query: What is porphyria?

title: Acute intermittent porphyria

text: medallion struck to commemorate his "curing". His great-great-great-great-grandson Prince William of Gloucester was reliably diagnosed with variegate porphyria in 1968. It is probable that the philosopher Jean-Jacques Rousseau suffered from porphyria. Acute intermittent porphyria Acute intermittent porphyria (AIP), also called Swedish porphyria, pyrroloporphyria, intermittent acute porphyria) is a rare autosomal dominant metabolic disorder affecting the production of heme resulting from a deficiency of the porphobilinogen deaminase. It is the most common of the acute porphyrias. The clinical presentation of AIP is highly variable and non-specific. The patients are typically asymptomatic, with most gene carriers having no family history because the

title: Acute intermittent porphyria

text: Acute intermittent porphyria Acute intermittent porphyria (AIP), also called Swedish porphyria, pyrroloporphyria, intermittent acute porphyria) is a rare autosomal dominant metabolic disorder affecting the production of heme resulting from a deficiency of the porphobilinogen deaminase. It is the most common of the acute porphyrias. The clinical presentation of AIP is highly variable and non-specific. The patients are typically asymptomatic, with most gene carriers having no family history because the condition had remained latent for several generations. The syndrome marked by acute attacks affects only 10% of gene carriers. The mean age at diagnosis is 33 years old. Like other porphyrias,

title: Variegate porphyria

text: transplant has been used in the treatment of this condition. In South Africa, the prevalence of variegate porphyria is approximately 1 in 300. In Finland, the prevalence is approximately 1 in 75,000. When it does occur in other populations (such as Switzerland), it can be with different mutations than in South Africa. It is also found in Argentina, Sweden, and Australia. Variegate porphyria is also known as mixed hepatic porphyria, mixed porphyria, South African genetic porphyria, and South African porphyria. "This article incorporates public domain text from The U.S. National Library of Medicine" Variegate porphyria Variegate porphyria, also known by

title: Porphyria

text: van Gogh may have had acute intermittent porphyria. It has also been speculated that King Nebuchadnezzar of Babylon had some form of porphyria as described in Daniel 4. However, the symptoms of the various porphyrias are so extensive that a wide constellation of symptoms can be attributed to one or more of them. Physician Archie Cochrane was born with porphyria, which caused health problems throughout his life. Paula Frías Allende, the daughter of the Chilean novelist Isabel Allende, fell into a porphyria-induced coma in 1991, which inspired Isabel to write the memoir "Paula," dedicated to her. Porphyria Porphyria is a

title: Porphyria

text: the bone marrow is affected. Diagnosis is typically by blood, urine, and stool tests. Genetic testing may be done to determine the specific mutation. Treatment depends on the type of porphyria and a person's symptoms. The treatment of porphyria of the skin generally involves the avoidance of sunlight. The treatment for acute porphyria may involve giving intravenous heme or a glucose solution. Rarely a liver transplant may be carried out. The frequency of porphyria is unclear. It is estimated that it affects 1 to 100 per 50,000 people. Rates vary around the world. Porphyria cutanea tarda is believed to be

title: Porphyria

text: a partial deficiency in PROTO oxidase, manifests itself with skin lesions similar to those of porphyria cutanea tarda combined with acute neurologic attacks. All other porphyrias are either skin- or nerve-predominant. Porphyria is diagnosed through biochemical analysis of blood, urine, and stool. In general, urine estimation of porphobilinogen (PBG) is the first step if acute porphyria is suspected. As a result of feedback, the decreased production of heme leads to increased production of precursors, PBG being one of the first substances in the porphyrin synthesis pathway. In nearly all cases of acute porphyria syndromes, urinary PBG is markedly elevated except

title: Porphyria

text: the most common type. The disease was described at least as early as 370 BC by Hippocrates. The underlying mechanism was first described by Felix Hoppe-Seyler in 1871. The name "porphyria" is from the Greek πορφύρα, "porphyra", meaning "purple", a reference to the color of the urine that may occur during an attack. The acute porphyrias are acute intermittent porphyria (AIP), variegate porphyria (VP), aminolevulinic acid dehydratase deficiency porphyria (ALAD) and hereditary coproporphyria (HCP). These diseases primarily affect the nervous system, resulting in episodic crises known as acute attacks. The major symptom of an acute attack is abdominal pain, often

title: Porphyria

text: to have other, unrelated conditions. For instance, the polyneuropathy of acute porphyria may be mistaken for Guillain–Barré syndrome, and porphyria testing is commonly recommended in those situations. The non-acute porphyrias are X-linked dominant protoporphyria (XLDPP), congenital erythropoietic porphyria (CEP), porphyria cutanea tarda (PCT), and erythropoietic protoporphyria (EPP). None of these are associated with acute attacks; their primary manifestation is with skin disease. For this reason, these four porphyrias—along with two acute porphyrias, VP and HCP, that may also involve skin manifestations—are sometimes called cutaneous porphyrias. Skin disease is encountered where excess porphyrins accumulate in the skin. Porphyrins are photoactive molecules,

title: Porphyria

text: and whether the intermediate is excreted (in the urine or feces). There are eight enzymes in the heme biosynthetic pathway, four of which—the first one and the last three—are in the mitochondria, while the other four are in the cytosol. Defects in any of these can lead to some form of porphyria. The hepatic porphyrias are characterized by acute neurological attacks (seizures, psychosis, extreme back and abdominal pain, and an acute polyneuropathy), while the erythropoietic forms present with skin problems, usually a light-sensitive blistering rash and increased hair growth. "Variegate porphyria" (also "porphyria variegata" or "mixed porphyria"), which results from

title: Porphyria cutanea tarda

text: Porphyria cutanea tarda Porphyria cutanea tarda (PCT) is the most common subtype of porphyria. The disease is named because it is a porphyria that often presents with skin manifestations later in life. The disorder results from low levels of the enzyme responsible for the fifth step in heme production. Heme is a vital molecule for all of the body's organs. It is a component of hemoglobin, the molecule that carries oxygen in the blood. Hepatoerythropoietic porphyria has been described as a homozygous form of porphyria cutanea tarda, although it can also be caused if two different mutations occur at the

title: Amanita porphyria

text: porphyria" usually grows near coniferous trees. "A. porphyria" is not suitable for consumption. It is considered slightly poisonous and, more importantly, can easily be confused with much more poisonous species such as the Panther cap "(Amanita pantherina)". Amanita porphyria Amanita porphyria, also known as the grey veiled amanita, is a fairly common, inedible basidiomycete mushroom of the genus "Amanita" found in Europe and North America. The cap is hemispherical when young, later flat. It is 4–10 cm in diameter, greyish brown to brown in colour, usually smooth, sometimes with grey patches. The gills are white and free from the stem.

