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Pituitary Nanism (dwarfism)

 
, medical expert
Last reviewed: 17.04.2020
 
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The term "hypophyseal nanism" (from the Greek nanos - dwarf; syn: dwarfism, nanosomy, microsomia) in the absolute sense means a disease, the main manifestation of which is a sharp lag in growth, associated with a violation of the secretion of growth hormone in the anterior pituitary gland.

More broadly, Nanism is a violation of growth and physical development, the occurrence of which can be due not only to the absolute or relative deficiency of the growth hormone in connection with the pathology of the pituitary itself, but also to the violation of the hypothalamic (cerebral) regulation of its functions, to the defects in the biosynthesis of the somatotropic hormone, disorders of tissue sensitivity to this hormone.

trusted-source[1], [2], [3], [4]

Epidemiology

According to various authors, nanism occurs with a frequency of 1: 3000-5000 to 1: 30,000. The incidence rate does not depend on sex.

trusted-source[5], [6], [7], [8], [9], [10]

Causes of the dwarfism

Most forms of pituitary Nanism refers to genetic diseases. The most common is panhypopituitory dwarfism, which is inherited mainly by the recessive type. It is assumed that there are 2 types of transmission of this form of pathology - autosomal and through the X chromosome. With this form of Nanism, along with a defect in the secretion of growth hormone, the secretion of gonadotropins and thyroid-stimulating hormone is most often upset. The secretion of ACTH is less frequent and to a lesser extent. Functional studies with releasing hormones, including synthetic somatotropin-releasing hormone (consisting of 29, 40 and 44 amino acid residues), similar to the pancreatic polypeptide, have shown that most such patients have pathology at the hypothalamic level, and an inadequate anterior lobe The pituitary gland has a secondary character. The primary pathology of the pituitary gland is less frequent.

Genetic nanism with isolated growth hormone deficiency, with a violation of its biological activity and sensitivity to it in Russia and the CIS countries occurs sporadically. It is more widespread on the American continent, in the countries of the Near and Middle East and in Africa. Based on the results of the study of the content of the somatotropic hormone in the blood and the sensitivity of the patients to the exogenous somatotropic hormone, the level of immunoreactive insulin (IRI), type I (somatomedin C) and type II insulin-like growth factors (IGF) and the IRF-1 response to treatment with growth hormone various variants of clinically similar types of nanism.

Recently, the pathogenesis of Laron's Nanism, which is caused by the deficiency of IRF-1 and IRF-II, has been deciphered, as well as the pathogenesis of dwarfism in African pygmies, due to the insufficiency of the former.

In 1984, a new variant of pseudohypophyseal nanism with a high level of somatotropic hormone and IRF-1 was described; genesis of nanism is explained by a defect in the receptors to it, which is proved by a sharp decrease in the binding of skin fibroblasts with IRF-1.

It should be emphasized that in modern conditions, in the presence of small families, many single cases ("idiopathic", sporadic) cases of the disease can also be genetic.

When analyzing 350 case histories, the etiology of nanism was unclear in 228 patients (65.2%). This group included patients from 57 families with a repeated incidence of nazism (2-4 cases in the family), which accounted for 28% of all patients. In 77% of families with etiologically unclear (mostly genetic) forms of nanism, there is an unequivocal connection with the inheritance of the absence of Rh factor. The distribution of Rh factor in the families of patients with nannism differs from that observed in the Rh-conflict between the mother and the fetus and, as a rule, is not accompanied by hemolytic disease of the newborn (Rh-negative may be the father, and in case of heterozygosity of the parents according to the Rhesus-affiliation - children, etc.). It is possible to assume a connection between the activity of genes responsible for the synthesis of somatotropic hormone (or somatotropin-releasing hormone) and the genes that determine Rh-accessory, especially since most forms of nanism and absence of Rh factor are autosomal recessive traits. This does not explain the relative rarity of the incidence of nazism compared to the frequency of Rhesus-negative individuals in the population. Probably, some additional factors not yet known are important, however, the distribution of Rh factor in families of patients with family and sporadic nazmom is unlikely to be random.

