Premature sexual development of a girl. What determines precocious puberty in children Premature puberty causes

premature sexual development girls are classified into:

• true, present - the presence of an increase in the mammary glands, pubic hair and axillary fossae and menstrual bleeding.
• false - the presence of growth of the mammary glands and adrenarche (the beginning of the secretion of androgens by the adrenal cortex) in the absence of menstruation.
• incomplete - the appearance of only the growth of the mammary glands or the secretion of androgens by the adrenal glands without accelerating the growth of the body and without menstruation.

Causes of true precocious puberty in girls:

1. Idiopathic early sexual development is due to premature impulse secretion of gonadoliberin, a hormone of the hypothalamus that stimulates the release of gonadotropins by the pituitary gland - follicle stimulating (FSH) And luteinizing (LH) hormones, which in turn stimulate the secretion of female sex hormones.

2. Some diseases cause excessive secretion of the GnRH, gonadotropins mentioned in the previous paragraph or disturbances in their regulation in the hypothalamic-pituitary system.

• tumors pituitary gland and the hypothalamus
• brain damage: trauma, encephalitis, meningitis, exposure to ionizing radiation, chemical factors,
• malformations of the central nervous system and congenital neurological disorders,
• hydrocephalus,
• delayed treatment of adrenogenital syndrome.

Causes of false precocious puberty in girls:

1. Isosexual precocious sexual development (with an excess of female sex hormones estrogen):

• estrogen-secreting ovarian tumors,
• estrogen-secreting tumors of the adrenal glands,
• iatrogenic (due to the use of estrogen or gonadotropin preparations).

2. Heterosexual precocious sexual development (with an excess of male sex hormones androgens):

• androgen-secreting ovarian tumors,
• androgen-secreting tumors of the adrenal glands.

Causes of incomplete precocious puberty in girls:

1. Premature growth of the mammary glands.

2. Premature onset of androgen secretion by the adrenal cortex.

Diseases accompanied by accelerated sexual development in girls:

1. Ovarian cysts.

2. Primary hypothyroidism (deficiency of thyroid hormones due to a pathological process or surgical removal), which is accompanied by hypersecretion of gonadotropic hormones of the pituitary gland that stimulates the ovaries, and hypersecretion prolactin, which affects the growth of the mammary glands.

3. Autonomous ovarian hyperfunction (McCune-Albright syndrome).

4. Hypersecretion of gonadotropins (Russell-Silver syndrome).

Diagnosis of premature sexual development in girls.

The diagnosis of idiopathic precocious sexual development, which occurs as a result of impulse secretion of GnRH and is manifested by the presence of regular menstruation and the absence of neurological, mental disorders, is established after the exclusion of all pathological causes of precocious sexual development.

With true premature sexual development, a short time interval is found between the onset of breast enlargement, the appearance of hair growth and the onset of menstruation. Normally, 1.5-2 years pass from the initial manifestations of sexual development to the first menstruation, and if this period is shortened to 0.5-1 year, pathological causes of premature sexual development should be sought. In a laboratory examination, the blood is found elevated levels luteinizing hormone LH, follicle-stimulating hormone FSH. To detect the tumor, computed tomography CT or magnetic resonance imaging MRI is prescribed.

With false precocious sexual development, an increase in the concentration of LH, FSH, thyroid-stimulating hormone TSH, as well as estrogens (due to autonomous hypersecretion of the latter by the ovaries, adrenal glands, the use of estrogens, choriogonin, and primary hypothyroidism) is detected. The presence of tumors is detected using CT, MRI.

Heterosexual precocious sexual development in girls appears in the pubertal period with signs of androgenization in the form of hirsutism (excessive hair growth), acne, accelerated growth, signs of a male physique, coarsening of the voice and hypertrophy of the clitoris, the presence of genitals of an indeterminate type.

Adrenosecreting tumors, virilizing forms of adrenogenital syndrome are diagnosed using ultrasound, CT or MRI of the pelvic organs and adrenal glands, as well as by examining the blood levels of LH, FSH, ACTH (adrenocorticotropic hormone) pituitary gland), cortisol, testosterone, dehydroepiandrosterone, dehydroepiandrosterone sulfate DEA-S.

Treatment of precocious puberty in girls.

With incomplete premature sexual development, which is manifested only by the growth of the mammary glands or the secretion of androgens by the adrenal glands without accelerating body growth and without menstruation, treatment is not carried out, the patient is under annual dispensary observation. In the idiopathic form, Androcur and GnRH antagonists are prescribed. In adrenogenital syndrome, glucocorticoids are used. Tumors of the brain, ovaries, adrenal glands are subject to surgical removal.

False PPR

False or LH-RH-independent PPR is understood as the development of secondary sexual characteristics associated with autonomous excess production of steroids by the adrenal glands and gonads. The most common cause of this form of PPR is congenital adrenal dysfunction (CHD). Less commonly, hormonally active tumors originating from the above organs, as well as tumors that secrete hCG (chorionepitheliomas, hepatomas, teratomas).

Congenital adrenal dysfunction is a group of autosomal recessive hereditary diseases caused by genetic defects in steroidogenesis enzymes. The main link in the pathogenesis is a violation of the synthesis of cortisol and / or aldosterone. Persistent cortisol deficiency according to the principle of negative feedback stimulates the secretion of adrenocorticotropic hormone (ACTH), which causes hyperplasia of the adrenal cortex, producing an excessive amount of androgens.

In the vast majority of cases, there is a deficiency of the enzyme 21-hydroxylase, 10 times less often - a deficiency of 11β-hydroxylase. At present, numerous point mutations of genes have been found that determine one or another deficiency, which correlates with the clinic of gluco- and mineralococticoid insufficiency and severe virilization.