title: Variegate porphyria

text: Variegate porphyria Variegate porphyria, also known by several other names, is an autosomal dominant porphyria that can have acute (severe but usually not long-lasting) symptoms along with symptoms that affect the skin. The disorder results from low levels of the enzyme responsible for the seventh step in heme production. Heme is a vital molecule for all of the body's organs. It is a component of hemoglobin, the molecule that carries oxygen in the blood. When symptoms occur, they can include acute attacks (similar to acute intermittent porphyria), skin damage, or both. Acute attacks usually begin in adulthood and cause abdominal

title: Porphyria

text: as far more probable. The theory is treated in "Purple Secret," which documents the ultimately unsuccessful search for genetic evidence of porphyria in the remains of royals suspected to have had it. In 2005, it was suggested that arsenic (which is known to be porphyrogenic) given to George III with antimony may have caused his porphyria. This study found high levels of arsenic in King George's hair. In 2010, one analysis of historical records argued that the porphyria claim was based on spurious and selective interpretation of contemporary medical and historical sources. The mental illness of George III is the

title: Porphyria

text: psychosis as a more likely diagnosis. The first suggestion that a physical illness was the cause of King George's mental derangement came in 1966, in a paper called "The Insanity of King George III: A Classic Case of Porphyria", with a follow-up in 1968, "Porphyria in the Royal Houses of Stuart, Hanover and Prussia". The papers, by a mother/son psychiatrist team, were written as though the case for porphyria had been proven, but the response demonstrated that many experts, including those more intimately familiar with the manifestations of porphyria, were unconvinced. Many psychiatrists disagreed with the diagnosis, suggesting bipolar disorder

title: Porphyria cutanea tarda

text: implicated in the origin of vampire myths. This is because people with the disease tend to avoid the sun due to photosensitivity (and therefore have a young complexion and pallor) and require blood. Hypertrichosis (i.e. A full head of hair) will also give a young appearance. Porphyria Cutanea Tarda is the name of a song by the rock band AFI on their fourth album "Black Sails in the Sunset", released on May 18, 1999. Porphyria cutanea tarda Porphyria cutanea tarda (PCT) is the most common subtype of porphyria. The disease is named because it is a porphyria that often presents

title: Erythropoietic porphyria

text: use Zn in its place, causing a buildup of zinc-protoporphyrin IX. Erythropoietic porphyria Erythropoietic porphyria is a type of porphyria associated with erythropoietic cells. In erythropoietic porphyrias, the enzyme deficiency occurs in the red blood cells. There are three types: X-linked dominant erythropoietic protoporphyria is a relatively mild version of porphyria with the predominant symptom being extreme photosensitivity causing severe itching and burning sensation of the skin due to the buildup of protoporphyrin IX. One possible treatment was discovered when treating an individual with supplemental iron for a gastric ulcer. Levels of free protoporphyrin decreased significantly as there was iron

title: Erythropoietic porphyria

text: Erythropoietic porphyria Erythropoietic porphyria is a type of porphyria associated with erythropoietic cells. In erythropoietic porphyrias, the enzyme deficiency occurs in the red blood cells. There are three types: X-linked dominant erythropoietic protoporphyria is a relatively mild version of porphyria with the predominant symptom being extreme photosensitivity causing severe itching and burning sensation of the skin due to the buildup of protoporphyrin IX. One possible treatment was discovered when treating an individual with supplemental iron for a gastric ulcer. Levels of free protoporphyrin decreased significantly as there was iron available for the FECH to produce heme. Levels of zinc-protoporphyrin, however

title: Porphyria

text: better evidence that George III's great-great-great-grandson Prince William of Gloucester was reliably diagnosed with variegate porphyria. It is believed that Mary, Queen of Scots, King George III's ancestor, also had acute intermittent porphyria, although this is subject to much debate. It is assumed she inherited the disorder, if indeed she had it, from her father, James V of Scotland. Both father and daughter endured well-documented attacks that could fall within the constellation of symptoms of porphyria. Maria I of Portugal—known as "Maria the Pious" or "Maria the Mad" because of both her religious fervor and her acute mental illness, which

title: Porphyria

text: made her incapable of handling state affairs after 1792 – is also thought to have had porphyria. Francis Willis, the same physician who treated George III, was even summoned by the Portuguese court but returned to England after the court limited the treatments he could oversee. Contemporary sources, such as Secretary of State for Foreign Affairs Luís Pinto, noted that the queen had ever-worsening stomach pains and abdominal spasms: hallmarks of porphyria. Vlad III was also said to have had acute porphyria, which may have started the notion that vampires were allergic to sunlight. Other commentators have suggested that Vincent

title: Porphyria

text: Porphyria Porphyria is a group of diseases in which substances called porphyrins build up, negatively affecting the skin or nervous system. The types that affect the nervous system are also known as acute porphyria, as symptoms are rapid in onset and last a short time. Symptoms of an attack include abdominal pain, chest pain, vomiting, confusion, constipation, fever, high blood pressure, and high heart rate. The attacks usually last for days to weeks. Complications may include paralysis, low blood sodium levels, and seizures. Attacks may be triggered by alcohol, smoking, hormonal changes, fasting, stress, or certain medications. If the skin

title: Porphyria

text: "Proceedings of the Royal Society of Medicine. "Later, Nancy Garden argued for a connection between porphyria and the vampire belief in her 1973 book, "Vampires". In 1985, biochemist David Dolphin's paper for the American Association for the Advancement of Science, "Porphyria, Vampires, and Werewolves: The Aetiology of European Metamorphosis Legends," gained widespread media coverage, popularizing the idea. The theory has been rejected by a few folklorists and researchers as not accurately describing the characteristics of the original werewolf and vampire legends or the disease, and as potentially stigmatizing people with porphyria. A 1995 article from the "Postgraduate Medical Journal" (via

title: Porphyria

text: carriers of the more common, dominantly inherited acute hepatic porphyrias (acute intermittent porphyria, hereditary coproporphyria, variegate porphyria) have been noted in DNA tests to be latent for classic symptoms and may require DNA or enzyme testing. The exception to this may be latent post-puberty genetic carriers of hereditary coproporphyria. As most porphyrias are rare conditions, general hospital labs typically do not have the expertise, technology, or staff time to perform porphyria testing. In general, testing involves sending samples of blood, stool, and urine to a reference laboratory. All samples to detect porphyrins must be handled properly. Samples should be taken

title: Porphyria

text: for the very rare ALA dehydratase deficiency or in patients with symptoms due to hereditary tyrosinemia type I. In cases of mercury- or arsenic poisoning-induced porphyria, other changes in porphyrin profiles appear, most notably elevations of uroporphyrins I & III, coproporphyrins I & III, and pre-coproporphyrin. Repeat testing during an attack and subsequent attacks may be necessary in order to detect a porphyria, as levels may be normal or near-normal between attacks. The urine screening test has been known to fail in the initial stages of a severe, life-threatening attack of acute intermittent porphyria. Up to 90% of the genetic

title: Aminolevulinic acid dehydratase deficiency porphyria

text: Aminolevulinic acid dehydratase deficiency porphyria Aminolevulinic acid dehydratase deficiency porphyria (also known as "Doss porphyria", and "plumboporphyria") is a neuropsychiatric condition, disease can present during early childhood (as well as in adulthood) with acute neurologic symptoms that resemble those encountered in acute intermittent porphyria. The condition is extremely rare, with fewer than 10 cases ever reported. ALA dehydratase deficiency is a rare cause of hepatic porphyria. It is an autosomal recessive disorder that results from inappropriately low levels of the enzyme ALA dehydratase (ALAD, also called porphobilinogen synthase), which is required for normal heme synthesis. ALA dehydratase deficiency is inherited

