Down syndrome or trisomy 21 (in British English: Down's syndrome) is a genetic disorder due to the presence of all or part of an extra 21st chromosome. It is named after John Langdon Down, the British doctor who first described it in 1866. The condition is characterized by a combination of major and minor abnormalities in body structure and function. Nearly all Down syndrome cases feature impairment of learning and physical growth as well as a recognizable facial appearance usually identified at birth.
Individuals with Down syndrome have lower than average cognitive ability, normally ranging from mild to moderate retardation. They generally have some developmental disability, such as a tendency toward concrete thinking or naïveté. Some have low intelligence. A small number suffer from severe to profound mental retardation. The incidence of Down syndrome is estimated at 1 per 800 to 1 per 1000 births.
The common physical features of Down syndrome also appear in people with a standard set of chromosomes. They include a single transverse palmar crease (a single instead of a double crease across one or both palms), an almond shape to the eyes caused by an epicanthic fold of the eyelid, shorter limbs, speech impairment, and protruding tongue. Health concerns for individuals with Down syndrome include a higher risk for congenital heart defects, gastroesophageal reflux disease, recurrent ear infections, obstructive sleep apnea, and thyroid dysfunctions.
Early childhood intervention, screening for common problems, medical treatment where indicated, a conducive family environment, and vocational training can improve the overall development of children with Down syndrome. While some of the genetic limitations of Down Syndrome cannot be overcome, education and proper care, initiated at any time, can improve quality of life.[1]
Contents [hide]
1 History
2 Characteristics
3 Genetics
3.1 Trisomy 21
3.2 Mosaicism
3.3 Robertsonian translocation
3.4 Duplication of a portion of chromosome 21
4 Incidence
5 Prenatal screening
6 Cognitive development
7 Health
8 Genetic research
9 Sociological and cultural aspects
10 Notable individuals
11 Portrayal in fiction
12 References
13 Bibliography
14 External links
14.1 Societies and associations
14.2 Conferences
[edit] History
Main article: History of Down syndrome
English physician John Langdon Down first characterized Down syndrome as a distinct form of mental retardation in 1862, and in a more widely published report in 1866 entitled "Observations on an ethnic classification of idiots".[2] Due to his perception that children with Down syndrome shared physical facial similarities (epicanthal folds) with those of Blumenbach's Mongolian race, Down used terms such as mongolism and mongolian idiocy.[3] Idiocy was a medical term used at that time to refer to a severe degree of intellectual impairment. Down wrote that mongolism represented "retrogression," the appearance of Mongoloid traits in the children of allegedly more advanced Caucasian parents.
By the 20th century, "mongolian idiocy" had become the most recognizable form of mental retardation. Most people with it were institutionalized. Few of the associated medical problems were treated, and most died in infancy or early adult life. With the rise of the eugenics movement, 33 of the 48 United States and a number of countries began programs of involuntary sterilization of individuals with Down syndrome and comparable degrees of disability. The ultimate expression of this type of public policy was the German euthanasia program "Aktion T-4" begun in 1940. Court challenges and public revulsion led to discontinuation or repeal of such programs during the decades after World War II.
Until the middle of the 20th century, the cause of Down syndrome remained unknown, although the presence in all races, the association with older maternal age, and the rarity of recurrence had been noticed. Standard medical texts assumed it was due to a combination of inheritable factors which had not been identified. Other theories focused on injuries sustained during birth.[4]
With the discovery of karyotype techniques in the 1950s it became possible to identify abnormalities of chromosomal number or shape. In 1959, Professor Jérôme Lejeune discovered that Down syndrome resulted from an extra chromosome.[5] The extra chromosome was subsequently labeled as the 21st, and the condition as trisomy 21.
In 1961, a group of nineteen geneticists wrote to the editor of The Lancet suggesting that mongolian idiocy had "misleading connotations," had become "an embarrassing term," and should be changed.[6] The Lancet supported Down's Syndrome. The World Health Organization (WHO) officially dropped references to mongolism in 1965 after a request by the Mongolian delegate.[7]
In 1975, the United States National Institute of Health convened a conference to standardize the nomenclature of malformations. They recommended eliminating the possessive form: "The possessive use of an eponym should be discontinued, since the author neither had nor owned the disorder."[8] While both the possessive and non-possessive forms are used in the general population, Down syndrome is the accepted term among professionals in the USA, Canada and other countries, while Down's syndrome continues to be used in the United Kingdom and other areas.[9]
[edit] Characteristics
Example of white spots on the iris known as Brushfield’s Spots.Individuals with Down syndrome may have some or all of the following physical characteristics:[10] oblique eye fissures with epicanthic skin folds on the inner corner of the eyes, muscle hypotonia, a flat nasal bridge, a single palmar fold (also known as a simian crease), a protruding tongue (due to small oral cavity, poor muscle tone, and an enlarged tongue near the tonsils), a short neck, white spots on the iris known as Brushfield spots,[11] excessive flexibility in joints, congenital heart defects, excessive space between large and second toe, and a single flexion furrow of the fifth finger. Most individuals with Down syndrome have mental retardation in the mild (IQ 50–70) to moderate range (IQ 35–50),[12] with scores for children having Mosaic Down syndrome (explained below) typically 10–30 points higher.[13] In addition, individuals with Down syndrome can have serious abnormalities affecting any body system.
