Premature sexual development in girls. Why does puberty begin prematurely in some children? Signs of early puberty

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Precocious sexual development (PPD) is the appearance of any secondary sexual characteristics in girls at an age that is 2 standard deviations less than the average age of their appearance in the population.

Taking into account the stages of sexual development, breast growth can be considered premature at the age of less than 8.9 years, pubic hair growth at the age of less than 9.0 years, and menarche at the age of less than 10.8 years.

In most countries of the world, PPD refers to the appearance of one or more secondary sexual characteristics in girls under 8 years of age.

The term “early puberty” is used when breast growth begins in girls between the 8th and 9th years of life.

Epidemiology

The frequency of PPR among all gynecological pathologies of childhood varies from 2.5 to 3%. In 1-3% of girls under the age of 8 years, the development of certain secondary sexual characteristics is noted.

The most common benign variant of PPR in girls is premature isolated thelarche, which occurs in approximately 1% of girls under 3 years of age. This form of PPR often occurs in premature babies.

Classification

There is currently no unified classification of PPR.

The working classification of PPR syndrome is based on a pathogenetic principle that takes into account the primary localization of the process in the hypothalamus-pituitary-gonads-adrenal system.

In accordance with this classification, there are:

■ central (true) forms of PPR;

■ peripheral (false) forms of PPR. A separate group includes gonadotropin-independent forms of PPR caused by genetic disorders (McCune-Albright-Braitsev syndrome).

With all of the listed forms of the disease, all the signs of the onset of puberty are noted: secondary sexual characteristics appear, the volume of the gonads increases, and the rate of bone growth and maturation increases. Clinical variants of PPR, which are characterized by this symptom complex, are defined as complete.

In addition, there are so-called incomplete (partial) forms of PPR, characterized by:

■ isolated hair growth corresponding to sex - isolated pubarche;

■ isolated enlargement of the mammary glands - isolated thelarche.

There are also variants of PPD that do not clearly fit into any of the listed forms of the disease:

■ PPR due to decompensated primary hypothyroidism;

■ PPR during late treatment of congenital adrenal dysfunction (CAD).

Etiology and pathogenesis

The central (true, gonadotropin-dependent) form of PPR is formed as a result of premature activation of the hypothalamic-pituitary-gonadal system as a result of various organic or functional disorders of the central nervous system. An increase in the secretion of sex hormones by the gonads in this case is a consequence of stimulation of the gonads by gonadotropins.

The peripheral (false) form of PPR is caused by hypersecretion of sex hormones by hormone-secreting tumors of the gonads and adrenal glands and does not depend on the level of gonadotropic hormones.

Depending on the hormones produced, the peripheral form of PPR can occur in an iso- or heterosexual type.

The most common cause of false PPR is CAH, a disease based on a violation of the synthesis of steroid hormones in the adrenal cortex due to a congenital defect in the 21-hydroxylase and 11β-hydroxylase enzyme systems, which are involved in the synthesis of gluco- and mineralocorticoids. In the classical form of CDCN, a sharp increase in the level of androgens in the blood serum leads to the formation of heterosexual type PPR. However, with prolonged elevated levels of sex hormones in the blood serum, transformation of the gonadotropin-independent form into a gonadotropin-dependent form is possible. In these cases, PPR proceeds according to the isosexual type.

The main mechanism for the development of McCune-Albright-Braitsev syndrome is considered to be a mutation of the Gsa protein gene. The appearance of mutant forms of protein G leads to constant activation of cyclic adenosine monophosphate, which does not require the presence of tropic hormones, and autonomous hormonal secretion, in particular estrogens, in the persistent follicle.

The cause of the development of isolated premature thelarche is considered to be a transient increase in the level of FSH, due to the immaturity of the system for regulating the secretion of gonadotropic hormones. Transient activation of FSH secretion can lead to maturation of the ovarian follicle and short-term estrogenic stimulation.

Premature isolated pubarche may be a manifestation of:

■ non-classical form of VDKN;

■ dysregulation of enzymatic processes in the ovaries, associated with hyperinsulinemia or manifested in older girls by the formation of PCOS. True premature menarche is extremely rare. The most common cause of bleeding from the genital tract in girls without any signs of puberty are tumors of the vagina and external genitalia (vulva), pathology of the cervix, foreign bodies in the vagina, trauma to the external genital area.