title: Porphyria

text: but is sometimes intractable. Hot baths and showers may lessen nausea temporarily, though caution should be used to avoid burns or falls. It is recommended that patients with a history of acute porphyria, and even genetic carriers, wear an alert bracelet or other identification at all times. This is in case they develop severe symptoms, or in case of accidents where there is a potential for drug exposure, and as a result they are unable to explain their condition to healthcare professionals. Some drugs are absolutely contraindicated for patients with any form of porphyria. Patients who experience frequent attacks can

title: Porphyria cutanea tarda

text: because of liver toxicity. Finally, due to the strong association between PCT and Hepatitis C, the treatment of Hepatitis C (if present) is vital to the effective treatment of PCT. Chloroquine, hydroxychloroquine, and venesection are typically employed in the management strategy. Porphyria cutanea tarda has a prevalence estimated at approximately 1 in 10,000. An estimated 80% of porphyria cutanea tarda cases are sporadic. The exact frequency is not clear because many people with the condition never experience symptoms and those that do are often misdiagnosed with anything ranging from idiopathic photodermatitis and seasonal allergies to hives. Porphyria cutanea tarda is

title: Porphyria

text: basis of the plot in "The Madness of King George", a 1994 British film based upon the 1991 Alan Bennett play, "The Madness of George III". The closing credits of the film include the comment that the illness that King George had has been attributed to porphyria and that it is hereditary. Among other descendants of George III theorized by the authors of "Purple Secret" to have had porphyria (based on analysis of their extensive and detailed medical correspondence) were his great-great-granddaughter Princess Charlotte of Prussia (Emperor William II's eldest sister) and her daughter Princess Feodora of Saxe-Meiningen. They uncovered

title: Porphyria

text: and exposure to light results in promotion of electrons to higher energy levels. When these return to the resting energy level or ground state, energy is released. This accounts for the property of fluorescence typical of the porphyrins. This causes local skin damage. Two distinct patterns of skin disease are seen in porphyria: The porphyrias are generally considered genetic in nature. Acute porphyria can be triggered by a number of drugs, most of which are believed to trigger it by interacting with enzymes in the liver which are made with heme. Such drugs include: Subtypes of porphyrias depend on which

title: Porphyria

text: is affected, blisters or itching may occur with sunlight exposure. Most types of porphyria are inherited from 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 dominant, autosomal recessive, or X-linked dominant manner. One type, porphyria cutanea tarda, may also be due to increased iron in the liver, hepatitis C, alcohol, or HIV/AIDS. The underlying mechanism results in a decrease in the amount of heme produced and a build-up of substances involved in making heme. Porphyrias may also be classified by whether the liver or

title: Amanita porphyria

text: Amanita porphyria Amanita porphyria, also known as the grey veiled amanita, is a fairly common, inedible basidiomycete mushroom of the genus "Amanita" found in Europe and North America. The cap is hemispherical when young, later flat. It is 4–10 cm in diameter, greyish brown to brown in colour, usually smooth, sometimes with grey patches. The gills are white and free from the stem. The spores are white. The stem is 5–12 cm high and 1-1.5 cm thick and has a grey to black ring. The stem has a basal bulb. The flesh is white with a smell of radish. "A.

title: Oliva porphyria

text: Oliva porphyria Oliva porphyria, common name the tent olive, is a species of sea snail, a marine gastropod mollusk in the family Olividae, the olives. Its shell pattern has been studied as an example of a Turing pattern that can be modeled with cellular automata. The length of the shell can vary between 30 mm and 135 mm. The flesh-colored shell is angularly marked with some large, and many small, crowded, deep chestnut lines. The fascicle is tinged with violet, with chestnut maculations. The interior of the aperture and columella is yellowish flesh-color. Sometimes the shell is very faintly, broadly

title: Variegate porphyria

text: such cases, the signs and symptoms are usually more severe than those starting later in life. In addition to the health problems described above, children with this disorder may have mental retardation and grow more slowly than other children. Mutations in the PPOX gene cause variegate porphyria. The PPOX gene makes a membrane bound mitochondrial enzyme called protoporphyrinogen oxidase, which is critical to the chemical process that leads to heme production. The activity of this enzyme is reduced by 50 percent in most people with variegate porphyria. In severe cases that begin early in life, the enzyme is almost completely

title: Oliva porphyria

text: two- or three-banded with bluish ash. This marine species occurs in the Panama Zone to Western Mexico (Gulf of California), to Northern Peru. Oliva porphyria Oliva porphyria, common name the tent olive, is a species of sea snail, a marine gastropod mollusk in the family Olividae, the olives. Its shell pattern has been studied as an example of a Turing pattern that can be modeled with cellular automata. The length of the shell can vary between 30 mm and 135 mm. The flesh-colored shell is angularly marked with some large, and many small, crowded, deep chestnut lines. The fascicle is

title: Porphyria

text: NIH) explains: As it was believed that the folkloric vampire could move about freely in daylight hours, as opposed to the 20th century variant, congenital erythropoietic porphyria cannot readily explain the folkloric vampire but may be an explanation of the vampire as we know it in the 20th century. In addition, the folkloric vampire, when unearthed, was always described as looking quite healthy ("as they were in life"), while due to disfiguring aspects of the disease, sufferers would not have passed the exhumation test. Individuals with congenital erythropoietic porphyria do not crave blood. The enzyme (hematin) necessary to alleviate symptoms

title: Porphyria

text: first described by Felix Hoppe-Seyler in 1871, and acute porphyrias were described by the Dutch physician Barend Stokvis in 1889. The links between porphyrias and mental illness have been noted for decades. In the early 1950s, patients with porphyrias (occasionally referred to as "porphyric hemophilia") and severe symptoms of depression or catatonia were treated with electroshock therapy. Porphyria has been suggested as an explanation for the origin of vampire and werewolf legends, based upon certain perceived similarities between the condition and the folklore. In January 1964, L. Illis's 1963 paper, "On Porphyria and the Aetiology of Werwolves," was published in

title: Porphyria

text: erythropoietic porphyria (C.E.P.), otherwise known as Gunther's disease. The signs may present from birth and include severe photosensitivity, brown teeth that fluoresce in ultraviolet light due to deposition of Type 1 porphyrins, and later hypertrichosis. Hemolytic anemia usually develops. Pharmaceutical-grade beta carotene may be used in its treatment. A bone marrow transplant has also been successful in curing CEP in a few cases, although long-term results are not yet available. In December 2014, afamelanotide received authorization from the European Commission as a treatment for the prevention of phototoxicity in adult patients with EPP. Rates of all types of porphyria taken

title: Porphyria

text: in porphyria may not respond to magnesium alone. The addition of hematin or heme arginate has been used during status epilepticus. Some liver diseases may cause porphyria even in the absence of genetic predisposition. These include hemochromatosis and hepatitis C. Treatment of iron overload may be required. Patients with the acute porphyrias (AIP, HCP, VP) are at increased risk over their life for hepatocellular carcinoma (primary liver cancer) and may require monitoring. Other typical risk factors for liver cancer need not be present. Hormonal fluctuations that contribute to cyclical attacks in women have been treated with oral contraceptives and luteinizing

