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 known as acute porphyria, as symptoms are rapid in onset and last a short time. Symptoms of an attack include abdominal pain, chest pain, confusion, fever, high blood pressure, high heart rate; the attacks last for days to weeks. Complications may include paralysis, low blood sodium levels, seizures. Attacks may be triggered by alcohol, hormonal changes, 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, are due to a mutation in one of the genes that make heme, they may be inherited in X-linked dominant manner. One type, porphyria cutanea tarda, may 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 be classified by whether the liver or the bone marrow is affected. Diagnosis is made by blood and stool tests. Genetic testing may be done to determine the specific mutation. Treatment depends on a person's symptoms; the treatment of porphyria of the skin involves the avoidance of sunlight. The treatment for acute porphyria may involve giving a glucose solution. A liver transplant may be carried out; the precise frequency of porphyria is unclear: it is estimated that it affects somewhere between 1 and 100 per 50,000 people. Rates vary around the world. Porphyria cutanea tarda is believed to be the most common type; the disease was described 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, meaning "purple", a reference to the color of the urine that may occur during an attack. The acute porphyrias are acute intermittent porphyria, variegate porphyria, aminolevulinic acid dehydratase deficiency porphyria and hereditary coproporphyria.
These diseases affect the nervous system, resulting in episodic crises known as acute attacks. The major symptom of an acute attack is abdominal pain accompanied by vomiting and tachycardia; the most severe episodes may involve neurological complications: motor neuropathy, which leads to muscle weakness and to quadriplegia and central nervous system symptoms such as seizures and coma. There may be short-lived psychiatric symptoms such as anxiety, confusion and rarely, overt psychosis. All these symptoms resolve. Given the many presentations and the low occurrence of porphyria, patients may be suspected to have other, unrelated conditions. For instance, the polyneuropathy of acute porphyria may be mistaken for Guillain–Barré syndrome, porphyria testing is recommended in those situations; the non-acute porphyrias are X-linked dominant protoporphyria, congenital erythropoietic porphyria, porphyria cutanea tarda, erythropoietic protoporphyria. None of these are associated with acute attacks. For this reason, these four porphyrias—along with two acute porphyrias, VP and HCP, that may involve skin manifestations—are sometimes called cutaneous porphyrias.
Skin disease is encountered. Porphyrins are photoactive molecules, 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: Immediate photosensitivity; this is typical of XLDPP and EPP. Following a variable period of sun exposure—typically about 30 minutes—patients complain of severe pain and discomfort in exposed areas; the effects are not visible, though there may be some redness and swelling of the skin. Vesiculo-erosive skin disease. This—a reference to the characteristic blistering and open sores noted in patients—is the pattern seen in CEP, PCT, VP, HCP; the changes are noted only in sun-exposed areas such as the back of the hands. Milder skin disease, such as that seen in VP and HCP, consists of increased skin fragility in exposed areas with a tendency to form blisters and erosions after minor knocks or scrapes.
These heal often leaving small scars that may be lighter or darker than normal skin. More severe skin disease is sometimes seen in PCT, with prominent lesions, darkening of exposed skin such as the face, hypertrichosis: abnormal hair growth on the face the cheeks; the most severe disease is seen in CEP and a rare variant of PCT known as hepatoerythropoietic porphyria. Patients may show deformed, discolored teeth or gum and eye abnormalities; the porphyrias are considered genetic in nature. Subtypes of porphyrias depend. Acute porphyria can be triggered by a
Van Zant II is an album released by American musical duo Van Zant. It was released in 2001 by Sanctuary Records; the single "Get What You Got Comin'" achieved chart success. This album has been released with the Copy Control protection system in some regions. "Oklahoma" - 5:27 "Get What You Got Comin'" - 4:15 "Heart Of An Angel" - 4:03 "Is It For Real" - 3:54 "Imagination" - 4:43 "At Least I'm Free" - 4:55 "Baby Get Blue" - 4:24 "What's The World Coming To" - 3:57 "Wildside" - 4:06 "Alive" - 5:09 Van ZantDonnie Van Zant - lead vocals, background vocals Johnny Van Zant - lead vocals, background vocalsAdditional MusiciansMike Brignardello - bass guitar Pat Buchanan - guitar Chris Carmichael - strings Carol Chase - background vocals Bill Cuomo - Hammond organ, piano Gary Dales - background vocals Shane Fontayne - electric guitar Janelle Guillot - voice over Vicki Hampton - background vocals Ioannis - background vocals Robert White Johnson - percussion, background vocals Jerry McPherson - acoustic guitar, electric guitar Tony Morra - drums Jimmy Nichols - strings Michael O'Hara - background vocals Dale Rossington - background vocals Kenny Wayne Shepherd - electric guitar on "Get What You Got Comin'" and "At Least I'm Free" Van Zant II at Allmusic
