Dwarfism

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Dwarfism refers to a condition of extreme small size of a person, animal, or plant. Any type of marked human smallness could be termed dwarfism in older popular and medical usage. Currently, in both general and technical usage, the term as related to human beings (the major subject of this article) is usually restricted to those forms of extreme shortness characterized by disproportion of body parts, typically due to an inheritable disorder of bone development.

[edit] Causes

Most forms of human dwarfism are caused by genetic disorders of cartilage or bone development. Many terms have been used in the medical literature for these conditions, including bone dysplasia, osseous dysplasia, chondrodystrophy, and osteochondrodystrophy (and other variations of those roots), and hundreds of individual forms have been described. Most involve defects of genes involved in cartilage or bone formation or growth, but some result from distortion of bone development by processes involving other systems of the body.

The most recognizable common form of dwarfism is achondroplasia, which produces markedly short limbs, increased spinal curvature, and distortion of skull growth. It accounts for 70% of dwarfism cases. Other relatively common types include spondyloepiphyseal dysplasia congenita (SED), diastrophic dysplasia, pseudoachondroplasia, hypochondroplasia, and osteogenesis imperfecta (OI). Severe shortness with skeletal distortion also occurs in several of the mucopolysaccharidoses and other storage diseases.

In contrast, severe proportional shortness is more often a result of deficiency of growth hormone deficiency or insulin-like growth factor-1, or due to malnutrition, or any of many other chronic diseases. "Pituitary dwarfism" is an old term for the shortness of growth hormone deficiency, but proportional shortness, even when severe, is currently rarely referred to as "dwarfism".^[1]

[edit] Diagnosis

Unusually short stature for a child's age is usually what brings the child to medical attention. Skeletal dysplasia ("dwarfism") is usually suspected because of obvious physical features (e.g., unusual configuration of face or shape of skull), because of an obviously affected parent, or because body measurements (arm span, upper to lower segment ratio) indicate disproportion. Bone x-rays are often the key to diagnosis of a specific skeletal dysplasia, but they are not the key diagnosis. Most children with suspected skeletal dysplasias will be referred to a genetics clinic for diagnostic confirmation and genetic counselling. (See External links, below, for a list of American referral centers with special expertise in skeletal dysplasias.) In the last decade, genetic tests for some of the specific disorders have become available.

During the initial medical evaluation for shortness, the absence of disproportion and the other clues above usually indicates other causes than bone dysplasias. Extreme shortness with completely normal proportions sometimes indicates growth hormone deficiency (pituitary dwarfism).

Short stature alone, in the absence of any other abnormalities, may simply be genetic, particularly if a person is born into a family of people who are relatively short.

[edit] Problems associated with dwarfism

The principal adverse effects of dwarfism can be divided into the physical and the social.

Physical effects of malformed bones vary according to the specific disease. Many involve pain resulting from joint damage from abnormal bone alignment, or from nerve compression (e.g, spinal stenosis).^[1]. Early degenerative joint disease, exaggerated lordosis or scoliosis, and constriction of spinal cord or nerve roots can cause pain and disability. Reduced thoracic size 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 disabling than the abnormal bone growth.

The psychosocial disadvantages may be more distressing 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.
• Numerous studies have demonstrated reduced employment opportunities. Severe shortness is associated with lower income.
• Self-esteem may be reduced and family relationships affected.
• Extreme shortness (in the low 2–3 foot [60–90 cm] range) can interfere with ordinary activities of daily living, like driving or even using countertops built for taller people.

[edit] Treatment and support

As the genetic defects of most forms of dwarfism due to bone dysplasia cannot be corrected, therapeutic interventions are typically aimed at (1) preventing or reducing pain or physical disability, (2) increasing adult height, or (3) mitigating psychosocial stresses and enhancing social adaptation.

Pain and disability may be ameliorated by physical therapy, by braces or other orthotic devices, or by surgical procedures.

The only simple interventions that increase perceived adult height are dress enhancements such as shoe lifts or hairstyle. Growth hormone is rarely used for shortness due to bone dysplasias, as the height benefit is typically small (less than 5 cm) and the cost high. The most effective means of increasing adult height by several inches is limb-lengthening surgery, though availability is limited and cost is high in terms of dollars, discomfort, and interruption of life. Most people with dwarfism do not avail themselves of this, and it remains controversial. ^[1]

[edit] Dwarfism in non-Western cultures

A number of Native American peoples believed that dwarves had special powers and often chose dwarves born among them as priests.

In the Talmud, it is said that the Egyptian Pharaoh of the Bible and the Babylonian king Nebuchadnezzar were dwarves.