title: Variegate porphyria

text: pain, vomiting, diarrhoea and constipation. During an attack, a person may also experience muscle weakness, seizures, and mental changes such as anxiety and hallucinations. These signs and symptoms are triggered by nongenetic factors such as certain drugs, dieting or fasting, certain hormones and stress. Some people with variegate porphyria have skin that is overly sensitive to sunlight (photosensitive). Areas of skin exposed to the sun develop severe blistering, scarring, changes in pigmentation, and increased hair growth. Exposed skin becomes fragile and is easily damaged. Rarely, the signs and symptoms of variegate porphyria can begin in infancy or early childhood. In

text: in an autosomal recessive manner. This means a defective gene responsible for the disorder is located on an autosome, and two copies of the defective gene (one inherited from each parent) are required in order to be born with the disorder. The parents of an individual with an autosomal recessive disorder both carry one copy of the defective gene, but usually do not experience any signs or symptoms of the disorder. Aminolevulinic acid dehydratase deficiency porphyria Aminolevulinic acid dehydratase deficiency porphyria (also known as "Doss porphyria", and "plumboporphyria") is a neuropsychiatric condition, disease can present during early childhood (as well

title: Porphyria cutanea tarda

text: the body. Certain mutations in the "HFE" gene cause hemochromatosis (an iron overload disorder). People who have these mutations are also at an increased risk of developing porphyria cutanea tarda. In the 20% of cases where porphyria cutanea tarda is inherited, it is inherited in an autosomal dominant pattern, which means one copy of the altered gene is sufficient to decrease enzyme activity and cause the signs and symptoms of the disorder. While inherited deficiencies in uroporphyrinogen decarboxylase often lead to the development of PCT, there are a number of risk factors that can both cause and exacerbate the symptoms

title: Porphyria

text: is not absorbed intact on oral ingestion, and drinking blood would have no beneficial effect on the sufferer. Finally, and most important, the fact that vampire reports were literally rampant in the 18th century, and that congenital erythropoietic porphyria is an extremely rare manifestation of a rare disease, makes it an unlikely explanation of the folkloric vampire. The mental illness exhibited by King George III in the regency crisis of 1788 has inspired several attempts at retrospective diagnosis. The first, written in 1855, thirty-five years after his death, concluded that he had acute mania. M. Guttmacher, in 1941, suggested manic-depressive

title: Porphyria

text: fatal. A high-carbohydrate diet is typically recommended; in severe attacks, a dextrose 10% infusion is commenced, which may aid in recovery by suppressing heme synthesis, which in turn reduces the rate of porphyrin accumulation. However, this can worsen hyponatraemia and should be done with extreme caution as it can prove fatal. Hematin (trade name Panhematin) and heme arginate (trade name NormoSang) are the drugs of choice in acute porphyria, in the United States and the United Kingdom, respectively. These drugs need to be given very early in an attack to be effective; effectiveness varies amongst individuals. They are not curative

title: Porphyria

text: during an acute attack; otherwise a false negative result may occur. Samples must be protected from light and either refrigerated or preserved. If all the porphyrin studies are negative, one must consider pseudoporphyria. A careful medication review often will find the cause of pseudoporphyria. Further diagnostic tests of affected organs may be required, such as nerve conduction studies for neuropathy or an ultrasound of the liver. Basic biochemical tests may assist in identifying liver disease, hepatocellular carcinoma, and other organ problems. Often, empirical treatment is required if the diagnostic suspicion of a porphyria is high since acute attacks can be

title: Variegate porphyria

text: of the defective gene. The entire PPOX gene has about 8kb with 13 exon sequences. It was successfully cloned from a cDNA library in 1995 revealing that, after processing, it is 477 nucleotides long. It has previously been thought that the PPOX gene was located on human chromosome 14, however mapping experiments (FISH) have shown that it is near 1q23. An additional aggravating mutation affecting variegate porphyria can be found at 6p21.3 on the HFE gene. Diagnosis is by finding raised urine porphyrins, raised faecal porphyrins, markedly raised plasma porphyrins (pathognomic) and finding photosensitive cutaneous lesions on clinical examination. Liver

title: Variegate porphyria

text: inactive. Nongenetic factors such as certain drugs, stress, and others listed above can increase the demand for heme and the enzymes required to make heme. The combination of this increased demand and reduced activity of protoporphyrinogen oxidase disrupts heme production and allows byproducts of the process to accumulate in the liver, triggering an acute attack. Variegate porphyria is inherited in an autosomal dominant pattern, which means the defective gene is located on an autosome, and inheriting one copy of the defective gene from an affected parent is sufficient to cause the disorder. More severe cases result from inheriting two copies

title: Porphyria

text: drugs are porphyrinogenic, limiting the therapeutic scope. Other psychiatric symptoms such as anxiety, restlessness, insomnia, depression, mania, hallucinations, delusions, confusion, catatonia, and psychosis may occur. Seizures often accompany this disease. Most seizure medications exacerbate this condition. Treatment can be problematic: barbiturates especially must be avoided. Some benzodiazepines are safe and, when used in conjunction with newer anti-seizure medications such as gabapentin, offer a possible regimen for seizure control. Gabapentin has the additional feature of aiding in the treatment of some kinds of neuropathic pain. Magnesium sulfate and bromides have also been used in porphyria seizures; however, development of status epilepticus

title: Porphyria

text: accompanied by vomiting, hypertension (elevated blood pressure), and tachycardia (an abnormally rapid heart rate). The most severe episodes may involve neurological complications: typically motor neuropathy (severe dysfunction of the peripheral nerves that innervate muscle), which leads to muscle weakness and potentially to quadriplegia (paralysis of all four limbs) and central nervous system symptoms such as seizures and coma. Occasionally, there may be short-lived psychiatric symptoms such as anxiety, confusion, hallucinations, and, very rarely, overt psychosis. All these symptoms resolve once the acute attack passes. Given the many presentations and the relatively low occurrence of porphyria, patients may initially be suspected

title: Porphyria cutanea tarda

text: same locus. Porphyria cutanea tarda (commonly referred to as PCT) is recognized as the most prevalent subtype of porphyritic diseases. The disease is characterized by onycholysis and blistering of the skin in areas that receive higher levels of exposure to sunlight. The primary cause of this disorder is a deficiency of uroporphyrinogen decarboxylase (UROD), a cytosolic enzyme that is a step in the enzymatic pathway that leads to the synthesis of heme. While a deficiency in this enzyme is the direct cause leading to this disorder, there are a number of both genetic and environmental risk factors that are associated