Clinical signs and symptoms

Full forms of PPR of central origin are characterized by:

■ premature appearance of all signs of maturity of the reproductive system;

■ acceleration of linear growth and differentiation of the skeleton (ahead of bone age by more than 2 years) or closure of growth zones in girls under 8 years of age while maintaining the physiological rate of maturation of other organs and systems.

At the same time, patients with complete forms of PPR of peripheral origin are characterized by a perverted sequence of appearance of signs of puberty. Thus, with undiagnosed CAH in girls, hair growth corresponding to gender first appears (normally, the first symptoms of the onset of puberty in girls are engorgement and enlargement of the mammary glands); with follicular ovarian cysts, menarche may appear after enlargement of the mammary glands (normally, after enlargement of the mammary glands girls develop sex-appropriate hair before menarche occurs).

In the classic form of CAH, pronounced enzymatic defects lead to hyperandrogenemia and virilization of the external genitalia even in utero. By the time a girl is born, the external genitalia have a bisexual structure (false female hermaphroditism).

Most children are characterized by hyperpigmentation of the skin. From the first months of life, these patients experience a significant acceleration in physical development and an increase in bone age. The degree of bone differentiation significantly outstrips the acceleration of linear growth; growth zones may be close to closing by the age of 9-10 years.

Mineralocorticoid deficiency is detected in 75% of patients with 21-hydroxylase deficiency. With a pronounced deficiency of mineralocorticoids in children, hyponatremia, hyperkalemia, acidosis, and fluid loss develop already in the first weeks of life, before the appearance of symptoms of androgenization. Dehydration is aggravated by frequent massive vomiting. With minor mineralocorticoid deficiency, the child may only have an excessive need for NaCl, but stress or severe intercurrent illness can lead to the development of a crisis.

With PV-hydroxylase deficiency, the pathognomonic symptom is arterial hypertension.

It is somewhat more difficult to diagnose the non-classical variant of CACN, which debuted before the onset of the physiological puberty and is characterized by premature isolated pubarche with an increase in basal and stimulated levels of adrenal androgens.

Clinical manifestations of McCune-Albright-Braitsev syndrome:

■ wave-like course of premature sexual development;

■ multiple fibrous osteodysplasia;

■ geographical coffee-colored spots on the skin;

■ ovarian cysts;

■ various endocrine pathologies. Some forms of PPR during the development of the pathological process can transform from a partial to a complete variant, while others are capable of spontaneous regression.

Mandatory examination methods:

■ physical examination;

■ determination of blood serum levels of LH, prolactin, FSH, estradiol, TSH, free T3, free T4;

■ Ultrasound of the genital organs;

■ determination of bone age;

If the level of LH, FSH, or estradiol in the blood serum increases, it is necessary to perform a test with a GnRH agonist: I Triptorelin IV 25-50 mcg/m2, once. The level of LH and FSH in the blood serum is determined before the injection, 30, 60, 90 and 120 minutes after it:

■ an increase in LH levels by more than 10 times from the initial values ​​indicates a central form of PPR;

■ a predominant increase in FSH levels indicates the absence of a central form of PPR, but is pathognomonic for isolated premature thelarche.

Additional examination methods:

■ in the central form - MRI of the brain;

■ for isolated thelarch - ultrasound of the mammary glands, thyroid gland;

■ with isolated pubarch:

Ultrasound, MRI of the adrenal glands;

The daily rhythm of hormone secretion (at 8.00 and at 23.00 the content of cortisol, testosterone, 17-OP, DHEA-S in the blood serum is determined).

At high levels of these hormones, a small test with dexamethasone is indicated:

Dexamethasone 0.5 mg 4 times a day, 2 days. Before starting dexamethasone and on the morning of the 3rd day, the level of 17-OP, DHEA-S, and testosterone in the blood serum is determined. The test allows you to exclude a tumor process in the adrenal glands.

Test with a synthetic analogue of ACTH

carried out with elevated serum levels of 17-OP, DEA-S and reduced or normal levels of cortisol.

Due to a possible sharp increase in blood pressure and the development of allergic reactions, the test is carried out only in a hospital setting:

Tetracosactide IM 0.25-1 mg, once at 8.00-9.00.

After receiving the results, the coefficient D is calculated:

D = 0.052 x 17-OP (after 9 hours) + 0.005 x cortisol/17-OP (initial) - 0.018 x cortisol/17-OP (after 9 hours).

When D is more than 0.069, there is a hidden enzymatic deficiency of 21-hydroxylase in the adrenal glands.

