Pituitary dwarfism (dwarfism)

April 19, 2013

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Pituitary dwarfism   (another often used name - dwarfism , and microsomia . nanosomes ) - A condition in which notes a serious delay in growth and physical development of man. This is due to lack of an organism growth hormone   (growth hormone, GH).

This disease is considered to be very rare. As the different data, the disease affects 1-4 people per 10,000 population. Doctors say that male patients suffer from dwarfism about two times more often than women. It is believed that the growth of dwarf males - a growth of less than 130 cm. In women, the figure is 120 cm.

Causes

The word " dwarfism "Comes from the Greek language and translated means" dwarf ". This clinical syndrome is manifested in humans as a consequence of violations that have endocrine and not endocrine character. In the body of patients suffering from dwarfism, there is a violation generation somatotropin   (the so-called growth hormone) by the anterior pituitary.

The cause of the manifestation of this disease in humans may be defeated pituitary   due to injuries, tumors, infection and the impact of toxins on mezhutochno- pituitary region. Sometimes it interferes with the normal regulation of pituitary function by hypothalamus .

The development of the disease is also influenced by genetic factors, loss or reduction of growth hormone of pituitary function, the absence of the biological activity of growth hormone or impaired sensitivity to this hormone by the peripheral tissues.

Pituitary dwarfism usually referred to diseases that are inherited genetically. In this case the person is important to know whether there were cases of his close relatives and ancestors. Sometimes recognize the disease in relatives of people help even old photos of relatives or inquiries.

But doctors isolated a number of factors that may aggravate the situation in the development of a human dwarfism. So, pituitary dwarfism in children is often exacerbated by malnutrition, the impact of negative environmental factors, the availability of certain systemic diseases. Treatment involves removal of pituitary dwarfism such factors.

The cause of dwarfism can be a birth trauma of the head, as well as syphilis . tuberculosis . sarcoidosis . Predisposing factor for dwarfism is sometimes radiation therapy or chemotherapy. However, about half the cases the cause of this disease and can not figure out.

Signs

Thus, the main feature of the disease is considered to be low stature - below 130 cm in men and less than 120 cm in women. So if the child normally grows by 7-8 cm per year, then the pituitary dwarfism growth of the child increases by only 3-4 cm per year. It should be noted that the constitutional short stature, that is the nature of the family, as well as growth retardation in healthy children, doctors are considering as an option the norm.

Dwarfism   - A condition in which there is a very sharp growth retardation and physical development of the body, compared with the average norms for a particular age. Signs suggest dwarfism is not only a pronounced lag, but also lowering the annual increase in the dynamics of growth and weight gain in this patient. Typically, these children are quite normal parameters as weight and growth at birth. But later, they observed a significant backlog from peers on these indicators. Sometimes the disease is manifested from the very first months of life. Yet most parents notice signs of dwarfism in children 2-4 years of age.

Patients who suffer from dwarfism, marked proportional to the shape and composition of the body. But while these proportions correspond to a child's physique. These people dry and pale skin. The reasons for this phenomenon - the lack of functioning of the parathyroid glands. If a person does not receive a regular and proper treatment, the skin very early age, they wrinkle.

Fatty tissue under the skin are also poorly developed, and some patients have excess fat on the abdomen, thighs, breasts. This very poorly developed muscles. In patients who develop gigantism Muscles are inherently strong, and later weakened, the patients with dwarfism muscle weakness observed immediately. Most often, such people have not observed secondary body hair. Another important feature, which manifests pituitary dwarfism, is the delay of ossification of the skeleton. This phenomenon determines the late shift at the permanent milk teeth.

In connection with the loss of gonadotropic pituitary function in people with dwarfism observed lack of sexual development. If you make a comparison with the age norm, the men reduced penis and gonads, there is the underdevelopment of the scrotum, and secondary sexual characteristics are absent. At the same time, women with pituitary dwarfism The expressed symptoms of hypogonadism. They may not have menstruation, breasts remain underdeveloped, do not show secondary sexual characteristics.

Mental development in these patients meet the criteria for standards, but can sometimes exhibit some juvenile features. In the course of the neurological examination might detect some signs suggesting an organic lesion of the nervous system. The internal organs in these patients reduced (this phenomenon is called splanhnomikriey ), They often suffer from symptoms hypotension . bradycardia , Marked muffled heart sounds. Sometimes there are secondary effects hypocorticoidism .