A large group of patients with nazism (primary cerebral, cerebro-gipophysial) are patients with various types of organic pathology of the central nervous system, which appeared in utero or in early childhood. The anatomical substrate that causes this pathology may be the hypoplasia or lack of development of the pituitary gland, its dystopia in the pathology of the formation of the Turkish saddle, cystic degeneration of the pituitary gland, its atrophy due to tumor compression (craniopharyngioma, chromophobic adenoma, meningioma, glioma). Nanizm can be caused by traumatic injuries of the hypothalamic-pituitary region (intrauterine, generic or postnatal), which often occurs in multiple pregnancies, as well as during labor in the gluteal, leg presentation or in the transverse position with a turn to the pedicle (this is the mechanism of delivery of more than 1 / 3 patients with nazmom). Infectious and toxic damage (fetal viral infections, tuberculosis, syphilis, malaria, toxoplasmosis, early illness, neonatal sepsis, meningo- and ara-noencephalitis, etc.) are important. These processes can damage the pituitary itself, regulate its function, hypothalamic centers, disrupt normal functional connections in the central nervous system.

Intrauterine lesions of the fetus can lead to the birth of patients with "nannism from birth" with the normal secretion of growth hormone (cerebral primordial nanism, microcephaly, Nanism Silver-Russell with hemiasymmetry of the body and high levels of gonadotropins, etc.).

Additional factors that aggravate the disruption of physical development in the case of nanism can be inadequate nutrition and unfavorable environmental conditions, as well as various chronic diseases, for example glomerulonephritis, in which azotemia affects the activity receptors of the liver or directly affects the metabolism of the hepatic cells, causing a decrease in the synthesis of somatomedine or cirrhosis, when the formation of atomedina.

trusted-source[11], [12], [13], [14], [15], [16]

Pathogenesis

In most patients with pituitary nanism, the changes are not limited to the pathology of secretion of the growth hormone and sensitivity to it, but spread to other tropic hormones of the pituitary gland, which causes various combinations of endocrine and metabolic disorders.

With isolated insufficiency of somatotropic hormone, morphological changes in the pituitary gland have been studied little. In the cases investigated, pathological disturbances are rarely found (craniopharyngioma or hyperostosis of the bones of the skull). In this type of nanism, congenital maldevelopment of peptidergic cells or defect of the neurotransmitter system in the hypothalamus can be observed. In such cases, a combination of nanism with dysplasia or hypoplasia of the optic nerves is possible. STG-insufficiency is caused by intrasellar cysts, pituitary and hypothalamic tumors that cause compression of the pituitary tissue, in particular, somatotrophs.

With dwarfism, thinning of the bones is noted, mainly due to the cortical layer, delay in differentiation and ossification of the skeleton. Internal organs are hypoplastic, sometimes atrophic, muscles are poorly developed.

trusted-source[17], [18], [19], [20], [21], [22], [23], [24], [25], [26], [27], [28]

Symptoms of the dwarfism

A sharp lag in growth and physical development are the main manifestations of pituitary Nanism. Patients are born with normal weight and body length and begin to lag behind in growth from 2-4 years of age.

Before the advent of the active therapy of nanism, the dwarf considered the growth of women less than 120 cm and men 130 cm. At present, the growth of the dwarf differs by not less than 2-3 sigma deviations from the average table norm for a given sex, age, population. There is also a method of graphical estimation of growth based on the Gaussian distribution curve. At the same time, dwarfs in terms of growth fall into the group, which includes the minimum number of persons in the corresponding population with the largest gap from the average growth rate.

For pituitary nanism not only small absolute body sizes are characteristic, but also a small annual dynamics of growth and physical development. The constitution is proportional, but the proportions of the patients' body are characteristic of childhood. Skin pale, often with a yellowish tinge, dry, which is caused by absolute or relative thyroid insufficiency, sometimes there is cyanosis - the "marbling" of the skin. In untreated patients, early appearance and wrinkling of the skin (geraderma) occurs. This is due to the inadequacy of the anabolic action of STH and the slow change in cellular generations.

