pituitary dwarfism

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Pituitary Dwarfism

 

Definition

Dwarfism is a condition in which the growth of the individual is very slow or delayed. There are many forms of dwarfism. The word pituitary is in reference to the pituitary gland in the body. This gland regulates certain chemicals (hormones) in the body. Therefore, pituitary dwarfism is decreased bodily growth due to hormonal problems. The end result is a proportionate little person, because the height as well as the growth of all other structures of the individual are decreased.

Description

Pituitary dwarfism is caused by problems arising in the pituitary gland. The pituitary gland is also called the hypophysis. The pituitary gland is divided into two halves: the anterior (front) and posterior (back) halves. The anterior half produces six hormones: growth hormone, adrenocorticotropin (corticotropin), thyroid stimulating homone (thyrotropin), prolactin, follicle stimulating hormone, and lutenizing hormone. The posterior pituitary gland only produces two hormones. It produces antidiuretic hormone (vasopressin) and oxytocin.
Most forms of dwarfism are a result of decreased production of hormones from the anterior half of the pituitary gland. The most common form is due to decreases of growth hormone which will be discussed here. These decreases during childhood cause the individual's arms, legs, and other structures to develop normal proportions for their bodies, but at a decreased rate.
When all of the hormones of the anterior pituitary gland are not produced, this is called panhypopituitarism. Another type of dwarfism occurs when only the growth hormone is decreased. Dwarfism can also result from a lack of somatomedin C (also called insulin like growth factor, IGF-1) production. Somatomedin C is a hormone produced in the liver that increases bone growth when growth hormone is present. The African pygmy and the Levi-Lorain dwarfs lack the ability to produce somatomedin C in response to growth hormone. All causes of dwarfism lead to a proportionate little person.
Growth is the body's response to different hormones. The forebrain contains a small organ called the hypothalamus, which is responsible for releasing hormones in response to the body's needs for purposes of regulation. Growth hormone is produced in the anterior pituitary gland when growth hormone-releasing hormone (GHRH), is released by the hypothalamus. Growth hormone is then released and stimulates the liver to produce IGF-1. In return, IGF-1 stimulates the long bones to grow in length. Thus, growth can be slowed down or stopped if there is a problem making any of these hormones or if there is a problem with the cells receiving these hormones.
Some estimates show that there are between 10,000 and 15,000 children in the United States who have growth problems due to a deficiency of growth hormone.

Causes and symptoms

Pituitary dwarfism has been shown to run in families. New investigations are underway to determine the specific cause and location of the gene responsible for dwarfism. The human cell contains 46 chromosomes arranged in 23 pairs. Most of the genes in the two chromosomes of each pair are identical or almost identical with each other. However, with dwarfism, there appears to be disruption on different areas of chromosome 3 and 7. Some studies have isolated defects for the production of pituitary hormones to the short arm (the "p" end) of chromosome 3 at a specific location of 3p11. Other studies have found changes on the short arm of chromosome 7.
A child with a growth hormone deficiency is often small with an immature face and chubby body build. The child's growth will slow down and not follow the normal growth curve patterns. In cases of tumor, most commonly craniopharyngioma (a tumor near the pituitary gland), children and adolescents may present with neurological symptoms such as headaches, vomiting, and problems with vision. The patient may also have symptoms of double vision. Symptoms such as truly bizarre and excessive drinking behaviors (polydipsia) and sleep disturbances may be common.

Diagnosis

The primary symptom of pituitary dwarfism is lack of height. Therefore, a change in the individual's growth habits will help lead to a diagnosis. Another diagnostic technique uses an x ray of the child's hand to determine the child's bone age by comparing this to the child's actual chronological age. The bone age in
Pituitary dwarfism is a condition of growth retardation characterized by patients who are very short but have normal body proportions. It is caused by a dysfunction of the pituitary gland, the pea-sized mass of tissue located at the base of the brain.
Pituitary dwarfism is a condition of growth retardation characterized by patients who are very short but have normal body proportions. It is caused by a dysfunction of the pituitary gland, the pea-sized mass of tissue located at the base of the brain.
(Illustration by Electronic Illustrators Group.)
affected children is usually two years or more behind the chronological age. This means that if a child is ten years old, his or her bones will look like they are those of an eight-year-old child. The levels of growth hormone and somatomedin C must also be measured with blood tests.
Hypopituitarism may be gained or acquired following birth for several reasons. It could be due to trauma to the pituitary gland such as a fall or following surgery to the brain for removal of a tumor. It may also be due to the child's environment (deprivational dwarfism).
On examination by the doctor there may be optic nerve atrophy, if the dwarfism is due to a type of tumor. X rays of the area where the pituitary gland is located (sella turcica) or more advanced imaging such as magnetic resonance imaging (MRI) or computed tomography CT may show changes of the pituitary gland itself. Computed tomography, is an advanced form of x ray that will help determine the integrity of the bone and how much calcification the tumor is producing. Magnetic resonance imaging, will also help in the diagnosis. MRI is a type of imaging device that can visualize soft tissues such as muscle and fat.
If the dwarfism is due to environmental and emotional problems, the individual may be hospitalization to monitor hormone levels. Following a few days of hospitalized, hormone levels may become normal due to avoidance of the original environment.