At birth, the external genital organs of girls have a heterosexual structure: varying degrees of clitoral hypertrophy, fused labia majora resemble the scrotum, forming a single urogenital opening at the base of the clitoris (urogenital sinus).

The formation of the external genitalia in boys takes place according to the isosexual type: the penis is enlarged, the scrotum is wrinkled and pigmented, erections appear early. In the first years of life, due to the anabolic action of androgens, children grow rapidly, their skeletal muscles develop, their voice coarsens, male-type hair appears on the face, chest, abdomen, limbs. In both sexes, the differentiation of the skeleton is significantly accelerated.

In aldosterone deficiency, the disease is acute. The disease manifests itself from the first weeks after birth and poses a serious threat to health. Clinically, this form is characterized by vomiting, dehydration, and a decrease in blood pressure (BP). In the blood, the amount of sodium decreases and potassium increases, the level of renin is high.

With a deficiency of 11β-hydroxylase, along with the above symptoms, an increase in blood pressure is detected, which can complicate the course of the disease. Girls in prepuberty and puberty lack secondary female sexual characteristics and menstruation.

In the blood, the level of renin is reduced, and sodium can be increased.

Hormonal diagnosis is based on determining the level of 17-hydroxyprogesterone. With 21-hydroxylase deficiency, it is many times higher than normal. In patients with 11β-hydroxylase deficiency, the increase in 17-hydroxyprogesterone is less.

The main goal of treatment is to suppress the excess production of ACTH. For this purpose, glucocorticoids are selected or combined with mineralcorticoids.

Van Wyck-Grombach syndrome occurs in children with long-term undiagnosed primary hypothyroidism. By the time PPR symptoms appear, children have a classic picture of severe hypothyroidism: chondrodystrophic physique, significant growth retardation, muscle hypotonia, a low, rough voice, and psychomotor retardation.

The diagnosis was confirmed by low levels of thyroid hormones (T3 and T4) and a sharp increase in pituitary thyroid stimulating hormone (TSH).

In girls, the first signs of PPR are an increase in the mammary glands, in some with lactorrhea, the appearance of menarche. Adrenarche (pubic and axillary hair growth) is uncharacteristic. All patients have high levels of prolactin, as for gonadotropins (LH and FSH), they moderately increased. Ultrasound examination (ultrasound) of the small pelvis in all cases visualizes polycystic ovaries.

A feature of the PPR clinic in boys with this syndrome is a moderate increase in testicles with a weak androgenization of the body, which corresponds to a moderate increase in testosterone levels.

In both sexes, bone maturation lagged behind biological age.

Replacement therapy with thyroid drugs causes a regression of the symptoms of PPR.

Androgen-secreting tumors of the adrenal glands (androsteromas) are, as a rule, adrenocarcinomas. Rarely seen in children. In the early adolescence the frequency of adrenocarcinomas increases in children with Wiedemann-Beckwith syndrome (visceromegaly, macroglossia, hemihypertrophy) and Li-Fraumeni syndrome (multiple malignant neoplasms).

In children with adrenocarcinomas, abnormal expression of tumor markers and a decrease in the expression of factors that suppress tumor growth, the genes of which are localized on the long arm of the 11th chromosome, were revealed. Abnormalities of this chromosome are found in most patients with adrenocorcinoma.

In boys, a clinical picture is observed according to the type of isosexual sexual development: muscle mass, growth rate, secondary hair growth, erections appear, the timbre of the voice changes. However, the volume of the testicles does not increase.

Girls show signs of virilization: apocrine glands (sweat, sebaceous, hair follicles) are activated, body weight increases due to muscle tissue, the clitoris is hypertrophied. Boys and girls grow faster.

Estrogen-producing tumors of the adrenal glands (corticoestromas) are very rare in children. In girls, in this case, they proceed according to the type of isosexual PPR, and in boys in the clinic, the leading symptom is gynecomastia.

The study of the hormonal profile is characterized by an increase in the level of dehydroepiandrosterone sulfate (DHEA-S) and juvenile levels of LH and FSH. In some cases, the concentration of testosterone, estradiol increases. Ultrasound is used in the diagnosis of adrenal tumors.

Steroid-secreting gonadal tumors are rare in childhood. In older girls, arrhenoblastomas (malignant tumors) are found, located in the cortical layer or hilum of the ovary. Undifferentiated tumors have a more pronounced virilizing effect, while differentiated ones have both a weakly pronounced masculinizing and feminizing effect. A granulosa cell tumor of the ovaries, often of benign origin, secretes a large amount of estrogen, causing isosexual PPR. Excess estrogen causes menstrual syndrome - from scanty to heavy bleeding, pigmentation of the areola, hardening of the glandular tissue, hypertrophy and swelling of the vulva. The amount of estradiol is sharply increased with pre-pubertal LH and FSH levels.

In boys, testosterone-secreting leidigoma is rare. This is a benign tumor that affects one testicle. Outwardly, it is enlarged, bumpy, dense consistency. Androgenization syndrome develops rapidly.

Sertolioma is a neoplasm containing Sertoli cells. In this case, the release of estradiol into the blood increases, which forms gynecomastia in boys, accelerates growth and bone maturation.

The level of gonadotropic hormones in both tumors of the testicles corresponds to the age of the children.

Follicular ovarian cysts are a common cause of PPR in girls. However, they are also found in healthy girls in the prepubertal period. The diameter of these cysts is from 0.5 to 1.5 cm. The presence of a cyst in the ovaries is not a sign of pathology. But in some cases, the cystic tissue begins to prematurely and excessively produce estradiol. As a rule, these cysts are 3-4 cm in size. Follicular cysts may be accompanied by irregular scanty sanious discharge from the genital tract, hypertrophy and swelling of the skin of the vulva, increased vaginal folding, moderate pigmentation and swelling of the nipples. The size of the uterus and bone maturation correspond to the passport age. The cause that causes the formation and persistence of follicular cysts may be a transient rise in gonadotropins (mainly FSH). Ovarian cysts are found on pelvic ultrasound. In most cases, follicular cysts spontaneously regress after 1.5-2 months and the PPR clinic disappears. Cysts are subject to surgical treatment large sizes or flowing with complications.

