Dwarfism

The most recognizable and most common form of dwarfism in humans is achondroplasia, which accounts for 70% of dwarfism cases, and occurs in 4 to 15 out of 100,000 live births.[7]

It produces rhizomelic short limbs, increased spinal curvature, and distortion of skull growth. In achondroplasia the body's limbs are proportionately shorter than the trunk (abdominal area), with a larger head than average and characteristic facial features. Achondroplasia is an autosomal dominant disorder caused by the presence of an altered allele in the genome. If a pair of achondroplasia alleles are present, the result is fatal. Achondroplasia is a mutation in the fibroblast growth factor receptor 3.[25] In the context of achondroplasia, this mutation causes FGFR3 to become constitutively active, inhibiting bone growth.[26]

Growth hormone deficiency (GHD) is a medical condition in which the body produces insufficient growth hormone. Growth hormone, also called somatotropin, is a polypeptide hormone which stimulates growth and cell reproduction. If this hormone is lacking, stunted or even halted growth may become apparent. Children with this disorder may grow slowly and puberty may be delayed by several years or indefinitely. Growth hormone deficiency has no single definite cause. It can be caused by mutations of specific genes, damage to the pituitary gland, Turner's syndrome, poor nutrition,[27] or even stress (leading to psychogenic dwarfism). Laron syndrome (growth hormone insensitivity) is another cause. Those with growth hormone issues tend to be proportionate.

Metatropic means "changing form" and refers to this form of skeletal dysplasia as there is an abnormality in the growth plates.[28] Skeletal changes continue over time and may need surgical intervention to help protect the lungs.[29] Symptoms starting at birth may be mild or can be fatal.[30] There are recognizable features in individuals with this genetic disorder. Some are short stature, narrow chest, " facial features such as a prominent forehead, underdevelopment of the upper jaw, cheekbones and eye sockets (midface hypoplasia), and a squared-off jaw."[29] It is considered a more severe skeletal dysplasia, but is very rare, with the exact number of those affected unknown.[30] Prognosis is largely on a case-by-case basis depending on the severity, and life expectancy may not be impacted unless there are respiratory complications.[31]

Other causes of dwarfism are spondyloepiphyseal dysplasia congenita, diastrophic dysplasia, pseudoachondroplasia, hypochondroplasia, Noonan syndrome, primordial dwarfism, Cockayne syndrome, Kniest dysplasia, Turner syndrome, osteogenesis imperfecta (OI), and hypothyroidism. Severe shortness with skeletal distortion also occurs in several of the Mucopolysaccharidoses and other storage disorders.[32] Hypogonadotropic hypogonadism may cause proportionate, yet temporary, dwarfism. NPR2 disproportionate dwarfism was discovered recently and is caused by a mutant gene.[33]

Serious chronic illnesses may produce dwarfism as a side effect. Harsh environmental conditions, such as malnutrition, may also produce dwarfism. These types of dwarfism are indirect consequences of the generally unhealthy or malnourished condition of the individual, and not of any specific disease. The dwarfism often takes the form of simple short stature, without any deformities, thus leading to proportionate dwarfism. In societies where poor nutrition is widespread, the average height of the population may be reduced below its genetic potential by the lack of proper nutrition. Sometimes there is no definitive cause of short stature.

Lavinia Warren, an actress with pituitary dwarfism (growth hormone deficiency)

In men and women, the sole requirement for being considered a dwarf is having an adult height under 147 cm (4 ft 10 in) and it is almost always sub-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:

• location
  • rhizomelic = root, i.e., bones of the upper arm or thigh
  • mesomelic = middle, i.e., bones of the forearm or lower leg
  • acromelic = end, i.e., bones of hands and feet.
  • micromelic = entire limbs are shortened
• source
  • chondro = of cartilage
  • osteo = of bone
  • spondylo = of the vertebrae
  • plasia = form
  • trophy = growth

Examples include achondroplasia and chondrodystrophy.

Two Norse dwarfs as depicted in a 19th-century edition of the Poetic Edda poem Völuspá (1895) by Lorenz Frølich

The appropriate term for describing a person of particularly short stature (or with the genetic condition achondroplasia) has developed euphemistically.

The noun dwarf stems from Old English dweorg, 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. The etymology 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-European root *dheur- (meaning 'damage'), the Indo-European root *dhreugh (whence modern Dutch droom 'dream' and bedrog '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.[37]

The terms "dwarf", "little person", "LP", and "person of short stature" are generally considered acceptable by most people affected by these disorders.[17] However, the plural "dwarfs" as opposed to "dwarves" is generally preferred in the medical context, possibly because the plural "dwarves" was popularized by author J. R. R. Tolkien, describing a race of characters in his The Lord of the Rings books resembling Norse dwarfs.[38]

"Midget", whose etymology indicates a "tiny biting insect",[39] came into prominence in the mid-19th century after Harriet Beecher Stowe used it in her novels Sunny Memories of Foreign Lands and Oldtown Folks where she described children and an extremely short man, respectively.[17] 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 show era.[13][40] 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.[41]

Dwarfs are supported to compete in sport by a number of organisations nationally and internationally.

Dwarfs are included in some events in the Athletics at the Summer Paralympics.

The Dwarf Athletic Association of America and the Dwarf Sports Association UK provide opportunities for dwarfs to compete nationally and internationally in the Americas and Europe, respectively.

The Dwarf Sports Association UK organises between 5 and 20 events per month for athletes with restricted growth conditions in the UK.[42]

For instance, swimming and bicycling are often recommended for people with skeletal dysplasias, since those activities put minimal pressure on the spine.[43]

Since its early days, professional wrestling has had the involvement of dwarf athletes. "Midget wrestling" had its heyday in the 1950s–'70s, when wrestlers such as Little Beaver, Lord Littlebrook, and Fuzzy Cupid toured North America, and Sky Low Low was the first holder of the National Wrestling Alliance's World Midget Championship. In the following couple of decades, more wrestlers became prominent in North America, including foreign wrestlers like Japan's Little Tokyo. Although the term is seen by some as pejorative, many past and current midget wrestlers, including Hornswoggle, have said they take pride in the term due to its history in the industry and its marketability.

Ivory statuette of a woman with dwarfism, Gerzeh culture (Naqada II), Prehistoric Egypt

In art, literature, and movies, dwarfs are rarely depicted as ordinary people who are very short but rather as a species apart. Novelists, artists, and moviemakers 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.[44][45]

The Bhagavat Purana Hindu text devotes nine chapters to the adventures of Vamana, a dwarf avatar of Lord Vishnu.

The Dwarf Don Sebastián de Morra, by Velázquez

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 the Egyptian Pharaoh of the Bible was a dwarf.[46] Recent scholarship has suggested that ancient Egyptians held dwarfs in high esteem.[47] Several important mythological figures of the North American Wyandot nation are portrayed as dwarfs.[48]

As popular media have become more widespread, the number of works depicting dwarfs have increased dramatically. Dwarfism is depicted in many books, films, and TV series such as Willow, The Wild Wild West, The Man with the Golden Gun (and later parodied in Austin Powers), Gulliver's Travels by Jonathan Swift,[49] The Wizard of Oz, Willy Wonka & the Chocolate Factory, Bad Santa, 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 films The Station Agent and Zero.

The Animal Planet TV series Pit Boss features 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 International Dwarf Fashion Show to raise awareness and boost self-confidence of people living with dwarfism.[50]

A number of reality television series on Lifetime, beginning with Little Women: LA in 2014, focused on showing the lives of women living with dwarfism in various cities around the United States.