Hair on the head can be either normal or dry, thin, brittle; long eyelashes are characteristic. Secondary hair is more often absent. The size of the Turkish saddle in most patients with nazism (70-75%) has not been changed, but the saddle often retains the children's form of a "standing oval", has a wide "juvenile" back, the sinus of the main bone lags in the timing of pneumatization. However, there are patients with an increase in the Turkish saddle, which is a sign of a tumor; with areas of calcification against its background or in the area of entry (with craniopharyngioma, residual neuroinfection phenomena) or its decrease (signs of underdevelopment, small pituitary size). There are symptoms of intracranial hypertension: thinning of the bones of the cranial vault, strengthening of the vascular pattern, the presence of finger impressions, etc. The most important sign of hypophyseal nanism is the delay in the timing of differentiation and ossification of the skeleton. In close connection with the differentiation of the skeleton there are also features of the dentoalveolar system: a late change of milk teeth is noted. The greatest lag in the development of the bone system is observed in patients with nannism with sexual insufficiency and hypothyroidism.

Genital organs in most patients are sharply underdeveloped, although developmental defects are rare. Cryptorchidism was observed in 5.8% of male patients. Sexual failure is accompanied by underdevelopment of secondary sexual characteristics and a decrease in sexual desire, the absence of menstruation. Normal spontaneous sexual development is observed only in patients with isolated growth hormone deficiency and in some patients with cerebral palsy.

Thyroid deficiency is a fairly common sign of Nanism. It should be noted that the external manifestations of hypothyroidism do not always reflect the true functional state of the thyroid gland. This is due to relative hypothyroidism due to a violation of the transition of thyroxine (T 4 ) to triiodothyronine (T 3 ) and the formation of inactive (reversible) T 3, which is typical for somatotropic insufficiency.

Adrenocorticotropic function in pituitary tuberculosis decreases less frequently and to a lesser extent than the sexual and thyrotropic, and in most patients does not require special correction.

In most cases, the intellect is not violated. There are emotional changes in the form of mental infantilism; In older patients without a loss of intelligence, reactive neuroses are sometimes noted.

In organic cerebral pathology, especially of a tumorous nature, nanism can occur with the phenomena of diabetes insipidus, bitemporal hemianopsia and with intellectual disabilities.

The study of the formation of brain bioelectric activity in patients without organic symptomatology from the side of the central nervous system showed that the EEG in them is characterized by features of immaturity, a long-term preservation of the high "child" voltage of the EEG; uneven alpha-rhythm in amplitude and frequency; a sharp increase in the content of slow θ- and δ-rhythms, especially in the frontal and central leads; a clear reaction to hyperventilation; shifting the range of EEG rhythms following the rhythms of light stimulation toward low frequencies (evidence of a decrease in the functional mobility of the neural structures of the brain). It was revealed that in elderly patients the immature character of brain electroactivity is caused by sexual underdevelopment, and in patients of all age groups - by hypothyroidism.

For carbohydrate metabolism of patients with nazmom a tendency to lowering blood glucose level on an empty stomach, lifting it during exercise, insufficiency of endogenous insulin, increased sensitivity to exogenous insulin with frequent development of hypoglycemic conditions is characteristic. The latter is mainly due to the inadequate content of patients with contrinsular hormones in the body.

On the part of the internal organs, there is a splanchnomycria, a decrease in their size. Specific for Nanism functional changes in internal organs are not described. Often observed arterial hypotension with a decrease in systolic and diastolic pressure, a decrease in pulse amplitude. Heart tones are muffled, functional noises of different topics are listened to in connection with trophic changes in the myocardium and autonomic disorders. ECG is characterized by low voltage (especially in the presence of hypothyroidism), sinus bradycardia or bradyarrhythmia; for FCG - a decrease in the amplitude of tones, additional tones, functional noise. The data of oxyhemometry indicate hypoxemia (baseline and with physical exertion), oxygen debt. Older patients sometimes develop hypertension.

trusted-source[29], [30], [31], [32]

Diagnostics of the dwarfism

Diagnosis and differential diagnosis of nanism is based on data of anamnesis and complex clinical-radiological, laboratory and hormonal examination. In addition to the absolute size of the body, to determine the growth of patients determine a growth deficit - the difference between the growth of the patient and his average rate for the corresponding sex and age; Growth age - conformity of growth of the patient to the certain specifications; standard deviation index

И = М - Мср / δ, where М - patient's growth, Мср - average normal growth for a given sex and age, δ - quadratic deviation from Мср; And less than 3 - is typical of Nanism, And more than 3 - for gigantism. This indicator can be used to assess the dynamics of development.