Incomplete PPR forms

Premature isolated thelarche (PT), an enlargement of the mammary glands in girls, is the most common benign variant of PPR. In most cases, it occurs at the age of 6-24 months in girls who are breastfed, in underweight and premature. Rarely found after reaching 3 years of age.

The reason for the increase in the mammary glands is considered to be a high level of gonadotropic hormones (especially FSH). The peak concentration of FSH after birth lasts up to 6 months and then slowly begins to decline by 2-3 years. IN preschool age in such patients, follicles are detected in the ovaries, reaching the size of adult women. Some authors associate this with dysfunction of the hypothalamic-pituitary system. FSH activates the enzyme aromatase, which leads to increased production of estrogens from testosterone in the granulosa tissue of the follicle. Other causes of isolated thelarche may be periodic estrogen surges or increased sensitivity of the receptor apparatus of the mammary glands to estrogens.

Enlarged mammary glands are palpable on one or both sides. Some girls have moderate estrogenization of the vulva. There are no other secondary sexual characteristics.

With isolated thelarch, the growth rate is not disturbed (5-6 cm per year), the bone age corresponds to the chronological one. Most often, the process regresses on its own and does not require medical intervention, but at the same time, thelarche that appears may be the first sign of true or false PPR, so all girls with thelarche must be re-examined (at least 2 times a year).

If thelarche is combined with accelerated bone age, but there are no other signs of precocious puberty, this condition is assessed as an intermediate form of PPR and requires more careful monitoring (quarterly) with monitoring of ovarian ultrasound, bone age.

Premature adrenarche (PA) is the appearance of isolated pilosis on the pubis and / or armpits in girls under 8 years old, and in boys up to 9 years old. It is more common in girls aged 6-8 years. PA may be a variant of the norm, given that the maturation of the reticular zone of the adrenal cortex begins at 6 years of age. While the secretion of GnRH, responsible for the onset of puberty, starts later. The cause of pubertal hair growth is an increase in the production of dehydroepiandrosterone (DHEA) and its DHEA-S, as well as delta-4-androstenedione, testosterone precursors that stimulate pubic and axillary hair growth. In girls, PA may be associated with excessive peripheral conversion of testosterone to dihydrotestosterone (increased aromatase activity). In the absence of other signs of androgenization of the body - acceleration of growth, maturation of the skeleton, prepubertal size of the uterus and ovaries, and in boys testicles, normal testosterone levels and moderately increased DHEA-S, the prognosis is favorable and sexual development does not deviate from the norm.

However, in some children, PA can be triggered by excessive production of ACTH (hydrocephalus, meningitis, etc.). There is more and more evidence of the association of PA with non-classical forms of congenital adrenal dysfunction (CHD) and, in particular, a deficiency in the activity of the enzyme 21-hydroxylase and less often 3β-hydroxysteroid dehydrogenase.

In the presence of a virilizing disease, clinical signs of androgenization appear: in girls - clitoral hypertrophy, high posterior perineal commissure, hirsutism, development of the muscular system; in boys - voice change, penis enlargement, activation of the sebaceous and sweat glands. These children show accelerated growth and bone age.

Girls with premature adrenarche should be at risk for developing polycystic ovary syndrome. This group of patients requires corrective therapy with glucocorticoids.

Differential Diagnosis

Primary diagnosis is based on a thorough history taking and assessment of the degree of sexual development of the child according to the Tanner-Marshall classification. Early puberty in males in the maternal and paternal family is characteristic of testotoxicosis. The presence in the family of brothers with PPR or sisters with symptoms of virilization is more common in VDKN.

From the anamnesis, it is necessary to find out the time of appearance of secondary sexual characteristics, the speed of their progression. In girls, the degree of development of the mammary glands and areola, the condition of the skin, external genitalia, and the presence of bloody discharge are assessed.

In boys, the degree of masculinization, the presence of pubic and axillary hair, the degree of change in the external genitalia (the size of the penis, testicles).

For both sexes, growth performance is assessed by calculating the coefficient of standard deviation (SD).

Early onset and rapid onset of symptoms are typical of testotoxicosis and hypothalamic hamartoma. Clinical symptoms of hypothyroidism, combined with PPR, suggest Van Wyck-Grombach syndrome.

When a history of congenital anomalies of the central nervous system, trauma, inflammation is indicated, one should think about the cerebral form of PPR.

The study of bone age (X-ray of the hand), more than another indicator that correlates with the stage of sexual development of the child, is mandatory for assessing the degree of PPR. If the bone age is more than 2 SD ahead of the passport age, this indicates an excess of sex steroids. A significant acceleration of bone maturation is characteristic of central forms of PPR, as well as androgen-secreting tumors of the adrenal glands, VDKN. In isolated forms of PPR (premature thelarche and adrenarche), bone age corresponds to chronological age.

The tumor variant of cerebral PPR is excluded using computed tomography (CT) and magnetic resonance imaging (MRI). These research methods are included in the mandatory standard of the survey plan.

Pelvic ultrasonography should be performed in all girls with suspected PPR. The size of the ovaries and uterus should be comparable to the level of sex hormones. Bilateral ovarian enlargement is a reliable sign of the central form of PPR.

Ovarian structure, follicle diameter, fundus-cervix ratio, uterine-endometrial length are important evaluative parameters, but many experts believe that they are not decisive in the differential diagnosis between PT and central forms of PPR. The ovaries may be asymmetrically enlarged in girls with peripheral forms of PPR.