This test allows us to exclude the non-classical form of CAH.

Differential diagnosis

Differential diagnosis of various forms of PPR is carried out based on the results of the examination (Table 41.1).

Table 41.1. Differential diagnosis of various forms of premature sexual development

Premature isolated thelarche must be differentiated from:

■ true PPR;

■ transient PPR against the background of persistence of the follicle.

In case of premature isolated adrenarche, it is necessary to carry out differential diagnosis between the following diseases:

■ non-classical form of 21-hydroxylase deficiency;

■ viril form of VDKN;

■ tumors of the adrenal glands and ovaries.

Main objectives of treatment:

■ suppression of the development of secondary sexual characteristics that cause emotional and psychological problems in the child and parents;

■ reduction in the rate of bone maturation, which improves growth prognosis.

For false forms caused by follicular cysts or hormone-producing tumors of the ovaries/adrenal glands that persist for more than 3 months, as well as intracranial tumors (except for hypothalamic hamartoma), surgical treatment is indicated.

There are 2 main requirements for pharmacotherapy of various forms of PPR:

■ absence of toxic effects of the drugs used;

■ reversibility of the hormone-suppressing effect.

Full form of precocious puberty of central origin

It is advisable to begin pharmacotherapy for the full form of PPR of central origin no later than 11.5 years of bone age.

Criteria for prescribing GnRH agonists:

■ rapid progression of disease symptoms (an increase in bone age that is 2 years or more ahead of the actual age, an increase in growth rate by more than 2 standard deviations over the previous year);

■ presence of repeated menstruation in girls under 7 years of age.

Currently, of the GnRH analogues, the only officially approved drug used for the treatment of true PPR is triptorelin.

LS of choice:

Danazol orally 100-200 mg 1 time per day continuously, no more than 6-8 months.

Premature sexual development due to congenital dysfunction of the adrenal cortex

In the heterosexual type of PPR against the background of the classic form of CAH without signs of NaCl loss, lifelong therapy is indicated according to the following scheme:

Hydrocortisone orally 10-20 mg/m2/day in 3-4 doses (at 7.00, 12.00, 17.00 and 3.00) or

Dexamethasone orally 0.5-0.75 mg/m2, once at night (only for closed growth areas) or

Prednisolone orally 2.5-5 mg/m2/day with 2/3 of the daily dose taken in the morning (at 6.00-8.00) and 1/3 in the early evening (at 17.00).

In the salt-wasting form of CAI, in addition to the use of corticosteroids, compensation for acute loss of NaCl by intravenous infusion of hypertonic NaCl solutions is indicated.

Premature sexual development with follicular ovarian cysts

Treatment for follicular ovarian cysts includes hormonal therapy:

Dydrogesterone orally 10 mg 2 times / day from the 16th to 25th day of the menstrual cycle, no more than 3 months or Progesterone orally 100 mg 3 times / day from the 16th to 25th day of the menstrual cycle, no more than 3 months. The absence of positive dynamics (reduction or disappearance of the formation according to ultrasound performed on the 7th-8th day of the menstrual cycle for 3 months) is an indication for surgical treatment - laparoscopy, enucleation of the cyst.

McCune-Albright-Braitsev syndrome

For McCune-Albright-Braitsev syndrome, treatment is indicated in the case of prolonged hyperestrogenism, accompanied by frequent and severe bleeding:

Cyproterone 50 mg orally 2 times a day until clinical manifestations subside.

Evaluation of treatment effectiveness

The criterion for the effectiveness of treatment is suppression of signs of PPR.

Hypothalamic hamartoma in children with central forms of PPD.

■ Use of long-acting synthetic corticosteroids as HRT in children with CAH with open growth zones;

■ The use of dopaminomimetics in patients with signs of PPD that developed against the background of primary congenital hypothyroidism.

Forecast

If pathogenetic therapy is started in a timely manner, the prognosis for life, final growth and fertility is favorable.

A clear regression of secondary sexual characteristics is observed after at least 3 months. In all girls, menstruation stops, the volume and intensity of development of the glandular tissue of the mammary glands decreases, but their complete disappearance should not be expected. The volume of the ovaries and the size of the uterus decrease. The content of gonadotropic and sex hormones decreases to the level characteristic of the prepubertal period. After 1-1.5 years, the growth rate decreases from 10-12 to 4-5 cm/year, which corresponds to the norm for the actual age of the child.

