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22-733: FSS may refer to: Education [ edit ] Faculty of Social Sciences, Charles University in Prague in the Czech Republic Fernie Secondary School , in British Columbia, Canada Friends Select School , in Philadelphia, Pennsylvania, United States Frontenac Secondary School , in Kingston, Ontario, Canada Government [ edit ] Federal Security Service ,

44-793: A Colombian sports apparel manufacturer Federal Signal Corporation , an American emergency services equipment manufacturer Fellow of the Royal Statistical Society Finland-Swedish Sign Language The Five Star Stories , a manga series by Mamoru Nagano Flinders Street railway station in Melbourne, Australia Foundation Stock Service Program , a dog breed registry Fox Sports South , an American regional sports network by Fox Sports Networks Freeman–Sheldon syndrome FSS Code , an international maritime fire code Standard Written Form (Cornish: Furv Skrifys Savonek ),

66-461: A Cornish orthographic standard Swiss Socialist Federation (French: Fédération socialiste suisse ), a defunct political party in Switzerland See also [ edit ] FS (disambiguation) Topics referred to by the same term [REDACTED] This disambiguation page lists articles associated with the title FSS . If an internal link led you here, you may wish to change

88-712: A principal security agency of Russia Federal statistical system , a decentralized network of federal agencies in the United States Federal Supply Service , part of the General Services Administration of the United States Financial Supervisory Service , South Korean supervisory agency on financial regulation Food Standards Scotland , a non-ministerial government department of the government of Scotland Forensic and Scientific Services , an agency of

110-508: A result of severe central nervous system involvement or respiratory complications. Several syndromes are related to the Freeman–Sheldon syndrome spectrum, but more information is required before undertaking such nosological delineation. One research priority is to determine the role and nature of malignant hyperthermia in FSS. Such knowledge would benefit possible surgical candidates and

132-464: A type of congenital myopathy . In March 2006, Stevenson et al. published strict diagnostic criteria for distal arthrogryposis type 2A (DA2A) or Freeman–Sheldon syndrome. These included two or more features of distal arthrogryposis: microstomia , whistling-face , nasolabial creases , and 'H-shaped' chin dimple. Patients must have early consultation with craniofacial and orthopaedic surgeons , when craniofacial, clubfoot , or hand correction

154-575: Is also intensively developing international cooperation beyond the framework of the European programs. This includes the maintenance of or entering into new cooperation with partner universities in North America, Asia and the Middle East. Freeman%E2%80%93Sheldon syndrome Freeman–Sheldon syndrome ( FSS ) is a very rare form of multiple congenital contracture (MCC) syndromes ( arthrogryposes ) and

176-766: Is feasible, in consideration of the tendency for fragile health. Early interventions hold the possibility to minimise developmental delays and negate the necessity of relearning basic functions. Due to the abnormal muscle physiology in Freeman–Sheldon syndrome, therapeutic measures may have unfavourable outcomes. Difficult endotracheal intubations and vein access complicate operative decisions in many DA2A patients, and malignant hyperthermia (MH) may affect individuals with FSS, as well. Cruickshanks et al. (1999) reports uneventful use of non-MH-triggering agents. Reports have been published about spina bifida occulta in anaesthesia management and cervical kyphoscoliosis in intubations. General health maintenance should be

198-427: Is indicated to improve function or aesthetics. Operative measures should be pursued cautiously, with avoidance of radical measures and careful consideration of the abnormal muscle physiology in Freeman–Sheldon syndrome. Unfortunately, many surgical procedures have suboptimal outcomes, secondary to the myopathy of the syndrome. When operative measures are to be undertaken, they should be planned for as early in life as

220-531: Is the most severe form of distal arthrogryposis (DA). It was originally described by Ernest Arthur Freeman and Joseph Harold Sheldon in 1938. As of 2007, only about 100 cases had been reported in medical literature. The symptoms of Freeman–Sheldon syndrome include drooping of the upper eyelids, strabismus , low-set ears, a long philtrum, gradual hearing loss, scoliosis and walking difficulties. Gastroesophageal reflux has been noted during infancy, but usually improves with age. The tongue may be small, and

242-571: The anaesthesiology and surgical teams who would care for them. MH may also be triggered by stress in patients with muscular dystrophies. Much more research is warranted to evaluate this apparent relationship of idiopathic hyperpyrexia, MH, and stress. Further research is wanted to determine epidemiology of psychopathology in FSS and refine therapy protocols. It is named for British orthopaedic surgeon Ernest Arthur Freeman (1900–1975) and British physician Joseph Harold Sheldon (1893–1972), who first described it in 1938. Melissa Blake (writer)

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264-813: The Faculty of Social Sciences are intended to prepare students for a professional career in the private sector, public administration, national governments, European institutions, academic research or the media. They are divided into three individual cycles: The Faculty offers degree programs at all levels in its subject specializations in both Czech and English. The English language degree programs offer one fully accredited English language bachelor´s degree program, ten fully accredited English language master's degree programs, and two double-degree programs in partnership with universities in London and Strasbourg. The Faculty offers summer school programs to its students, visiting students and