X-ray study of patients with nazmom allows to identify the presence of signs of intracranial hypertension, residual phenomena of neuroinfection, calcification, craniostenosis. The study of the size, shape and structure of the Turkish saddle is considered as an indirect indicator characterizing the size of the pituitary gland. One of the most important manifestations of pathological growth retardation is the violation of the differentiation of the skeleton. To assess the degree of maturity of the skeleton, the bone (x-ray) age is determined, to which the differentiation of bone tissue corresponds; Deficiency of ossification (ossification) - the degree of ossification of the ossification from the norm (in years), the coefficient of ossification - the quotient from the division of bone age into chronological and other parameters.

Modern diagnostics of nanism is impossible without studying the secretion of somatotropic hormone, its basal level, circadian rhythm, release under stimulation conditions. For most patients with pituitary nazmom characterized by a reduced content of somatotropic hormone in the blood serum. When determined by the radioimmunoassay method, it is (from the data of different authors) from (0.87 ± 0.09) to (1.50 ± 0.64) ng / ml, at an average rate (3.81 ± 0.29) ng / ml. The study of diurnal (circadian) rhythms of secretion of somatotropic hormone showed that its level in healthy people is maximal during the first 2 hours of sleep and at 4-6 hours. During nanism and during these hours, the content of growth hormone is reduced.

To study the reserves of somatotropic function, various stimulants are used, by examining the content of growth hormone before and after their administration. Blood for examination is taken for 2-3 hours every 30 minutes. Normal release of a somatotropic hormone after stimulation is not less than 7-10 ng / ml, sometimes it reaches 20-40 ng / ml. If there is no reaction in one of the samples, repeated tests with other stimulants are carried out. Insufficient growth hormone is considered proven in the absence of a release of growth hormone in 2-3 different samples.

The following stimulating samples are most commonly used: with intravenous injection of 0.1 U (0.75-1.5 U) of insulin per kg of body weight of the patient and achievement of hypoglycemia (decrease of blood glucose level by 50% to the baseline level), serum hormone the above scheme. With the development of severe hypoglycemia, the sample is interrupted, the patient is given intravenous glucose. This is the most common, classical method of diagnosis.

TGH in a dose of 200-500 μg intravenously. Effectively reveals the reserves of the hormone, does not give complications. In combination with the insulin test allows you to judge the level of damage to the hypothalamic-pituitary system. A positive reaction to TRH in its absence on insulin hypoglycemia testifies to the intactness of the pituitary gland and lesion at the hypothalamic level, negative reactions to TRH and hypoglycemia - about the defeat of the pituitary itself.

TGH, LH-RG at a dose of 300 μg is intravenously similar to the previous one.

The human SRH is a synthetic analogue of a biologically active compound isolated from a pancreatic tumor. At present, there are 3 types of synthetic SRH: with 29, 40 and 44 amino acid residues. Applied intravenously in doses from 1 to 3 mcg / kg of body weight of the patient. The release of STH is observed 15-20 min after administration, the test more efficient than others reveals the reserves of endogenous growth hormone. The positive reaction of the STH testifies to the hypothalamic level of the lesion of the somatotropic function and the intactness of the pituitary gland; with amino acids (L-arginine-monochloride, ornithine, tryptophan, glycine, leucine) intravenously at a dose of 0.25-0.5 g per kg of body weight of the patient. Effective for the study of STG reserves. May cause allergic reactions.

L-dopa orally in a dose of 250-500 μg. Effective, well tolerated by patients.

Samples with glucagon, bromergocryptine (parlodel), lysine-vasopressin, clonidine, with a dosed bicycle ergometric load are also used.

The study of the state of somatotropic function is necessary not only for diagnosis of nanism, but also for a reasonable choice of the method of therapy, since treatment with somatotropin is rational only in the case of an inadequate endogenous growth hormone.

For the diagnosis of the form of nanism, it is very important to study the content of insulin-like growth factors, or somatomedins (especially IGF-1, or somatomedin C), the mediators of the action of somatotropic hormone at the tissue level. It is known that the content of somatomedin C in the case of nanism is reduced, and with acromegaly, it is increased in comparison with the norm. The form of nanism described by Laron is a type of disease with normal production of STH, but with a violation of the formation of IRF-1 and IRF-P. Treatment of such patients with somatotropin is futile.