For malignant neoplasms of the central nervous system, ovaries and adrenal glands, the prognosis depends on the histomorphological characteristics of the tumor and the extent of the process.

IN AND. Kulakov, V.N. Serov

Puberty, otherwise known as puberty, begins at the age of 8–13 years in girls, and at 9–14 years in boys. This process means the beginning of the transformation of the child’s body into an adult organism endowed with the function of reproduction. A comprehensive understanding of the period of puberty implies not only the physiological side of growing up, but also the psychological and social adaptation of a teenager.

What can be considered precocious puberty?

So, puberty is considered to be early if it occurs in girls before they reach 8 years of age, and in boys - before they reach 9 years of age. However, it should be noted that such norms do not apply to everyone - for example, among people living in countries with hot climates, children reach puberty somewhat earlier. It has been observed that children who are overweight are more susceptible to early puberty.

There are factors that can influence the timing of puberty, including:

• genetic predisposition;
• environmental (climate) features;
• belonging to one race or another;
• socio-economic standard of living;
• eating habits and characteristics;
• endocrinological disorders (hypothyroidism, adrenal hyperplasia) or frequent use of hormonal drugs;
• pathologies of the genital organs: ovaries in girls and testicles in boys.

In some cases, puberty occurs earlier due to dysfunction of the central nervous system. The following diseases can also contribute to the acceleration of puberty:

• neoplasms of the brain (brain and spinal cord);
• inflammatory diseases of the meninges - meningitis, encephalitis;
• hypothalamic hamartoma;
• malformations of intrauterine brain development - hydrocephalus;
• brain injuries and damage;

Other diseases that are quite rare can also affect the timing of puberty. As an example, we can mention McCune-Albright-Braitsev syndrome, in which skin pigmentation and bone structure are disrupted.

Experts distinguish two main forms of premature puberty:

1. True, or central, which is associated with the early activity of the hypothalamus and pituitary gland;
2. False, or peripheral, caused by hormones of the adrenal cortex or neoplasms of the genital glands, which causes increased secretion of sex hormones.

In many cases, the cause of early puberty is premature activity of the pituitary gland

How can you recognize premature onset of puberty?

The reproductive system of both boys and girls is formed during the period of intrauterine development. Each child at the time of birth receives a full set of necessary genital organs, but they “wake up” just at the onset of puberty. By about the age of six in girls, and by the age of eight in boys, the adrenal glands begin to intensively produce androgens, which affect changes in the child’s body. This process precedes the onset of puberty.

So, on the basis of what signs can we conclude that a child has begun puberty?

In boys

• an increase in the size of the external genital organ, especially the testicles;
• the appearance of facial hair (a cannon above the upper lip), pubic and armpit hair;
• intensive growth;
• change and breaking of voice;
• change in the smell of sweat.

For girls:

• breast formation;
• pubic and armpit hair growth;
• change in body contours, intensive growth;
• the beginning of menstruation;
• the appearance of acne on the face;
• change in the smell of sweat.

The appearance of secondary sexual characteristics in a girl

Acne is one of the signs of puberty

How is it diagnosed?

First of all, it is necessary to carefully examine the child; if the signs mentioned above are found, it is best to contact an endocrinologist who will conduct the necessary examinations and decide what is the cause of the child’s condition and whether it needs treatment or correction.

To establish an accurate diagnosis, a special blood test is performed with the child first given an injection of a special hormone that releases gonadotropin. Depending on the result obtained, the doctor determines the form of premature puberty.

In addition, to diagnose early puberty, an X-ray examination of the child’s hands is used. The data obtained make it possible to determine the biological age of bone tissue and its correspondence to chronological age. By comparing the biological age with the size of the hands and wrists, one can come to a conclusion about whether the child’s skeleton is developing normally and whether the bones are increasing in size too quickly.

If there is a suspicion that brain pathologies are the cause of early puberty, children are examined using an MRI - magnetic resonance imaging scanner. To identify possible disorders of the thyroid gland and reproductive organs, the doctor may order an ultrasound and a blood test for TSH levels.

Is it necessary to treat

First of all, the main goal of treating precocious puberty is to stop the intense manifestation of secondary sexual characteristics, which sometimes causes psychological trauma to children. In addition, a skeleton that develops too quickly outpaces the growth of internal organs and leads to the development of fragility of bone tissue.

The choice of treatment method will depend on the cause of the child’s condition and the extent to which this condition is pathological.

