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Rare disease

A rare disease is any disease that affects a small percentage of the population. In some parts of the world, an orphan disease is a rare disease whose rarity means there is a lack of a market large enough to gain support and resources for discovering treatments for it, except by the government granting economically advantageous conditions to creating and selling such treatments. Orphan drugs are ones so sold. Most rare diseases are genetic and thus are present throughout the person's entire life if symptoms do not appear. Many rare diseases appear early in life, about 30% of children with rare diseases will die before reaching their fifth birthday. With only three diagnosed patients in 27 years, ribose-5-phosphate isomerase deficiency is considered the rarest known genetic disease. No single cut-off number has been agreed upon for. A disease may be considered rare in one part of the world, or in a particular group of people, but still be common in another; the US organisation Global Genes has estimated that more than 300 million people worldwide are living with one of the 7,000 diseases they define as "rare" in the United States.

There is no single accepted definition for rare diseases. Some definitions rely on the number of people living with a disease, other definitions include other factors, such as the existence of adequate treatments or the severity of the disease. In the United States, the Rare Diseases Act of 2002 defines rare disease according to prevalence "any disease or condition that affects fewer than 200,000 people in the United States", or about 1 in 1,500 people; this definition is the same as that of the Orphan Drug Act of 1983, a federal law, written to encourage research into rare diseases and possible cures. In Japan, the legal definition of a rare disease is one that affects fewer than 50,000 patients in Japan, or about 1 in 2,500 people. However, the European Commission on Public Health defines rare diseases as "life-threatening or chronically debilitating diseases which are of such low prevalence that special combined efforts are needed to address them"; the term low prevalence is defined as meaning fewer than 1 in 2,000 people.

Diseases that are statistically rare, but not life-threatening, chronically debilitating, or inadequately treated, are excluded from their definition. The definitions used in the medical literature and by national health plans are divided, with definitions ranging from 1/1,000 to 1/200,000; because of definitions that include reference to treatment availability, a lack of resources, severity of the disease, the term orphan disease is used as a synonym for rare disease. But in the United States and the European Union, "orphan diseases" have a distinct legal meaning; the United States' Orphan Drug Act includes both rare diseases and any non-rare diseases "for which there is no reasonable expectation that the cost of developing and making available in the United States a drug for such disease or condition will recovered from sales in the United States of such drug" as orphan diseases. The European Organization for Rare Diseases includes both rare diseases and neglected diseases into a larger category of "orphan diseases".

Prevalence, rather than incidence, is used to describe the impact of rare diseases. The Global Genes Project estimates; the European Organization for Rare Diseases estimates that as many as 5,000 to 7,000 distinct rare diseases exist, as much as 6% to 8% of the population of the European Union is affected by one. Only about 400 rare diseases have therapies and about 80% have a genetic component according to Rare Genomics Institute. Rare diseases can vary in prevalence between populations, so a disease, rare in some populations may be common in others; this is true of genetic diseases and infectious diseases. An example is cystic fibrosis, a genetic disease: it is rare in most parts of Asia but common in Europe and in populations of European descent. In smaller communities, the founder effect can result in a disease, rare worldwide being prevalent within the smaller community. Many infectious diseases are rare everywhere else. Other diseases, such as many rare forms of cancer, have no apparent pattern of distribution but are rare.

The classification of other conditions depends in part on the population being studied: All forms of cancer in children are considered rare, because so few children develop cancer, but the same cancer in adults may be more common. About 40 rare diseases have a far higher prevalence in Finland. There are rare genetic diseases among the Amish religious communities in the US and among ethnically Jewish people. A rare disease is defined as one that affects fewer than 200,000 people across a broad range of possible disorders. Chronic genetic diseases are classified as rare. Among numerous possibilities, rare diseases may result from bacterial or viral infections, chromosome disorders and proliferative causes, affecting any body organ. Rare diseases may be chronic or incurable, although many short-term medical conditions are rare diseases; the NIH's Office of Rare Diseases Research was established by H. R. 4013/Public Law 107-280 in 2002. H. R. 4014, signed the same day, refers to the "Rare Diseases Orphan Product Development Act".

Similar initiatives have been proposed in Europe. The ORDR runs the Rare Disea

Knud Holscher

Knud Helmuth Holscher is a Danish architect and industrial designer. For many years he was a partner in KHR Architects with Svend Axelsson and designed many of their works together. Holscher studied with Erik Christian Sørensen and professor Arne Jacobsen at the Royal Danish Academy of Fine Arts, School of Architecture, graduating in 1957, he joined the office of Arne Jacobsen in 1960 and moved to England to oversee construction of Jacobsen's St Catherine's College, Oxford in 1962. Holscher was awarded the British Design Award in 1965 and 1966 for work done in collaboration with Alan Tye before accepting a partnership in established Danish architectural office Krohn and Hartvig Rasmussen renamed KHRAS. Knud Holscher has since headed his own office Knud Holscher Design. Though starting out as an architect, it is his work as industrial designer which has brought Knud Holscher international attention and recognition, his designs have been linked to the Hochschule für Gestaltung Ulm and to the work of HfG Ulm student Dieter Rams.

