Heart-hand syndromes are a group of rare diseases that manifest with both heart and limb deformities. As of July 2013, known heart-hand syndromes include Holt–Oram syndrome, Berk–Tabatznik syndrome, heart-hand syndrome type 3, brachydactyly-long thumb syndrome, patent ductus arteriosus-bicuspid aortic valve syndrome and heart hand syndrome, Slovenian type. Heart-hand syndrome type 1 is more known as Holt–Oram syndrome. Is the most prevalent form of heart-hand syndrome, it is an autosomal dominant disorder that affects bones in the arms and hands and may cause heart problems. The syndrome includes an absent radial bone in the arms, an atrial septal defect, a first degree heart block. Heart-hand syndrome type 2 is known as Berk–Tabatznik syndrome. Berk–Tabatznik syndrome is a condition with an unknown cause that shows symptoms of short stature, congenital optic atrophy and brachytelephalangy; this condition is rare with only two cases being found. Heart-hand syndrome type 3 is rare and has been described only in three members of a Spanish family.
It is known as Heart-hand syndrome, Spanish type. Several people in multiple generations have suffered this type, it causes sudden cardiac death due to ventricular tachycardia, it prominently causes a unique type of brachydactyly with mild hand involvement and more severe foot involvement. Described in one family. A unique feature in the syndrome is symmetric brachydactyly with long thumbs, it has been confirmed that Patent ductus arteriosus-bicuspid aortic valve syndrome is a distinct syndrome from an allelic variant of Char syndrome. Hand anomalies include metacarpal hypoplasia, it is unknown if heart-hand syndromes are caused by distinct genetic defects. It has been claimed that congenital heart diseases are caused by a limited number of shared genetic defects. Holt–Oram syndrome, Brachydactyly-long thumb syndrome, Patent ductus arteriosus-bicuspid aortic valve syndrome and Heart-hand syndrome, Slovenian type are known to be autosomally dominant disorders. Brachydactyly-long thumb syndrome is known to have been transmitted from male-to-male in a single instance.
Medicine portal Cardiovascular disease Congenital heart defect Congenital limb deformities Hand deformity
Jakobstad is a town and municipality in Ostrobothnia, Finland. The town covers a land area of 88.31 square kilometres. The population density is 218.4 inhabitants per square kilometre. Neighbour municipalities are Larsmo, Pedersöre, Nykarleby; the Swedish name means Jacob's City or Jacob's Town, in reference to Jacob De la Gardie. The town was founded at the old harbour of the parish Pedersöre and this name lives on in the Finnish name of the municipality, Pietarsaari Peter's Island; the town was founded in 1652 by Ebba Brahe, the widow of the military commander Jacob De la Gardie, was granted city privileges by Queen Christina of Sweden. The town was founded at the old harbour of the parish Pedersöre. Pedersöre remains an independent municipality neighbouring Jakobstad; the city grew at first, with the authorities scarcely promoting any growth. In 1680 the inhabitants were ordered to relocate to the cities of Karleby, Uleåborg and Nykarleby, but the order was rescinded. Wars contributed to the slow growth, the city was invaded by Russian troops twice during the Greater Wrath, large parts of the town were burnt to the ground.
A majority of the inhabitants fled the city. While those with means moved across the sea to the Swedish side, others took shelter in the forest or in the archipelago. Many were killed. During the 1720s, some of the previous inhabitants returned, while newcomers added to the population; the subsequent decades were marked by a period of growth, the current church was built in 1731. The economic foundation was laid in the mid 18th century, with tar manufacturing and tobacco packaging at its centre. Trade started to develop in Jakobstad as of 1765, when the cities along the Finnish shore of the Gulf of Bothnia were granted privileges by the Swedish crown to trade directly with foreign countries; this led to shipbuilding becoming a major activity in Jakobstad. The first ships to sail with goods to foreign countries were the galeas Jacobstads Wapen and the brig Enigheten. Trade and shipbuilding made Jakobstad a wealthy city, a notable businessman of that time was the merchant and shipbuilder Adolf Lindskog, who became one of the richest men in Finland.
The early 19th century was a time of upheaval, which saw the 1808–1809 war between Sweden and Russia, as well as a devastating fire in 1835 that destroyed half of the city. Despite this, the economic progress continued, a brewery, a matchstick factory and several banks were founded after 1850. In 1859, the merchant and shipowner Peter Malm started a steam powered sawmill, only the second such installation in Finland; the Crimean War was a major setback to shipping industry, as the British navy puts up an effective blockade and the shipping fleet in Jakobstad was reduced from 26 ships to 9. Notable businessmen in the 19th century were Otto Malm and Wilhelm Schauman, the latter founding a chicory factory in Jakobstad in 1883; this moment in time is considered as the start of industrialization in Jakobstad. In 1900, the Strengberg tobacco factory was the largest employer in Jakobstad. An artillery school was located in Jakobstad during the Finnish civil war. During World War II, the city was bombed once by Soviet bomber planes.