286-434: The diagnosis of Freeman–Sheldon syndrome were drawn up, assigning Freeman–Sheldon syndrome as distal arthrogryposis type 2A (DA2A). On the whole, DA1 is the least severe; DA2B is more severe with additional features that respond less favourably to therapy. DA2A (Freeman–Sheldon syndrome) is the most severe of the three, with more abnormalities and greater resistance to therapy. Freeman–Sheldon syndrome has been described as

308-702: The faculties of Charles University. Each year it welcomes around 500 exchange students. International mobility at the Faculty is mainly based on the European Higher Education Area which is coordinated by the Bologna Process . Its most visible demonstration is the ERASMUS Programme . In addition to the Erasmus Programme, the Faculty participates in other smaller student exchange programs, such as Erasmus Mundus , CEEPUS or AKTION. The Faculty

330-411: The general public. For international students, the Faculty organizes Spring University Prague and Summer University Prague each April and September. This program is intended for students who are enrolled in a college or university or have recently graduated. It is open to approximately 30 students from across the world. The Faculty has one of the highest international mobility participation rates of all

352-1112: The limited movement of the soft palate may cause nasal speech. Often there is an H- or Y-shaped dimpling of the skin over the chin. FSS is caused by genetic changes. Krakowiak et al. (1998) mapped the distal arthrogryposis multiplex congenita (DA2B; MIM #601680) gene , a syndrome very similar in phenotypic expression to classic FSS, to 11p15.5-pter. Other mutations have been found as well. In FSS, inheritance may be either autosomal dominant , most often demonstrated. or autosomal recessive (MIM 277720). Alves and Azevedo (1977) note most reported cases of DA2A have been identified as new allelic variation . Toydemir et al. (2006) showed that mutations in embryonic myosin heavy chain 3 (MYH3; MIM *160270), at 17p-13.1-pter, caused classic FSS phenotype, in their screening of 28 (21 sporadic and 7 familial) probands with distal arthrogryposis type 2A. In 20 patients (12 and 8 probands, respectively), missense mutations (R672H; MIM *160270.0001 and R672C; MIM *160270.0002) caused substitution of arg672, an embryonic myosin residue retained post-embryonically. Of

374-662: The link to point directly to the intended article. Retrieved from " https://en.wikipedia.org/w/index.php?title=FSS&oldid=1256197137 " Category : Disambiguation pages Hidden categories: Articles containing Cornish-language text Articles containing French-language text Short description is different from Wikidata All article disambiguation pages All disambiguation pages Faculty of Social Sciences, Charles University in Prague The Faculty enrolls around 5,000 full-time students, including 1,000 international students. Studies at

396-527: The remaining 6 patients in whom they found mutations, 3 had missense private de novo (E498G; MIM *160270.0006 and Y583S) or familial mutations (V825D; MIM *160270.0004); 3 other patients with sporadic expression had de novo mutations (T178I; MIM *160270.0003), which was also found in DA2B; 2 patients had no recognized mutations. Freeman–Sheldon syndrome is a type of distal arthrogryposis , related to distal arthrogryposis type 1 (DA1). In 1996, more strict criteria for

418-688: The sports teams of Florida State University Football Association of Serbia FS Seoul , a South Korean futsal club Technology [ edit ] Fish School Search Fixed-satellite service Fixed Service Structure , a launch tower leased by SpaceX at Kennedy Space Center, Florida, United States Flats Sequencing System , a mail sorting system used by the United States Postal Service Flight service station Flying-spot scanner Food Service Solutions , an American software company Frequency selective surface Other uses [ edit ] FSS (brand) ,

440-663: The state government of Queensland, Australia Forensic Science Service , a defunct British crown corporation Foreign Service Specialist , Department of State Military [ edit ] Fast Sealift Ship of the United States Navy Fire Support Specialist , an artillery observer in the United States Army RAF Kinloss , a former Royal Air Force station in Scotland Sport [ edit ] Florida State Seminoles ,

462-518: The syndrome's primary involvement is limited to the musculoskeletal systems, and satisfactory quality and length of life can be expected with proper care. There are little data on prognosis. Rarely, some patients have died in infancy from respiratory failure; otherwise, life expectancy is considered to be normal. By 1990, 65 patients had been reported in the literature, with no sex or ethnic preference notable. Some individuals present with minimal malformation; rarely patients have died during infancy as

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484-410: The therapeutic emphasis in Freeman–Sheldon syndrome. The focus is on limiting exposure to infectious diseases because the musculoskeletal abnormalities make recovery from routine infections much more difficult in FSS. Pneumonitis and bronchitis often follow seemingly mild upper respiratory tract infections . Though respiratory challenges and complications faced by a patient with FSS can be numerous,

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