[edit] Genetics
Main article: Genetic origins of Down syndrome
Karyotype for trisomy Down syndrome. Notice the three copies of chromosome 21.Down syndrome is a chromosomal abnormality characterized by the presence of an extra copy of genetic material on the 21st chromosome, either in whole (trisomy 21) or part (such as due to translocations). The effects of the extra copy varies greatly from individual to individual, depending on the extent of the extra copy, genetic background, environmental factors, and random chance. Down syndrome occurs in all human populations, and analogous effects have been found in other species such as chimpanzees and mice. Recently, researchers have been able to create transgenic mice with most of human chromosome 21 (in addition to the normal mouse chromosomes).[14] The extra chromosomal material can come about in several distinct ways. A normal human karyotype is designated as 46,XX or 46,XY, indicating 46 chromosomes with an XX arrangement for females and 46 chromosomes with an XY arrangement for males.[15]
[edit] Trisomy 21
Trisomy 21 (47,XX,+21) is caused by a meiotic nondisjunction event. With nondisjunction, a gamete (i.e., a sperm or egg cell) is produced with an extra copy of chromosome 21; the gamete thus has 24 chromosomes. When combined with a normal gamete from the other parent, the embryo now has 47 chromosomes, with three copies of chromosome 21. Trisomy 21 is the cause of approximately 95% of observed Down syndromes, with 88% coming from nondisjunction in the maternal gamete and 8% coming from nondisjunction in the paternal gamete.[16]
[edit] Mosaicism
Trisomy 21 is generally caused prior to conception, and all cells in the body are affected. However, when some of the cells in the body are normal and other cells have trisomy 21, it is called Mosaic Down Syndrome (46,XX/47,XX,+21).[17] This can occur in one of two ways: A nondisjunction event during an early cell division in a normal embryo leads to a fraction of the cells with trisomy 21; or a Down syndrome embryo undergoes nondisjunction and some of the cells in the embryo revert back to the normal chromosomal arrangement. There is considerable variability in the fraction of trisomy 21, both as a whole and among tissues. This is the cause of 1–2% of the observed Down syndromes.[16] There is evidence that mosaic Down syndrome may produce less developmental delay, on average, than full trisomy 21.[18]
[edit] Robertsonian translocation
The extra chromosome 21 material that causes Down syndrome may be due to a Robertsonian translocation. In this case, the long arm of chromosome 21 is attached to another chromosome, often chromosome 14 (45,XX,t(14;21q)) or itself (called an isochromosome, 45,XX,t(21q;21q)). Normal disjunction leading to gametes have a significant chance of creating a gamete with an extra chromosome 21. Translocation Down syndrome is often referred to as familial Down syndrome. It is the cause of 2-3% of observed cases of Down syndrome.[16] It does not show the maternal age effect, and is just as likely to have come from fathers as mothers.
[edit] Duplication of a portion of chromosome 21
Rarely, a region of chromosome 21 will undergo a duplication event. This will lead to extra copies of some, but not all, of the genes on chromosome 21 (46,XX,dup(21q)).[19] If the duplicated region has genes that are responsible for Down syndrome physical and mental characteristics, such individuals will show those characteristics. This cause is very rare and no rate estimates are available.
[edit] Incidence
Graph showing increased chance of Down syndrome compared to maternal age.[20]The incidence of Down syndrome is estimated at 1 per 800 to 1 per 1000 births.[21] In 2006, the Center for Disease Control estimated the rate as 1 per 733 live births in the United States (5429 new cases per year).[22] Approximately 95% of these are trisomy 21, making it the most common human aneuploidy[citation needed]. Down syndrome occurs in all ethnic groups and among all economic classes.
Maternal age influences the risk of conceiving a baby with Down syndrome. At maternal age 20 to 24, the risk is 1/1490, while at age 40 the risk is 1/106, and at age 49 the risk is 1/11.[23] Although the risk increases with maternal age, 80% of children with Down syndrome are born to women under the age of 35,[24] reflecting the overall fertility of that age group. Other than maternal age, no other risk factors are known. There does not appear to be a paternal age effect.
Many standard prenatal screens can discover Down syndrome. Genetic counseling along with genetic testing, such as amniocentesis, chorionic villus sampling (CVS), or percutaneous umbilical blood sampling (PUBS) are usually offered to families who may have an increased chance of having a child with Down syndrome, or where normal prenatal exams indicate possible problems. Genetic screens are often performed on pregnant women older than 30 or 35.
[edit] Prenatal screening
Pregnant women can be screened for various complications in their pregnancy, or due to risk factors such as advanced maternal age. There are several common non-invasive screens that can indicate a fetus with Down syndrome, and are normally performed in the late first trimester or early second trimester. Due to the nature of screens, each has a significant chance of a false positive, suggesting a fetus with Down syndrome when, in fact, the fetus does not have this genetic abnormality. Screen positives must be verified before a Down syndrome diagnosis is made. Common screening procedures for Down syndrome are given in Table 1.
Table 1: Common first and second trimester Down syndrome screens Screen When performed (weeks gestation) Detection rate False positive rate Description
Triple screen 15–20 75% 8.5% This test measures the maternal serum alpha feto protein (a fetal liver protein), estriol (a pregnancy hormone), and human chorionic gonadotropin (hCG, a pregnancy hormone).[25]
Quad screen 15–20 79% 7.5% This test measures the maternal serum alpha feto protein (a fetal liver protein), estriol (a pregnancy hormone), human chorionic gonadotropin (hCG, a pregnancy hormone), and high inhibin-Alpha (INHA).[25]
AFP/free beta screen 13–22 80% 2.8% This test measures the alpha feto protein, produced by the fetus, and free beta hCG, produced by the placenta.
