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Erythropoietic Protoporphyria
Erythropoietic protoporphyria (or commonly called EPP) is a form of porphyria, which varies in severity and can be very painful. It arises from a deficiency in the enzyme ferrochelatase, leading to abnormally high levels of protoporphyrin in the red blood cells (erythrocytes), plasma, skin, and liver. The severity varies significantly from individual to individual. A clinically similar form of porphyria, known as X-Linked dominant protoporphyria, was identified in 2008. Presentation EPP usually presents in childhood with the most common mode of presentation as acute photosensitivity of the skin. It affects areas exposed to the sun and tends to be intractable. A few minutes of exposure to the sun induces pruritus, erythema, swelling and pain. Longer periods of exposure may induce second degree burns. After repetitive exposure, patients may present with lichenification, hypopigmentation, hyperpigmentation and scarring of the skin. EPP usually first presents in childhood, and most ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Porphyria
Porphyria ( or ) is a group of disorders in which substances called porphyrins build up in the body, adversely affecting the skin or nervous system. The types that affect the nervous system are also known as Porphyria#Acute porphyrias, acute porphyria, as symptoms are rapid in onset and short in duration. Symptoms of an attack include abdominal pain, chest pain, vomiting, confusion, constipation, fever, hypertension, high blood pressure, and tachycardia, high heart rate. The attacks usually last for days to weeks. Complications may include paralysis, hyponatraemia, low blood sodium levels, and seizures. Attacks may be triggered by Alcohol (drug), alcohol, smoking, hormonal changes, fasting, stress, or certain medications. If the skin is affected, blisters or itching may occur with sunlight exposure. Most types of porphyria are inherited from one or both of a person's parents and are due to a mutation in one of the genes that make heme. They may be inherited in an autosomal dom ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Sun Protective Clothing
Sun protective clothing is clothing specifically designed for sun protection and is produced from a fabric rated for its level of ultraviolet (UV) protection. A novel weave structure and denier (related to thread count per inch) may produce sun protective properties. In addition, some textiles and fabrics employed in the use of sun protective clothing may be pre-treated with UV-inhibiting ingredients during manufacture to enhance their effectiveness. In addition to special fabrics, sun protective clothing may also adhere to specific design parameters, including styling appropriate to full coverage of the skin most susceptible to UV damage. Long sleeves, ankle-length trousers, knee- to floor-length skirts, knee- to floor-length dresses, and collars are common styles for clothing as a sun protective measure. Regular clothing can leave you exposed to the damaging effects of UV radiation. However, a number of fabrics and textiles in common use today need no further UV-blocking enha ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Kidney Dialysis
Kidney dialysis is the process of removing excess water, solutes, and toxins from the blood in people whose kidneys can no longer perform these functions naturally. Along with kidney transplantation, it is a type of renal replacement therapy. Dialysis may need to be initiated when there is a sudden rapid loss of kidney function, known as acute kidney injury (previously called acute renal failure), or when a gradual decline in kidney function, chronic kidney failure, reaches stage 5. Stage 5 chronic renal failure is reached when the glomerular filtration rate is less than 15% of the normal, creatinine clearance is less than 10 mL per minute, and uremia is present. Dialysis is used as a temporary measure in either acute kidney injury or in those awaiting kidney transplant and as a permanent measure in those for whom a transplant is not Indication (medicine), indicated or not possible.Pendse S, Singh A, Zawada E. "Initiation of Dialysis". In: ''Handbook of Dialysis''. 4th ed. ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Acetylcysteine
''N''-acetylcysteine, also known as Acetylcysteine and NAC, is a mucolytics that is used to treat paracetamol (acetaminophen) overdose and to loosen thick mucus in individuals with chronic bronchopulmonary disorders, such as pneumonia and bronchitis. It has been used to treat lactobezoar in infants. It can be taken intravenously, orally (swallowed by mouth), or inhaled as a mist by use of a nebulizer. It is also sometimes used as a dietary supplement. Common side effects include nausea and vomiting when taken orally. The skin may occasionally become red and itchy with any route of administration. A non-immune type of anaphylaxis may also occur. It appears to be safe in pregnancy. For paracetamol overdose, it works by increasing the level of glutathione, an antioxidant that can neutralize the toxic breakdown products of paracetamol. When inhaled, it acts as a mucolytic by decreasing the thickness of mucus. Acetylcysteine was initially patented in 1960 and came into m ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Liver Transplantation
Liver transplantation or hepatic transplantation is the replacement of a diseased liver with the healthy liver from another person (allograft). Liver transplantation is a treatment option for end-stage liver disease and acute liver failure, although availability of donor organs is a major limitation. Liver transplantation is highly regulated, and only performed at designated transplant medical centers by highly trained transplant physicians. Favorable outcomes require careful screening for eligible recipients, as well as a well-calibrated live or deceased donor match. Medical uses Liver transplantation is a potential treatment for acute or chronic conditions which cause irreversible and severe ("end-stage") liver dysfunction. Since the procedure carries relatively high risks, is resource-intensive, and requires major life modifications after surgery, it is reserved for dire circumstances. Judging the appropriateness/effectiveness of liver transplant on case-by-case basis is ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Bone Marrow Transplantation