title: Acute intermittent porphyria

text: all cells, but 80% of all heme is produced in erythropoietic cells in bone marrow and 15% in parenchymal cells in the liver, where turnover of hemoproteins is high. In AIP, over 100 mutations have been identified on the long arm of chromosome 11 at the "HMBS" gene, which codes for the cytoplasmic enzyme porphobilinogen deaminase. This deficiency prevents heme synthesis, which can not be completed and the metabolite porphobilinogen accumulates in the cytoplasm. AIP is an autosomal dominant porphyria resulting in about 50% normal activity of the affected enzyme. The penetrance of AIP is incomplete with only 10% of

title: Acute intermittent porphyria

text: called port-wine urine), but this is a nonspecific symptom. Physical examination often shows no abnormalities. Hyponatremia is the most common electrolyte abnormality during acute attacks, occurring in 40% of patients and presenting as SIADH. Hypomagnesemia is also common. There are no pathognomonic signs or symptoms. The most common identified triggers for acute attacks are medications, weight loss diets, and surgery. Many medications have been associated with AIP including antibiotics, hormonal contraceptives, seizure medications, anesthetics, and HIV treatment drugs. The initial diagnosis of acute porphyria is confirmed by urinalysis. Elevated urine porphobilinogen confirms diagnosis of AIP, hereditary coproporphyria (HCP), or variegate

title: Acute intermittent porphyria

text: porphyria (VP). A positive test should be indicated with an increase of five times normal, not just a slight increase which can which can occur with dehydration. To distinguish between AIP from HCP and VP, fecal porphyrin levels are normal in AIP but elevated in HCP and VP. Rapid, accurate diagnosis is important. Delays in diagnosis may result in permanent neurological damage or death. Porphyrias are caused by mutations in genes that encode enzymes in heme synthesis. In normal physiology, heme synthesis begins in the mitochondrion, proceeds into the cytoplasm, and finishes back in the mitochondrion. Heme is produced in

title: Porphyria cutanea tarda

text: of this disease. One of the most common risk factors observed is infection with the Hepatitis C virus. One review of a collection of PCT studies noted Hepatitis C infection in 50% of documented cases of PCT. Additional risk factors include alcohol abuse, excess iron (from iron supplements as well as cooking on cast iron skillets), and exposure to chlorinated cyclic hydrocarbons and Agent Orange. It can be a paraneoplastic phenomenon. Porphyria cutanea tarda is primarily caused by uroporphyrinogen decarboxylase deficiency (UROD). Uroporphyrinogen decarboxylase occurs in nature as a homodimer of two subunits. It participates in the fifth step in

title: Porphyria cutanea tarda

text: entire complex is more water soluble allowing the kidneys to preferentially remove it from the blood stream and expel it through urination. It should be noted that chloroquine treatment can induce porphyria attacks within the first couple of months of treatment due to the mass mobilization of porphyrins from the liver into the blood stream. Complete remission can be seen within 6–12 months as each dose of antimalarial can only remove a finite amount of porphyrins and there are generally decades of accumulation to be cleared. Originally, higher doses were used to treat the condition but are no longer recommended

title: Porphyria cutanea tarda

text: It is also felt to increase the uptake of iron in liver cells, leading to further oxidation of uroporphyrinogen by the release of activated oxygen species. Additionally, exposure to chlorinated cyclic hydrocarbons can lead to a deficiency in the activity of uroporphyrinogen decarboxylase, causing the buildup of excess uroporphyrinogen. Additionally, alcohol has been shown to increase the activity of the delta-aminolevulinic acid synthetase (ALA synthetase), the rate-limiting enzymatic step in heme synthesis in the mitochondria, in rats. Therefore, alcohol consumption may increase the production of uroporphyrinogen, exacerbating symptoms in individuals with porphyria cutanea tarda. While the most common symptom of

title: Porphyria cutanea tarda

text: is usually reduced by 50% in all tissues in people with the inherited form of the condition. Nongenetic factors such as alcohol abuse, excess iron, and others listed above can increase the demand for heme and the enzymes required to make heme. The combination of this increased demand and reduced activity of uroporphyrinogen decarboxylase disrupts heme production and allows byproducts of the process to accumulate in the body, triggering the signs and symptoms of porphyria cutanea tarda. The "HFE" gene makes a protein that helps cells regulate the absorption of iron from the digestive tract and into the cells of

title: Porphyria cutanea tarda

text: of factors. In addition to the symptomatic manifestation of the disease in the skin, chronic liver problems are extremely common in patients with the sporadic form of PCT. These include hepatic fibrosis (scarring of the liver), cirrhosis, and inflammation. However, liver problems are less common in patients with the inherited form of the disease. Additionally, patients will often void a wine-red color urine with an increased concentration of uroporphyrin I due to their enzymatic deficiency. Certain vitamin and minerals deficiencies are commonly found in people with porphyria cutanea tarda. The most frequently cited deficiencies are those of beta-Carotene, retinol, vitamin

title: What Is the What

text: What Is the What What Is the What: The Autobiography of Valentino Achak Deng is a 2006 novel written by Dave Eggers. It is based on the life of Valentino Achak Deng, a Sudanese child refugee who immigrated to the United States under the Lost Boys of Sudan program. It was a finalist for the National Book Award. As a boy, Achak is separated from his family during the Second Sudanese Civil War when the Arab militia, referred to as "murahaleen" (which is Arabic for the deported), wipes out his Dinka village, Marial Bai. During the assault, he loses sight

title: What Is the What

text: That Happens Will Happen Today". Tom Tykwer plans to adapt the novel into a film. In 2009, the novel received the Prix Médicis étranger in France. What Is the What What Is the What: The Autobiography of Valentino Achak Deng is a 2006 novel written by Dave Eggers. It is based on the life of Valentino Achak Deng, a Sudanese child refugee who immigrated to the United States under the Lost Boys of Sudan program. It was a finalist for the National Book Award. As a boy, Achak is separated from his family during the Second Sudanese Civil War when

title: Here Is What Is

text: Here Is What Is Here Is What Is is the fifth studio album by Canadian songwriter and record producer Daniel Lanois. It was first released in December 2007 as a high-quality download, and later released on CD on March 18, 2008. The album is the result of the same project that led to the 2007 documentary "Here Is What Is" that premiered at the 2007 Toronto International Film Festival in September. The movie documents the aesthetics and creative process behind Lanois' approach to music making and recording. The album has been presented as a direct soundtrack to this film, and

title: (What Is) Love?