If premature puberty develops as a result of early activity of the pituitary gland and hypothalamus, then it is quite possible, by taking small doses of hormonal drugs, to slightly slow down this process, bringing the child on par with his peers. Here, a psychological problem is solved to a greater extent than a physiological one.

In the case where the cause of premature puberty is tumors of the brain and spinal cord, the doctor will consider either the possibility of conservative treatment of tumors, or, in extreme cases, the need to remove them. Modern medicine has high-tech methods that make it possible to rid a patient of tumors without the use of a surgical scalpel, followed by rapid rehabilitation.

Premature puberty most often becomes the cause of psychological discomfort for the child.

Alas, many natural factors, such as racial and genetic characteristics, cannot be eliminated. However, if your child is taught a healthy lifestyle from childhood, his risk of such disorders will be significantly reduced.

To do this, the baby must eat properly and not overuse medications, and especially hormonal ones. Parents should strictly control the weight of their child.

Unfortunately, there is still a widespread misconception about what a well-groomed baby should look like - chubby arms and legs, a round tummy and bright “diathesis” cheeks. Here he is, the idol of grandparents! Parents must understand that the consequence of this “beauty” will be a hormonal disorder of their beloved child.

It is also necessary to know that a child whose puberty occurred too early experiences psychological trauma from the fact that he is not like his comrades. It is no secret that in children's groups he will be subject to ridicule, and in the worst cases, bullying. Parents should help their child cope with their condition, and for this it is best to contact a child psychologist or psychotherapist.

It is quite possible that the child’s premature puberty has no reason to worry, but parents should pay great attention to this factor, since it may be based on serious illnesses that require immediate treatment.

Girls are said to have premature sexual development when they develop sexual characteristics before the age of 7 - mammary glands grow, hair is noticeable on the pubis and armpits, and female forms begin to actively form. In addition, the girl begins to menstruate early. 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 getting examined to prevent various consequences.

Symptoms

You can suspect something is wrong based on 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.

It is provoked by increased production of hormones - the pituitary gland, hypothalamus, which stimulate the work of the girl’s ovaries. In the case of complete true sexual development, the girl appears all the symptoms described above. If a child has an incomplete true form, the signs are still the same, only menstruation begins at 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 develop 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, but also has a uterus and 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 passed on from mother to daughter. If this is 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:

• Consequence of traumatic brain injury.
• Congenital structure of the brain.
• Complication after a virus or bacterial infection.
• A brain tumor.
• Poisoning by poisons.
• Radiation therapy.
• Cyst, ovarian cancer.

As a rule, girls experience problems with puberty if their mother:

• She used drugs and alcohol.
• She suffered from endocrine disorders.

Diagnostics

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

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

The mother's medical history is of no small importance. The doctor asks her about past gynecological diseases, operations, as well as about the number of abortions and pregnancies.

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

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

It is important for the doctor to determine bone age; for this purpose, an x-ray is prescribed. This examination makes it possible to find out about age-related disorders. Hormone analysis is of no small importance. Most of all it turns to sex hormones, pituitary gland, adrenal hormones. An X-ray of the skull may be taken to find out if there is a tumor of the hypothalamus or pituitary gland.

To exclude anatomical abnormalities of the brain, an MRI is prescribed. Additionally, the girl must undergo an ultrasound of the female organs, where they carefully check whether there are any abnormalities of the genital organs, cysts, or tumors.

In severe cases, laparoscopy using an endoscope is prescribed. This examination is necessary to check the fallopian tubes, uterus, and ovaries. If cancer is suspected, a biopsy may be ordered, during which samples of ovarian tissue 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 puberty. Recommended:

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

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

Consequences

If you don’t start treating your child in a timely manner, everything will end:

• Short in stature.
• Changes in body proportions - short legs, arms, long body, wide chest.

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

Prevention

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

• Watch the girl's diet. Her diet should include foods rich in fiber - fruits, vegetables, herbs. Do not give your child fried, hot, canned or spicy foods.
• Regularly monitor your newborn baby with a pediatrician or endocrinologist.

Carefully monitor your child's health!

Precocious puberty is called when secondary sexual characteristics appear in boys under 9 years of age, and in girls under 8 years of age. According to statistics, this symptom complex is common among children all over the world - about 0.5% of children suffer from diseases accompanied by it. However, this syndrome is most often recorded in America and African countries.