Argonaut Glacier is a tributary glacier about 10 miles long in the Mountaineer Range of Victoria Land, Antarctica. It flows east to enter Mariner Glacier just north of Engberg Bluff, it was named by the New Zealand Geological Survey Antarctic Expedition, 1962–63, in association with Aeronaut and Cosmonette Glaciers. List of glaciers in the Antarctic Glaciology This article incorporates public domain material from the United States Geological Survey document "Argonaut Glacier"
The'Underpasses of Taganrog is an underground passageway which, according to city legends, is located beneath the city of Taganrog of the Rostov Oblast. There are several legends of the origin of tunnels in Taganrog; some researchers believe that they could have been dug by Turks, others — that ancient Romans were their builders. Versions move forward that underpasses based at Peter I or Catherine II, but all these versions did not find reliable confirmation. The writer Anton Pavlovich Chekhov was interested in city underpasses in Taganrog and legends of their existence about what there are confirmed facts. According to one legend, there was an underpass earlier from the territory of modern trolleybus management, located down the street from Aleksandrovska to port, it intended for pro-closed sleighs of bags with flour. In some houses of Taganrog, there were deep cellars which by means of the underpass connected to the embankment. During the researches which were conducted in the territory of Taganrog traces of the existence of underpasses were found.
The small underpass passed from the cellar of the house of Alferaki, located down the street Greek, 76 to Anton Glushko Lane, 13, to the place where now the museum of local lore is located. In references, there are data on the existence of the underpass which conducted with the sea coast to the cellar of the house of the millionaire Valvano. Now in this place, the building of aviation technical school, located at the intersection of Chekhov Street and Turgenevsky Lane is located. At the construction of one TRTI case "B", builders found the underpass, in it, there were ancient things. Many historians slopes to a recognition of the existence of tunnels in Taganrog. At construction of Petrovsky fortress, numerous underground passages which conducted from bastions to ravelins from bastions towards fortress were built, it was necessary in order that in case of a possible siege people had opportunities to move in such ways. These vaults had to remain as at destruction of a fortress, only a land part was destroyed.
Under bastions, underground casemates were located. In 1926 in one of the reports of a museum of local lore it was reported that in destroyed to a shaft of Petrovsky fortress the top part of an entrance for which construction the stone and lime was used was found; the similar courses were found about the building of Southern Federal University. In 1927 in one of the inhabited yards of Taganrog dug a hole for lime — its bottom failed and at a depth of 5 meters there was underground a course, it managed to be investigated on 20 meters in length. The former fortress university cases stand still; when the hostel No. 5 on Garibaldi Lane was under construction, the round ancient powder cellar and the underpass, half filled up was found
The Bellelli Family known as Family Portrait, is an oil painting on canvas by Edgar Degas, painted c. 1858–1867, housed in the Musée d'Orsay. A masterwork of Degas' youth, the painting is a portrait of his aunt, her husband, their two young daughters. While finishing his artistic training in Italy, Degas drew and painted his aunt Laura, her husband the baron Gennaro Bellelli, their daughters Giulia and Giovanna. Although it is not known for certain when or where Degas executed the painting, it is believed that he utilized studies done in Italy to complete the work after his return to Paris. Laura, his father's sister, is depicted in a dress which symbolizes mourning for her father, who had died and appears in the framed portrait behind her; the baron was an Italian patriot exiled from Naples, living in Florence. Laura Bellelli's countenance is dignified and austere, her gesture connected with those of her daughters, her husband, by contrast, appears to be separated from his family. His association with business and the outside world is implied by his position at his desk.