In boys, MRI or CT is preferable for detecting adrenal masses.

To clarify the form of PPR, the levels of gonadotropic hormones, estrogens and androgens are determined. The levels of LH, FSH and estradiol reflect the state of the hypothalamic-pituitary-gonadal system, the concentration of DHEA and DHEA-S - the secretory activity of the adrenal glands.

For differential diagnosis between the central and false forms of PPR, in all cases, a functional test with LH-WG should be performed. With true PPR, the test with Diferelin causes a pubertal response of LH and FSH. In children with peripheral forms of PPR, gonadotropins do not respond to stimulation.

An increase in DHEA-S is characteristic of premature adrenarche. An excess of adrenal androgens is possible with virilizing forms of VDKN, tumors of the adrenal glands and ovaries.

The tumor cause of PPR requires a study for the presence of AFP, beta-hCG, CEA.

Treatment

The main goal of PPR treatment is the elimination of the clinical symptoms of the disease, the normalization of the secretion of steroid hormones that accelerate bone maturation and the closure of growth zones to achieve socially acceptable growth.

Treatment of true PPR involves blocking the impulse secretion of LH-RH. The indication for the appointment of synthetic analogues of GnRH is early age and rapid dynamics of bone maturation. With a slowly progressive disease, this treatment should be carefully approached.

Triptorelin has been clinically tested in Russia. The drug is administered intramuscularly, the frequency of administration is 1 time every 28 days. Children weighing less than 20 kg - 1.875 mg, more than 20 kg - 3.75 mg.

Normalization of the FSH level is noted after 3 weeks, a decrease in the size of the testicles and uterus from the 6th month of treatment. Inhibition of growth rate and skeletal maturation is observed by the end of the 1st year of treatment. The growth forecast is improving. The drug is well tolerated by patients. During treatment, constant monitoring of changes in bone age, growth rate, standard deviation coefficient (SDS) of growth is necessary.

The data confirm the expediency of drug-induced isolated thelarche against the background of reduced thyroid function, with Van Wyck-Grombach syndrome, hormone replacement therapy with thyroid hormones is indicated. The criterion for the adequacy of treatment are normal readings of TSH and free T4.

With McCune-Albright-Braytsev syndrome, pathogenetic therapy has not been developed. In cases of frequent massive bleeding, it is possible to use cyproterone in a daily dose of 70-100 mg. The drug has an antiproliferative effect on the endometrium, which leads to the cessation of menstruation. To reduce hyperestrogenemia, an inhibitor of aromatase activity is used - testolactone at a dose of 20-40 mg / kg per day or tamoxifen, which blocks estrogen receptors.

Tactics for the treatment of testotoxicosis involves the appointment, firstly, medroxyprogesterone (inhibition of testosterone synthesis), secondly, ketoconazole (inhibition of the synthesis of hormones of the sex glands and adrenal glands) or a combination of testolactone and spironolactone (aromatase inhibition and blockade of androgen receptors). Ketoconazole is prescribed at a dose of 30 mcg/kg per day per os. The use of the drug may be accompanied by adrenal insufficiency and impaired liver function. With a late start of treatment, with a bone age that has reached 12-13 years, a picture of true PPR may develop, in this case, therapy with synthetic analogues of LH-RH is performed.

Functional ovarian cysts in most cases undergo spontaneous regression within four months. With the formation of follicular cysts in utero or in newborn girls, treatment is usually not carried out. Resection of the ovary or laparoscopic exfoliation with suturing of the walls is performed when cysts with a diameter of more than 8 cm are detected.

Surgical methods of treatment are used in children with PPR, which develops against the background of hormonally active tumors of the adrenal glands, ovaries, and volumetric formations of the central nervous system, however, in some patients, the removal of neoplasms does not lead to regression of PPR. Hypothalamic hamartoma is removed only for strict neurosurgical indications. In the presence of focal and cerebral symptoms, surgery or radiation therapy corresponding to the type of tumor is performed. It must be remembered that radiation exposure or surgical intervention on the bottom of the 3rd ventricle can provoke PPR. For this reason, such children should be constantly monitored by an endocrinologist. In cases where the leading clinical manifestation of the disease is only the symptoms of PPR, only conservative treatment is possible.

In girls with heterosexual precocious puberty against the background of VDKN, if necessary, surgical correction of the external genital organs is performed. A penis-shaped or hypertrophied clitoris is recommended to be resected immediately after diagnosis, regardless of the child's age.

Further management of patients

All children diagnosed with precocious puberty should be monitored continuously (at least once every 3-6 months) before and throughout the entire period of physiological puberty. Treatment of true PPR with triptorelin is carried out continuously until the onset of puberty, since the cessation of its administration causes the resumption of the disease. The study of bone age is controlled with any form of PPR once a year.

Literature

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V. V. Smirnov 1 doctor of medical sciences, professor
A. A. Nakula

GBOU VPO RNIMU them. N. I. Pirogov Ministry of Health of the Russian Federation, Moscow

abstract. Precocious sexual development is the frequent violation of puberty in children and in their etiology and pathogenesis is a heterogeneous disease. The article summarizes the current data on the causes of violations of formation of the hypothalamic-pituitary-gonadal relationships cause premature sexual development. A classification of diagnosis and treatment of this pathology.

• true forms.
  • For girls:
    • breast enlargement;
    • pubic hair;
    • acne (acne);
    • menstruation that is regular. However, more often in girls with true precocious puberty, menstruation may be absent for 5-6 years after the onset of secondary sexual characteristics.
  • For boys:
    • testicles and penis increase in size;
    • acne, increased sweating, a specific pungent smell of sweat appear;
    • sexual hair growth;
    • voice roughens, muscle mass increases.

