Psychosocial short stature

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Psychosocial short stature
Other namesPsychosocial dwarfism, [1] [2] psychogenic or stress dwarfism
Specialty Endocrinology

Psychosocial short stature (PSS) is a growth disorder that is observed between the ages of 2 and 15, caused by extreme emotional deprivation or stress.

Contents

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, their 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.

Cause

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-released 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.

Society and culture

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. [3]

When a police raid in 1987 released the children held by an Australian group 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. [4]

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" [5] 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 is 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 is adopted by Jean Valjean and is of average height as an adult. [6]

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 from seeing white men rape his mother. [7]

In the novel Flowers in the Attic by V.C. Andrews, twins Cory and Carrie Dollanganger are locked in an attic (with their two older siblings). The stress of their grandmother's abuse and lack of attention from their mother—along with arsenic poisoning and lack of outdoor play opportunities—stunts the twins' growth. [8] Later in the series, Carrie is described as being eight years old, yet her physical appearance is that of a three-year-old. [9]

Related Research Articles

Achondroplasia is a genetic disorder with an autosomal dominant pattern of inheritance whose primary feature is dwarfism. In those with the condition, the arms and legs are short, while the torso is typically of normal length. Those affected have an average adult height of 131 centimetres for males and 123 centimetres (4 ft) for females. Other features can include an enlarged head and prominent forehead. Complications can include sleep apnea or recurrent ear infections. Achondroplasia includes short-limb skeletal dysplasia with severe combined immunodeficiency.

<span class="mw-page-title-main">Catecholamine</span> Class of chemical compounds

A catecholamine is a monoamine neurotransmitter, an organic compound that has a catechol and a side-chain amine.

<span class="mw-page-title-main">Dwarfism</span> Small size of an organism, caused by growth deficiency or genetic mutations

Dwarfism is a condition wherein an organism is exceptionally small, and mostly occurs in the animal kingdom. In humans, it is sometimes defined as an adult height of less than 147 centimetres, regardless of sex; the average adult height among people with dwarfism is 122 centimetres. Disproportionate dwarfism is characterized by either short limbs or a short torso. In cases of proportionate dwarfism, both the limbs and torso are unusually small. Intelligence is usually normal, and most have a nearly normal life expectancy. People with dwarfism can usually bear children, though there are additional risks to the mother and child depending upon the underlying condition.

<span class="mw-page-title-main">Growth hormone</span> Peptide hormone that stimulates growth, cell reproduction and cell regeneration

Growth hormone (GH) or somatotropin, also known as human growth hormone in its human form, is a peptide hormone that stimulates growth, cell reproduction, and cell regeneration in humans and other animals. It is thus important in human development. GH also stimulates production of IGF-1 and increases the concentration of glucose and free fatty acids. It is a type of mitogen which is specific only to the receptors on certain types of cells. GH is a 191-amino acid, single-chain polypeptide that is synthesized, stored and secreted by somatotropic cells within the lateral wings of the anterior pituitary gland.

<span class="mw-page-title-main">Hypothalamic–pituitary–adrenal axis</span> Set of physiological feedback interactions

The hypothalamic–pituitary–adrenal axis is a complex set of direct influences and feedback interactions among three components: the hypothalamus, the pituitary gland, and the adrenal glands. These organs and their interactions constitute the HPA axis.

<span class="mw-page-title-main">Fight-or-flight response</span> Physiological reaction to a perceived threat or harmful event

The fight-or-flight or the fight-flight-or-freeze-fawn is a physiological reaction that occurs in response to a perceived harmful event, attack, or threat to survival. It was first described by Walter Bradford Cannon. His theory states that animals react to threats with a general discharge of the sympathetic nervous system, preparing the animal for fighting or fleeing. More specifically, the adrenal medulla produces a hormonal cascade that results in the secretion of catecholamines, especially norepinephrine and epinephrine. The hormones estrogen, testosterone, and cortisol, as well as the neurotransmitters dopamine and serotonin, also affect how organisms react to stress. The hormone osteocalcin might also play a part.

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<span class="mw-page-title-main">Zvi Laron</span>

Zvi Laron is an Israeli paediatric endocrinologist. Born in Cernăuţi, Romania, Laron is a professor emeritus at Tel Aviv University. In 1966, he described the type of dwarfism later called Laron syndrome. His research opened the way to the treatment of many cases of growth hormone disorders. He was the first to introduce the multidisciplinary treatment for juvenile diabetes.