In this article, we will introduce the reader to the types of precocious puberty (PPP), the causes and mechanisms of its development, and also talk about the symptoms, principles of diagnosis and treatment of this pathology.

Kinds

The classification of precocious puberty syndrome is very conditional, since some of its forms under certain conditions can transform into others. However, there are 4 main types of this disorder, each of which includes several subtypes. Let's look at them.

True precocious puberty

It occurs due to increased activity of the pituitary-hypothalamus system. It, in turn, is divided into 3 types: idiopathic (the causes of which cannot be identified), cerebral and arising under the influence of sex hormones.

Cerebral true PPS can be caused by:

• tumors of the central nervous system (optic tract glioma, hypothalamic hamartoma and others);
• cysts of the third ventricle;
• birth injuries of the central nervous system;
• hydrocephalus;
• infectious diseases of the brain (, toxoplasmosis);
• surgical interventions on the brain;
• radiation therapy.

True precocious puberty, resulting from prolonged exposure to sex hormones on the child’s body, can occur in the following cases:

• with late treatment of congenital adrenal cortex dysfunction;
• as a result of the removal of tumors that produce hormones.

False PPP

In girls, it usually occurs against the background of:

• tumors (carcinomas) of the ovaries;
• ovarian follicular cysts.

In boys, the causes of this pathology are:

• tumors secreting hCG (human chorionic gonadotropin);
• tumors (androsteromas) of the adrenal glands;
• tumors (leydigoma) of the testicles;
• congenital dysfunction of the adrenal cortex (namely, deficiency of the enzymes 11-beta and 21-hydroxylase).

Gonadotropin-independent forms

Such variants of premature puberty occur with testotoxicosis and McCune-Albright syndrome. The activity of the gonads in this case is determined by genetic disorders; it is not related to the level of follicle-stimulating and luteinizing hormones in the blood.

Incomplete (partial) forms of teaching staff

They manifest themselves as premature secondary hair growth or isolated early enlargement of the mammary glands.

A separate variant of PPS is that against the background of the primary one in the stage of decompensation. It cannot be attributed to any of the above forms of the disease, since the mechanism of development of this pathology is different.

Causes and mechanism of development

As is clear from the data provided above, premature puberty is not an independent pathology, but only a syndrome, a set of symptoms that arise as a result of other disorders in the body, against the background of a variety of diseases.

True PPS occurs due to premature activation of the secretion of GnRH, a hormone synthesized by the cells of the hypothalamus. The main function of this hormone is to stimulate the release of the anterior pituitary gland of gonadotropic hormones - FSH (follicle-stimulating hormone) and LH (luteinizing hormone). They, in turn, stimulate the functioning of the gonads - their release of sex hormones with all the ensuing symptoms (development of secondary sexual characteristics). In a healthy body, all these processes are activated during puberty, and in pathology - ahead of schedule. In most cases, the cause of early activation of GnRH secretion is a tumor of the hypothalamus - hamartoma.

False PPS occurs, as a rule, as a result of a deficiency of the enzymes 11-beta and 21-hydroxylase, which results in increased secretion of androgen hormones by the adrenal glands. In some cases, an excess of sex hormones in the body occurs in the presence of tumors that secrete them.

Gonadotropin-independent precocious puberty is a consequence of genetic mutations. They cause hyperproduction of hormones by the gonads without the participation of FSH and LH in this process.

Clinical manifestations

True PPS in girls is manifested by the following symptoms:

• breast growth;
• “adult-like” appearance of the external genitalia;
• hair growth in the pubic and axillary areas is activated, but it is still less than in healthy girls during puberty;
• increased sweating;
• , often even regular (observed in 40-50% of girls, and in the rest they may be absent even against the background of all other symptoms of PPS for several years after the appearance of the latter).

At the age of 6-7 years, the ability of the adrenal glands to produce androgens is activated, therefore, starting at this age, symptoms of premature sexual development appear.

Symptoms of true PPS in boys:

• an increase in the size of the external genitalia (penis) and glands (testicles);
• acne;
• increased sweating;
• hair growth of the external genitalia, hair growth in the armpits;
• deepening of the voice;
• muscle growth.

With false forms of PPS in boys, the size of the testicles remains the same, or they are slightly enlarged.

Also, this pathology in both girls and boys is accompanied by rapid growth rates of the child - as in puberty. Such children grow by 10-15 cm in 1 year. Accelerated differentiation of bones takes place - this leads to early closure of growth zones and the person’s overall height is below average.

