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Pituitary nanism (dwarfism)
The pituitary nanism (other often used name – dwarfism, and also a microsomia, a nanosomia) is a state at which the serious growth inhibition and physical development of the person is noted. It occurs owing to a lack of an organism of somatotropic hormone (a growth hormone, STG).
This illness is considered to be very rare. As different data testify, the disease affects 1-4 people on 10000 population. Physicians note that male patients are ill dwarfism approximately twice more often than women. It is considered to be that dwarfish growth at men is growth below, than 130 cm. At women this indicator makes 120 cm.
The word "nanism" comes from Greek and in transfer "dwarf" designates. This clinical syndrome is shown at the person as an effect of the disturbances having endocrine and not endocrine character. In an organism of the patients suffering from dwarfism disturbance of development of Somatotropinum (a so-called growth hormone) is noted by a front share of a hypophysis.
The reason of display of this illness the person can have a damage of a hypophysis owing to injuries, tumors, influences of toxins and an infection on interstitial and pituitary area. Normal regulation of functions of a hypophysis from a hypothalamus is sometimes broken.
Development of a disease is influenced by also genetic factors, loss or decrease in somatotropic function of a hypophysis, lack of biological activity of a growth hormone or the broken sensitivity to this hormone from peripheral fabrics.
The pituitary nanism can be carried to diseases which are inherited genetically. In this case it is important to person to know whether his close relatives and ancestors had cases. Sometimes will help to distinguish a disease at close people even old photos or inquiries of relatives.
But physicians allocate also a number of factors which can aggravate a situation at development in the person of dwarfism. So, the pituitary nanism at children is often aggravated owing to defective food, influence of negative factors of environment, existence of some somatopathies. Treatment of a pituitary nanism assumes elimination of such factors.
The birth trauma of the head, and also syphilis, tuberculosis, sarcoidosis can become the reason of development of dwarfism. The factor contributing to a nanism sometimes is radiation therapy or chemotherapy. However, approximately in half of cases the reason of development of this pathology does not manage to be found out.
So, low human height is considered the main symptom of a disease — men have lower than 130 cm and women have lower than 120 cm. So, if normal the child grows by 7-8 cm a year, then at a pituitary nanism growth of the child increases by only 3-4 cm a year. It should be noted that low-tallness constitutional, that is family character, and also doctors consider lag in growth at healthy children as norm option.
Dwarfism is a state at which very sharp lag in growth and physical development of an organism in comparison with average values of norms for a certain age is noted. Signs of dwarfism assume at the same time not only pronounced lag, but also lowering of annual dynamics of increase in growth and weight at such patient. As a rule, such children have quite normal indicators both the weight, and growth at the birth. But later they observe considerable lag from peers on these indicators. Sometimes the illness is shown since the very first months of life. But nevertheless most often parents notice dwarfism symptoms at children at 2-4 summer age.
At patients who suffer from a nanism, the proportional shape and addition of a body is noted. But at the same time such proportions more correspond to a children's constitution. Such people have a dry and pale skin. The reasons of this phenomenon – insufficient functioning of epithelial bodies. If the person does not receive regular and correct treatment, then integuments grow old very much early, on them wrinkles develop.
The fatty tissue under skin is also developed poorly, and at some patients excess fatty deposits on a stomach, hips, a breast are observed. At the same time muscles are developed very poorly. If at patients at whom giantism develops muscles initially are strong, and weaken later, then at patients with a nanism weakness of muscles is noted at once. Most often at such people secondary pilosis is not observed. One more important sign which shows a Lorain's disease is a delay of ossification of a skeleton. This phenomenon defines late shift of milk teeth with constants.
Due to the loss of gonadotropic function of a hypophysis at people with dwarfism insufficiency of sexual development is observed. If to carry out comparison with age norms, then at men the penis and gonads is reduced, underdevelopment of a scrotum is observed, and secondary sexual characteristics are absent. At the same time at women with a pituitary nanism the expressed manifestations of a hypogonadism are noted. At them can not be periods, mammary glands remain underdeveloped, secondary sexual characteristics are not shown.
Intellectual development in such patients answers criteria of norm, however some juvenile lines can sometimes be shown. In the course of carrying out neurologic inspection detection of some signs testimonial of organic lesion of a nervous system is possible. Internals at such patients reduced (this phenomenon is called a splankhnomikriya), they often suffer from displays of hypotension, bradycardia, the muffled cardiac sounds are noted. The phenomena of a secondary hypocorticoidism are sometimes observed.