Hematopoietic stem-cell transplantation (HSCT) is the transplantation of multipotent hematopoietic stem cells, usually derived from bone marrow, peripheral blood, or umbilical cord blood, in order to replicate inside a patient and produce additional normal blood cells. HSCT may be autologous (the patient's own stem cells are used), syngeneic (stem cells from an identical twin), or allogeneic (stem cells from a donor). It is most often performed for patients with certain cancers of the blood or bone marrow, such as multiple myeloma, leukemia, some types of lymphoma and immune deficiencies. In these cases, the recipient's immune system is usually suppressed with radiation or chemotherapy before the transplantation. Infection and graft-versus-host disease are major complications of allogeneic HSCT. HSCT remains a dangerous procedure with many possible complications; it is reserved for patients with life-threatening diseases. As survival following the procedure has increa ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Activated Carbon
Activated carbon, also called activated charcoal, is a form of carbon commonly used to filter contaminants from water and air, among many other uses. It is processed (activated) to have small, low-volume pores that greatly increase the surface area available for ''adsorption '' or chemical reactions. (Adsorption, not to be confused with Absorption (chemistry), absorption, is a process where atoms or molecules adhere to a surface). The pores can be thought of as a microscopic "sponge" structure. Activation is analogous to making popcorn from dried corn kernels: popcorn is light, fluffy, and its kernels have a high surface-area-to-volume ratio. ''Activated'' is sometimes replaced by ''active''. Because it is so porous on a microscopic scale, one gram of activated carbon has a surface area of over , as determined by gas absorption and its porosity can run 10ML/day in terms of treated water per gram. Researchers at Cornell University synthesized an ultrahigh surface area activated ca ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Resin
A resin is a solid or highly viscous liquid that can be converted into a polymer. Resins may be biological or synthetic in origin, but are typically harvested from plants. Resins are mixtures of organic compounds, predominantly terpenes. Common resins include amber, hashish, frankincense, myrrh and the animal-derived resin, shellac. Resins are used in varnishes, adhesives, food additives, incenses and perfumes. Resins protect plants from insects and pathogens, and are secreted in response to injury. Resins repel herbivores, insects, and pathogens, while the volatile natural phenol, phenolic compounds may attract benefactors such as predators of insects that attack the plant. Composition Most plant resins are composed of terpenes. Specific components are alpha-Pinene, alpha-pinene, pinene, beta-pinene, carene, delta-3 carene, and sabinene, the monocyclic terpenes limonene and terpinolene, and smaller amounts of the tricyclic sesquiterpenes, longifolene, caryophyllene, and cad ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Cholestyramine
Colestyramine ( INN) or cholestyramine ( USAN) (trade names Questran, Questran Light, Cholybar, Olestyr, Quantalan, Vasosan) is a bile acid sequestrant, which binds bile in the gastrointestinal tract to prevent its reabsorption. It is a strong ion exchange resin, which means it can exchange its chloride anions with anionic bile acids in the gastrointestinal tract and bind them strongly in the resin matrix. The functional group of the anion exchange resin is a quaternary ammonium group attached to an inert styrene- divinylbenzene copolymer. Colestyramine removes bile acids from the body by forming insoluble complexes with bile acids in the intestine, which are then excreted in the feces. As a result of this loss of bile acids, more plasma cholesterol is converted to bile acids in the liver to normalise levels. This conversion of cholesterol into bile acids lowers plasma cholesterol levels. Medical uses Bile acid sequestrants such as colestyramine were first used to treat hype ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Plasmapheresis
Plasmapheresis (from the Greek language, Greek πλάσμα, ''plasma'', something molded, and ἀφαίρεσις ''aphairesis'', taking away) is the removal, treatment, and return or exchange of blood plasma or components thereof from and to the circulatory system, blood circulation. It is thus an extracorporeal therapy, a medical procedure performed outside the body. Three general types of plasmapheresis can be distinguished: * ''Autologous'', removing blood plasma, treating it in some way, and returning it to the same person, as a therapy. * ''Exchange'', a patient's blood plasma is removed, while blood products are given in replacement. This type is called plasma exchange (PE, PLEX, or PEX) or plasma exchange therapy (PET). The removed plasma is discarded and the patient receives replacement donor plasma, albumin, or a combination of albumin and saline (medicine), saline (usually 70% albumin and 30% saline). * ''Donation'', removing blood plasma, separating its components, and ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Hematin
Haematin (also known as hematin, ferriheme, hematosin, hydroxyhemin, oxyheme, phenodin, or oxyhemochromogen) is a dark bluish or brownish pigment containing iron in the ferric state, obtained by the oxidation of haem. Haematin inhibits the synthesis of porphyrin (by repressing ALAS1 synthesis), and stimulates the synthesis of globin. For this reason, it is used in the treatment of porphyrias. It is a component of cytochromes and peroxidases Peroxidases or peroxide reductases ( EC numberbr>1.11.1.x are a large group of enzymes which play a role in various biological processes. They are named after the fact that they commonly break up peroxides, and should not be confused with other .... Haematin derived synthetically from hemin is used as a reagent. References Iron(III) compounds Pigments {{organic-compound-stub ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Bile Acid
Bile acids are steroid acids found predominantly in the bile of mammals and other vertebrates. Diverse bile acids are synthesized in the liver in peroxisomes. Bile acids are conjugated with taurine or glycine residues to give anions called bile salts. Primary bile acids are those synthesized by the liver. Secondary bile acids result from bacterial actions in the colon. In humans, taurocholic acid and glycocholic acid (derivatives of cholic acid) and taurochenodeoxycholic acid and glycochenodeoxycholic acid (derivatives of chenodeoxycholic acid) are the major bile salts. The salts of their 7-alpha-dehydroxylated derivatives, deoxycholic acid and lithocholic acid, are also found, with derivatives of cholic, chenodeoxycholic and deoxycholic acids accounting for over 90% of human biliary bile acids. Description Bile acids comprise about 80% of the organic compounds in bile (others are phospholipids and cholesterol). An increased secretion of bile acids produces an increase ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
