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Management Of Thalassemia
Treatment of the inherited blood disorder thalassemia depends upon the level of severity. For mild forms of the condition, advice and counseling are often all that are necessary. For more severe forms, treatment may consist in blood transfusion; chelation therapy to reverse iron overload, using drugs such as deferoxamine, deferiprone, or deferasirox; medication with the antioxidant indicaxanthin to prevent the breakdown of hemoglobin; or a bone marrow transplant using material from a compatible donor, or from the patient's mother. Removal of the spleen (splenectomy) could theoretically help to reduce the need for blood transfusions in people with thalassaemia major or intermedia but there is currently no reliable evidence from clinical trials about its effects. Population screening (medicine), screening has had some success as a preventive measure. Levels of severity * Mild thalassemia : patients with thalassemia Phenotypic trait, traits do not require medical or follow-up care afte ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Thalassemia
Thalassemias are a group of Genetic disorder, inherited blood disorders that manifest as the production of reduced hemoglobin. Symptoms depend on the type of thalassemia and can vary from none to severe, including death. Often there is mild to severe anemia (low red blood cells or hemoglobin) as thalassemia can affect the production of red blood cells and also affect how long the red blood cells live. Symptoms include fatigue (medical), tiredness, pallor, bone problems, an splenomegaly, enlarged spleen, jaundice, pulmonary hypertension, and dark urine. A child's growth and development may be slower than normal. Thalassemias are genetic disorders. Alpha thalassemia is caused by deficient production of the Hemoglobin subunit alpha, alpha globin component of hemoglobin, while beta thalassemia is a deficiency in the Hemoglobin subunit beta, beta globin component. The severity of alpha and beta thalassemia depends on how many of the four genes for alpha globin or two genes for beta ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Transferrin
Transferrins are glycoproteins found in vertebrates which bind and consequently mediate the transport of iron (Fe) through blood plasma. They are produced in the liver and contain binding sites for two Iron(III), Fe3+ ions. Human transferrin is encoded by the ''TF'' gene and produced as a 76 Dalton (unit), kDa glycoprotein. Transferrin glycoproteins bind iron tightly, but reversibly. Although iron bound to transferrin is less than 0.1% (4 mg) of total body iron, it forms the most vital iron pool with the highest rate of turnover (25 mg/24 h). Transferrin has a molecular weight of around 80 atomic mass unit, kDa and contains two specific high-affinity Fe(III) binding sites. The affinity of transferrin for Fe(III) is extremely high (association constant is 1020 M−1 at pH 7.4) but decreases progressively with decreasing pH below neutrality. Transferrins are not limited to only binding to iron but also to different metal ions. These glycoproteins are located in various b ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Ferric
In chemistry, iron(III) or ''ferric'' refers to the chemical element, element iron in its +3 oxidation number, oxidation state. ''Ferric chloride'' is an alternative name for iron(III) chloride (). The adjective ''ferrous'' is used instead for iron(II) salts, containing the cation Fe2+. The word ''wikt:ferric, ferric'' is derived from the Latin word , meaning "iron". Although often abbreviated as Fe3+, that naked ion does not exist except under extreme conditions. Iron(III) centres are found in many compounds and coordination complexes, where Fe(III) is bonded to several Ligand, ligands. A molecular ferric complex is the anion ferrioxalate, , with three bidentate oxalate ions surrounding the Fe core. Relative to lower oxidation states, ferric is less common in organoiron chemistry, but the ferrocenium cation is well known. Iron(III) in biology All known forms of life require iron, which usually exists in Fe(II) or Fe(III) oxidation states. Many proteins in living beings cont ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Ligand
In coordination chemistry, a ligand is an ion or molecule with a functional group that binds to a central metal atom to form a coordination complex. The bonding with the metal generally involves formal donation of one or more of the ligand's electron pairs, often through Lewis acids and bases, Lewis bases. The nature of metal–ligand bonding can range from covalent bond, covalent to ionic bond, ionic. Furthermore, the metal–ligand bond order can range from one to three. Ligands are viewed as Lewis bases, although rare cases are known to involve Lewis acids and bases, Lewis acidic "ligands". Metals and metalloids are bound to ligands in almost all circumstances, although gaseous "naked" metal ions can be generated in a high vacuum. Ligands in a complex dictate the reactivity (chemistry), reactivity of the central atom, including ligand substitution rates, the reactivity of the ligands themselves, and redox. Ligand selection requires critical consideration in many practical are ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Chelating