Indirect indicators of somatotropic function of the pituitary gland are the activity of alkaline phosphatase and the content of inorganic phosphorus of serum. With hyposomatotropic conditions, these indicators are reduced. When pangipopituitarnoy form of dwarfism reduced secretion of gonadotropins often TTG, which is accompanied by a corresponding reduction in gonadal function (deficiency of androgens or estrogens), thyroid (falling levels of T 3, T 4, protein-bound iodine - PBI, accumulation of 131 I thyroid gland), adrenal gland ( decrease in the amount of cortisol and 17-ACS in plasma, excretion of 17-KC and 17-ACS in urine, lymphocytosis).

For all types of hypophyseal (hypothalamic-pituitary) genetic nanism, a recurrent disease of children in the family with autosomal recessive (more often) or autosomal dominant type inheritance is characteristic, delay in growth and physical development from 2-4 years with a delay of not less than 2- 3 o from the average growth rates for a given sex, age, population, with a low spontaneous annual growth dynamics, a delay in ossification. At a low level of somatotropic hormone (in 2-3 stimulating tests below 7 ng / ml) therapy with growth hormone is highly effective (gives an increase in growth of at least 7 cm per year). At a normal or high level of growth hormone (with its biological inactivity), the sensitivity to hormone can be maintained. Changes in intelligence are not observed

In genetic nanism with tissue insensitivity to somatotropic hormone, the clinical picture is analogous to isolated growth hormone deficiency, but somatotropin therapy is ineffective. In this group, the following main forms can be distinguished according to the level of IGF: with their normal content (receptor defect in the IGF) and reduced ones - Laron type (IRF-1 and IRP-P deficiency) and type detected in African pygmies (IGF-1 deficiency ).

Cerebral nanism is characterized by single diseases in the family that are associated with intrauterine or postnatal injuries of the central nervous system, with the presence of obvious organic changes in the central nervous system, often combined with the pathology of the organ of vision, the presence of diabetes insipidus, the preservation of gonadotropic functions, and changes in the intellect.

The expressed dormancy is accompanied by some kinds of dysgenesis and agenesis of the gonads, in particular Shereshevsky-Turner syndrome and the "turnaround" (mosaic) form of the testicular dysgenesis syndrome. Differential diagnostics is assisted by cytogenetic studies (sexual chromatin, karyotype) that detect chromosomal defects, as well as characteristic defects of somatic and sexual development, normal or elevated levels of endogenous growth hormone, and insensitivity to treatment with somatotropin.

Among endocrine disorders that occur with short stature, primary hypothyroidism due to congenital hypoplasia or aplasia of the thyroid gland, its dystopia, enzyme defects in the biosynthesis of thyroid hormones, early autoimmune thyroid disease is to be distinguished . For all these conditions, signs of hypothyroidism with a high level of TSH, a decrease in T 4 and T 3 in the blood serum dominate . With myxedem autoimmune genesis, antibodies to thyroglobulin, microsomal and nuclear fractions of thyroid tissue are detected in the blood, the level of normal growth hormone is normal or decreased. The clinical effect can be achieved only by compensating for hypothyroidism.

Low growth accompanied by premature sexual development and adrenogenital syndrome due to early closure of growth zones; Itenko-Cushing's disease, which arose in childhood due to the inhibitory effect of glucocorticoids on the secretion of growth hormone and their catabolic effect; Mauriac's syndrome is a short stature and infantilism of patients with insulin-dependent diabetes mellitus of severe form.

Gipofizarnyj nanizm it is necessary to differentiate with somatogenic delay of the physical development caused by chronic infringements of an exchange (at diseases of a liver, kidneys, a gastrointestinal tract), a chronic hypoxia (at diseases of cardiovascular and respiratory systems, at anemias); with systemic diseases of the musculoskeletal system (chondrodystrophy, imperfect osteogenesis, exostosis disease), etc.

Functional (constitutional) growth retardation is sometimes observed in the late onset of pubertal period in practically healthy adolescents; we found that it is primarily associated with transient insufficiency of gonadotropic activity. Secretion of growth hormone is usually not disturbed or reduced slightly. Stimulation of gonadotropins can cause acceleration of both sexual development and growth.