Nuchal translucency/free beta/PAPPA screen 10–13.5 91%[26] 5%[26] Uses ultrasound to measure Nuchal Translucency in addition to the freeBeta hCG and PAPPA (pregnancy-associate plasma protein A, Mendelian Inheritance in Man (OMIM) 176385 ). NIH has confirmed that this first trimester test is more accurate than second trimester screening methods.[27]
Ultrasound of fetus with Down syndrome and megacystis.Even with the best non-invasive screens, the detection rate is 90%–95% and the rate of false positive is 2%–5%. False positives can be caused by undetected multiple fetuses (very rare with the ultrasound tests), incorrect date of pregnancy, or normal variation in the proteins.
Confirmation of screen positive is normally accomplished with amniocentesis. This is an invasive procedure and involves taking amniotic fluid from the mother and identifying fetal cells. The lab work can take a couple of weeks but will detect over 99.8% of all numerical chromosomal problems with a very low false positive rate.[28]
Due to the low incidence of Down syndrome, the vast majority of early screen positives are false.[29] Since the risk of spontaneous abortion is approximately 1/200 to 1/300,[30] amniocentesis confirmation presents a risk of spontaneously aborting a healthy fetus (while testing from a false positive).
A 2002 literature review of elective abortion rates found that 91–93% of pregnancies with a diagnosis of Down syndrome were terminated.[31] Physicians and ethicists are concerned about the ethical ramifications,[32] with some commentators calling it "eugenics by abortion".[33] Many members of the disability rights movement "believe that public support for prenatal diagnosis and abortion based on disability contravenes the movement's basic philosophy and goals."[34]
[edit] Cognitive development
Cognitive development in children with Down syndrome is quite variable. It is not possible at birth to predict their capabilities. The identification of the best methods of teaching each particular child ideally begins soon after birth through early intervention programs.[35] Since children with Down syndrome have a wide range of abilities, many can be successful in school, while others struggle. Because of this variability in expression of Down syndrome, it is important to evaluate children individually. The cognitive problems that are found among children with Down syndrome can also be found among typical children. This means that parents can take advantage of general programs that are offered through the schools or other means.
Language skills show a difference between understanding speech and expressing speech. It is common for children with Down syndrome to need speech therapy to help with expressive language.[36] Fine motor skills are delayed[37] and often lag behind gross motor skills and can interfere with cognitive development. Occupational therapy can address these issues.[38]
In education, mainstreaming of children with Down syndrome is controversial. Mainstreaming is when students of differing abilities are placed in classes with their chronological peers. Children with Down syndrome do not age emotionally/socially and intellectually at the same rates as children without Down syndrome, so eventually the intellectual and emotional gap between children with and without Down syndrome widens. Complex thinking as required in sciences but also in history, the arts, and other subjects is often beyond their abilities, or achieved much later than in most children. Therefore, if they are to benefit from mainstreaming without feeling inferior most of the time, special adjustments must be made to the curriculum.[39]
Some European countries such as Germany and Denmark advise a two-teacher system, whereby the second teacher takes over a group of children with disabilities within the class. A popular alternative is cooperation between special education schools and mainstream schools. In cooperation, the core subjects are taught in separate classes, which neither slows down the typical students nor neglects the students with disabilities. Social activities, outings, and many sports and arts activities are performed together, as are all breaks and meals.[40]
[edit] Health
Main article: Health aspects of Down syndrome
The medical consequences of the extra genetic material in Down syndrome are highly variable and may affect the function of any organ system or bodily process. The health aspects of Down syndrome encompass anticipating and preventing effects of the condition, recognizing complications of the disorder, managing individual symptoms, and assisting the individual and his/her family in coping and thriving with any related disability or illnesses.[41]
The most common manifestations of Down syndrome are the characteristic facial features, cognitive impairment, congenital heart disease, hearing deficits, short stature, thyroid disorders, and Alzheimer's disease. Other less common serious illnesses include leukemia, immune deficiencies, and epilepsy. Down syndrome can result from several different genetic mechanisms. This results in a wide variability in individual symptoms due to complex gene and environment interactions. Prior to birth, it is not possible to predict the symptoms that an individual with Down syndrome will develop. Some problems are present at birth, such as certain heart malformations. Others become apparent over time, such as epilepsy.
These factors contribute to a significantly shorter lifespan for people with Down syndrome. One study, carried out in the United States, shows an average lifespan of 49 years.[42]
[edit] Genetic research
Main article: Research of Down syndrome-related genes
Down syndrome disorders are based on having too many copies of the genes located on chromosome 21. In general, this leads to an overexpression of the genes.[43][44] Understanding the genes involved may help to target medical treatment to individuals with Down syndrome. It is estimated that chromosome 21 contains 200 to 250 genes.[45] Recent research has identified a region of the chromosome that contains the main genes responsible for the pathogenesis of Down syndrome,[46] located proximal to 21q22.3. The search for major genes involved in Down syndrome characteristics is normally in the region 21q21–21q22.3.
Recent use of transgenic mice to study specific genes in the Down syndrome critical region is has yielded some results. APP (Mendelian Inheritance in Man (OMIM) 104760
, located at 21q21) is an Amyloid beta A4 precursor protein. It is suspected to have a major role in cognitive difficulties.[47] Another gene, ETS2 (Mendelian Inheritance in Man (OMIM) 164740
, located at 21q22.3) is Avian Erythroblastosis Virus E26 Oncogene Homolog 2. Researchers have "demonstrated that overexpression of ETS2 results in apoptosis. Transgenic mice overexpressing ETS2 developed a smaller thymus and lymphocyte abnormalities, similar to features observed in Down syndrome."[48]
[edit] Sociological and cultural aspects
Advocates for people with Down syndrome point to various factors, such as special education and parental support groups, that make life easier for parents. There are also great strides being made in education, housing, and social settings to create "Down-friendly" environments. In most developed countries, since the early twentieth century many people with Down syndrome were housed in institutions or colonies and excluded from society. However, in the twenty first century there is a change among parents, educators and other professionals generally advocating a policy of "inclusion",[49] bringing people with any form of mental or physical disability into general society as much as possible. In many countries, people with Down syndrome are educated in the normal school system and there are increasingly higher-quality opportunities to mix "special" education with regular education settings.