• False forms.
  • For girls:
    • menstruation begins simultaneously with an increase in the mammary glands. The discharge is erratic, may be profuse or "spotting";
    • isolated thelarche (premature enlargement of the mammary glands) - the only secondary sexual characteristic in girls under 2 years old;
    • isolated adrenarche (pubic hair in girls from 3 to 8 years old, not combined with the development of other signs of puberty).
  • For boys:
    • progression of secondary sexual characteristics;
    • the pre-pubertal (before puberty) volume of the testicles is preserved.

In all forms of the disease in children of both sexes, growth accelerates, reaching 10-15 cm per year. Growth acceleration can be noted 6-12 months before the appearance of secondary sexual characteristics. There is also a premature closure of the growth plates in the bones and a significant decrease in the final growth.

Forms

• True or central- due to premature activity of the hypothalamic-pituitary system (a region of the brain that regulates the activity of the endocrine glands). An increase in the production of sex hormones by the sex glands in this case is a consequence of stimulation of the internal sex glands by gonadotropins (hormones of the pituitary gland (brain area) that regulate the functioning of the sex glands).
• False, or peripheral- due to premature production of sex hormones by tumors of the gonads or adrenal glands, independent of the production of gonadotropins.
• Gonadotropin-independent- premature sexual development, in which the activation of the activity of the gonads is due to genetic disorders.

Depending on the degree of manifestation of premature sexual development, it can be:

• complete- an increase in the mammary glands in girls, penis and testicles in boys, hair growth of the armpits, pubic area, change in voice timbre, formation of a figure according to the female type in girls (breast and hip enlargement) and according to the male type in boys (increase in the width of the shoulder girdle, reduction of fat deposition on the buttocks), growth acceleration;
• partial or incomplete- characterized by an isolated development of hair growth in the axillary and pubic areas (premature pubarche) and an isolated increase in the mammary glands (premature thelarche).

Causes

• Causes of true precocious puberty.
  • Tumors of the central nervous system.
  • Non-tumor brain damage (eg, birth trauma, meningitis (inflammation of the meninges)).
  • congenital syndromes:
    • neurofibromatosis type 1 (hereditary disease predisposing to the occurrence of tumors of the nervous system);
    • Russell-Silver syndrome (a syndrome accompanied by moderate production of gonadotropins (hormones that regulate the functioning of the gonads) from early childhood);
    • Van Weik-Grumbach syndrome (develops in children with primary hypothyroidism (low levels of thyroid hormones)).
  • true premature puberty with prolonged exposure to sex hormones.
  • Idiopathic (the exact cause of precocious puberty has not been established).
• False precocious puberty.
  • For boys:
    • testicular tumors (leidigoma);
    • tumors of the adrenal glands (androsteromas);
    • congenital dysfunction of the adrenal cortex (changes in the body that develop with excessive production of androgens (male sex hormones) or estrogens (female sex hormones) by the adrenal cortex).
  • For girls:
    • ovarian tumors;
    • adrenal tumors;
    • ovarian cysts.
• Gonadotropin-independent forms:
  • McCune-Albright-Braytsev syndrome (premature puberty occurs as a result of a congenital gene mutation that causes uncontrolled activation of estrogen production (increases the production of female sex hormones));
  • testotoxicosis (activation of testicular function without the participation of gonadotropins (hormones that regulate the functioning of the sex glands)).

Diagnostics

• Analysis of complaints and anamnesis of the disease, general examination.
  • Early onset of puberty (8-9 years):
    • breast enlargement in girls;
    • menses;
    • an increase in the size of the testicles and penis;
    • sexual hair growth;
    • voice coarsens, muscle mass increases;
    • acne (acne);
    • acceleration of growth up to 10-15 cm per year.
  • Comparison of the degree of physical and puberty according to Tanner (a scale describing the puberty of children, adolescents. Tanner's stages are based on the observation of changes in external primary and secondary sexual characteristics: changes in the size of the breasts, male and female genital organs, development of pubic hair) with age standards.
• Laboratory and instrumental research methods.
  • Determination of the level of follicle-stimulating and luteinizing hormones (hormones necessary for normal reproductive function).
  • Determination of levels of prolactin, thyroid-stimulating hormone (pituitary hormones).
  • Determination of levels of estradiol (female sex hormone), testosterone (male sex hormone), cortisol (adrenal hormone), 17-OH-progesterone (an intermediate product of cortisol synthesis), dehydroepiandrosterone sulfate (adrenal hormone), free thyroxine and triiodothyronine.
  • Carrying out tests that stimulate and suppress the production of steroid hormones:
    • a test with a synthetic analogue of gonadotropin-releasing hormone (a hormone that regulates the synthesis of follicle-stimulating and luteinizing hormones);
    • a small test with glucocorticoids is performed in girls with premature pubarche (hair growth of the pubic area) when an increased content of 17-OH-progesterone and / or dehydroepiandrosterone sulfate and testosterone in the blood is detected. Normally, there is a decrease in the level of 17-OH-progesterone, dehydroepiandrosterone sulfate and testosterone by 50% or more. The absence of hormone concentration dynamics suggests the presence of an androgen-producing tumor.
  • Ultrasound of the internal genital organs with an assessment of the degree of maturity of the uterus and ovaries in girls.
  • Ultrasound of the mammary glands, thyroid gland and adrenal glands.
  • X-ray of the hand and wrist joint with determination of the biological age of the child.
  • Electroencephalographic and echoencephalographic study with the identification of non-specific changes (the appearance of a pathological rhythm, increased convulsive readiness), most often accompanying precocious puberty against the background of disorders of the central nervous system.
  • Magnetic resonance imaging (MRI) of the brain in order to exclude volumetric formations of the brain.
  • CT, MRI of the adrenal glands to exclude mass formations.
  • Molecular genetic testing to detect genetic mutations.
• Consultation, (for girls), (for boys) is also possible.