Treatment

The main course of therapy is growth hormone replacement therapy when there is lack of growth hormone in the body. A pediatric endocrinologist, a doctor specializing in the hormones of children, usually administers this type of therapy before a child's growth plates have fused or joined together. Once the growth plates have fused, GH replacement therapy is rarely effective.
Growth hormone used to be collected from recently deceased humans. However, frequent disease complications resulting from human growth hormone collected from deceased bodies, lead to the banning of this method. In the mid-1980s, techniques were discovered that could produce growth hormones in the lab. Now, the only growth hormone used for treatment is that made in a laboratory.
A careful balancing of all of the hormones produced by the pituitary gland is necessary for patients with panhypopituitarism. This form of dwarfism is very difficult to manage.

Prognosis

The prognosis for each type of dwarfism varies. A panhypopituitarism dwarf does not pass through the initial onset of adult sexual development (puberty) and never produces enough gonadotropic hormones to develop adult sexual function. These individuals also have several other medical conditions. Dwarfism due to only growth hormone deficiency has a different prognosis. These individuals do pass through puberty and mature sexually, however, they remain proportionately small in stature.
If the individual is lacking only growth hormone then growth hormone replacement therapy can be administered. The success of treatment with growth hormone varies however. An increase in height of 4-6 in (10-15 cm) can occur in the first year of treatment. Following this first year, the response to the hormone is not as successful. Therefore the amount of growth hormone administered must be tripled to maintain this rate. Long-term use is considered successful if the individual grows at least 0.75 in (2 cm) per year more than they would without the hormone. However, if the growth hormone treatment is not administered before the long bones—such as the legs and arms—fuse, then the individual will never grow. This fusion is completed by adult age.
Improvement for individuals with dwarfism due to other causes such as a tumor, varies greatly. If the dwarfism is due to deprevational causes, then removing a child from that environment should help to alleviate the problem.

Key terms

Adrenocorticotropin (corticotrophin) — A hormone that acts on cells of the adrenal cortex, causing them to produce male sex hormones and hormones that control water and mineral balance in the body.
Antidiuretic hormone (vasopressin) — A hormone that acts on the kidneys to regulate water balance.
Craniopharyngioma — A tumor near the pituitary gland in the craniopharyngeal canal that often results in intracranial pressure.
Deprivational dwarfism — A condition where emotional disturbances are associated with growth failure and abnormalities of pituitary function.
Follicle-stimulating hormone (FSH) — A hormone that in females stimulates estrogen and in males stimulates sperm production.
Growth hormone — A hormone that eventually stimulates growth. Also called somatotropin.
Hormone — A chemical messenger produced by the body that is involved in regulating specific bodily functions such as growth, development, and reproduction.
Luteinizing hormone — A hormone secreted by the pituitary gland that regulates the menstrual cycle and triggers ovulation in females. In males it stimulates the testes to produce testosterone.
Oxytocin — A hormone that stimulates the uterus to contract during child birth and the breasts to release milk.
Panhypopituitarism — Generalized decrease of all of the anterior pituitary hormones.
Prolactin — A hormone that helps the breast prepare for milk production during pregnancy.
Puberty — Point in development when the gonads begin to function and secondary sexual characteristics begin to appear.
Thyroid stimulating hormone (thyrotropin) — A hormone that stimulates the thyroid gland to produce hormones that regulate metabolism.

Resources

Books

Beers, Mark H., Robert Berkow, and Mark Burs. "Pituitary Dwarfism." In MerckManual. Rahway, NJ: Merck & Co., Inc., 2004.