Despite this change, reduced abilities of people with Down syndrome pose a challenge to their parents and families. While living with their parents is preferable to institutionalization for most people with Down syndrome, they often encounter patronising attitudes and discrimination in the wider community. In the past decade, many couples with Down Syndrome have married and started homes of their own, overcoming many of the stereotypes associated with this condition.
The first World Down Syndrome Day was held on 21 March 2006. The day and month were chosen to correspond with 21 and trisomy respectively. It was proclaimed by Down Syndrome International.[50]
[edit] Notable individuals
Chris Burke (far right) was an actor on Life Goes OnNotable people with Down syndrome include:
Chris Burke, actor (Life Goes On) and autobiographer
Anne de Gaulle (1928-1948), daughter of Charles de Gaulle
Stephane Ginnsz, actor (Duo) First actor with Down syndrome in the lead part of a motion picture.
Joey Moss, Edmonton Oilers Locker room attendant
Judith Scott, artist
Miguel Tomasin, singer with Argentinian avant-rock band Reynols
Isabella Pujols, adopted daughter of St. Louis Cardinals first baseman Albert Pujols
The Down Syndrome Association of Los Angeles maintains a list of individuals with Down syndrome in roles in TV and movies.[51]
[edit] Portrayal in fiction
Bret Lott: Jewel
Bernice Rubens: A Solitary Grief
Paul M Belous & Robert Wolterstorff: Quantum Leap: Jimmy
Emily Perl Kingsley: Welcome to Holland
The Kingdom and its American counterpart, Kingdom Hospital
Stephen King: Dreamcatcher
Dean Koontz: The Bad Place
Jeffrey Eugenides: The Virgin Suicides
Petal Mitchell, character in EastEnders
Kim Edwards: The Memory Keeper's Daughter
[edit] References
^ Roizen NJ, Patterson D.Down's syndrome. Lancet. 2003 12 April;361(9365):1281-9. Review. PMID 12699967
^ Down, J.L.H. (1866). "Observations on an ethnic classification of idiots". Clinical Lecture Reports, London Hospital 3: 259-262. Retrieved on 2006-07-14. For a history of the disorder, see OC Ward (1998). John Langdon Down, 1828-1896. Royal Society of Medicine Press. ISBN 1-85315-374-5. or Conor, Ward. John Langdon Down and Down's syndrome (1828 - 1896). Retrieved on 2006-06-02.
^ (1999) "John Langdon Down: The Man and the Message". Down Syndrome Research and Practice 6 (1): 19-24. Retrieved on 2006-06-02.
^ Warkany, J. (1971). Congenital Malformations. Chicago: Year Book Medical Publishers, Inc, 313-314. ISBN 0-8151-9098-0.
^ Jérôme Lejeune Foundation. Retrieved on 2006-06-02.
^ Allen, Gordon, C.E. Benda, J.A. Böök, C.O. Carter, C.E. Ford, E.H.Y. Chu, E. Hanhart, George Jervis, W. Langdon-Down, J. Lejeune, H. Nishimura, J. Oster, L.S. Penrose, P.E. Polani, Edith L. Potter, Curt Stern, R. Turpin, J. Warkany, and Herman Yannet (1961). "Mongolism (Correspondence)". The Lancet 1 (7180): 775.
^ Howard-Jones, Norman (1979). "On the diagnostic term "Down's disease"". Medical History 23 (1): 102-104. PMID 153994.
^ A planning meeting was held on 20 March 1974, resulting in a letter to The Lancet. (1974) "Classification and nomenclature of malformation (Discussion)". The Lancet 303 (7861): 798. The conference was held 10 February-11 February 1975, and reported to The Lancet shortly afterward. (1975) "Classification and nomenclature of morphological defects (Discussion)". The Lancet 305 (7905): 513.
^ Leshin, Len (2003). What's in a name. Retrieved on 2006-05-12.
^ Debra Wood (2005). Down syndrome. Retrieved on 2006-06-30.
^ Definition of Brushfield's Spots.
^ Keep Kids Healthy article on Down syndrome.
^ Strom, C. FAQ from Mosaic Down Syndrome Society. Retrieved on 2006-06-03.
^ BBC News (22 September 2005). Down's syndrome recreated in mice. Retrieved on 2006-06-14.
^ For a description of human karyotype see Mittleman, A. (editor) (1995). An International System for Human Cytogenetic Nomeclature. Retrieved on 2006-06-04.
^ a b c Down syndrome occurrence rates (NIH). Retrieved on 2006-06-02.
^ Mosaic Down Syndrome on the Web
^ Leshin, L. (2000). Mosaic Down Syndrome. Retrieved on 2006-06-02.
^ Petersen MB, Tranebjaerg L, McCormick MK, Michelsen N, Mikkelsen M, Antonarakis SE. Clinical, cytogenetic, and molecular genetic characterization of two unrelated patients with different duplications of 21q. Am J Med Genet Suppl. 1990;7:104-9. PMID 2149934
^ Hook, E.B. (1981). "Rates of chromosomal abnormalities at different maternal ages". Obstet Gynecol 58: 282.