text: (What Is) Love? "(What Is) Love?" is a song recorded by American entertainer Jennifer Lopez. Originally entitled "What Is Love?", the song appeared on the soundtrack to "The Back-up Plan" (2010), a film in which Lopez stars. The song was later included on Lopez's seventh studio album "Love?" (2011), as the album's title track. "(What Is) Love?" is a midtempo electropop song written by Diana "Wynter" Gordon, with the song's producer Emile "D'Mile" Dernst II. The song is about "not knowing what love is," according to Gordon. A remix of "(What Is) Love?", entitled "What Is Love? Part II", was

title: Here Is What Is

text: some of the tracks ("Beauty" and "Chest of Drawers") are conversations with Brian Eno. All songs written by Daniel Lanois unless otherwise noted. "Where Will I Be" is a new version of a song which had been previously released on Emmylou Harris' album "Wrecking Ball" in 1995. "Lovechild" and "Sacred And Secular" tracks incorporates, respectively, the pedal steel guitar melody used in "Carla", on Belladonna album, and the pedal steel guitar melody used in "Transmitter", on Shine album. Here Is What Is Here Is What Is is the fifth studio album by Canadian songwriter and record producer Daniel Lanois. It

title: What Is the What

text: writes: "Over the course of many years, Dave and I have collaborated to tell my story... I told [him] what I knew and what I could remember, and from that material he created this work of art." The book is typical of Eggers' style: blending non-fictional and fictional elements into a non-fiction novel or memoir. By classifying the book a novel, Eggers says, he freed himself to re-create conversations, streamline complex relationships, add relevant detail and manipulate time and space in helpful ways—all while maintaining the essential truthfulness of the storytelling. However, not all critics were impressed. Lee Siegel sees

title: What is Living and What is Dead in Indian Philosophy

text: What is Living and What is Dead in Indian Philosophy What is Living and What is Dead in Indian Philosophy is a 1976 book by Debiprasad Chattopadhyaya. Explaining the relationship between the soul and consciousness in the Nyaya-Vaisesika philosophy, Debiprasad Chattopadhyaya makes three points. First, consciousness in this view is knowledge (jnana) in an empirical sense. Second, consciousness is one of the multiple qualities of the soul. Third, consciousness is a transient quality, not a permanent quality. By itself, the soul is without consciousness; consciousness is produced in the soul only when it is conjoined with certain other entities. Hiriyanna,

title: What Is Life

text: of "What Is Life" on his 2005 tribute CD "Something for George". "What Is Life" was performed live at George Fest 2014 by "Weird Al" Yankovic. The recording is available on YouTube. The following musicians are believed to have played on "What Is Life": What Is Life "What Is Life" is a song by the English musician George Harrison, released on his 1970 triple album "All Things Must Pass". In many countries, it was issued as the second single from the album, in February 1971, becoming a top-ten hit in the United States, Canada and elsewhere, and topping singles charts

title: This Is What You Want... This Is What You Get

text: in the film. The album was seen as a step down from the band's efforts. Pitchfork Media named the album "maligned but salvageable". Allmusic said the album as "the most tentative and least powerful of PiL's recordings." This Is What You Want... This Is What You Get This Is What You Want... This Is What You Get is a 1984 album by Public Image Ltd. It is the band's fourth official studio album and includes the single "Bad Life" and a re-recorded version of a "This Is Not a Love Song", which had been a No. 5 UK and international

text: This Is What You Want... This Is What You Get This Is What You Want... This Is What You Get is a 1984 album by Public Image Ltd. It is the band's fourth official studio album and includes the single "Bad Life" and a re-recorded version of a "This Is Not a Love Song", which had been a No. 5 UK and international hit when released as a single in 1983. An early version of the album was released in 1983 by founding PiL guitarist Keith Levene as "Commercial Zone". The album was then re-recorded after Levene's departure from the

text: commenting on this point, notes: Hiriyanna expresses his perplexity in reviewing the Nyaya-Vaisesika view of consciousness, and then comes out with a comment equating this view with the Charvaka view of consciousness: What is Living and What is Dead in Indian Philosophy What is Living and What is Dead in Indian Philosophy is a 1976 book by Debiprasad Chattopadhyaya. Explaining the relationship between the soul and consciousness in the Nyaya-Vaisesika philosophy, Debiprasad Chattopadhyaya makes three points. First, consciousness in this view is knowledge (jnana) in an empirical sense. Second, consciousness is one of the multiple qualities of the soul. Third,

title: What Is History?

text: and were instead products of their own places and times, which in turn decided what "facts of the past" they determined into "facts of history". British historian Richard J. Evans said "What Is History?" caused a revolution in British historiography in the 1960s. Australian historian Keith Windschuttle, a critic of Carr, said "What Is History?" is one of the most influential books written about historiography, and that very few historians working in the English language since the 1960s had not read it. What Is History? What Is History? is a study that was written by the English historian E. H.

title: What Is Art?

text: in Tolstoy and his problems: essays. London: Grant Richards. pp. 66–101. Maude, Aylmer (1901b). “"What is Art?" - Tolstoy’s Theory of Art”, in Tolstoy and his problems: essays. London: Grant Richards. pp. 102–127. Noyes, George (1918). Tolstoy. London: Duffield. Pevear, Richard (1995). "Preface" in Tolstoy, Leo (1995 [1897]). "What is Art?" (Translated by Richard Pevear and Larissa Volokhonsky). London: Penguin. Simmons, Ernest (1973). "What is Art?", in Tolstoy. London: Routledge and Kegan Paul. pp. 175–189. What Is Art? What is Art? ( "Chto takoye iskusstvo?") is a book by Leo Tolstoy. It was completed in Russian in 1897 but first

text: He then flew over to London and presented them to Richard Branson as the finished new PiL album for Virgin Records: "Commercial Zone". For his part, John Lydon decided to completely abandon the tapes and re-record the whole album from scratch with session musicians. This new version of "Commercial Zone" became "This Is What You Want... This Is What You Get" in 1984. "The Order of Death" is a reference to the film "Copkiller", also known as "The Order of Death". The line "This is what you want... This is what you get", which gives title to the album, appears

text: were not re-recorded for "This Is What You Want...This Is What You Get" (although a remixed version of "Blue Water" was included as the B-side on the "This Is Not a Love Song" 12" single). Songs on "This Is What You Want...This Is What You Get" which did not appear in any form on "Commercial Zone" are "Tie Me to the Length of That", "The Pardon" and "1981", and are the only songs on the album which do not credit Keith Levene as a co-writer. PiL was supposed to score the soundtrack for the 1983 suspense film "Copkiller", starring Harvey

title: What Is Love

text: What Is Love "What Is Love" is a song recorded by Trinidadian-German Eurodance artist Haddaway for his debut album, "The Album". The song is recognizable by its refrain "What is love? Baby don't hurt me, don't hurt me... no more." It was a massive hit in Europe, becoming a number-one hit in at least 13 countries and reaching number two in Germany, Sweden, and the United Kingdom. Outside Europe, the song was moderately successful, reaching number 11 in the United States, number 12 in Australia, number 17 in Canada, and number 48 in New Zealand. "What Is Love" was written

title: What Is Art?

text: What Is Art? What is Art? ( "Chto takoye iskusstvo?") is a book by Leo Tolstoy. It was completed in Russian in 1897 but first published in English due to difficulties with the Russian censors. Tolstoy cites the time, effort, public funds, and public respect spent on art and artists as well as the imprecision of general opinions on art as reason for writing the book. In his words, "it is difficult to say what is meant by art, and especially what is good, useful art, art for the sake of which we might condone such sacrifices as are being

title: (What Is) Love?

