growth hormone deficiency

August 12, 2017 17:51 | Genetic Diseases

growth hormone deficiency (growth hormone deficiency) occurs when a large number of diseases and syndromes.In most cases, the disease manifests itself dwarfism syndrome (from the Greek nanos -. «Dwarf").Dwarfism is a condition characterized by a sharp delay child growth and physical development of their peers, which is associated with an absolute or relative deficiency in the body of the growth hormone.Since growth hormone is produced by the endocrine gland of the brain, which is called the pituitary gland, and the pituitary dwarfism it is.To people

dwarf growth include men who have growth below 130 cm and females -.. Less than 120 cm Smallest described in the literature dwarf growth was 38 cm pituitary dwarfism occurs with a frequency of 1 in 5000 newborns.Differences in incidence between men and women is not marked.The most common form of growth hormone deficiency is idiopathic (65-75%).It should be noted that with the introduction of the practice of MRI research and genetic improvement of research m

ethods the proportion of patients with idiopathic growth hormone deficiency is gradually reduced, as is increasingly possible to identify the specific cause of growth hormone deficiency.In addition to violations of the formation of growth hormone in the pituitary gland to the pituitary dwarfism may lead and some other reasons.These include the formation of a hormone from the wrong chemical structure and congenital defect receptors sensitive to this hormone, whereby they do not respond to the production of growth hormone by the pituitary gland.

the most part, growth hormone deficiency due to a genetic defect.However, other causes of this disease may be: hypoplasia of the pituitary gland, its incorrect location in the brain, cyst formation, compression of the tumor, trauma of the central nervous system.In addition, a certain value are infectious and toxic damage to the central nervous system in early childhood: intrauterine viral infections, tuberculosis, syphilis, malaria, toxoplasmosis, newborn sepsis, inflammation of the brain and its membranes.Changes at the internal organs are in dwarfism thinning bones, stunted growth and skeletal ossification.The internal organs, muscles, and subcutaneous fat is poorly developed.

For a long time, growth hormone deficiency was regarded only as a problem of pediatric endocrinology.The main goal of treatment in this case was considered to achieve a normal child growth.Only relatively recently, it was found that the presence of growth hormone deficiency in adults is a serious cause of a whole range of metabolic disorders.This condition requires constant supervision of specialists and the necessary medical treatment.Growth hormone deficiency, first emerged into adulthood, occurs with a frequency of 1 per 10 000 population.

The main features of dwarfism is a sharp growth retardation and physical development of the child.Children with classic growth hormone deficiency are born with normal weight and body length.They begin to lag behind in development starting with 2-4 years of age.This development of the disease attributed to the fact that the hormone prolactin, entering the body of the child with the mother's milk during the first years in early childhood can give children an effect similar to growth hormone.In the case of genetically caused in children with delayed growth and sexual development of the pathology of growth hormone in most cases, the questioning can not identify similar cases of stunting in the family of one of the parents.In adults who did not receive the necessary treatment in childhood, celebrated children's body proportions.

small facial features ( "doll face"), the bridge sinks.Pale skin with a yellowish tinge, dry, sometimes observed bluish coloration of the skin marbling.In untreated patients with early to appear senile appearance, the skin becomes thin, wrinkled.The distribution of subcutaneous fat varies from starvation to obesity, in which excess fat tissue deposited mainly in the upper half of the body.Hair can be both normal and dry, thin, brittle.Re-growth of body hair, which should appear in puberty, in most cases, is absent.The muscular system is underdeveloped.Boys tend to have extremely small penis size, sexual development is delayed.Most of the children with growth hormone deficiency have a collateral deficit of hormones that promote the development of the genital organs (gonadotropins).

Laron syndrome - endocrine disease, in which is based on the loss of sensitivity of body cells to growth hormone as a result of genetic mutation.The manifestations of this deviation is roughly the same as in the case of pituitary dwarfism.Especially in this case are: high degree of growth retardation since birth, bone age is behind the passport, but ahead of the child's growth, sexual development begins in a relatively normal as possible in 50% of children may cause surges of growth.In addition, at a syndrome Laron there is a high risk of various birth defects, the most common of which are: the shortening of the phalanges, cataracts, involuntary eye movement (nystagmus), narrowing of the aortic lumen, split upper lip, congenital dislocation of the hip, bluesclera.

main methods by which you can identify and confirm pituitary dwarfism are: anthropometry (measurement of growth) and a comparison of its results with the proper values ​​for the test the child's age;dynamic monitoring of the growth of the child.In children with growth hormone deficiency growth rate of less than 4 cm per year.To eliminate various congenital disorders of skeletal dysplasia is necessary to assess body composition.During the X-ray study of bones of the hands and wrist joints is determined by the so-called bone (X-ray) age.In the case of pituitary dwarfism revealed a significant delay of ossification.When X-rays of the skull reveals the size and shape of sella (pituitary bone receptacle), characteristic of childhood.Regardless of the fact that all of the above methods of inspection are highly informative, the most accurate method for the correct diagnosis of pituitary dwarfism is to determine the level of growth hormone in the blood serum.A single determination of blood level of growth hormone for the diagnosis of somatotropic deficiency does not matter because the hormone is released episodically during the day, which can lead to the determination of low levels even in healthy children.

Growth hormone deficiency in adults is accompanied by violation of all types of metabolism and very diverse manifestations.As a result of violations of fat metabolism develops obesity.Violation of protein synthesis results in a reduction of weight and strength of the skeletal muscles, cardiac muscles marked depletion.Quite often it is possible to note the appearance of hypoglycemic states (there are at deficiency of blood glucose), which are accompanied by excessive sweating during the night and the appearance of a headache in the morning.

case of insufficiency of growth hormone are the most vivid manifestation of changes in the human psyche.There is a tendency to frequent depressions, anxiety, a person gets tired quickly, suffered overall health, broken emotional reactions.As time begins to clear tendency to social isolation of people suffering from this disease.

Among people with growth hormone deficiency who receive treatment without growth hormone, a two-fold increase in mortality from cardiovascular disease.The reason for this is to change the composition of blood, the increase in its amount of fat, which begins to settle on the inner surface of blood vessels, leading to the development of atherosclerosis.When

somatotropin deficiency bone loss occurs as a result of all kinds of disorders of metabolism, including mineral.This increases the fragility of bones, resulting in increased frequency of fractures.

treatment. basis therapy of pituitary dwarfism is replacement therapy with growth hormone.The drug of choice in this case is the human growth hormone produced by genetic engineering.Somatotropin daily by subcutaneous injection in the evening (20.00-22.00) is inserted in the treatment of classical growth hormone deficiency.Application hubbub growth with Laron syndrome does not bring any effect.