^ Based on estimates by National Institute of Child Health & Human Development Down syndrome rates. Retrieved on 2006-06-21.
^ Center for Disease Control (6 January 2006). "Improved National Prevalence Estimates for 18 Selected Major Birth Defects, United States, 1999-2001". Morbidity and Mortality Weekly Report 54 (51 & 52): 1301-1305.
^ Hook, E.B. (1981). "Rates of chromosomal abnormalities at different maternal ages". Obstet Gynecol 58: 282.
^ Estimate from National Down Syndrome Center. Retrieved on 2006-04-21.
^ a b For a current estimate of rates, see Benn, PA, J Ying, T Beazoglou, JFX Egan. "Estimates for the sensitivity and false-positive rates for second trimester serum screening for Down syndrome and trisomy 18 with adjustments for cross-identification and double-positive results". Prenatal Diagnosis 21 (1): 46-51.
^ a b Some practices report adding Nasal Bone measurements and increasing the detection rate to 95% with a 2% False Positive Rate.
^ NIH FASTER study (NEJM 2005 (353):2001). See also J.L. Simplson's editorial (NEJM 2005 (353):19).
^ Fackler, A. Down syndrome. Retrieved on 2006-09-07.
^ Assume the false positive rate is 2% (at the low end), the incidence of Down syndrome is 1/500 (on the high side) with 95% detection, and there is no ascertainment bias. Out of 100,000 screens, 200 will have Down syndrome, and the screen will detect 190 of them. From the 99,800 normal pregnancies, 1996 will be given a positive result. So, among the 2,186 positive test results, 91% will be false positives and 9% will be true positives.
^ Benke, P, V. Carver, R Donahue. Risk and Recurrence Risk of Down Syndrome. Retrieved on 2006-09-07.
^ Caroline Mansfield, Suellen Hopfer, Theresa M. Marteau (1999). "Termination rates after prenatal diagnosis of Down syndrome, spina bifida, anencephaly, and Turner and Klinefelter syndromes: a systematic literature review". Prenatal Diagnosis 19 (9): 808-812. This is similar to 90% results found by David W. Britt, Samantha T. Risinger, Virginia Miller, Mary K. Mans, Eric L. Krivchenia, Mark I. Evans (1999). "Determinants of parental decisions after the prenatal diagnosis of Down syndrome: Bringing in context". American Journal of Medical Genetics 93 (5): 410 - 416.
^ Glover, NM and Glover, SJ (1996). "Ethical and legal issues regarding selective abortion of fetuses with Down syndrome". Ment. Retard. 34 (4): 207-214. PMID 8828339.
^ George Will (14 April 2005). "Eugenics By Abortion: Is perfection an entitlement?". Washington Post: A37. Retrieved on 2006-07-03.
^ Erik Parens and Adrienne Asch (2003). "Disability rights critique of prenatal genetic testing: Reflections and recommendations". Mental Retardation and Developmental Disabilities Research Reviews 9 (1). Retrieved on 2006-07-03.
^ New Parent Guide. National Down Syndrome Society. Retrieved on 2006-05-12. Also Research projects - Early intervention and education. Retrieved on 2006-06-02.
^ Bird, G. and S. Thomas (2002). "Providing effective speech and language therapy for children with Down syndrome in mainstream settings: A case example". Down Syndrome News and Update 2 (1): 30-31. Also, Kumin, Libby (1998). “Comprehensive speech and language treatment for infants, toddlers, and children with Down syndrome”, Hassold, T.J.and D. Patterson: Down Syndrome: A Promising Future, Together. New York: Wiley-Liss.
^ Development of Fine Motor Skills in Down Syndrome. Retrieved on 2006-07-03.
^ M. Bruni. Occupational Therapy and the Child with Down Syndrome. Retrieved on 2006-06-02.
^ S.E.Armstrong. Inclusion: Educating Students with Down Syndrome with Their Non-Disabled Peers. Retrieved on 2006-05-12. Also, see Debra L. Bosworth. Benefits to Students with Down Syndrome in the Inclusion Classroom: K-3. Retrieved on 2006-06-12. Finally, see a survey by NDSS on inclusion, Gloria Wolpert (1996). The Educational Challenges Inclusion Study. National Down Syndrome Society. Retrieved on 2006-06-28.
^ There are many such programs. One is described by Action Alliance for Children, K. Flores. Special needs, "mainstream" classroom. Retrieved on 2006-05-13. Also, see Flores, K.. Special needs, "mainstream" classroom. Retrieved on 2006-05-13.
^ American Academy of Pediatrics Committee on Genetics (Feb 2001). "American Academy of Pediatrics: Health supervision for children with Down syndrome". Pediatrics 107 (2): 442-449. PMID 11158488.
^ Young, Emma. "Down's syndrome lifespan doubles", New Scientist, 2002-03-22. Retrieved on 2006-10-14.
^ R Mao, CL Zielke, HR Zielke, J Pevsner (2003). "Global up-regulation of chromosome 21 gene expression in the developing Down syndrome brain". Genomics 81 (5): 457-467.
^ Rong Mao, X Wang, EL Spitznagel, LP Frelin, JC Ting, H Ding, J Kim, I Ruczinski, TJ Downey, J Pevsner (2005). "Primary and secondary transcriptional effects in the developing human Down syndrome brain and heart". Genome Biology 6 (13): R107.
^ See Leshin, L. (2003). Trisomy 21: The Story of Down Syndrome. Retrieved on 2006-05-21.