Low-growing family character should be considered as a variant of physiological development.

trusted-source[33], [34], [35], [36]

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Treatment of the dwarfism

Treatment of dwarfism is a long process. This causes the physician to distribute the means of influencing the growth in time so as to obtain the greatest clinical effect while observing the two basic principles:

  • the maximum approximation of treatment-induced development to physiological conditions;
  • sparing epiphyseal growth zones.

Many years of experience in the treatment of dwarfism makes it possible to consider the following scheme of stage therapy expedient. The diagnosis of nanism in adult patients is usually not in doubt. In young children, if the clinical picture is not clear, a diagnostic period is needed: 6-12 months under observation without hormone therapy. At this time, complex general restorative treatment is prescribed; full nutrition with an increase in the content of animal protein, vegetables and fruits in the diet, vitamins A and D, calcium and phosphorus. Absence of sufficient changes in growth and physical development on this background and detection of endocrine disorders during examination is the basis for the beginning of hormone therapy.

The main type of pathogenetic therapy of pituitary nanism is the use of human growth hormone, since the occurrence of the majority of cases of dwarfism from one form or another of its insufficiency is doubtless dependent. In connection with the specific specificity peculiar to this hormone, only human somatotropin and primates are active for humans. Widely used in the clinic drug, isolated from the pituitary gland of people who died from non-infectious and non-tumor diseases. Human somatotropin was obtained by bacterial synthesis using Escherichia coli by genetic engineering. Human somatotropin is also synthesized chemically, but it is extremely expensive and in the clinic it is practically not used. For the treatment of somatotropin, patients with proven endogenous growth hormone deficiency are selected, with differentiation of the skeleton not exceeding the level characteristic of 13-14 years. Age restrictions for treatment are not established.

The minimum effective doses that can be used in the first treatment period are 0.03-0.06 mg / kg body weight. The most effective dose - 2-4 mg 3 times a week. An increase in the single dose to 10 mg was not accompanied by an adequate increase in the growth effect, but caused the rapid formation of antibodies to the somatotropin.

In our country, work on the study of human growth hormone has been conducted since 1960. Two treatment regimens have been tested: continuous and intermittent with courses for 2-3 months and at the same intervals between them. The average increase in the growth of patients for the first year of treatment was 9.52 ± 0.39 cm, an increase in body weight of 4.4 ± 0.14 kg. With long-term continuous treatment, the average increase in growth is 0.82 cm / month, body weight is 0.38 kg / month; at intermittent - 0.75 cm / month and 0.4 kg / month, respectively. Continuous treatment gave a faster increase in growth with a sharp decrease in the effect after 1-1.5 years, with intermittent treatment, the efficacy persisted for 3-4 years, which makes the course of treatment more appropriate. Determination of the level of IGF-I (somatomedin C) can serve as a reliable indicator of the sensitivity of the patient to treatment with drugs of somatotropin. An increase in the content of IGF-I after the administration of somatotropic hormone allows predicting the positive effect of therapy. An important advantage of treatment with somatotropin is the absence on its background of acceleration of ossification of the skeleton.