Giulia holds a livelier pose than that of her sister Giovanna, whose restraint appears to underscore the familial tensions. In 1856 Degas left his home in Paris to study art and visit family relations in Italy, arriving in Naples on 17 July. In 1857 he traveled between Naples, where he stayed with his grandfather, Hilaire Degas, Rome. At the end of July 1858 Laura Bellelli wrote to Degas from Naples, inviting him to stay with her in Florence. Degas arrived in Florence by 4 August, living with his uncle Gennaro and making studies in the Uffizi. By September he had become bored, did not get along well with Gennaro, remained only to see Laura and Giulia, who had prolonged their stay in Naples following the death of Degas' grandfather Hilaire on 31 August; that there were strains within the Bellelli household at the time was certainly noticed by Degas, confirmed by another uncle: "The domestic life of the family in Florence is a source of unhappiness for us. As I predicted, one of them is much at fault and our sister a little, too."
Laura subsequently confided to Degas that, living in exile, she missed her Neapolitan family, further, that her husband was "immensely disagreeable and dishonest... Living with Gennaro, whose detestable nature you know and who has no serious occupation, shall soon lead me to the grave." Laura Bellelli was pregnant at the time, it has been suggested that this circumstance, the subsequent death of the child in infancy, may have contributed to her unhappiness and to domestic tensions in general. These conflicts would provide both content for the painting. After his aunt and cousins returned in early November 1858, Degas undertook a series of works that would culminate in The Bellelli Family, it appears that he planned to paint a vertical composition depicting his aunt and her two daughters in a pyramidical grouping. He painted his cousins in their black dresses and white pinafores, while his father wrote letters from Paris, offering advice on how best to proceed with the project, impatiently awaited his return.
Degas wrote of Giovanna: "The elder one was in fact a little beauty. The younger one, on the other hand, was smart as can kind as an angel. I am painting them in mourning dress and small white aprons, which suit them well…I would like to express a certain natural grace together with a nobility that I don't know how to define...." At year's end, Degas stopped work on the double portrait of his young cousins in order to begin a larger painting. The preparatory works include portrait studies and compositional details in pencil and oil. One drawing indicates Degas' initial intention to have Gennaro Bellelli seated at the end of the table, an oil sketch placed him standing behind his daughters. In late March 1859, Degas left Florence to return to Paris. Other than the conclusion that Degas worked on the picture "for several years", there is no documentation to confirm the actual time or place at which the picture was painted. Supporting this conclusion is the observation that the Bellelli's apartment was too small to host such a large work, there were no studio facilities.
In March 1860, Degas returned to Italy, in part to conduct family business, in April he again visited the Bellellis and made several drawings of his uncle. There is a family account, once accepted but more deemed unlikely, that offers a different version: a Neapolitan lawyer who married one of Degas' grandnieces claimed that the painting was completed in Italy, brought back to France only some forty to fifty years but this is contradicted by evidence that the painting was exhibited in the Paris Salon of 1867; the work of many artists provided inspiration: at this time Degas included in his correspondence mention of Anthony van Dyck and Botticelli, among others. Other prototypes whose influence have been cited in terms of composition, include 17th-century Dutch genre and portrait painting, the portrait studies of Ingres, Velázquez's Las Me
The Mitsogo or Tsogo are an ethno-cultural group from the highlands of Gabon. They reside in Ngounié Province to the north and east of Mouila. Numbering around 13,000, they speak the Tsogo language. In the late 19th and early 20th century they were known for their fierce resistance to the French. There are about 13,000 Mitsogo people, they reside in Ngounié Province in southern-central Gabon, to the north and east of Mouila. The region is named after the major river, Ngounié River, a tributary of the Ogooué River, is so associated with the Mitsogo that it is referred to as "Mitsogo country", it is sometimes known as Mitsogo. 90% of them are Christian. The French first encountered the Mitsogo people in 1857, when they totalled 5000 people, they become known for their skills in cloth manufacturing. In the 1890s the Tsogo-speaking clans of the Matèndè, Waka districts along the Ikoy River clashed with Kele invaders; the Kele took their children to increase their own numbers and fertility. As a result, Mitsogo clans settled in districts inhabited by Apindji speaking clans.
In 1899, the French established a military post and Roman Catholic mission in the region and the Mitsogo people came under their control. In the early 20th century the Mitsogo the conflicted with the Bakele people, they put up a strong resistance in 1907, with the Mitsogo-Kamba clan fighting a fierce battle with the Bakele near Mount Motende. The conflict solidified Tsogo identity; the Mitsogo chief Mbombe was known for his freedom fighting against the French. One major uprising broke out in 1904, he was captured in 1913 and executed at the prison in Mouila. Beti-Pahuin peoples