Diagnostic principles

At the diagnosis stage, the doctor faces the following tasks:

• determine the form of the disease;
• find out whether the function of the gonads is active (that is, there is a gonadotropin-dependent or gonadotropin-independent version of PPS);
• understand why the secretion of gonadotropins and sex hormones is increased.

To answer all these questions, a specialist listens to the patient’s complaints, collects an anamnesis of his life and illness, conducts an objective examination, and determines the need for additional examination - laboratory and instrumental.

History of life and illness

The most important thing at this stage is to find out what sexual development was like in the patient’s close relatives.

• Thus, testotoxicosis is characterized by early puberty in men in families of both mother and father.
• Congenital dysfunction of the adrenal cortex will be supported by PPS in the patient’s brothers or symptoms of virilization in his sisters.
• Hypothalamic hamartoma is characterized by early onset of puberty and rapid progression of this process.

Objective examination

Actually, the fact of premature puberty is clearly visible “to the eye.” When examining the girl, the doctor will pay attention to:

• an increase in the volume of the mammary glands, darkening of the areolas;
• changes in physique (the girl’s figure becomes more rounded and feminine);
• changes in the area of ​​the external genitalia;
• presence, frequency, duration and volume of menstruation.

Examining the boy, the specialist:

• assess the size of the testicles and penis;
• will clarify the presence of erections and their frequency;
• will pay attention to the degree of muscle development of his patient;
• will evaluate the timbre of the voice and check with the child’s parents whether the voice has become rougher.

In children of both sexes, the doctor will pay attention to the presence of rashes on the face (acne), hair growth in the axillary and pubic areas, and determine height and body weight.

In addition, during an objective examination, symptoms of various diseases can be detected, the manifestation of which is PPS (coffee-colored spots, subcutaneous fibromas, signs of hypothyroidism, and so on).

Laboratory diagnostics

Patients with PPS may be prescribed the following laboratory diagnostic methods:

Instrumental diagnostic methods

The following methods can be used to diagnose precocious puberty:

• radiography of the hands to determine bone age (high levels of sex hormones in the blood increase bone age);
• computer or magnetic resonance imaging of the skull (allows to exclude tumors of the central nervous system);
• Ultrasound of the pelvic organs (it is used to determine the degree of enlargement of the genital organs - uterus, ovaries, detect follicular cysts, tumors, adenomatous nodes);
• magnetic resonance or computed tomography of the adrenal glands and kidneys (allows us to identify tumors of these organs).

Principles of treatment

It pursues the following goals:

• stop the process of development of secondary sexual characteristics;
• suppress the rate of bone maturation to delay the closure of growth plates, thereby improving its prognosis.

True gonadotropin-dependent precocious puberty is treated by taking drugs from the GnRH agonist group. The most commonly used drug is diferelin, which actively binds to GnRH receptors in the pituitary gland, preventing the hormone itself from binding to them. This drug is administered intramuscularly, once every 28 days. The dose is calculated individually, based on the body weight of the sick child.

Forms of precocious puberty that progress slowly, occurring at the age of 5-6 years in girls, usually cannot be treated.

A tumor of the hypothalamus - hypothalamic hamartoma - does not threaten the patient’s life, since, as a rule, it does not increase in size. However, it is accompanied by very unpleasant neurological symptoms for a person, including seizures, which worsen the quality of his life and require treatment using radiosurgical methods and radiofrequency thermocoagulation. If PPS is the only sign of this pathology, surgery is not performed, but GnRH analogues are used.

Gliomas of the chiasmal optic region may be an indication for surgical and radiation treatment, but sometimes patients suffering from them are simply under dynamic observation by a doctor. This is due to the fact that in some cases such tumors can involute (undergo reverse development) on their own. Therefore, if the glioma does not manifest itself with any life-threatening or very unpleasant symptoms for the patient, it is not touched.

McCuin-Albright syndrome can be treated if it is accompanied by a prolonged increase in estrogen levels in the blood and frequent bleeding with loss of large volumes of blood. To normalize estrogen levels, the drugs Arimidex and Faslodex are used.

For testotoxicosis, ketoconazole is used. Its leading effect is antifungal, but this drug acts by inhibiting steroidogenesis (blocks the conversion of 21-steroids into androgens, reducing the level of the latter in the blood). During treatment, some patients may develop insufficiency of adrenal and liver function. Cyproterone acetate, Arimidex and Bicalutamide, which do not affect testosterone synthesis, can also be used.

