Psychosocial short stature

Psychosocial short stature
Classification and external resources
Specialty endocrinology
ICD-10 E34.3

Psychosocial short stature (PSS) or psychosocial dwarfism,[1][2] sometimes called psychogenic or stress dwarfism, or Kaspar Hauser Syndrome,[3] is a growth disorder that is observed between the ages of 2 and 15, caused by extreme emotional deprivation or stress.

The symptoms include decreased growth hormone (GH) and somatomedin secretion, very short stature, weight that is inappropriate for the height, and immature skeletal age. This disease is a progressive one, and as long as the child is left in the stressing environment, his or her cognitive abilities continue to degenerate. Though rare in the population at large, it is common in feral children and in children kept in abusive, confined conditions for extended lengths of time. It can cause the body to completely stop growing but is generally considered to be temporary; regular growth will resume when the source of stress is removed.

Etiology

Children with PSS have extremely low levels of growth hormone. These children possibly have a problem with growth hormone inhibiting hormone (GHIH) or growth hormone releasing hormone (GHRH). The children could either be unresponsive to GHRH, or too sensitive to GHIH.

Children who have PSS exhibit signs of failure to thrive. Even though they appear to be receiving adequate nutrition, they do not grow and develop normally compared to other children of their age.

An environment of constant and extreme stress causes PSS. Stress releases hormones in the body such as epinephrine and norepinephrine engage what is known as the 'fight or flight' response. The heart speeds up and the body diverts resources away from processes that are not immediately important; in PSS, the production of growth hormone (GH) is thus affected. As well as lacking growth hormone, children with PSS exhibit gastrointestinal problems due to the large amounts of epinephrine and norepinephrine, resulting in their bodies lacking proper digestion of nutrients and further affecting development.

While the cure for PSS is questionable, some studies show that placing the child affected with the disease in a foster or group home increases growth rate and socialization skills.

Cases

One case was a child who was admitted to a hospital with an extremely low weight. One nurse took over his care and he began to rapidly gain weight and his growth hormone levels increased during this time. The child was so dependent on the nurse emotionally that when she left, his levels returned to what they had been when he was admitted to the hospital, and once she returned, they stabilized once more.[4][5]

When a police raid in 1987 released the children held by an Australian cult known as The Family, one twelve-year-old girl weighed under 20 kg (44 lbs) and was under 120 cm (4 ft) tall. She grew 11 cm (4 in) in the following year and her growth hormone levels returned to normal.[6]

Fictional characters

In Günter Grass's 1959 novel The Tin Drum (Die Blechtrommel), the character Oskar Matzerath "willfully stunted his growth at three feet tall as a three-year-old, although later in the novel he grows to four feet one inch" [7] in reaction to the stress he experiences – the petit-bourgeois German society.

In the novel Les Misérables by Victor Hugo, eight-year old Cosette had been abused, neglected and enslaved by the Thènardier family. The extreme abuse and neglect she faces is described as very stressful for her, and as a result she's described as simultaneously being as short as a child half her age and having an expression more appropriate for an older woman. She recovers after she's adopted by Jean Valjean and is of average height as an adult.

In One Flew Over the Cuckoo's Nest, Chief Bromden claims that one of Nurse Ratched's orderlies, Williams, a black man with dwarfism, gained his short stature as the result of seeing white men rape his mother.

References

  1. ↑ Duché DJ (2002). "[Consequences of family violence on children's health]". Bull. Acad. Natl. Med. (in French). 186 (6): 963–9; discussion 969–70. PMID 12587335.
  2. ↑ Sirotnak, Andrew P (March 19, 2008). "Child Abuse & Neglect: Psychosocial Dwarfism". eMedicine. WebMD. Retrieved January 17, 2010.
  3. ↑ Money, John (July 1992). The Kaspar Hauser Syndrome of "Psychosocial Dwarfism": Deficient Statural, Intellectual, and Social Growth Induced by Child Abuse. Prometheus Books. ISBN 978-0-87975-754-0.
  4. ↑ Sapolsky, Robert M. (1998). Why Zebras Don't Get Ulcers: An Updated Guide to Stress, Stress-Related Diseases, and Coping. New York: W.H. Freeman and Company. pp. 106–7.
  5. ↑ Saenger P, Levine LS, Wiedemann E, Schwartz E, Korth-Schutz S, Pareira J, Heinig B, New MI (1977). "Somatomedin and growth hormone in psychosocial dwarfism". Pädiatrie und Pädologie. Supplementum. Springer Verlag (5): 1–12. ISSN 0300-9556. PMID 917570.
  6. ↑ Hamilton-Byrne, S. (1995). "Hierarchies of organisation within cults" (PDF). The Skeptic. 15 (3): 25–7. Retrieved January 17, 2010. My name is Sarah Hamilton-Byrne. I grew up in a cult called 'The Family'... Cassandra, my youngest sister, was 12 years old but under 120 cm and weighed under 20 kg. She looked like a four- or five-year-old.
  7. ↑ Grass's own description
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