^ Zohra Rahmani, Jean-Louis Blouin, Nicole Créau-Goldberg, Paul C. Watkins, Jean-François Mattei, Marc Poissonnier, Marguerite Prieur, Zoubida Chettouh, Annie Nicole, Alain Aurias, Pierre-Marie Sinet, Jean-Maurice Delabar (2005). "Down syndrome critical region around D21S55 on proximal 21q22.3". American Journal of Medical Genetics 37 (S2): 98-103.
^ Chandra Shekhar (6 July 2006). Down syndrome traced to one gene. The Scientist. Retrieved on 2006-07-11.
^ OMIM, NIH. V-ETS Avian Erythroblastosis virus E26 Oncogene Homolog 2. Retrieved on 2006-06-29.
^ See the pamphlet: Inclusion: Educating Students with Down Syndrome with Their Non-Disabled Peers. National Down Syndrome Society. Retrieved on 2006-05-21.
^ World Down Syndrome Day. Retrieved on 2006-06-02.
^ Down Syndrome Association of Los Angeles. Media Archive: Television and Film that include individuals with Down Syndrome. Retrieved 1 December 2006.
[edit] Bibliography
Beck, M.N. (1999). Expecting Adam. New York: Berkley Books.
Buckley, S. (2000). Living with Down Syndrome. Portsmouth, UK: The Down Syndrome Educational Trust.
Down Syndrome Research Foundation (2005). Bright Beginnings: A Guide for New Parents. Buckinghamshire, UK: Down Syndrome Research Foundation.
Hassold, T.J., D. Patterson (1999). editors,: Down Syndrome: A Promising Future, Together. New York: Wiley Liss.
Kingsley, J., M. Levitz (1994). Count us in — Growing up with Down Syndrome. San Diago: Harcourt Brace.
Pueschel, S.M., M. Sustrova (1997). editors,: Adolescents with Down Syndrome: Toward a More Fulfilling Life. Baltimore, MD USA: Paul H. Brookes.
Selikowitz, M. (1997). Down Syndrome: The Facts, 2nd edition, Oxford: Oxford University Press.
Van Dyke, D.C., P.J. Mattheis; S. Schoon Eberly; and J. Williams (1995). Medical and Surgical Care for Children with Down Syndrome. Bethesda, MD USA: Woodbine House.
Zuckoff, M. (2002). Choosing Naia: A Family's Journey. New York: Beacon Press.
[edit] External links
Wikimedia Commons has media related to:
Down syndrome
For comprehensive lists of Down syndrome links see
Directory of Down Syndrome Internet Sites (US based, but contains international links)
UK resources for Down's syndrome
[edit] Societies and associations
Down Syndrome International
The Down Syndrome Educational Trust
By country
Canadian Down Syndrome Society (Canada)
Down Syndrome Research Foundation (Canada)
Down's Syndrome Scotland (Scotland)
Down's Syndrome Association UK (Not including Scotland)
Down's Syndrome Research Foundation (UK)
National Down Syndrome Society (USA)
National Down Syndrome Congress (USA)
International Mosaic Down Syndrome Association (USA)
Down's Heart Group (heart conditions related to Down's Syndrome)
[edit] Conferences
9th World Down Syndrome Congress (2006, Vancouver)
Bright Beginnings Conference for Parents and Professionals (2006, London) -- Report
Retrieved from "http://en.wikipedia.org/wiki/Down_syndrome"
Categories: Articles with unsourced statements | Genetic disorders | Disability | Eponymous diseases | Pediatrics | Syndromes
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Matanitu Tu-Vaka-i-koya ko Viti
फ़िजी فِجی
Republic of the Fiji Islands
Flag Coat of arms
Motto: Rerevaka na Kalou ka Doka na Tui
(English: Fear God and honour the Queen)
Anthem: God Bless Fiji
Capital
(and largest city) Suva
18°10′S 178°27′E
Official languages English, Bau Fijian, and Hindustani (Hindi/Urdu)
Government Republic
- President Ratu Josefa Iloilo
- Prime Minister Laisenia Qarase
- GCC Chairman Ratu Ovini Bokini
- Great Chief Queen Elizabeth II1
Independence from UK
- Date 10 October 1970
Area
- Total 18,274 km² (155th)
7,056 sq mi
- Water (%) negligible
Population
- July 2006 estimate 905,949 (156th)
- Density 46/km² (148th)
119/sq mi
GDP (PPP) 2005 estimate
- Total $5.447 billion (149th)
- Per capita $6,375 (93rd)
HDI (2006) 0.752 (medium) (90th)
Currency Fijian dollar (FJD)
Time zone (UTC+12)
Internet TLD .fj
Calling code +679
1 recognised by the Great Council of Chiefs.
Fiji (Fijian: Viti; Hindustānī: फ़िजी فِجی), officially the Republic of the Fiji Islands, is an island nation in the South Pacific Ocean east of Vanuatu, west of Tonga and south of Tuvalu. The country occupies an archipelago of about 322 islands, of which 106 are permanently inhabited, and 522 islets. The two major islands, Viti Levu and Vanua Levu, account for 87% of the population. The name Fiji is the old Tongan word for the islands, which in turn is derived from the Fijian name Viti.
