Classification and external resourcesSpecialtyEndocrinology, medical geneticsICD-10E23.0, E34.2, E45.0,Q77.4ICD-9-CM253.3, 259.4DiseasesDB80MedlinePlus001176MeSHD004392
Dwarfism (UK: /ˈdwɔːfɪsəm/; US:/ˈdwɔrfɪzəm/) occurs when an individual person or animal is short in stature resulting from a medical condition caused by slow growth. In humans, dwarfism is sometimes defined as an adult height of less than 4 feet 10 inches (58 in; 147 cm).[1]Dwarfism can be caused by more than 300 distinct medical conditions,[2] such that the symptoms and characteristics of individuals with dwarfism vary greatly. Disproportionate dwarfism is characterized by one or more body parts being relatively large or small in comparison to those of an average-sized adult, with growth variations in specific areas being apparent. In cases of proportionate dwarfism, the body appears normally proportioned, but is unusually small.
There is no single treatment for dwarfism. Individual differences, such as bone growth disorders, sometimes can be treated through surgery, some hormone disorders can be treated through medication, and by hormone replacement therapy; this treatment must be done before the child's growth plates fuse. Individual accommodations, such as specialized furniture, are often used by people with dwarfism.[3] Many support groups provide services to aid individuals with dwarfism and the discrimination they may face because of their dwarfism.[4]
For people, in addition to the medical aspect of the condition, there are social and sociological aspects as well. For a person with dwarfism, heightism can lead to ridicule in childhood and discrimination in adulthood.[5][6] In theUnited States, Canada, the United Kingdom, and other English-speaking countries, some people with dwarfism prefer to be called little people.[7][8]
Historically, the term midget was used to describe "proportionate dwarfs"; however, this term is now regarded as offensive and pejorative[9] (seeTerminology). Another definition for midget is any thing (especially an animal) that is small in proportion to a typical specimen[citation needed].Hypotonia, or low muscle tone, is common in dwarfs, but intelligence and lifespan are usually normal. Defining dwarfism by height alone is problematic because short stature in itself is not a disorder. For example, pygmies have adult male heights of less than 150 cm (4 feet 11 inches) on average.[10]
Classification
Lavinia Warren, an actress with pituitary dwarfism (growth hormone deficiency).
In men and women, the sole requirement for being considered a little person is having an adult height under 147 cm (4 ft 10 in) and it is almost always classified with respect to the underlying condition that is the cause of the short stature. Dwarfism is usually caused by a genetic variant;achondroplasia is caused by a mutation on chromosome 4. If dwarfism is caused by a medical disorder, the person is referred to by the underlying diagnosed disorder. Disorders causing dwarfism are often classified by proportionality. Disproportionate dwarfism describes disorders that cause unusual proportions of the body parts, while proportionate dwarfism results in a generally uniform stunting of the body.
Disorders that cause dwarfism may be classified according to one of hundreds of names, which are usually permutations of the following roots:
locationrhizomelic = root, e.g., bones of the upper arm or thighmesomelic = middle, e.g., bones of the forearm or lower legacromelic = end, e.g., bones of hands and feet.micromelic = entire limbs are shortenedsourcechondro = of cartilageosteo = of bonespondylo = of the vertebraeplasia = formtrophy = growth
Examples include achondroplasia,osseous dysplasia, chondrodystrophy, and osteochondrodystrophy.[11]
Characteristics
A typical defining characteristic of dwarfism is an adult height of less than 147 cm (4 ft 10 in). Since those with dwarfism have such a wide range of physical characteristics, variations in individuals are identified by diagnosing and monitoring the underlying disorders.
Short stature can be inherited without any coexisting disease. Short stature in the absence of a medical condition is not generally considered dwarfism. For example, a short man and a short woman with average health will tend to produce children who are also short and with average health. While short parents tend to produce short children, persons with dwarfism may produce children of average height, if the cause of their dwarfism is not genetically transmissible or if the individual does not pass on the genetic variation.
Dwarfism occurs in animals as well as humans; horses can haveachondroplastic symptoms, as shown here next to a person with dwarfism.