Treatment of precocious puberty

• Gonadotropin-dependent precocious puberty:
  • long-acting analogs of gonadotropin-releasing hormone (a hormone that regulates the reproductive system);
  • progestogens are used to prevent uterine bleeding in girls.
• Gonadotropin-independent precocious puberty (isosexual), premature isolated thelarche (breast enlargement). Annual observation and temporary abstinence from vaccinations in girls with premature thelarche are shown, given the possibility of breast enlargement after their implementation.
• Premature pubarche (hair growth of the pubic area). Carry out preventive measures aimed at the formation of a stereotype healthy eating and prevention of weight gain:
  • reduction in the diet of foods high in carbohydrates (sugar, sweets, jam) and fats (butter, fatty meat, lard);
  • regular exercise;
  • observance of the duration of night sleep for at least 8 hours.
• Gonadotropin-independent precocious puberty (heterosexual): glucocorticoids (adrenal hormones).
• Surgical methods of treatment are used in children with precocious sexual development, which develops against the background of hormonally active tumors of the adrenal glands, ovaries, as well as volumetric formations of the brain (neoplasms are removed).

Complications and consequences

In the absence of treatment:

• infertility (in the future);
• osteoporosis (decrease in bone density);
• increased arterial (blood) pressure;
• obesity;
• psychological complexes among peers due to the early development of secondary sexual characteristics (breast enlargement in girls, hair growth of the armpits and face in boys, etc.);
• in patients with neoplasms, there is a high risk of malignancy (degeneration into cancer) of tumors. Irradiation of intracranial tumors can lead to the development of pituitary insufficiency (a disease that develops as a result of a violation of the secretion of pituitary hormones (a region of the brain that regulates the activity of the endocrine glands), which is manifested by a lack of function of the organs of the peripheral endocrine system (thyroid gland, adrenal glands, gonads)).

They say about premature sexual development of girls when they have sexual characteristics before the age of 7 - the mammary glands grow, hair is visible on the pubis and armpits, and female forms begin to actively form. In addition, the girl has early menstruation. This process is not the norm. Puberty should occur from 12 to 14 years of age, a year difference is allowed. If mothers notice all the signs in little girls, it is worth being examined to prevent various consequences.

Symptoms

You can suspect something is wrong by the following signs: in little girls who are not even 7 years old, their hips are noticeably rounded, an hourglass figure is noticeable, irregular menstruation occurs, and the child’s growth is slowed down.

In medicine, such forms of the disease are distinguished.

true sexual development

It is provoked by increased production of hormones - the pituitary gland, the hypothalamus, which stimulate the work of the girl's ovaries. In the case of complete true sexual development, the girl has all the symptoms described above. If the child has an incomplete true form, the signs are still the same, only menstruation begins to go from the age of 10.

Ovarian sexual development

The problem is associated with increased production of sex hormones. In this case, only disruptions in menstruation are of concern, while other signs do not appear.

hereditary form

The girl has no health problems at all, only puberty occurs 2 years earlier than her peers: the first menstruation appears, the mammary glands grow faster.

Heterosexual form

One of the unpleasant pathologies in which a girl begins to form sexual characteristics characteristic of men. Puberty here is associated with an increased amount of male hormones, it can still begin in utero. A girl can have male-type external genitalia, while there is a uterus, ovaries. This pathology is explained by adrenogenital syndrome - a disease of the adrenal glands.

Causes

Most often, the pathology is associated with heredity, it is transmitted from mother to daughter. If so, the girl's reproductive function will not be impaired. It is more dangerous when the disease is associated with disorders of the pituitary gland, hypothalamus, and hormonal excess. The main factors include:

• Sequelae of traumatic brain injury.
• The innate structure of the brain.
• Complication after a virus, bacterial infection.
• A brain tumor.
• Poison poisoning.
• Radiation therapy.
• Cyst, ovarian cancer.

As a rule, girls have problems with puberty if their mother during pregnancy:

• used narcotic substances, alcohol.
• She suffered from endocrine disorders.

Diagnostics

The doctor must collect a medical history, find out when the first menstruation began, what kind of discharge the girl has. He is also interested in when the breasts began to grow, the first pubic hair appeared.

Then the doctor analyzes the genetic predisposition. The doctor is interested in who had violations of this nature - grandmothers, sisters, mothers or aunts, cousins.

The mother's medical history is also important. The doctor asks her about the transferred gynecological diseases, operations, as well as the number of abortions, pregnancies.

After the interview, the doctor examines the child, feels the genitals, he is interested in the ovaries, cervix, size, the presence of pain. During a general examination, attention is drawn to secondary sexual characteristics:

• Body type.
• Striae.
• Hair growth.
• Discharge from nipples.
• How do the mammary glands develop, is there any discharge from the nipples.

It is important for the doctor to determine the bone age for this, an x-ray is prescribed. This examination provides an opportunity to learn about age-related disorders. Equally important is the analysis of hormones. Most drawn to the sex hormones, pituitary, adrenal hormones. An x-ray of the skull can be taken to find out if there is a tumor of the hypothalamus, pituitary gland.

To exclude anatomical abnormalities of the brain, an MRI is prescribed. In addition, the girl must undergo an ultrasound scan of the female organs, where they carefully check whether there are anomalies of the genital organs, cysts, or a tumor.

In severe cases, laparoscopy using an endoscope is prescribed. This examination is necessary to check the fallopian tubes, uterus, ovaries. If cancer is suspected, a biopsy may be ordered, during which tissue samples from the ovaries are examined.

Important! In addition to the gynecologist, the girl is examined by an endocrinologist.