Contents [hide]
1 History
2 Politics
3 Administrative divisions
4 Geography
5 Economy
6 Demographics
6.1 Ethnic groups
6.2 Languages
6.3 Religion
7 Sport
8 Culture
9 Miscellaneous topics
10 External links
[edit] History
Levuka, 1842.Main article: History of Fiji
The first inhabitants of Fiji arrived from South East Asia long before contact with European explorers in the 17th century. Pottery excavated from Fijian towns show that Fiji was settled before or around 1000 BC. This question of Pacific migration still lingers.[1] The Dutch explorer Abel Tasman visited Fiji in 1643 while looking for the Great Southern Continent.[2] It was not until the 19th century, however, that Europeans settled the islands permanently.[3] The islands came under British control as a colony in 1874, and the British brought over Indian contract laborers. It was granted independence in 1970. Democratic rule was interrupted by two military coups in 1987 because the government was perceived as dominated by the Indo-Fijian (Indian) community. The second 1987 coup saw the British Monarchy and the Governor General replaced by a non-executive President, and the country changed the long form of its name from Dominion of Fiji to Republic of Fiji (and to Republic of the Fiji Islands in 1997). The coups contributed to heavy Indian emigration; the population loss resulted in economic difficulties but ensured that Melanesians became the majority.
For a country of its size, Fiji has exceptionally capable armed forces, and has been a major contributor to UN peacekeeping missions in various parts of the world.
[edit] Politics
Main articles on politics and government of Fiji can be found at the Politics and government of Fiji series.
The Politics of Fiji take place in a framework of a parliamentary representative democratic republic, whereby the Prime Minister of Fiji is the head of government, and of a pluriform multi-party system. Executive power is exercised by the government. Legislative power is vested in both the government and the Parliament of Fiji. The Judiciary is independent of the executive and the legislature.
[edit] Administrative divisions
Main article: Local government of Fiji
Fiji is divided into four parts, called divisions (capitals in parentheses):
Central Division (Suva)
Northern Division (Labasa)
Eastern Division (Levuka)
Western Division (Lautoka)
These divisions are further subdivided into fourteen provinces. Additionally, the island of Rotuma, north of the main archipelago, has the status of a dependency. It is officially included in the Eastern Division for statistical purposes, but administratively has a degree of internal autonomy.
Municipal governments, with City and Town Councils presided over by Mayors, have been established in Suva, Lautoka, and ten other towns.
[edit] Geography
Map of FijiMain article: Geography of Fiji
Fiji consists of 322 islands, of which 110 are inhabited, and 522 smaller islets. The two most important islands are Viti Levu and Vanua Levu. The islands are mountainous, with peaks up to 1,300 metres (4,250 ft), and covered with tropical forests. Viti Levu hosts the capital city of Suva, and is home to nearly three quarters of the population. Other important towns include Nadi (the location of the international airport), and Lautoka. The main towns on Vanua Levu are Labasa and Savusavu. Other islands and island groups include Taveuni and Kadavu (the third and fourth largest islands respectively), the Mamanuca Group (just outside Nadi) and Yasawa Group, which are popular tourist destinations, the Lomaiviti Group, outside of Suva, and the remote Lau Group. Rotuma, some 500 kilometres (310 mi) north of the archipelago, has a special administrative status in Fiji.
[edit] Economy
Main article: Economy of Fiji
Fiji, endowed with forest, mineral, and fish resources, is one of the more developed of the Pacific island economies, though still with a large subsistence sector. Fiji experienced a period of rapid growth in the 1960s and 70s but stagnated in the early 1980s. The coups of 1987 caused further contraction. Economic liberalisation in the years following the coup created a boom in the garment industry and a steady growth rate despite growing uncertainty of land tenure in the sugar industry. The expiration of leases for sugar cane farmers (along with reduced farm and factory efficiency) has led to a decline in sugar production despite a subsidised price. Subsidies for sugar have been provided by the EU and Fiji has been the second largest beneficiary after Mauritius.
Urbanization and expansion in the service sector have contributed to recent GDP growth. Sugar exports and a rapidly growing tourist industry — with 430,800 tourists in 2003 and increasing in the subsequent years — are the major sources of foreign exchange. Sugar processing makes up one-third of industrial activity. Long-term problems include low investment and uncertain property rights. The political turmoil in Fiji has had a severe impact on the economy, which shrank by 2.8% in 2000 and grew by only 1% in 2001. The tourism sector recovered quickly, however, with visitor arrivals reaching pre-coup levels again during 2002, which has since resulted in a modest economic recovery. This recovery continued into 2004 but grew by 1.7% in 2005 and is projected to grow by 2.0% in 2006. Although inflation is low, the policy indicator rate of the Reserve Bank of Fiji was raised by 1% to 3.25% in February 2006 due to fears of excessive consumption financed by debt. Lower interest rates have so far not produced greater investment for exports. However, there has been a housing boom from declining commercial mortgage rates.
Until recently, the tallest building in Fiji was the 14-story Reserve Bank of Fiji Building in Suva, which opened in 1984. As of November 2005, the 17 story Suva Central commercial centre is now the tallest building in Fiji.
[edit] Demographics
Main article: Demographics of Fiji
[edit] Ethnic groups
The population of Fiji is mostly made up of native Fijians, a people of mixed Polynesian (partly Tongan) and Melanesian ancestry (54.3%), and Indo-Fijians (38.1%), descendants of Indian contract labourers brought to the islands by the British in the 19th century. The percentage of the population of Indian descent has declined significantly over the last two decades because of emigration.
About 1.2% are Rotuman — natives of Rotuma Island, whose culture has more in common with countries such as Tonga or Samoa than with the rest of Fiji. There are also small, but economically significant, groups of Europeans, Chinese and other minorities.
Relationships between ethnic Fijians and Indo-Fijians have often been strained, and the tension between the two communities has dominated politics in the islands for the past generation. The level of tension varies between different regions of the country.