Disproportionate dwarfism is characterized by one or more body parts being unusually large or small compared to the rest of the body. Inachondroplasia one's trunk is usually of average size, one's limbs being proportionately shorter, one's head usually larger, and a prominent forehead.[12] In at least one case achondroplasia resulted in a significantly smaller trunk and head.[13]Facial features are often affected and individual body parts may have problems associated with them. Orthopedic problems can result from multiple conditions such as diastrophic dysplasia and pseudoachondroplasia.
Proportionate dwarfism is marked by body parts being proportional but smaller.[14] Height is significantly below average and there may be long periods without any significant growth. Sexual development is often delayed or impaired into adulthood. Unlike disproportionate dwarfism, in some cases intellectual disability may be a part of proportionate dwarfism.
Physical maleffects of malformed bones vary according to the specific disease. Many involve joint pain caused by abnormal bone alignment, or fromnerve compression.[7] Early degenerative joint disease, exaggeratedlordosis or scoliosis, and constriction ofspinal cord or nerve roots can cause pain and disability.[15] Reduced thoracicsize can restrict lung growth and reduce pulmonary function. Some forms of dwarfism are associated with disordered function of other organs, such as the brain or liver, sometimes severely enough to be more of an impairment than the unusual bone growth.[16][17]
Mental effects also vary according to the specific underlying syndrome. In most cases of skeletal dysplasia, such as achondroplasia, mental function is not impaired.[14] However, there are syndromes which can affect the cranial structure and growth of the brain, severely impairing mental capacity. Unless the brain is directly affected by the underlying disorder, there is little to no chance of mental impairment that can be attributed to dwarfism.[18]
The psychosocial limitations of society may be more disabling than the physical symptoms, especially in childhood and adolescence, but people with dwarfism vary greatly in the degree to which social participation and emotional health are affected.
Social prejudice against extreme shortness may reduce social and marital opportunities.[19][20]
See also: heightism
Numerous studies have demonstrated reduced employment opportunities. Severe shortness is associated with lower income.[20]Self-esteem may suffer and family relationships may be affected.Extreme shortness (in the low 2–3 foot [60–90 cm] range) can, if not accommodated for, interfere withactivities of daily living, like driving or using countertops built for taller people. Other common attributes of dwarfism such as bowed knees and unusually short fingers can lead to back problems, and difficulty in walking and handling objects.Children with dwarfism are particularly vulnerable to teasing and ridicule from classmates. Because dwarfism is relatively uncommon, children may feel isolated from their peers.[16]
Causes
Diagnosis
Prevention
Management
Society and culture
Terminology
Two Norse dwarves as depicted in a 19th-century edition of the Poetic Eddapoem Völuspá (1895) by Lorenz Frølich
The appropriate term for describing a person of particularly short stature (or with the genetic condition achondroplasia) has historically been ambiguous, and has developed euphemistically over the past few centuries.
The noun dwarf stems from Old Englishdweorg, originally referring to a being from Germanic mythology—a dwarf—that dwells in mountains and in the earth, and is associated with wisdom, smithing, mining, and crafting. Theetymology of the word dwarf is contested, and scholars have proposed varying theories about the origins of the being, including that dwarfs may have originated as nature spirits or as beings associated with death, or as a mixture of concepts. Competing etymologies include a basis in the Indo-Europeanroot *dheur- (meaning 'damage'), the Indo-European root *dhreugh (whence modern German Traum 'dream' and Trug'deception'), and comparisons have been made with the Old Indian dhvaras(a type of demonic being). The being may not have gained associations with small stature until a later period.[32]
The terms "dwarf", "little person", "LP", and "person of short stature" are now generally considered acceptable by most people affected by these disorders.[12] However, the plural "dwarfs" as opposed to "dwarves" is generally preferred in the context of the medical condition, probably due to the fact that the plural "dwarves" was conceived of by author J.R.R. Tolkien to describe a race of characters in his The Lord of the Rings books resemblingNorse dwarves.[33]
"Midget", whose etymology indicates a "tiny biting insect",[34] came into prominence in the mid-19th century after Harriet Beecher Stowe used it in her novels Sunny Memories of Foreign Lands and Old Town Folks where she described children and an extremely short man, respectively.[12] Later some people of short stature considered the word to be offensive because it was the descriptive term applied to P. T. Barnum's dwarfs used for public amusement during the freak showera.[7][35] It is also not considered accurate as it is not a medical term or diagnosis, though it is sometimes used as a slang term to describe those who are particularly short, whether or not they have dwarfism.[36]
Dwarf sports
American dwarfs are supported to compete in sport by the Dwarf Athletic Association of America. There is also the opportunity to take part in international competitions at the Dwarf Games.