Up until the 1960s, the town was overwhelmingly Swedish speaking, but as a consequence of industrial expansion in the 1960s and 1970s, the need for additional work force caused a large influx of Finnish speakers. The town remains bilingual with 56 % being 40 % Finnish speakers. Results of the 2017 Finnish municipal election in Jakobstad: At the end of November a chamber music festival called Rusk is held annually in Pietarsaari/Jakobstad. At the heart of this festival embracing superb chamber music and various other genres of the arts is the Schauman Hall in the centre of town, but the events spread out into the surrounding urban environment. Jakobstad City Hall is a historic building in the city, completed in 1875; the current look of the building dates from 1890. Jakobstad is the home of the galeas Jacobstads Wapen, a full-scale replica built between 1987 and 1992, based on the original 1755 drawings by Swedish naval architect Fredrik Henrik af Chapman Jakobstad Museum is the main museum in the city, concentrating on maritime and ship building history The Arctic museum Nanoq is located outside Jakobstad.
Chicorymuseum is the only chicorymuseum in Finland. In the former chicory factory from the 19th century visitors can familiarize themselves with the production of chicory, get acquainted with Wilhelm Schaumans early industrial career and experience authentic old factory conditions; the museum in Pietarsaari will offer art exhibitions and other events. The botanical garden Skolparken, with 1,000 plant species, is renowned both as one of the most northerly botanical gardens in the world and for its classical park architecture; the foundation was laid in 1915 and the park was completed in 1932. The funding was provided by the Schauman family, who wanted to honour the memory of Elise and Viktor Schauman; the park was designed by the prominent Finnish garden architect Bengt Schalin. Skata is a protected residential area close to the city centre which dates from 1783. Up until the late 19th century, Skata was home to sailors and their families; as of the 1890s, it transformed into a working class area, providing housing to a large part of the work force employed by the Strengberg tobacco factory.
Joshua Colley is an American musical theatre actor. Joshua Colley and his twin brother Cameron were born on January 20, 2002, to Brad and Robbie Lynn Colley. Colley was raised in Florida, his parents own a business called Play'N Around Theatre Company that performs educational musicals at local schools and community centers. Cameron Colley is an actor. In January 2014, it was announced that Colley would play the role of Gavroche in the Broadway revival of Les Miserables, alternating in that role with Gaten Matarazzo. Colley started with the show when it began previews on March 1, 2014 and performed until March 1, 2015; the Huffington Post called Colley's performance "'grounded and believable and anything but an outtake from Oliver!"In 2014, Colley was cast as the voice of Pig Robinson in the Nick Jr. animated television show Peter Rabbit. He voiced the role from 2014 to 2015. Colley played the role of Young Calogero in the Alan Menken musical A Bronx Tale during its world premiere production at the Paper Mill Playhouse.
The show opened on February 4, 2016. Colley played the lead role in You're a Good Man, Charlie Brown at the York Theater Company from May 24, 2016 to June 26, 2016; the cast album was released on November 2016 by Broadway Records. He took one week off of the run to perform in The Little Mermaid at the Hollywood Bowl. Colley played the role of Flounder, alongside Sara Bareilles, Tituss Burgess, John Stamos, Rebel Wilson in the first week of June 2016. In 2016, Colley participated in MisCast at the MCC Theater, performing The Schuyler Sisters from the Broadway hit Hamilton, he sang the part of Angelica Schuyler alongside fellow performers Luca Padovan, Douglas Baldeo and Ana Villafañe
Apophantic is a term Aristotle coined to mean a specific type of declaratory statement that can determine the truth or falsity of a logical proposition or phenomenon. It was adopted by Martin Heidegger as part of phenomenology. Marcuse defines it as "the logic of judgment". In Aristotle's usage, the Greek term ἀποφαντικὸς λόγος describes a statement that, by examining a proposition in itself, can determine what is true about a statement by establishing whether or not the predicate of a sentence may logically be attributed to its subject. For example, logical propositions may be divided into ones that are semantically determinate, as in the sentence "All penguins are birds," and those that are semantically indeterminate, as in the sentence "All bachelors are unhappy." In the first proposition, the subject is penguins and the predicate is birds, the set of all birds is a category into which the subject of penguins should be put. In the second proposition, the subject is bachelors and the predicate is unhappy.