Treatment Methods

The course of therapy depends on the cause of premature sexual development. Recommended:

• Diet to help you regain weight.
• Consultation with a psychotherapist, taking sedatives.
• Taking medications to reduce the production of the pituitary gland. After taking the drug, the ovaries do not mature so quickly and actively work, thus sexual development slows down. If you stop taking the medicine, maturation is activated again.
• Operation in case of diagnosis of a tumor of the ovaries, pituitary gland, adrenal glands, hypothalamus, as well as congenital anomalies.

Important! The hereditary form is not treated, but the girl must be registered with a gynecologist, always be under supervision.

Consequences

If you do not start treating the child in a timely manner, everything will end:

• Low stature.
• Change in body proportions short legs, arms, long body, wide chest.

Most often, sexual disorders are associated with the pathology of the ovaries, brain, adrenal glands, so timely diagnosis and treatment is necessary.

Prevention

It is easier to prevent pathology than to watch your child suffer, so follow these rules:

• Watch the girl's diet. In her diet should be foods rich in fiber - fruits, vegetables, herbs. Do not give your child fried, hot, canned, spicy.
• Regularly observe a newborn child with a pediatrician, endocrinologist.

Take good care of your child's health!

Why do children sometimes enter puberty much earlier than their peers? Doctors and a psychologist shared their opinions.

Modern children grow up earlier, this fact is noted by many doctors. But what does premature sexual development mean, how does it happen, what features should parents pay attention to?

Several experts answered these and many other questions for Letidor at once: a gynecologist, endocrinologists and a psychologist.

Elena Alexandrovna Pisareva

What is considered early puberty

Under early or premature puberty understand the appearance of secondary sexual characteristics in girls before 8 years, in boys before 9 years.

Secondary sexual characteristics are an increase in the mammary glands (in girls) and an increase in the volume of the testicles (more than 4 ml) and the size of the genitals (in boys), the appearance of pubic and axillary hair growth. There may be an early appearance of one or more components.

The cause of premature puberty may be the early activation of the production of sex hormones.

There are two conditional groups.

The first includes states when the appearance of signs of early maturation is temporary and passes on its own without our intervention. It is not considered a pathology and does not require treatment.

For example, a condition is often encountered when in girls in the first years of life there is an increase in the mammary glands, the so-called "isolated thelarche", which later disappears.

The second group includes conditions when the production of sex hormones began ahead of time and will not stop without treatment, and the signs of puberty will progress.

To understand what option we are dealing with and whether treatment is necessary, you need to undergo a specialized examination and contact a pediatric endocrinologist.

Can we say that this is a modern trend

Problems of early puberty have always been. They are more common among girls. There is evidence of earlier puberty in children living in the southern regions, but even among them, early puberty can be pathological.

How does this affect the growth of the child?

With early puberty, there is an acceleration in growth rates, and children with this condition tend to be taller than their peers. However, due to the excessive production of sex hormones, the growth zones close very quickly, and as a result, the child may be low relative to peers.

With closed growth zones, we cannot change the height of the child.

Also, with early puberty, given the increased secretion of hormones, social maladjustment of the child is possible.

Is it hereditary

Early puberty, as a rule, is not hereditary. In world practice, only isolated cases of inheritance in the family are known.

Advice to parents

If you notice the early appearance of secondary sexual characteristics, which were mentioned above, you should contact a pediatric endocrinologist to clarify the causes and determine the need for treatment or, possibly, the choice of observational tactics.

By the time of admission, be sure to prepare growth indicators for previous observation periods so that the doctor can assess the dynamics of development.

Comments by Natalya Alexandrovna Chekenova, gynecologist-endocrinologist, pediatric gynecologist, reproductologist, ultrasound diagnostician (14 years of experience)

Natalia Alexandrovna Chekenova

Causes of early sexual development

Violation of sexual development, like any pathological condition, cannot arise just like that. Many factors can precede this, for example:

⁃ chronic intrauterine fetal hypoxia;

⁃ acute hypoxia in childbirth;

⁃ traumatic brain injury in a child at any age;

⁃ tumors of the central nervous system;

⁃ tumors and hyperplasia of the adrenal glands;

⁃ tumors and dysfunction of the gonads;

⁃ thyroid disease (primary hypothyroidism);

⁃ congenital genetic pathology (Russell-Silver, McCune-Albright-Braytsev syndromes).

If suddenly you realize that your child began to grow and mature faster than all his peers - the girls began to grow mammary glands, hair, behavior changed, menstruation began; the boys had an increase in the genitals, hair growth, coarsening of the voice, polylution, which means it's time to see a doctor.

The deeper and more severe the child's lesions are, the more severe and earlier the manifestations will be.

Condition Diagnostics

First of all, the specialist needs to get important information about the child's health:

⁃ collection of anamnesis (how pregnancy and childbirth occurred and proceeded);

⁃ examination of the child;

⁃ blood chemistry;

⁃ blood hormones;

⁃ functional tests (with gonadotropins);

⁃ genetic research;

⁃ Ultrasound of the genital organs, adrenal glands, brain;

⁃ electroencephalography;

⁃ X-ray of the hands;

⁃ MRI, etc.

The child must be examined by narrow specialists. After that, the doctor will be ready to decide on the tactics of treatment. Sometimes this requires more frequent monitoring, and sometimes correction with serious drugs, surgical treatment.

Yulia Anatolyevna Galkina

What other factors affect sexual development

The physiological development of a person occurs in each individual. It is related to the following factors:

hereditary (how his parents, grandparents, brothers, sisters developed);

ethnic (developmental features of different nationalities);

socio-economic (under what conditions a person develops, his standard of living);

nutritional factors (nutrition, eating habits);

climatic conditions of the living environment;

the presence of various chronic diseases of the endocrine system (dysfunction of the thyroid gland, adrenal glands, pathology of the ovaries, testicles) and the use of hormonal drugs.

