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Dwarfism

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Dwarfism
SpecialtyMedical genetics Edit this on Wikidata

Dwarfism is a typically pathological condition in which the physical size of a person, animal, or plant is well below normal. This article primarily discusses dwarfism in human beings.

Symptoms

The distinguishing symptom of dwarfism is abnormally and significantly shorter stature with respect to other persons in the same age cohort, and especially in adults who have reached their final heights. Many conditions that cause dwarfism also have other distinctive symptoms.

Causes

There are approximately 200 different types of dwarfism. [1]

Of the hundreds of causes of dwarfism in humans, most are genetic, and most involve single-gene variations that affect the structure or metabolism of bone, cartilage, or connective tissue. This class of disorder is referred to by physicians as skeletal dysplasia. Chondrodystrophy, chondrodysplasia, osteochondrodystrophy are also used (fairly interchangeably) in the medical literature to refer to most of these conditions. In some conditions, the bones are the primary affected body part, and the person is otherwise healthy. In others, the genetic difference affects other body systems, causing unusual features or other major problems. Achondroplasia is one of the most common and perhaps the most easily recognized skeletal dysplasia. It affects approximately 1 in 40,000 children, both males and females, as it is due to a mutation of an autosomal gene.

Some people are naturally short, because they have inherited small stature from their parents and ancestors. Apart from their unusually short stature, they are normal in every other way. Relatively few people in this category are short enough to be considered dwarfs.

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. 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.

Treatment

Treatment of most dwarfism arising from genetic defects is limited to palliation of symptoms and side effects; there is no cure.

Pituitary dwarfism can be effectively treated through administration of human growth hormone, if treatment begins early enough (before skeletal development is complete). The patient may still end up slightly shorter than average, but the inconveniences of extremely short stature can usually be avoided.

Short stature arising from normal genetic inheritance requires no treatment. The use of growth hormone to increase adult height in children who are short but otherwise normal is sometimes attempted for aesthetic or practical reasons, but remains controversial and risky. This controversy arises from the fact that there is nothing inherently bad about being extremely short statured.

Insular dwarfism

Small size is not necessarily a disadvantage. For instance, insular dwarfism is the dwarfism of species due to isolated environment, and it helps to reduce the need for food.

If Homo floresiensis, discovered in 2003, turns out to be a true species, then it will be the first known example of insular dwarfism in human beings.

Problems faced by dwarfs

  • Social and employment opportunities are greatly reduced, regardless of anti-discrimination laws. Self-esteem and family relationships are often affected, although not necessarily if the person(s) involved are emotionally healthy.
  • 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.
  • Many problems can arise from other abnormalities associated with pathological dwarfism, such as abnormal bone structure. 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.
  • Many modern causes of dwarfism are associated on rare occasions with disordered function of other organs, such as the brain or liver. These problems can be more disabling than the abnormal bone growth.

Terminology

When applied to people, the term dwarfism usually implies not only extremely short stature, but also a pathological cause for the short stature. Persons who are simply very short in the absence of pathology (short people born into a short family, for example) are not usually referred to as dwarfs. According to the Little People of America, the human definition of dwarfism is "a medical or genetic condition that usually results in an adult height of 4'10" (147 cm) or shorter, among both men and women, although in some cases a person with a dwarfing condition may be slightly taller than that."

Little person (as opposed to big person), and short-statured are politically correct terms to refer to a person with extreme, disproportionate shortness. Dwarf is sometimes perceived as having negative connotations, although the term is often used by those affected. The plural is dwarfs as in "Peter Dinklage and Wee Man are famous, handsome dwarfs." The plural term dwarves is used only for the imaginary creature. In the 19th century both dwarf and midget were ordinary medical terms referring to persons of disproportionate and proportionate shortness, respectively. Like many other older medical terms, they became primarily pejorative as they entered popular use. Midget is now considered offensive in all contexts to most little people, because of its connotation with little people being gawked at in circus/sideshows. Additionally, since proportionate short stature is usually due to pituitary dwarfism—a condition that is readily treatable—there are few people today who would qualify as midgets in developed countries.

File:Velazquez-dwarf-don-sebastian-de-morra.jpg
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.

Dwarfism is such an obvious difference that it attracts curiosity, humor, and imagination. People have paid just to look at dwarfs or to keep them around for amusement (for example, dwarf tossing). In many times and places this may be the most attractive employment option available, as was the case for some jesters of medieval Europe.

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. 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, not necessarily realistically:

Leslie Fiedler's Freaks: Myths and Images of the Secret Self (1979) is considered an intelligent and sensitive exploration of the cultural significance and artistic treatments of differentness. Other readers may feel that he valued physically unusual people for their differentness, not for their personhood, when he laments 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:

The actor and stunt man Verne Troyer has become famous playing the character "Mini-Me" in two Austin Powers movies.

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

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.

In 2006, The Learning Channel began airing Little People Big World, a reality series that profiles a family in which both parents and one of the four children have dwarfism.

Famous people with dwarfism

See also

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