If dwarfism develop in patients due to organic brain damage, cerebral symptoms is present, reduced intelligence. Often, when this condition develops diabetes insipidus .

Diagnostics

Suspicions about the stunt a child's parents can appear in the first months or years of baby's life. The features of the development, a pediatrician evaluates not only the growth and weight of the child, but also their dynamics.

If you suspect that pituitary dwarfism is assigned to X-ray examination. On radiographs of the hands and wrist joints may be considered signs of delayed differentiation and ossification of the skeleton.

Computed tomography to determine the underdeveloped areas of ossification of the skeleton. In the normal development of the body's bones are formed from cartilage, ossification of their passes slowly. Such processes begin with the points of ossification. They are completely terminated when a person turns 20-25 years. If the production of growth hormone is broken, then the study of such points can be determined throughout a person's life.

Patients were allocated to laboratory testing. In particular, it is carried out Insulin test At which no marked increased secretion of growth hormone (this is typical for normal human health). Sometimes secretion increases for a short period only slightly. Other tests are also used to detect the level of growth hormone in the blood. For this purpose, the active compounds and certain medications.

Treatment

Since the treatment of dwarfism, the doctor must be sure to identify the cause that provoked the development of this pathology. If pituitary dwarfism unable to timely identify and confirm the diagnosis, the replacement therapy with growth hormone gives good results. In the treatment of hormone use, both natural and synthetic origin.

Treatment somatotropin   It held to 13-14 years of age of the patient. This must take into account the effect of the hormone on the growth of the concentration somatomedins   the patient's blood, as without somatomedins   somatotropin hormone acts is limited. If after a period of treatment increases the concentration in the blood somatomedins You can draw conclusions about the success of therapy.

The increase in the growth of the patient in the treatment process - another important criterion. The treatment is considered effective if, after years of therapy in a specific pattern person grows 8-12 cm.

The drug takes place several times a week for 2-3 months. This is followed by a short break. It is important to adhere to this scheme, because prolonged use of this hormone in large doses can trigger the production of the body antibodies . Therefore, to control the process of therapy must experienced endocrinologist.

If the patient primary processes occur in the hypothalamus, it shows the treatment somatoliberin Which stimulates the pituitary gland.

Sometimes the treatment regimen include dwarfism receiving steroid hormones. They can not fully replace the effect that gives somatotropin . However, this treatment can enhance the growth and development of the skeleton, and thus contributes to the production of internal growth hormone. This therapy should begin with 5-7 years of age But to hold it later than 18 years Ineffective.

If the patient is mixed form of the disease and the body is marked shortage of other types of hormones, the growth hormone is practiced in parallel with the reception of synthetic analogues of the hormones that are experiencing a shortage.

At insufficiency of thyroid function is assigned   L thyroxine . Thyreocombum . If there is a need to adjust the function of sex hormones in adolescence patients assigned to receive special preparations. Male patients assigned to receive chorionic gonadotropin   or methyltestosterone . Girl showing welcome estrogen . sinestrola   or chorionic gonadotropin . If there is a violation of the CNS , Patients prescribed cardiovascular drugs. Sometimes it is necessary to carry out dehydration, resolving treatment.

Patients with pituitary dwarfism have fully fed. The diet should be as many products containing animal protein . calcium . phosphorus All groups of vitamins.

Pituitary dwarfism Triggered brain tumors, pituitary, hypothalamus, difficult to treat, as access to the localization of the tumor very difficult. Such people often acquire a disability due to limited physical abilities.

But in general, the prognosis is mainly determined depending on the cause of the disease. If there are certain genetic defects or hereditary transmission of the disease, the correct approach to treatment helps to stop the process of its development. People whose treatment started in a timely manner may subsequently significantly add to growth.

Prevention

The effectiveness of prevention measures to prevent dwarfism does not exist. Nevertheless, it should prevent harmful influence factors ( poisons . Toxins   et al.) during pregnancy   and then breast-feeding . The development of the illness can affect injuries during delivery . The child should be provided the most nutritious food in the diet should be foods high in vitamins and minerals. It is important to promptly treat all somatic diseases of the body.