When evaluating sexual development, it is necessary to take into account the nationality of the child, belonging to a particular race, climatic and social conditions of his residence.

For middle lane In Russia, the beginning of puberty (puberty) in boys 9-14 years old, in girls 8-13 years old.

In peoples living in a hot climate, puberty of children occurs earlier.

But there is also a hereditary predisposition for the onset of puberty, so the age of its onset in parents, and possibly grandparents, is taken into account.

What are the signs that parents can understand that development is premature

It is possible to suspect early sexual development in a child already with visible changes in his body that are not typical for his peers:

a jump in growth of more than 10-12 cm per year, which may be accompanied by weight gain;

hair growth in the armpits, on the pubis;

increased greasiness of the skin and hair of the head;

increased sweating, the smell of sweat may change;

skin rashes, acne, seborrhea of ​​the scalp;

in girls: enlargement of the mammary glands, darkening of the halo of the nipples, the formation of a more feminine figure (the appearance of a waist, an increase in hips), the appearance of menstruation.

If sexual development begins ahead of time, the child's body is not ready for the appearance of such an amount of hormones.

He does not undergo psychological adaptation. Often such children experience ridicule from their peers. They can withdraw into themselves, experience a sharp change in mood, tantrums, aggression. Subsequently, there may be a violation of sexual behavior.

Which doctors should be contacted

In each case, the cause of premature sexual development is individual. It is not an independent pathology, but occurs as a result of the manifestation of certain diseases in children under 6-7 years old. Therefore, an examination by a number of specialists is required: a pediatrician, a pediatric endocrinologist, a pediatric gynecologist, a neurologist, an ophthalmologist.

I also recommend the following tests: blood test for hormones (LH, FSH, estradiol, prolactin, TSH, free T3, free T4, cortisol, testosterone, 17-OH progesterone, DEA-c), MRI of the brain (to rule out brain tumors brain, cysts, hydrocephalus), CT or MRI of the kidneys and adrenal glands (to rule out adrenal enlargement, tumors), ultrasound of the pelvic organs (to rule out cysts, ovarian tumors, adenomatous nodes), as well as assess bone age.

Victoria Romanova, practicing psychologist, leading trainer of the ALMA Academy, female trainer, life coach, master of the Indo-Tibetan method of treatment, comments

Victoria Romanova

Today's teenagers mature earlier than their parents once did, both physically and psychologically. Here's what parents need to know.

Physiological and psychological maturation of the child

From a physiological point of view, early puberty is expressed in the formation of secondary sexual characteristics: the genitals increase, active hair growth on the body begins, and in girls - breast growth.

Normally, these processes occur when the child reaches 10 years of age.

If secondary sexual characteristics appear earlier, it is best to consult a doctor to rule out possible diseases brain or hormonal system of the body.

In psychological terms, the early puberty of children consists of three phases: autosexuality, homosexuality and heterosexuality. It must be understood that every child goes through them, regardless of gender, race, religious or other beliefs of the parents.

Only the correct attitude of adults to this process will help the correct formation of sexuality in a child.

Let's take a closer look at these stages.

autosexuality

Autosexuality is expressed in the fact that the child begins to explore his body, touch it, play with the genitals. The kid does not see anything bad and shameful in this, for him this is only a stage of getting to know his own body, and globally - the initial phase of the formation of his sexuality.

At this moment, the main task of parents is not to frighten the child, not to shame, and even more so not to punish him for his excessive interest in certain parts of the body.

On the contrary, you need to unobtrusively explain to the baby how boys and girls work, without going into unnecessary details. To help moms and dads - a huge amount of children's literature on this topic with vivid illustrations that are understandable to a child of any age.

However, more often than not, parents who were brought up in strictness and are themselves sexually illiterate start to panic. It seems to them that such games testify to the abnormality of the baby. They punish the child, forbid him to touch himself, scream. As a result, the baby feels bad, spoiled, unworthy of the love of mom and dad.

Ultimately, all this leads the grown child to serious problems in the sexual life.

As a rule, children who are prevented from going through the phase of autosexuality by their parents are guided only by the satisfaction of their own needs.

Sex for them is just a mechanical process of self-satisfaction. Masturbation becomes the only source of sexual satisfaction, even during sex with a partner. Such people are unhappy personal life, it is difficult for them to find a soul mate, they cannot please their partner.

Homosexuality

If a child has moved to the homosexual phase of sexual development, then his parents did everything right at the first stage. The second phase is just as natural as the first.

Puberty is arranged according to the principle "from simple to complex". First, the child studies the simplest and most accessible “material” for him - his own body. Then - an identical body, but of another person. And only after that - the body of a person of the opposite sex.

That is why there is nothing dangerous in the homosexual stage of children's development.

It is very difficult for parents to go through this stage and adequately respond to the behavior of the child. They try to ban such contacts, punish children, condemn them.

At the same time, society itself creates the prerequisites for homosexuality, dividing the younger generation by gender in everyday life. These are sports sections only for boys or only for girls, and closed educational establishments where children of different sexes do not study together, and much more.

But it is precisely the constant bans on communication with representatives of the same sex that provoke a teenager to rebellion.

As a result, he does not go through the stage of homosexuality and can “get stuck” in it for life.

Parents should treat the child with understanding, talk a lot with him about everything that worries him. If adults are of the "old school" and it is difficult for them to have such conversations, then you need to turn to competent psychologists who will help the teenager move to the heterosexual stage of sexual development.

Heterosexuality

If both initial phases of puberty are successful and adults do not interfere with the natural development of the child's sexuality, then he enters the phase of heterosexuality.

It is characterized by interest in the opposite sex, the formation of sexual attraction. At this stage, teenagers begin to comprehend the characteristics of the opposite sex, learn love and harmony in relationships. This is the final point of becoming a healthy sexuality.

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