[edit] In popular culture and the arts

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The Dwarf Don Sebastián de Morra, by Velázquez. In his portraits of the dwarfs of Spain's royal court, the artist preferred a serious tone that emphasized their human dignity.

When depicted in art, literature, or movies, dwarfs are rarely depicted as "regular people who are very short" but often as a species apart. Novelists, artists, and moviemakers attach special moral or aesthetic significance to the "apartness" or the misshapenness.

Artistic representations of dwarfism can be found on Greek vases and other ancient artifacts, including ancient Egyptian art. Documentation of dwarfs can also be found on European paintings and many pictures. Many European paintings (especially Spanish) of the 16th–19th centuries depict dwarfs by themselves or with others.

Several novels have treated dwarfism as a major theme, although not necessarily realistically:

• The Tin Drum (Die Blechtrommel) by Günter Grass
• Stones from the River by Ursula Hegi
• The Dwarf by Pär Lagerkvist
• A Prayer for Owen Meany by John Irving
• A Son of the Circus by John Irving
• Hop-Frog, or The Eight Chained Ourang-Outangs by Edgar Allan Poe
• Maybe the Moon by Armistead Maupin
• Swords and Crowns and Rings by Ruth Park
• Tale of the Wind by Kay Nolte Smith
• Memoir of a Dwarf in the Sun King's Court by Paul Weidner
• The Eye of Night by Pauline Alama
• Mendel's Dwarf by Simon Mawer

Leslie Fiedler's Freaks: Myths and Images of the Secret Self (1979) explored the value of differentness of "freaks" to "normal" people, lamenting medical treatment for reducing the number of picturesquely different people around.

Several 20th and 21st century movies & TV shows have addressed the topic or made much use of dwarfs:

• Freaks (1932)
• The Wizard of Oz (1939)
• Even Dwarfs Started Small (Auch Zwerge haben klein angefangen) (1970)
• Willy Wonka & the Chocolate Factory (1971)
• Time Bandits (1981)
• Willow (1988)
• Austin Powers: The Spy Who Shagged Me (1999)
• Austin Powers in Goldmember (2002)
• Monster Garage (2002-2006)
• "The Station Agent" (2003)
• Little People, Big World (2006) – Reality TV series following the daily lives of a family with two dwarf parents and one dwarf child (as well as three other children of average height)

The actor and stunt man Verne Troyer has become famous playing the character "Mini-Me" in two Austin Powers movies, as has fellow stuntman, and Jackass castmember, Wee-Man .

The 1960s television series The Wild Wild West featured a dwarf, Michael Dunn, as the recurring character Dr. Miguelito Loveless, the brilliant but insane arch-enemy of Secret Service agents James West and Artemus Gordon.

In the mid-1970's, Sid and Marty Krofft built an indoor theme park in Atlanta, Georgia called The World of Sid and Marty Krofft. This had a live stage production that was at that time the largest gathering of "little people" since the filming of The Wizard of Oz in 1937-38 as well as being the largest indoor theme park built to that time. The facility that was built to house this theme park is today the studios of CNN, the Cable News Network, and CNN Headline News.

In the 1990s, the immensely popular series Seinfeld featured a dwarf character, Mickey Abbott, in seven episodes; Mickey was played by actor Danny Woodburn. Notably, few references were made to his height, indicating an improved acceptance of little people in entertainment.

From 1999 until 2003, the popular television series The Man Show featured dwarfs in many of their segments.

In Monster Garage, Chris "Body Drop" Artiaga made his debut as a contestant in episode 'Ramp Rage', but later became parts runner for the series. In addition, there are 2 episodes featuring all-dwarf build teams.

In George R.R. Martin's A Song of Ice and Fire series, one of the main characters, Tyrion Lannister, is a dwarf. Though a brilliant and well-read man (some would say genius), he struggles with acceptance by "normal" people, who pejoratively refer to him as "the Imp," or "half-man". This is especially true of his father, Lord Tywin Lannister, who holds Tyrion in contempt, especially when compared to Tyrion's handsome, talented older brother Jaime, and Jaime's equally beautiful and talented twin sister, Cersei. Tyrion often wonders if any woman could ever truly love him in spite of his condition.

[edit] See also

• Achondroplasia
• Chondrodystrophy
• List of people with dwarfism
• Primordial dwarfism
• Psychogenic dwarfism
• Gigantism
• Insular dwarfism

[edit] References

[edit] External links

• Little People of America
• Restricted Growth Association UK
• www.shortsupport.org for a variety of information related to short stature, and a somewhat cautionary view of limb lengthening surgery.
• Little People: Learning to See the World Through My Daughter's Eyes, a book by Dan Kennedy
• Photo Gallery of Little People
• What is Normal Raising a child with Achondroplasia, a form of Dwarfism by Tonya Sweat