Chelation () is a type of bonding of ions and their molecules to metal ions. It involves the formation or presence of two or more separate coordinate bonds between a polydentate (multiple bonded) ligand and a single central metal atom. These ligands are called chelants, chelators, chelating agents, or sequestering agents. They are usually organic compounds, but this is not a necessity. The word ''chelation'' is derived from Greek χηλή, ''chēlē'', meaning "claw"; the ligands lie around the central atom like the claws of a crab. The term ''chelate'' () was first applied in 1920 by Sir Gilbert T. Morgan and H. D. K. Drew, who stated: "The adjective chelate, derived from the great claw or ''chele'' (Greek) of the crab or other crustaceans, is suggested for the caliperlike groups which function as two associating units and fasten to the central atom so as to produce heterocyclic rings." Chelation is useful in applications such as providing nutritional supplements, in ch ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Hexadentate
A hexadentate ligand in coordination chemistry is a ligand that combines with a central metal atom with six bonds. One example of a hexadentate ligand that can form complexes with soft metal ions is TPEN. A commercially important hexadentate ligand is EDTA. The denticity of hexadentate ligands is often denoted with the prefix κ6. Topology The way the donor atoms are joined together in the molecule is its topology. Some topologies are simple, such as the linear or ring shapes. The molecule can also be branched, either at a donor atom, or at a non-donor atom. Example shapes are the tripod, and amplector, with a bifurcation at each end. Rigid molecules can be used to force unusual coordination such as trigonal prism In chemistry, octahedral molecular geometry, also called square bipyramidal, describes the shape of compounds with six atoms or groups of atoms or ligands symmetrically arranged around a central atom, defining the vertices of an octahedron. The oc .... F. Lions identif ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Streptomyces Pilosus
''Streptomyces pilosus'' is a bacterium species from the genus of ''Streptomyces'' which has been isolated from soil in Rome in Italy.Deutsche Sammlung von Mikroorganismen und Zellkulturenbr>/ref> ''Streptomyces pilosus'' produces piloquinone and the antidote desferrioxamine B. Further reading * * * * * * * * See also * List of Streptomyces species References External linksType strain of ''Streptomyces pilosus'' at Bac''Dive'' - the Bacterial Diversity Metadatabase pilosus ''Anthodiscus pilosus'' (chamisa, tahuari, botón caspi, botón huayo or tahuarí amarillo) is a plant species in the genus ''Anthodiscus'' found in Amazonian Colombia and Peru. ''Anthodiscus pilosus'' is added to some versions of the hallucinog ... Bacteria described in 1958 ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Actinomycetota
The Actinomycetota (or Actinobacteria) are a diverse phylum of Gram-positive bacteria with high GC content. They can be terrestrial or aquatic. They are of great importance to land flora because of their contributions to soil systems. In soil they help to decompose the organic matter of dead organisms so the molecules can be taken up anew by plants. While this role is also played by fungi, Actinomycetota are much smaller and likely do not occupy the same ecological niche. In this role the colonies often grow extensive mycelia, as fungi do, and the name of an important order of the phylum, Actinomycetales (the actinomycetes), reflects that they were long believed to be fungi. Some soil actinomycetota (such as '' Frankia'') live symbiotically with the plants whose roots pervade the soil, fixing nitrogen for the plants in exchange for access to some of the plant's saccharides. Other species, such as many members of the genus ''Mycobacterium'', are important pathogens. Beyond th ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Deferoxamine
Deferoxamine (DFOA), also known as desferrioxamine and sold under the brand name Desferal, is a medication that binds iron and aluminium. It is specifically used in iron overdose, hemochromatosis either due to multiple blood transfusions or an underlying genetic condition, and aluminium toxicity in people on dialysis. It is used by injection into a muscle, vein, or under the skin. Common side effects include pain at the site of injection, diarrhea, vomiting, fever, hearing loss, and eye problems. Severe allergic reactions including anaphylaxis and low blood pressure may occur. It is unclear if use during pregnancy or breastfeeding is safe for the baby. Deferoxamine is a siderophore from the bacteria '' Streptomyces pilosus''. Deferoxamine was approved for medical use in the United States in 1968. It is on the World Health Organization's List of Essential Medicines. Medical uses Deferoxamine is used to treat acute iron poisoning, especially in small children. This agent i ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Lipid Peroxidation
Lipid peroxidation, or lipid oxidation, is a complex chemical process that leads to oxidative degradation of lipids, resulting in the formation of peroxide and hydroperoxide derivatives.{{Cite journal , last1=Ayala , first1=Antonio , last2=Muñoz , first2=Mario F. , last3=Argüelles , first3=Sandro , date=2014 , title=Lipid Peroxidation: Production, Metabolism, and Signaling Mechanisms of Malondialdehyde and 4-Hydroxy-2-Nonenal , journal=Oxidative Medicine and Cellular Longevity , language=en , volume=2014 , pages=1–31 , doi=10.1155/2014/360438 , doi-access=free , issn=1942-0900 , pmc=4066722 , pmid=24999379 It occurs when free radicals, specifically reactive oxygen species (ROS), interact with lipids within cell membranes, typically polyunsaturated fatty acids (PUFAs) as they have carbon–carbon double bonds. This reaction leads to the formation of lipid radicals, collectively referred to as lipid peroxides or lipid oxidation products (LOPs), which in turn react with other ox ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