text: she was dissatisfied with the circumstances, but was in "no way dissing Lopez." A mastered version of "What Is Love?" was included on the soundtrack to "The Back-up Plan" (2010), a film in which Lopez stars. "What Is Love?" was re-titled as "(What Is) Love?" and appeared on "Love?" (2011). "(What Is) Love?" was due to be released as a promotional single from "Love?" on April 26, 2011, but was "unlocked" and released four days earlier, on April 22, 2011 by through a campaign on Lopez's Facebook page. It was released in many European countries on April 25. The song

title: What You Get Is What You See

text: What You Get Is What You See "What You Get Is What You See" is a song by recording artist Tina Turner from her album "Break Every Rule" (1986). The song was written by the Terry Britten and Graham Lyle team and was notably different from the three previous singles that they had written for Turner, "What's Love Got to Do with It", "We Don't Need Another Hero" and "Two People", as it was an up-tempo country-tinged rock track featuring Eric Clapton on guitars. Tina Turner said in an interview that "What You Get Is What You See" is her

title: What Is and What Should Never Be

text: What Is and What Should Never Be "What Is and What Should Never Be" is a song by English rock band Led Zeppelin. It was written by Jimmy Page and Robert Plant and was included as the second track on "Led Zeppelin II" (1969). "What is and What Should Never Be" was one of the first songs on which Page used his soon-to-become trademark Gibson Les Paul for recording. The production makes liberal use of stereo as the guitars pan back and forth between channels. Robert Plant's vocals were phased during the verses. Record producer Rick Rubin has remarked, "The

title: What Is Love

text: An official music video was released directed by Soulvizion. It features the Dutch professional basketball player Don Rigters who plays the role of David Rose, a basketball player who is severely injured trying to make a comeback to the game with encouragement from his girlfriend, (played by Melissa Kanza), his three teammates, (Alkenah Wansing, Jeroen Jansen and Lindy Chippendel) and by his basketball coach (played by J E Rigters). What Is Love "What Is Love" is a song recorded by Trinidadian-German Eurodance artist Haddaway for his debut album, "The Album". The song is recognizable by its refrain "What is love?

title: What Is It?

text: What Is It? What Is It? is a 2005 American surrealist film written, edited, co-produced and directed by Crispin Glover. It stars Crispin Glover and Fairuza Balk and Steven C. Stewart. As of 2008, the film has only been shown at independent theaters, typically accompanied by a question-and-answer session, a one-hour dramatic narration of eight different profusely illustrated books as a slideshow, and a meet-and-greet/book signing with Glover. "What Is It?" is the first entry in a planned trilogy directed by Glover, followed by "It Is Fine! Everything Is Fine." and continued with "It Is Mine". The "adventures" of a

title: What Is Literature?

text: The art of writing is deeply linked to freedom and thus ventures into the fields of politics and democracy. The poet frees himself by disassociation, and the prose writer fulfills a duty to utilize language for the end of a conceived free society. What Is Literature? What Is Literature? (), also published as "Literature and Existentialism",) is an essay by French philosopher and novelist Jean-Paul Sartre, published by Gallimard in 1948. Initially published in freestanding essays across French literary journals "Les Temps modernes", "Situations I" and "Situations II", essays "What is Writing?" and "Why Write?" were translated into English and

title: What Work Is

text: terribly damaged conditions of American labor, whose circumstance has perhaps never been more wrecked.” After Levine was named Poet Laureate, "What Work Is" reached the top of Amazon.com's "Movers and Shakers" list. What Work Is What Work Is is a collection of poetry by Philip Levine. The collection has many themes that are representative of Levine's writing including physical labor, class identity, family relationships and personal loss. Its primary focus on work and the working class led to it being studied with emphasis on Marxist literary criticism. The focus on work is expressed in thematically different ways throughout the collection.

title: What Work Is

text: What Work Is What Work Is is a collection of poetry by Philip Levine. The collection has many themes that are representative of Levine's writing including physical labor, class identity, family relationships and personal loss. Its primary focus on work and the working class led to it being studied with emphasis on Marxist literary criticism. The focus on work is expressed in thematically different ways throughout the collection. Furthermore, much of the collection was shaped by concerns for blue collar workers as well as nationwide political events. "What Work Is" was first published in 1991 by Alfred A. Knopf, Inc.

title: What Is and What Should Never Be

text: and What Should Never Be" was performed live at Led Zeppelin concerts between 1969 and 1973. A live version taken from a performance at the Royal Albert Hall in 1970 can be seen on the "Led Zeppelin DVD". Another was included on disc two of the live triple album "How The West Was Won". Two more versions were included in BBC Sessions. What Is and What Should Never Be "What Is and What Should Never Be" is a song by English rock band Led Zeppelin. It was written by Jimmy Page and Robert Plant and was included as the second

title: What Is Life

text: What Is Life "What Is Life" is a song by the English musician George Harrison, released on his 1970 triple album "All Things Must Pass". In many countries, it was issued as the second single from the album, in February 1971, becoming a top-ten hit in the United States, Canada and elsewhere, and topping singles charts in Australia and Switzerland. In the United Kingdom, "What Is Life" appeared as the B-side to "My Sweet Lord", which was the best-selling single there of 1971. Harrison's backing musicians on the song include Eric Clapton and the entire Delaney & Bonnie Friends band,

title: What Is Love

text: in "Just Dance 2017". In 2009, German DJ Klaas remixed the song under the title "Klaas meets Haddaway – What Is Love 2K9". This remix charted in several European countries. In 2016, Belgian DJ Lost Frequencies released a cover titled "What Is Love 2016", as a single from his debut album "Less Is More". It was actually already produced back in 2014 as a remix for Jaymes Young's cover version of "What is Love". This version was remade for the album and became a hit on a number of European singles charts and topped the Belgian Ultratop Official Singles Chart.

title: What Is Literature?

text: What Is Literature? What Is Literature? (), also published as "Literature and Existentialism",) is an essay by French philosopher and novelist Jean-Paul Sartre, published by Gallimard in 1948. Initially published in freestanding essays across French literary journals "Les Temps modernes", "Situations I" and "Situations II", essays "What is Writing?" and "Why Write?" were translated into English and published by the Paris-based literary journal Transition 1948. The English translation by Bernard Frechtman was published in 1950. Sartre attempts to devise an understanding of the effect literature has on those who are subjected to it. In the foreword Sartre addresses his critics

title: (What Is) Love?

text: the internet on April 20, 2013. According to Jessica Sager of PopCrush, it picks up where the original song left off. Sager wrote that the song "likely isn't about [Marc] Anthony — but for those new to the song, it's surely easy to interpret it as such. Lopez gets pretty revealing in the new song, addressing a divorce directly and explicitly". Credits adapted from the liner notes of "Love?". (What Is) Love? "(What Is) Love?" is a song recorded by American entertainer Jennifer Lopez. Originally entitled "What Is Love?", the song appeared on the soundtrack to "The Back-up Plan" (2010),

title: The World Is What It Is

text: The World Is What It Is The World Is What It Is: The Authorized Biography of V. S. Naipaul is a biography of the Nobel Prize-winning author V. S. Naipaul by Patrick French. It was published in 2008 (by Picador in the UK and Knopf in the USA). The title is a quotation from Naipaul's book "A Bend in the River". "The world is what it is; men who are nothing, who allow themselves to become nothing, have no place in it." French deals with Naipaul's family background and his life from his birth in 1932 until his second marriage