Organizations

Human Growth Foundation. 997 Glen Cove Ave., Glen Head, NY 11545. (800) 451-6434. Fax: (516) 671-4055. http://www.hgfound.org.
Little People of America, Inc. National Headquarters, PO Box 745, Lubbock, TX 79408. (806) 737-8186 or (888) LPA-2001. lpadatabase@juno.com. http://www.lpaonline.org.

Other

"Clinical Growth Charts by the National Center for Health Statistics." Center for Disease Control. http://www.cdc.gov/nchs/about/major/nhanes/growthcharts/clinical_charts.htm.
"Entry 312000: Panhypopituitarism; PHP." OMIM—Online Mendelian Inhericance in Man. National Institutes of Health. http://www.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?312000.
Hill, Mark. "Development of the Endocrine System—Pituitary." The University of New South Wales, Sydney, Australia—Department of Embryology. 〈http://anato-my.med.unsw.edu.au/CBL/Embryo/OMIMfind/endocrine/pitlist.htm〉.

dwarfism

 [dwor´fizm]
underdevelopment of the body; the state of being a dwarf. It may be the result of a developmental anomaly, of nutritional or hormone deficiencies, or of other diseases. The size of pygmies found in some parts of the world, such as the Philippines and equatorial Africa, is not the result of dwarfism; their small stature is a hereditary trait. Called also nanism and nanosomia.

A dwarf in adulthood may be as small as 75 cm (30 inches) tall. The proportions of body to head and limbs may be normal or abnormal. In certain conditions the body may be deformed or the person may suffer from mental retardation.

achondroplasia is a developmental anomaly that affects the growth of the bones. The person's trunk is usually normal, but the head is unusually large and the limbs unusually small. Most fetuses with achondroplastic dwarfism are stillborn. Those who reach adulthood do not suffer lessening of their mental or sexual abilities, and may have unusual muscular strength. The condition does not significantly shorten the life span.

An infant who suffers from an insufficiency of thyroxine, a hormone secreted by the thyroid gland, may develop the symptoms of cretinism, including an enlarged head, short limbs, puffy eyes, a thick and protruding tongue, dry skin, and lack of coordination. This can be treated by giving the patient an extract of thyroxine; early treatment can result in normal growth and development. If the condition is not treated, however, the child will grow up dwarfed, mentally retarded, and sexually sterile.

Pituitary dwarfism occurs when the pituitary gland does not produce enough growth hormone. This hormone plays a major role in growth of the skeleton and viscera; if it is not produced in large enough quantities, growth of the trunk will be curtailed, and the head and limbs will be in normal proportion to the small torso. Administration of purified human growth hormone has been shown to induce skeletal growth in these patients.
achondroplastic dwarfism dwarfism due to achondroplasia; see dwarfism.
pituitary dwarfism dwarfism due to inadequate secretion of growth hormone by the pituitary gland; see dwarfism.
renal dwarfism dwarfism caused by renal failure.
rhizomelic dwarfism the autosomal recessive form of chondrodysplasia punctata.

pi·tu·i·tar·y dwarf·ism

a rare form of dwarfism caused by the absence of a functional anterior pituitary gland; may be present at birth or develop during early childhood.

pi·tu·i·tar·y dwarf·ism

(pi-tū'i-tar-ē dwōrf'izm)
A rare form of dwarfism caused by the absence of a functional anterior pituitary gland; may be present at birth or develop during early childhood.

pituitary dwarfism

Stunted growth due to deficiency of pituitary growth hormone during the early years of life. This is now seldom allowed to occur as treatment with growth hormone is readily available.

Lorain,

Paul, French physician, 1827-1875.
Lorain disease - dwarfism generally associated with hypogonadism. Synonym(s): idiopathic infantilism
Lorain-Lévi dwarfism - a rare form of dwarfism caused by the absence of a functional anterior pituitary gland. Synonym(s): Lorain-Lévi infantilism; Lorain-Lévi syndrome; pituitary dwarfism
Lorain-Lévi infantilism - Synonym(s): Lorain-Lévi dwarfism
Lorain-Lévi syndrome - Synonym(s): Lorain-Lévi dwarfism
pituitary dwarfism rare cause of very short stature due to lack of pituitary growth hormone; subjects' trunk and limbs are of normal proportion, but there is overall growth failure

dwarfism

the state of being a dwarf; underdevelopment of the body. Dwarfism may be the result of a developmental anomaly, of nutritional or hormone deficiencies, or of other diseases. See also achondroplasia, cretinism.