Art and media depictions
See also: List of Dwarfism media depictions andList of people with dwarfism
The Dwarf Don Sebastián de Morra, by Velázquez.
In art, literature, and movies, dwarfs are rarely depicted as ordinary people who are very short but rather as a species apart. Novelists, artists, andmoviemakers may attach special moral or aesthetic significance to their "apartness" or misshapenness.
Artistic representations of dwarfism can be found on Greek vases and other ancient artifacts, including ancient Egyptian art in which dwarfs are likely to have been seen as a divine manifestation, with records indicating they could reach high positions in society.[37][38]
The Bhagavat Purana Hindu text devotes nine chapters to the adventures of Vamana, a dwarf avatar of Lord Vishnu.
Depictions of dwarfism are also found in European paintings and many illustrations. Many European paintings (especially Spanish) of the 16th–19th centuries depict dwarfs by themselves or with others. In the Talmud, it is said that the second born son of theEgyptian Pharaoh of the Bible was a dwarf.[39] Recent scholarship has suggested that ancient Egyptians held dwarfs in high esteem.[40] Several important mythological figures of the North American Wyandot nation are portrayed as dwarfs.[41]
As popular media have become more widespread, the number of works depicting dwarfs have increased dramatically. Some feel that dwarf characters are often depicted based on the novelty factor of their stature rather than on other personal attributes. Dwarfism is depicted in many books, films, and TV series such as Willow,Austin Powers, Gulliver's Travels byJonathan Swift,[42] The Wizard of Oz,Willy Wonka & the Chocolate Factory, A Son of the Circus, Little People Big World,The Little Couple, A Song of Ice and Fire(and its TV adaptation Game of Thrones),Seinfeld, The Orator, In Bruges, The Tin Drum by Günter Grass, the short-lived reality show The Littlest Groom, and the film The Station Agent.
The Animal Planet TV series Pit Bossfeatures dwarf actor "Shorty" Rossi and his talent agency, "Shortywood Productions", which Rossi uses to provide funding for his pit bull rescue operation, "Shorty's Rescue". Rossi's three full-time employees, featured in the series, are all little people and aspiring actors.
In September 2014 Creative Business House along with Donnons Leur Une Chance created the The National Dwarf Fashion Show to raise awareness and boost self-confidence of people living with dwarfism. [43]
See also
References
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Retrieved 2008-11-18.^ Escamilla RF, Hutchings JJ, Li CH, Forsham P (August 1966)."Achondroplastic dwarfism. Effects of treatment with human growth hormone". Calif Med 105 (2): 104–10.PMC 1516352. PMID 5946547. Retrieved 2008-11-17.^ "The Pituitary Gland & Growth Disorders: An Overview". Retrieved2009-02-22.^ Hall, Judith A.; BETTY M. ADELSON (2005). Dwarfism: medical and psychosocial aspects of profound short stature. Baltimore: Johns Hopkins University Press. ISBN 0-8018-8121-8.^ a b Gollust SE, Thompson RE, Gooding HC, Biesecker BB (August 2003). "Living with achondroplasia in an average-sized world: an assessment of quality of life". American journal of medical genetics. Part A 120A (4): 447–58. doi:10.1002/ajmg.a.20127.PMID 12884421.^ "Ancient Egypt:Kingdom of the Pharaohs", R. Hamilton, p. 47, Paragon, 2006, ISBN 1-4054-8288-5^ [1]^ FGFR3^ "Achondroplasia - Genetics Home Reference". Genetics Home Reference. National Institute of Health. 2008-09-26. 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