This is a subjective, contingent connection that does not follow. An apophantic conclusion would, by examining the two statements—and not any evidence supporting or denying them—make a judgment between them that identifies "All penguins are birds" as more truthful than "All bachelors are unhappy." One would reach this conclusion because of the propositions' nature, not because any penguins or bachelors had been consulted. In phenomenology, Martin Heidegger argues that apophantic judgements are the most reliable means of obtaining truth, as they do not rely upon subjective comparisons. Before Heidegger, his former teacher Husserl had centralized the role of apophantic judgment in his phenomenological'transcendental logic', during the course lectures on passive synthesis in the mid 1920s; the concept appears in the Arabic Aristotelian tradition as jâzim, or'truth-apt'. "Benedetto Croce, Aesthetic as Science of Expression and General Linguistic"
The 2007 Uttarakhand Legislative Assembly election were the 2nd Vidhan Sabha election of the state of Uttarakhand in India. The elections were held on 21 February 2007, when the Bharatiya Janata Party emerged as the largest party with 35 seats in the 70-seat legislature. One seat short of forming a majority, the BJP have had to rely on support from the Uttarakhand Kranti Dal and three Independents to form the government; the Indian National Congress became the official opposition. The Bharatiya Janata Party emerged as the largest party with 35 seats in a house of 70, they were still one short of the majority to form a government. After much wrangling it was announced that the Uttarakhand Kranti Dal and the three Independents would be supporting the government; the incumbent Indian National Congress Government lost. After protracted discussions it was announced the B. C. Khanduri would be Chief Minister and B. S. Koshyari was to manage party work. 2nd Uttarakhand Assembly First Khanduri ministry Pokhriyal ministry Second Khanduri ministry Elections in Uttarakhand Politics of Uttarakhand 2007 elections in India Elected Members of Legislative Assembly of Uttarakhand, Official list Govt. of Uttarakhand
Action semantics is a framework for the formal specification of semantics of programming languages invented by David Watt and Peter D. Mosses in the 1990s, it is a mixture of denotational and algebraic semantics. Action Semantics aims to be pragmatic. Action-Semantic Descriptions are designed to scale up to handle realistic programming languages; this is aided by the modifiability of ASDs. This helps to ensure that changes do not require too many changes in the description; this is in contrast to the typical case when extending denotational or operational semantics, which may require reformulation of the entire description. The Action Semantics framework was developed at the University of Aarhus and the University of Glasgow. Groups and individuals around the world have since contributed further to the approach. An important part of action semantics that gives it a modularity not seen in previous programming language semantics is the use of first-order semantic entities. First-order refers to how, unlike in denotational semantics, where a semantic function can be applied to another semantic function, in action semantics, a semantic entity cannot be applied to another semantic entity of its kind.
Furthermore, the semantic entities utilized by action semantics broaden the framework’s ability to describe a programming language’s constructs by serving to denote both program behavior, independent of any particular implementation and the way in which parts of a program influence the overall performance of the whole. The appropriately named action notation is employed to express the three types of semantic entities found in action semantics: actions and yielders; the central semantic entity in this framework is actions, with data and yielders occupying supplementary roles. More actions are the mechanisms through which yielders and data are processed. An action, which can occur within another action, is a step-by-step representation of program behavior, where each step accesses current information, changes current information, or does both. Yielders appear within only access current information. A yielder entity is capable of being evaluated, when it is, the product is a datum entity. Action entities can directly represent programs’ semantics by describing possible program behaviors or represent, in a more indirect way, the impact that individual pieces of a program, like statements or expressions, have on the semantics of the program as a whole.
They model computational behavior by indicating changes in state through their generation of new values from passed values. An action accepts data passed to it via the current information — the transient data given to it, the bindings received by it, the current state of storage — and, from this, gives new transient data, creates new bindings, updates the state of storage, or any combination of these. An action entity can culminate in four possible ways, it can: complete, fail, or diverge. There are four categories of information. Transient information corresponds to intermediate results and is accessible for immediate use by the action; the data that comprises transient information encompasses the values given by expressions. If these values are not used, they are lost. Scoped information corresponds to symbol tables and can be referenced from anywhere within the action and its sub-actions, it is possible for such information to be hidden within a sub-action, via the creation of an inner scope, in which case it would be only locally accessible within that scope, to that sub-action.
Stable information corresponds to values assigned to variables and can be modified in the action performance. Because alterations to storage during the performance of an action are persistent, only explicit actions can cause such modifications. In accordance with this, stable information is available. And, unlike scoped information, it cannot be hidden. Permanent information corresponds to data exchanged between actions and can be extended but not modified. Transient information is produced only when an action completes or escapes, scoped information is produced only when an action completes; the modification of stable information and the extension of permanent information must take place during action performance. An action entity has five different facets, one for processing that does not rely on information, four for processing each of the four different types of information; the basic facet, an example of which would be control flows, is not tied to information of any kind. The functional facet deals with the processing of transient information and is characterized by actions giving and accepting data.
The declarative facet deals with the processing of scoped information and is characterized by actions creating and receiving bindings. The imperative facet deals with the processing of stable information and is characterized by actions allocating and freeing storage cells, fetching and modifying the data stored in them; the communicative facet deals with processing permanent information and is characterized by actions sending and receiving messages and “offer contracts to agents.” There are two different kinds of actions in terms of their effect on the information in each facet. Primitive actions only affect the information in one facet. Action combinators permit actions that involve multiple facets, governing how control and information flows for each facet involved in a combined action. In combining actions, action combinators governor the sequencing of sub-action performances and the incoming and outgoing flows of data for each sub-action. Dat