achondroplastic dwarfism
an inherited defect in cattle caused by defective cartilage growth which is effectively lethal because the calves do not grow well and die before 6 months of age. Typical signs are short legs, large, wide, short head, protruding lower jaw, depression of the maxilla with obstruction of respiration and stertorous breathing. The tongue protrudes and the eyes bulge, the abdomen is distended and there is chronic bloat. Urine levels of glycosaminoglycans are much higher than normal in some of the calves. Called also snorter dwarfs. The condition has also been seen in dogs and cats.
chondrodystrophic dwarfism with hydrocephalus
constitutional dwarfism
a proportional dwarfism due to a generalized genetic defect.
disproportionate dwarfism
the skeleton is dystrophic, the soft tissues are normal. The animal is pot-bellied, dyspneic and the tongue protrudes. Characteristic of achondroplastic and chondrodystrophic dwarfs.
German shepherd dog dwarfism
an inherited juvenile panhypopituitarism caused by a defect in differentiation of the oropharyngeal ectoderm of Rathke's pouch. Affected puppies appear normal at birth but soon show a reduced rate of growth, retention of deciduous teeth and puppy hair, alopecia, delayed closure of epiphyseal growth plates, infantile genitalia and shortened life span.
Enlarge picture
A 6-month-old dwarf German shepherd dog.By permission from Ettinger SJ, Feldman E, Textbook ofVeterinary Internal Medicine, Saunders, 2004
pituitary dwarfism
see German shepherd dog dwarfism (above).
primordial dwarfism
general proportional dwarfism of all organs of the kind that has produced Kerry cattle and Miniature pinschers.
proportional dwarfism
primordial dwarfism (above).
thyroid dwarfism
hypothyroidism in an immature animal causes retarded growth and development of bones with disproportionate dwarfism. See cretinism.

pituitary

1. a neuroepithelial endocrine gland of dual origin at the base of the brain in the sella turcica, attached by a stalk to the hypothalamus; called also hypophysis. It is composed of two main lobes, the anterior lobe (anterior pituitary, adenohypophysis), secreting several important hormones that regulate the proper functioning of the thyroids, gonads, adrenal cortex, and other endocrine glands, and the posterior lobe (posterior pituitary, neurohypophysis) whose cells serve as a reservoir for hormones having antidiuretic and oxytocic action, releasing them as needed, and in response to signals from the hypothalamus, itself responding to incoming signals from the nervous system received by the thalamus. Called also hypophysis. See also pituitrin, vasopressin, oxytocin, antidiuretic hormone, thyrotropin releasing hormone, adrenocorticotropic hormone, follicle-stimulating hormone, luteinizing hormone, melanocyte-stimulating hormone, prolactin and growth hormone.
2. pertaining to the pituitary gland.
3. a preparation of the pituitary glands of animals, used therapeutically.

pituitary abscess
abscess in the rete mirabile of the pituitary is recognizable clinically in cattle. The syndrome begins with a characteristic inability to close the mouth. Saliva drools, the tongue is prolapsed slightly, and there may be blindness, opisthotonos, loss of balance and recumbency.
pituitary-adrenal axis
the interactions between hypothalamus, pituitary and adrenal cortex, involving releasing factors, tropic hormones and negative feedback mechanisms.
pituitary alopecia
see growth hormone-responsive dermatosis.
pituitary cachexia
see pituitary cachexia.
pituitary dwarfism
congenital dwarfism with all parts properly proportioned. Affected animals are miniatures of normals. There is delayed bone development and epiphyseal fusion is retarded. It is inherited in cattle and German shepherd dogs. See also German shepherd dog dwarfism.
pituitary giantism
acromegaly.
fetal pituitary hormones
in sheep, cows and goats fetal pituitary ACTH stimulates fetal adrenal cortisol production inducing in turn placental estrogen secretion. Hence fetal placental hormone is important in the induction of parturition.
pituitary hypoplasia
congenital absence (aplasia) or incomplete growth (hypoplasia) of gland. Occurs in one form of inherited prolonged gestation in cattle and in poisoning by the weed Salsola tuberculata var. tomentosa. It is an inherited trait in German shepherd dogs.
posterior pituitary
1. the posterior lobe of the pituitary gland; the neurohypophysis.
2. a preparation of animal posterior pituitary having the pharmacological actions of its hormones, oxytocin and vasopressin; used mainly as an antidiuretic in the treatment of diabetes insipidus and as a vasoconstrictor.
pituitary rete mirabile abscess
see pituitary abscess.
pituitary tumor
includes adenoma, carcinoma and craniopharyngioma. All cause pressure on surrounding tissue and some cause endocrinological disturbances.
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