ] Iron toxicity occurs as a result of the ferrous reactive forms of iron that reacts with oxidants, forming a complex that rapidly degrades proteins and DNA of a cell. (medscape.com)