In 2003, he said of his work: "Design should be like buttons on a shirt. With character to catch your attention, but no more so than you can use it without thinking about it". Holscher was professor of architecture at the Royal Danish Academy of Fine Arts from 1968–1988 and professor of design from 1994, he has received numerous awards for his work and is an Honorary RDI, Royal Designer for Industry since 2004. Dynamic and aware, Knud Holscher's design mantra revolves around constant innovation, with his efforts directed at satisfying the end user's needs. Knud believes. In his own words, "Design should not be defined. Competition 1967, construction 1971 onwards Royal Theatre, Denmark. 1. Prize, competition 1979. Not built. Bahrain National Museum, Bahrain, 1982–1988 Copenhagen Airport, Terminal B, Denmark, 1986 Copenhagen Airport, Domestic Terminal, Denmark, 1988–1989Industrial Design d line, architectural hardware, since 1971 ERCO track lights Quinta, since 1990 2001 Dreyer Honorary Award Knud Holscher Design d line international, architectural hardware

Tiga Island, Malaysia

Tiga Island is one of a group of small uninhabited islands in Kimanis Bay off the western coast of Sabah, Malaysia. The islands were formed on 21 September 1897, when an earthquake on Mindanao caused a volcanic eruption near Borneo; the island is 607 hectares in size and has a couple of active mud volcanos at the highest part of the island. Tiga Island is one of the three islands; the Park Headquarters are on the island, comprising an office complex, accommodation for the park staff and visiting scientists. Located 48 km south of Kota Kinabalu and the Tunku Abdul Rahman National Park, Tiga Island National Park is reached by driving the 140 km to Kuala Penyu, a small settlement on the tip of the Klias Peninsula. From here it is about 30 minutes, by boat. Another way of getting there is by chartering a speed boat from Kota Kinabalu and cruise to Tiga Island, or fly to Labuan and charter a speed boat from Labuan. Tiga Island became well known through the Survivor television series, it was the setting of Survivor: the first American season of the show.

It was the setting of the first seasons of the British shows. The island was rumoured to be the setting of the upcoming third season of Australian Survivor. However, it was revealed that Samoa would be used as the location for the series. List of islands of Malaysia Sabah Parks page about Tiga Island updated 5.15.2019 The Guardian interavtive web page about Tiga Island


Pentachlorobenzene is a chemical compound with the molecular formula C6HCl5, a chlorinated aromatic hydrocarbon. It consists of a benzene ring substituted with five chlorine atoms. PeCB was once used industrially for a variety of uses, but because of environmental concerns there are no large scale uses of PeCB. Pentachlorobenzene is a known persistent organic pollutants, classified among the "dirty dozen" and banned globally by the Stockholm Convention on Persistent Organic Pollutants as of 2011. PeCB can be produced as a byproduct of the manufacture of carbon benzene, it is extracted by crystallization. The direct production of pure PeCB is not practical because of the simultaneous production of other chlorinated compounds. Since PeCB is produced in small quantities in the chlorination of benzene, it is contained in other chlorobenzenes Today, a majority of the PeCB released into the environment is a result of backyard trash burning and municipal waste incineration. PeCB was used as an intermediate in the manufacture of pesticides the fungicide pentachloronitrobenzene.

Pentachloronitrobenzene is now made by the chlorination of nitrobenzene in order to avoid the use of PeCB. PeCB was a component of a mixture of chlorobenzenes added to products containing polychlorinated biphenyls in order to reduce viscosity. PeCB has been used as a fire retardant. PeCB is a persistent organic pollutant. Pentachlorobenzene was added in 2009 to the list of chemical compounds covered by the Stockholm Convention, an international treaty which restricts the production and use of persistent organic pollutants. PeCB has been banned in the European Union since 2002. PeCB is toxic to aquatic organisms, decomposes on heating or on burning with the formation of toxic, corrosive fumes including hydrogen chloride. Combustion of PeCB may result in the formation of polychlorinated dibenzodioxins and polychlorinated dibenzofurans. Chlorobenzene Dichlorobenzene Trichlorobenzene Hexachlorobenzene Pentafluorobenzene Pentachlorobenzenethiol

Oregon Rail Heritage Foundation

The Oregon Rail Heritage Foundation is a registered non-profit organization based in Portland, United States. Composed of several all-volunteer groups dedicated to maintaining vintage railroad equipment, the ORHF is committed "to secure a permanent home for the City of Portland’s steam locomotives, preserve the Brooklyn Roundhouse, establish a Rail and Industrial Heritage Museum.”At present, the ORHF is constructing a new restoration shop for the city's steam locomotives, the first of a multi-phase project aimed at housing them permanently. When the new Oregon Rail Heritage Center is completed, this will permit the ORHF to continue operating steam-powered excursions while displaying the locomotives to the public. Access to the locomotives is limited as the equipment is stored on the private property of the Union Pacific Railroad, out of visitors' reach; the ORHF is tasked with completing the construction of the engine house and moving the locomotives before UP razes its current roundhouse in mid-2012.