title: What Is Life

text: in the UK as perhaps the strongest of all of Harrison's singles. Writing in 1981, "NME" critic Bob Woffinden grouped "What Is LIfe" with "My Sweet Lord", "Isn't It a Pity" and "Awaiting on You All" as "all excellent songs". Reviewing the 2001 reissue of "All Things Must Pass", for "Rolling Stone", James Hunter wrote of how the album's music "exults in breezy rhythms", among which "the colorful revolutions of 'What Is Life' ... [move] like a Ferris wheel". The following year, in Rolling Stone Press's "Harrison" tribute book, David Fricke included "What Is Life" among his selection of "essential

title: What Is Life

text: love song – perhaps a "lovingly crafted paen" to Harrison's wife Pattie, as Alan Clayson puts it – or a devotional song like many of Harrison's compositions. Ian Inglis writes that the song title suggests a "philosophical debate about the meaning of life", yet its rendering as ""what is my life"" in the choruses "reshapes [the meaning] completely". Theologian Dale Allison finds no religious content in "What Is Life" but notes the "failure of words to express feelings" implied in the opening line (""What I feel, I can't say""), a recurring theme of Harrison's spiritual songs such as "That Is

title: What Is This?

text: project while the former was seen as side endeavor. What Is This released their debut EP "Squeezed" in 1984. Around this time, Hillel Slovak had become uncertain of his role in the band and decided to rejoin the Red Hot Chili Peppers. He left What Is This in 1985, and later that year the band would release its only full-length studio album "What Is This?" and its final EP "3 Out of 5 Live". After this, the band broke up. Alain Johannes later met Natasha Shneider and the duo formed Walk The Moon, whose sole studio album featured Jack Irons

title: What Is This?

text: following week. The group decided to change their name to the Red Hot Chili Peppers, played several more shows at various L.A. clubs and musical venues, and eventually recorded a demo tape, all while Slovak and Irons were still playing with What Is This?. A few months later, What Is This received a record deal with MCA Records and the Red Hot Chili Peppers received a joint deal with EMI America and Enigma Records. Hillel Slovak and Jack Irons decided to leave the Red Hot Chili Peppers and stay with What Is This? as the latter was their primary musical

title: What Is This?

text: What Is This? What Is This (known prior to 1980 as Anthym) was a rock band that originated in Fairfax High School in California and would play numerous shows along the coast of California. It was formed by guitarist Hillel Slovak, drummer Jack Irons, vocalist Alain Johannes, and bassist Todd Strassman. Originally named Chain Reaction, the band would later change its name to Anthem, then later to Anthym (as there was already a band using the former moniker), and then finally to What Is This, which was a tongue-in-cheek reference to the reaction they usually received from first-time listeners. During

title: What Is Art?

text: Aylmer Maude, translator of many of Tolstoy's writings, calls it "probably the most masterly of all Tolstoy's works", citing the difficulty of the subject matter and its clarity. For a comprehensive review of the reception at the time of publication, see Maude 1901"b". Bayley, John (1986 [1966]). ""What is Art?" - excerpt from Tolstoy and the Novel" in Bloom, Harold (ed.). Leo Tolstoy. New York: Chelsea House. p. 141-152. Jahn, Gary R. (1975). "The aesthetic theory of Leo Tolstoy's "What is Art?"". "Journal of Aesthetics and Art Criticism" 34 (1): 59-65. Maude, Aylmer (1901a). “"What is Art?" - An Introduction”,

title: (What Is) Love?

text: song. Lopez's vocals for the song were recorded and produced by Kuk Harrell, with recording assistance from Jim Annunziato and Josh Gudwin at Conway Studios in Hollywood, California. Annunziato and Eric Eylands handled audio engineering of "(What Is) Love?", while the song was later mixed by Mike "Handz" Donaldson at Chalice Recording Studios in Los Angeles, California. "(What Is) Love?" deals with the topic of "not knowing what love is". The song's co-writer Gordon stated that: "I felt like so many woman have that same story. No fathers, families, abusive boyfriends and husbands..no parental support, they feel alone .. I've

title: What Is Intelligence?

text: What Is Intelligence? What Is Intelligence?: Beyond the Flynn Effect is a book by psychologist James R. Flynn which outlines his model for an explanation of the eponymous Flynn effect. The book summarizes much of the work of Flynn in this area, as well as that of his colleague William Dickens of the Brookings Institution. The book begins with a description of what has generally become known as "the Flynn effect": the steady rise from one generation to the next in average scores on IQ tests over time. Flynn notes that this effect apparently contradicts some fundamental beliefs about IQ

title: What U See Is What U Get

text: What U See Is What U Get "What U See Is What U Get" is the first single from Xzibit's second album, "40 Dayz & 40 Nightz". In its first week the song peaked at #50 on the "Billboard" Hot 100, being Xzibit's highest charting single. It peaked at #3 on Hot Rap Singles, his highest on that chart. A music video directed by Gregory Dark was filmed in Los Angeles. The deceptively simplistic premise of the video is Xzibit's trek to a nearby shop after being asked to pick up some milk. On his way, Xzibit greets several friends,

title: What Is Life

text: All", "Mystical One" and "Pisces Fish". Joshua Greene, another religious academic, identifies the song as part of its parent album's "intimately detailed account of a spiritual journey": where "Awaiting on You All" shows Harrison "convinced of his union with God", "What Is Life" reveals him to be "uncertain that he deserved such divine favor". The song's second verse repeats what Inglis refers to as the "somewhat confusing promise" from Harrison (in lines 3 and 4) should his love be "rejected": Musically, Simon Leng describes "What Is Life" as "Motown-spiced" and a comparatively rare example of its composer's willingness to embrace

title: What You Get Is What You See

text: where in 1989 it was used in a campaign for the New South Wales Rugby League. They later used the Tina Turner song "The Best" in another campaign. The song has been covered many times including by Canadian country music group Straight Clean & Simple and a single for the band taken from their 1992 album "Iron Lady". It was also covered by Scottish / Irish country artist Lisa McHugh. Her cover appears in her 2014 album "A Life That's Good". What You Get Is What You See "What You Get Is What You See" is a song by recording

title: What U See Is What U Get

text: the music videos for Xzibit's songs "Paparazzi" and "The Foundation". The music video peaked at 1 during 6 weeks at BET's "Rap City". 12" Promo What U See Is What U Get "What U See Is What U Get" is the first single from Xzibit's second album, "40 Dayz & 40 Nightz". In its first week the song peaked at #50 on the "Billboard" Hot 100, being Xzibit's highest charting single. It peaked at #3 on Hot Rap Singles, his highest on that chart. A music video directed by Gregory Dark was filmed in Los Angeles. The deceptively simplistic premise

title: What Is a Nation?

text: What Is a Nation? "What is a Nation?" ("Qu'est-ce qu'une nation?") is an 1882 lecture by French historian Ernest Renan (1823–1892), known for the statements that a nation is "a daily referendum", and that nations are based as much on what the people jointly forget, as what they remember. It is frequently quoted or anthologized in works of history or political science pertaining to nationalism and national identity. Renan wrote "What is a Nation" in order to symbolize the nationalism which was born in France as a result of the French Revolution of 1789. Renan begins his essay by noting