Among non-drug treatment methods, a healthy lifestyle and proper nutrition are extremely important. Patients should avoid external sources of testostenone and estrogen. They should also maintain a normal weight through regular exercise.

Since children suffering from premature puberty feel different from their peers, they often develop complexes and other psychological problems. In such cases, they are advised to consult and treat with a psychotherapist.

Premature sexual development

Precocious sexual development (PPD) is a pathological condition of a girl’s body, which is characterized by early - before the age of 8 years - development of one or more secondary sexual characteristics: enlargement of one or both mammary glands, the appearance of hair in the pubic area, the manifestation of cyclic blood discharge from genital tract such as premature menstruation.

If your child is showing signs of “growing up” that are uncharacteristic for his age, be sure to consult with a specialist. Timely diagnosis and treatment is very important, since precocious puberty can lead to full sexual development, cessation of growth in preschool girls, as well as a number of health problems that can significantly affect the quality of life and reproductive function in the future.

Premature sexual development: causes

PPD can be caused by a number of different factors, both disruption of internal systems and organs, and negative external influences. The simultaneous appearance of mammary glands and bleeding from the genital tract in girls under 8 years of age can occur against the background of congenital diseases, as well as in the absence of diagnosis and treatment of one or another acquired pathology.

Reasons for PPR:

• family predisposition (idiopathic option);
• tumors and/or pathological processes in the hypothalamic-pituitary region;
• tumors and cysts (follicular) of the ovaries;
• excessive secretion of androgens due to congenital dysfunction of the adrenal cortex;
• pathology of the thyroid gland (hypothyroidism);
• inadequate hormonal therapy;
• food containing growth hormones, androgens and estrogens.

IMPORTANT: pediatric doctors of the Center for Obstetrics, Gynecology and Perinatology named after Academician V.I. Kulakov strongly recommends that parents under no circumstances give their children medications without a doctor’s prescription.

Premature sexual development: symptoms

The clinical manifestations of PPR correspond to the physiological changes that occur in the body of every girl during puberty. Normally, breast enlargement begins at 10-11 years of age, and the first menstrual cycle begins at 15-16 years of age. In parallel with these stages of growing up, a change in the figure occurs, it becomes more feminine, as well as a change in the hairline - the appearance of hair in the armpits and pubic area, often a change in the structure of the hair. If one or more of these stages occur at once in a girl of preschool age or in the lower grades, this may be premature sexual development, which requires competent diagnosis and therapy.

Symptoms of PCR:

• enlargement of the mammary glands (thelarche);
• feminization of the figure;
• the appearance of sexual hair growth (pubarche);
• the appearance of menstruation (menarche).

IMPORTANT: in modern society there is a misconception that today girls “mature” faster and this is a manifestation of the norm. This misconception poses a danger to the child's health. If you observe one or more of the above symptoms in your daughter, immediately visit a pediatric gynecologist.

Premature sexual development: diagnosis

Diagnosis of PPR includes determination of hormonal status; echographic examination of the uterus and ovaries, mammary glands, thyroid gland and adrenal glands; radiography of the hands (determining the biological or bone age of the child); electroencephalographic study of the brain; MRI of the retroperitoneum and adrenal glands (for girls with premature pubic hair); ophthalmological examination; stimulation test with a synthetic analogue of gonadotropins.

The Center uses advanced equipment from leading manufacturers that corresponds to the expert class to diagnose children's gynecological diseases and pathological conditions. Doctors at the Children's Center have unprecedented experience in diagnosing and treating girls of any age. Each therapy program is created with the participation of doctors of various specialties: in addition to the gynecologist, the process may include a therapist, endocrinologist, geneticist and other specialists who are competent to provide the assistance the child needs.

Premature sexual development: treatment

There are various methods for overcoming PPD; the choice of treatment tactics and treatment regimens are prescribed based on the results of the diagnosis, taking into account the girl’s age, the characteristics of her body and physical health. The treatment program in most cases includes controlled administration of medications strictly as prescribed by the doctor, exclusively in recommended dosages.

Treatment may be adjusted depending on its effectiveness within the expected time frame and the individual reactions of the child.

The service is provided by: specialists from the 2nd gynecological department (gynecology of childhood and adolescence), taking into account the standards for specialized gynecological care for children with PPD that are appropriate for federal healthcare institutions approved by the Ministry of Health of the Russian Federation.