The ORHF oversees the preservation of three city-owned steam locomotives: Southern Pacific 4449, Portland & Seattle 700, Oregon Railroad & Navigation 197. All three engines were donated to the city in 1958 and displayed in Oaks Park in Southeast Portland until restored to operation; each engine has its own cadre of volunteers dedicated to its upkeep. They give Portland the distinction of being the only city in the United States to own operating mainline steam locomotives. In addition to these engines, several vintage passenger cars owned and operated by the Great Northern Railway and the Southern Pacific Railroad, are owned and maintained by Northwest Rail Museum and the Pacific Northwest Chapter of the National Railway Historical Society; the steam locomotives are stored in the Brooklyn Roundhouse, in the middle of a former Southern Pacific yard now owned by Union Pacific. The roundhouse is in the midst of a needed expansion by UP on its intermodal facilities, necessitating the movement of the locomotives to a permanent location and the eventual demolition of the roundhouse.

UP has stipulated that the roundhouse must be vacated by the end of June 2012, underscoring the ORHF's determination to build a new facility. The ORHF and its member groups are working to organize an excursion train or special appearance by one of the steam locomotives originating in Portland and running distances of up to 2,000 or more miles over the course of several days, weeks, or months. Excursions are planned months or years in advance in accordance with the destination, dependent upon the approval of the host railroad. An excursion may be as brief as never leaving the Portland city limits, or as long as the SP 4449's journey to Owosso, Michigan for TrainFestival 2009, hosted by the Steam Railroading Institute; the SP 4449 has frequently appeared at the classic automobile show "Cruisin' Sherwood" in Sherwood Old Town, Ore. while the SP&S 700 participates in the annual Burlington Northern Santa Fe Railway holiday celebration in Vancouver, Washington. Both engines teamed up in July 2005 to doublehead the National Railway Historical Society "Western Star" from Portland to Wishram, Washington.

Each engine has taken a turn on "Montana by Steam", a round trip between Portland and Billings, during the past decade. The 700 pulled a number of short excursions on the Oregon Pacific Railroad in 2006 and 2007, along with appearances at the Salem Safety Faire in Salem, Oregon. In the summer of 2011, the SP 4449 hauled two excursions: one to Tacoma and Stampede Pass for the NRHS annual convention, another up the Columbia River Gorge to Wishram to celebrate its 70th anniversary and help to raise funds for an upcoming 15-year boiler certification; the holiday season is the most popular time for both viewing and riding, as the ORHF operates "Holiday Express" excursion trains pulled by the 4449 and/or 700 on weekends in December. The Holiday Express has enjoyed enormous success to date with as many as 10,000 passengers each year. Holiday Express trains run along the Oregon Pacific main line near the bank of the Willamette River, while children on board are greeted by Santa Claus and company. Over its seven years of operation, the Holiday Express has been instrumental in raising the needed capital for construction of the rail heritage museum.

In 2011, the Holiday Express operated on the first two weekends of December, raising $75,000. The ORHF consists of the following groups: Friends of SP 4449 Inc. Friends of OR&N 197 Pacific Railroad Preservation Association Northwest Rail Museum Pacific Northwest Chapter of the National Railway Historical Society City of Portland Parks & Recreation Hosford-Abernethy Neighborhood Development Retired SP/UP locomotive engineer Doyle McCormack serves as the ORHF's president. Former State of Oregon rail planner Ed Immel, who founded the Northwest Rail Museum in 1986, serves as vice-president; the volunteer members of the restoration groups spend several hundred hours each week doing mechanical or cosmetic work on the locomotives and rolling stock. The estimated value and quantity of volunteer labor comes to $2.5 million over 115,000 hours since 1975, it has been stated that every hour of operation is the result of 100 volunteer hours of work. About 150 people volunteer for the ORHF itself every year with the Holiday Express, National Train Day celebrations, mainline excur

Fosi Pala'amo

Fosi Pala'amo is a New Zealand professional rugby player of Samoan descent. Internationally, he plays for Samoa, he was born in New Zealand to Samoan parents. He was a player for the Irish team Leinster Rugby, but now works at Pfizer for a living and coaches the forwards on the Pfizer tag rugby team. Now coaching Monkstown u20s Pala'amo is a product of the famous Australian rugby club Randwick, where he was selected for the Australian Under 21 team, playing alongside Wallabies such as Tom Bowman and Stirling Mortlock. However, despite being hailed a Wallaby in the making, he switched his allegiance to Samoa, in 1998 at the age of 21, he made his debut for Samoa in the Oceania qualifying games for the 1999 world cup. However, he withdrew from the Samoa squad for the 1999 Rugby World Cup due to a serious knee injury which threatened to end his playing career. Pala'amo did not play international rugby again for eight years, he was recalled to the squad for the 2007 World Cup, despite not participating in the warm up games.

He was thrown in at the deep end against eventual runners-up, England. In total, Pala'amo has nine international caps for Samoa, he now lives in Dublin. He is the son of a Samoan church minister who has retired in Australia