The most important means of treating dwarfism should be considered the use of anabolic steroids, which stimulate growth, enhancing protein synthesis and increasing the level of endogenous growth hormone. Treatment is carried out for several years, with a gradual replacement of some drugs by others, from less active to more active compounds. The change in anabolic drugs is indicated with a decrease in the growth effect in 2-3 years, which leads to an additional increase in growth. Treatment is carried out by courses (rest period should be half the term of treatment). When accustomed to show longer breaks (up to 4-6 months). Simultaneously prescribe only one of the anabolic steroids. Combine 2 or more drugs is inappropriate, since this does not enhance their metabolic and growth activity. The latter depends primarily on the age of patients and the degree of differentiation of the bones of the skeleton to the beginning of treatment. The best effect is observed in patients up to 16-18 years with ossification of the skeleton, not exceeding the level characteristic of 14-year-old age. It is advisable to begin treatment immediately upon diagnosis, usually from 5-7 years. Prior to treatment, gonadotropins and sex hormones should be avoided, which, while stimulating growth, simultaneously accelerate the differentiation of the skeleton. The principle of dosing anabolic steroids - from minimal effective doses to gradually increasing. Recommended doses of the most common drugs: nerobol (methandrostenol, dianabol) - 0.1-0.15 mg per 1 kg of body weight per day orally; nerobolil (durabolin) - 1 mg per 1 kg of body weight per month intramuscularly, the monthly dose is administered for 2-3 doses, respectively, after 15 or 10 days; retabolil (deca-durabolin) - 1 mg per 1 kg of body weight once a month intramuscularly. Excess of these doses can lead to androgenization. In physiological doses, these compounds do not significantly affect the condition of the genital organs and the differentiation of the bones of the skeleton, which allows them to be applied to long-term patients of both sexes. Girls should be under the supervision of a gynecologist, since in case of an overdose or with increased individual sensitivity, some signs of virilization may appear in some patients, which quickly regress when treatment is withdrawn. Methylated in the 17th position, oral preparations can sometimes cause a cholestatic effect, so for liver diseases, preference should be given to anabolic compounds of parenteral use, or the combination of oral preparations with choleretic agents. Very rarely, treatment with anabolic steroids can cause allergic phenomena (itching, rash). In the absence of complications, anabolic steroids are used as long as the growth effect is observed (up to 16-18 years, and sometimes longer). Treatment is conducted against a background of general strengthening therapy.

If there are signs of hypothyroidism in patients, thyroidin preparations (thyroxine, thyroidin, thyrotome) at the individually selected doses are prescribed simultaneously.

In the treatment of boys, the next step is the appointment of a chorionic gonadotropin. This drug is used not earlier than 15-16 years, and often at a later age to stimulate Leydig cells, which accelerates both sexual development and growth (due to the anabolic activity of native androgens). Doses from 1000 to 1500 units 1-2 times a week intramuscularly with courses for 2 months no more than 2-3 times a year are applied. With incomplete effect, treatment with chorionic gonadotropin in boys aged 16 years and older alternates with the administration of small doses of androgens (methyltestosterone 5-10 mg / day sublingually).

Girls over 16 can start treatment with small doses of estrogen, while simulating a normal sexual cycle. Treatment is carried out for 3 weeks each month with a subsequent break. In the 2 nd phase of the cycle from the third week, chorionic gonadotropin can be administered at a dose of 1000-1500 units 3-5 times per week or preparations of gestagenic action (pregnin, progesterone).

The final stage of treatment (after the closure of growth zones) is the permanent appointment of therapeutic doses of sex hormones corresponding to the sex of the patient, with the goal of fully developing the sexual organs, secondary sexual characteristics, providing libido and sexual potency. For the treatment of female patients, combined estrogen-inhagen preparations (non-vellon, bisekurin, infecoundin, and rigevidon) are convenient in the use of the female, and prolonged-action androgen preparations (testanate, sustanon-250, omnadren-250) for male patients.

There is a general restorative treatment (regimen, protein-vegetable diet, vitamin therapy, biostimulants). The use of zinc preparations is shown, in the mechanism of action of which the main role is played by the increase in the activity of IGF-1 (insulin-like growth factor I).

In the presence of organic pathology, the central nervous system performs anti-inflammatory, resorptive, dehydration therapy. Targeted systematic therapy gives an encouraging effect. Out of 175 patients with both sexes, 148 (80.4%) managed to achieve growth of more than 130 cm as a result of long-term treatment, 92 (52.5%) - more than 140 cm and in 32 (18.3%) - 150- 160 cm and more. At the same time, the growth in 30 patients increased in 37 patients (21.2%), by 31-50 cm - in 107 (61.1%). On 51-60 cm and more - in 31 (17.7%).

Forecast

The forecast depends on the form of Nanism. With genetic types of dwarfism, the prognosis for life is favorable. In the presence of a pituitary tumor and an organic lesion of the central nervous system, it is determined by the dynamics of the development of the underlying pathological process. Modern methods of therapy significantly increased the physical capacity and ability to work of patients, extended their life. During active treatment, patients need a doctor's examination every 2-3 months, with maintenance therapy - every 6-12 months.

Corresponding to intellectual and physical opportunities for employment of patients is of paramount importance for their social adaptation.

It is advisable to choose the professions that are not associated with high physical loads, but allow to show intellectual abilities, ability to work accurately, languages.

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