If the nanism developed at the patient owing to organic lesion of a brain, then there is an all-brain symptomatology, the intelligence decreases. Often at such state not diabetes mellitus develops.
Suspicions concerning dwarfism at the child at parents can appear in the first months or years of life of the kid. Defining features of development, the pediatrician estimates not only indicators of growth and child's weight, but also their dynamics.
At suspicion X-ray inspection is appointed to a pituitary nanism. On x-ray films of brushes and radiocarpal joints it is possible to consider signs of a delay of a differentiation and ossification of a skeleton.
Carrying out a computer tomography allows to define underdeveloped zones of ossification of a skeleton. At normal development of an organism of a bone are formed of cartilages, their ossification takes place gradually. Such processes begin with ossification centers. They completely come to the end when to the person 20-25 years are executed. If development of Somatotropinum is broken, then at research such points can be defined throughout all human life.
To the patient laboratory researches are appointed. In particular, the insulin test at which growth hormone hypersecretion is not noted is carried out (it is characteristic of a normality of health of the person). Sometimes its secretion increases only for the short period and is insignificant. Also other tests for identification of level of somatotropic hormone in blood are used. Biologically active agents and some medicines are for this purpose applied.
Beginning dwarfism treatment, the doctor has to define surely that reason which provoked development of this pathology. If the pituitary nanism is possible to distinguish and confirm timely the diagnosis, then replacement therapy using somatotropic hormone yields good results. In the course of treatment hormone, both a natural, and synthetic origin is used.
Treatment by Somatotropinum is carried out till 13-14 summer age of the patient. At the same time influence of hormone on growth of concentration of somatomedins in the patient's blood as without somatomedins hormone Somatotropinum works is surely considered it is limited. If after a certain period of treatment concentration of somatomedins increases in blood, it is possible to draw conclusions about success of therapy.
Increase in growth of the patient in the course of treatment – one more important criterion. Treatment is considered effective if after a year of therapy according to a certain scheme of people grows by 8-12 cm.
Administration of drug happens several times a week for 2-3 months. After that the small break follows. It is important to adhere to such scheme, for a long time use of this hormone in high doses can provoke a producing in an organism of antibodies. Therefore, the experienced endocrinologist shall control therapy process.
If the patient has primary processes in a hypothalamus, treatment is shown it by somatoliberin which makes the stimulating impact on a hypophysis.
Sometimes include reception of steroid hormones in the scheme of treatment of dwarfism. They cannot replace completely that effect which gives Somatotropinum. However this treatment allows to activate growth and a skeletogeny, and at the same time promotes a producing internal Somatotropinum. It is reasonable to begin such therapy with 5-7 summer age, however to carry out it later, than in 18 years, inefficiently.
If at the patient the combined form of an illness is observed and in an organism shortage of other types of hormones is noted, then in parallel with Somatotropinum reception of synthetic analogs of those hormones which shortage is observed practices.
At insufficiency of functions of a thyroid gland the L thyroxine, Thyreocombum is appointed. If there is a need to correct functions of sex hormones, then at youthful age to patients reception of special drugs is appointed. To male patients reception of a chorionic gonadotrophin or methyltestosterone is appointed. Reception of estrogen, hexestrol or a chorionic gonadotrophin is shown to girls. If dysfunction of TsNS is observed, to the patient vascular drugs are appointed. Sometimes it is necessary to carry out dehydrational, resorptional treatment.
Patients with a pituitary nanism have to eat fully. In a diet has to be as much as possible products containing animal protein, calcium, phosphorus, all groups of vitamins.
The pituitary nanism provoked by tumors of a brain, hypophysis, a hypothalamus will more difficultly respond to treatment as access to localization of a tumor very difficult. Such people often receive disability in view of limitation of physical abilities.
But in general the forecast of a disease generally is defined depending on the illness reason. If certain genetic defects or hereditary transfer of an illness take place, then the correct approach to treatment helps to suspend process of its development. People whose treatment began timely can add considerably afterwards in growth.
Effective measures of prevention for prevention of dwarfism do not exist. Nevertheless, it is necessary not to allow influences of harmful factors (poisons, toxins, etc.) during pregnancy and the subsequent breastfeeding. The injuries got at the time of delivery can influence development of an illness. The child should provide the best nutrition, in a diet there have to be products with the high content of vitamins and minerals. It is important to treat timely all somatopathies of an organism.