Short stature refers to a height of a human which is below typical. Whether a person is considered short depends on the context. Because of the lack of preciseness, there is often disagreement about the degree of shortness that should be called short. Dwarfism is the condition of being very short, often caused by a medical condition.

<span class="mw-page-title-main">Child abuse</span> Maltreatment or neglect of a child

Child abuse is physical, sexual, and/or psychological maltreatment or neglect of a child or children, especially by a parent or a caregiver. Child abuse may include any act or failure to act by a parent or a caregiver that results in actual or potential harm to a child and can occur in a child's home, or in the organizations, schools, or communities the child interacts with.

Chondrodystrophy refers to a skeletal disorder caused by one of myriad genetic mutations that can affect the development of cartilage. As a very general term, it is only used in the medical literature when a more precise description of the condition is unavailable.

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<span class="mw-page-title-main">Laron syndrome</span> Medical condition

Laron syndrome (LS), also known as growth hormone insensitivity or growth hormone receptor deficiency (GHRD), is an autosomal recessive disorder characterized by a lack of insulin-like growth factor 1 production in response to growth hormone. It is usually caused by inherited growth hormone receptor (GHR) mutations.

<span class="mw-page-title-main">Growth-hormone-releasing hormone receptor</span> Receptor protein that binds with somatcrinin

The growth-hormone-releasing hormone receptor (GHRHR) is a G-protein-coupled receptor that binds growth hormone-releasing hormone. The GHRHR activates a Gs protein that causes a cascade of cAMP via adenylate cyclase. GHRHR is distinct from the growth hormone secretagogue receptor, where growth hormone releasing peptides act to release growth hormone.

Robert M. Blizzard was an American pediatric endocrinologist and a founding member of the Lawson Wilkins Pediatric Endocrine Society.

Institutional abuse is the maltreatment of a person from a system of power. This can range from acts similar to home-based child abuse, such as neglect, physical and sexual abuse, and hunger, to the effects of assistance programs working below acceptable service standards, or relying on harsh or unfair ways to modify behavior. Institutional abuse occurs within emergency care facilities such as foster homes, group homes, kinship care homes, and pre-adoptive homes. Children who are placed in this type of out of home care are typically in the custody of the state. The maltreatment is usually caused by an employee of the facility.

Early childhood is a critical period in a child's life that includes ages from conception to five years old. Psychological stress is an inevitable part of life. Human beings can experience stress from an early age. Although stress is a factor for the average human being, it can be a positive or negative molding aspect in a young child's life.

Child neglect, often overlooked, is the most common form of child maltreatment. Most perpetrators of child abuse and neglect are the parents themselves. A total of 79.4% of the perpetrators of abused and neglected children are the parents of the victims, and of those 79.4% parents, 61% exclusively neglect their children. The physical, emotional, and cognitive developmental impacts from early childhood neglect can be detrimental, as the effects from the neglect can carry on into adulthood.

<span class="mw-page-title-main">Kowarski syndrome</span> Medical condition

Kowarski syndrome describes cases of growth failure, despite the presence of normal or slightly high blood growth hormone by radioimmunoassay (RIA-GH) and low serum IGF1, and who exhibit a significant increase in growth rate following recombinant GH therapy.

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. ↑ 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. Pädiatrie und Pädologie. Springer Verlag. 5 (5): 1–12. doi:10.1007/978-3-7091-8491-2_1. ISBN   978-3-211-81440-6. ISSN   0300-9556. PMID   917570.
  4. ↑ 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.
  5. ↑ Grass's own description
  6. ↑ "Les Misérables: "Cosette," Book Three: Chapter VIII". SparkNotes. Retrieved 6 June 2022.
  7. ↑ Kesey, Ken (1963). One Flew Over the Cuckoo's Nest . Signet. p.  31. ISBN   978-0451163967.
  8. ↑ "Flowers In The Attic Plot Summary" (PDF). Lady Xanax. Retrieved 28 May 2017.
  9. ↑ Andrews, V.C. (November 1990). Petals On The Wind. Pocket Books. p. 4. ISBN   0671729470.
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