[edit] Languages
Three official languages are prescribed by the constitution: English, which was introduced by the former British colonial rulers, Bau Fijian, spoken by ethnic Fijians, and Hindustani, the main language spoken by Indo-Fijians. Citizens of Fiji have the constitutional right to communicate with any government agency in any of the official languages, with an interpreter to be supplied on request.
The use of English is one of the more enduring legacies of almost a century of British rule. Widely spoken by both ethnic Fijians and Indo-Fijians, English is the main medium of communication between the two communities, as well as with the outside world. It is the language in which the government conducts most of its business, and is the main language of education, commerce, and the courts.
Fijian belongs to the Austronesian family of languages. Fijian proper is closely related to the Polynesian languages, such as Tongan. There are many dialects, but the official standard is the speech of Bau, the most politically and militarily powerful of the many indigenous kingdoms of the 19th Century.
"Hindustani" is considered an umbrella term in India for the standard languages Hindi (preferred by Hindus) and Urdu (preferred by Muslims), as well as many closely related tongues that are sometimes considered separate languages. A patois known as Fiji Hindi descends from one of the eastern forms of Hindustani, called Awadhi and has synthesized a number of North Indian languages and adopted a considerable number of loanwords from English and Fijian. It has developed some unique features that differentiate it from the Awadhi spoken on the Indian subcontinent, although not to the extent of hindering mutual understanding. It is spoken by nearly the entire Indo-Fijian community regardless of ancestry, except for a few elders.
In addition to the three official languages, several other languages are spoken. On the island of Rotuma, Rotuman is used; this is more closely related to the Polynesian languages than to Fijian. Some Fijian dialects, especially in the west of the country, differ markedly from the official Bau standard, and would be considered separate languages if they had a codified grammar or a literary tradition. Among the Indo-Fijian community, there a proportion of Gujarati speaking and Punjabi speaking communities, and a few older Indo-Fijians still speak Telugu and Tamil, with smaller numbers of Bihari, Bengali, and others.
In the Fijian alphabet, some of the letters have unusual values. For one, the "c" is a voiced "th" sound, [ð]. (For example, the name of Fiji-born New Zealand rugby player Joe Rokocoko is often mis-pronounced. The correct pronunciation is IPA: [rɒkɒˈðɒkɒ].) Another difference is that the letters "b" and "d" are always pronounced with a nasal before them, [mb, nd], even at the beginning of a word. The "q" is pronounced like a "g" with a nasal "ng" before it, [ŋg] as in the word "finger", while the "g" is pronounced like the "ng" of the word "singer", [ŋ].
[edit] Religion
Religion is one of the faultlines between indigenous Fijians and Indo-Fijians, with the former overwhelmingly Christian (99.2% at the 1996 census), and the latter mostly Hindu (76.7%) and Muslim (15.9%).
The largest Christian denomination is the Methodist Church of Fiji and Rotuma. With 36.2% of the total population (including almost two-thirds of ethnic Fijians), its share of the population is higher in Fiji than in any other nation. Roman Catholics (8.9%), the Assemblies of God (4%), and Seventh-day Adventists (2.9%) are also significant. These and other denominations also have small numbers of Indo-Fijian members; Christians of all kinds comprise 6.1% of the Indo-Fijian population. Much major Roman Catholic missionary activity was conducted through the Vicariate Apostolic of Fiji.
Hindus belong mostly to the Sanatan sect (74.3% of all Hindus) or else are unspecified (22%). The small Arya Samaj sect claims the membership of some 3.7% of all Hindus in Fiji. Muslims are mostly Sunni (59.7%) or unspecified (36.7%), with an Ahmadiya minority (3.6%) regarded as heretical by more orthodox Muslims.
The Sikh religion comprises 0.9% of the Indo-Fijian population, or 0.4% of the national population in Fiji. Their ancestors came from the Punjab region of India.
[edit] Sport
The national sport of Fiji is considered to be rugby union (see Rugby union in Fiji). The national team is quite successful, and has competed at four Rugby World Cups, the first being in 1987, which has also thus far been their best result, reaching the quarter finals. Fiji also competes in the Pacific Tri-Nations and the Pacific Nations Cup. The sport is governed by the Fiji Rugby Union which is a member of the Pacific Islands Rugby Alliance, and contributes to the Pacific Islanders rugby union team. At club level there is the Colonial Cup and the Pacific Rugby Cup. The Fiji sevens team is also one of the most successful rugby 7s teams in the world.
Rugby league is also played in Fiji (see Rugby league in Fiji).
Vijay Singh, one of the best golfers in the world, is from Fiji.
[edit] Culture
Main article: Culture of Fiji
See also: Music of Fiji and Festivals in Fiji
[edit] Miscellaneous topics
List of Fiji-related topics
List of Fijians
Communications in Fiji
Transportation in Fiji
Military of Fiji
Foreign relations of Fiji
Fiji Meteorological Service
[edit] External links
Find more information on Fiji by searching Wikipedia's sister projects:
Dictionary definitions from Wiktionary
Textbooks from Wikibooks
Quotations from Wikiquote
Source texts from Wikisource
Images and media from Commons
News stories from Wikinews
Learning resources from Wikiversity
WikiMapia has one or more wiki satellite maps of Fiji.
* Fiji travel guide from Wikitravel
Official Website of the Government of Fiji
Official Website of the Parliament of Fiji
Bulafiji.com. Official Fiji Visitors Bureau website
The Fiji Times - Fiji News, Sport and Weather from Fiji's leading newspaper
Open Directory Project - Fiji directory category
The CIA World Factbook: Fiji
Interactive maps of Fiji
Map of Fiji
Finding Fiji
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