Puberty early and late in boys. Early development of preschool children

Violations of sexual development in boys are associated with pathology of the secretion or action of androgens. Clinical picture depends on the age at which the problem started.

The formation of the male reproductive system goes on continuously until the end of adolescence. Doctors distinguish 3 stages of differentiation of the genital organs. Each of them is characterized by its dominant influences and a certain physiological meaning.

Stages of formation:

• intrauterine;
• prepubertal;
• pubertal.

prenatal period

The intrauterine period begins with conception and ends with the birth of a child. At the time of fertilization of the egg, the chromosomal sex of the child is determined. The obtained genetic information remains unchanged and influences further ontogeny. In humans, the XY set determines the male sex. Up to 5-6 weeks, female and male embryos develop in the same way. Primary germ cells have the ability to differentiate both in one way and in another way up to the 7th week of pregnancy. Before this period, two internal ducts are laid: wolf (mesonephric) and mullerian (paramesonephric). The primary gonad up to 7 weeks is indifferent (indistinguishable in boys and girls). It consists of a cortex and a medulla.

After 6 weeks of development, sexual differences appear in differentiation. Their occurrence is due to the influence of the SKY gene, which is located on the short arm of the Y chromosome. This gene encodes a specific "male membrane protein" H-Y antigen (testicular development factor). The antigen affects the cells of the primary indifferent gonad, causing it to transform into a male pattern.

Testicular embryogenesis:

• the formation of sex cords from the cortical substance of the primary gonad;
• the appearance of Leydig and Sertoli cells;
• the formation of convoluted seminiferous tubules from sex cords;
• formation albuginea from the cortex.

Leydig cells begin to secrete testosterone, and Sertoli - anti-Mullerian factor.

At 9 weeks prenatal development the reproductive ducts are affected by the influence of the chromosomal and gonadal sex. Anti-Müllerian factor causes atrophy of the paramesonephric duct. Without this influence, the uterus, fallopian tubes, and the upper third of the vagina are formed from the duct. The regression factor leaves in male body just rudiments.

Testosterone stimulates the development of wolf ducts. By the beginning of the 14th week, the epididymis, seminal vesicles, vas deferens and ejaculatory ducts are formed in the fetus. Primary germ cells are transformed into spermatogonia.

At the intrauterine stage big influence belongs dihydrotestosterone. This hormone is produced from testosterone by the enzyme 5a-reductase. Dihydrotestosterone is involved in the formation of external organs (penis, scrotum).

In the prenatal period, the testicles descend into the scrotum. By birth, this process is completed in 97% of full-term boys and in 79% of premature ones.

• guide ligament defects;
• gonadal dysgenesis;
• hypogonadism in the prenatal period;
• immaturity of the femoral-genital nerve;
• anatomical barriers to the movement of the testicle;
• weakening of the tone of the muscles of the abdominal wall;
• violation of the synthesis and action of testosterone.

prepubertal period

The prepubertal period is characterized by relative functional rest. In the first months after birth, high levels in the blood of a child can be determined (due to maternal intake). Further, the concentration of FSH and LH, as well as testosterone, drops to the limit low values. The prepubertal period is called the "juvenile pause". It lasts until the end of prepuberty.

puberty

In the pubertal stage, testosterone synthesis is activated in the testis. First, at the age of 7-8, the level of androgens in the blood of the boy rises due to the adrenal glands (adrenarche). Then, at the age of 9-10 years, inhibition in the centers of the hypothalamus responsible for sexual development decreases. This increases the levels of GnRH, LH and FSH. These hormones affect the testicle by increasing testosterone production.

Male sex steroids:

• enhance the growth of internal and external genital organs;
• affect the development of accessory glands;
• form sexual characteristics (secondary, tertiary);
• enhance the linear growth of the body;
• increase the percentage of muscle tissue;
• affect the distribution of subcutaneous fat.

In puberty, the maturation of germ cells and the formation of mature spermatozoa begins.

Normal onset of sexual development and definition of its delay

Puberty in boys starts with an increase. Average age the appearance of this symptom - 11 years.

Table 1 - Average values ​​​​of the volume of the testicles in different age periods(according to Jockenhovel F., 2004).

The rate of puberty is the rate at which signs of puberty appear.

Possible rates:

• medium (all signs are formed in 2-2.5 years);
• accelerated (formation occurs in less than 2 years);
• slow (formation takes 5 or more years).

The normal sequence of signs of puberty at puberty is:

1. testicular enlargement (10-11 years);
2. penis enlargement (10-11 years);
3. development of the prostate, an increase in the size of the larynx (11-12 years);
4. a significant increase in the testicles and penis (12-14 years);
5. pubic hair according to the female type (12-13 years);
6. knotting in the area mammary glands, (13-14 years old);
7. beginning of voice mutation (13-14 years old);
8. the appearance of hair in the armpits, on the face (14-15 years);
9. pigmentation of the skin of the scrotum, first ejaculation (14-15 years);
10. maturation of spermatozoa (15-16 years);
11. male-type pubic hair (16-17 years old);
12. stop the growth of the bones of the skeleton (after 17 years).

The stage of puberty is assessed according to Tanner.

Table 2 - Assessment of the stage of sexual development according to Tanner.

Retarded puberty in boys

Delayed sexual development is determined if the boy has a testicular volume of less than 4 ml by the age of 14, there is no growth of the penis in length and an increase in the scrotum. In this case, it is required to start an examination to identify the cause of the pathology.

The reasons

Delayed sexual development may be due to:

• constitutional features (family);
• violations of the hypothalamic-pituitary regulation ();
• primary insufficiency of testicular tissue ();
• severe somatic pathology.

Diagnostics

• collection of anamnesis;
• assessment of heredity;
• grade bone age by radiograph;
• general inspection;
• examination of the external genital organs, assessment of the volume of the testicles and the size of the scrotum;
• hormonal profile (LH, FSH, testosterone, prolactin, TSH);
• tomography of the brain, x-ray of the skull;
• cytogenetic study.

Treatment

Treatment depends on the causes of delayed sexual development.

Family forms of delayed sexual development can be corrected with the help of. Anabolic steroids are prescribed to adolescents with this form of the disease to prevent short stature.

In secondary hypogonadism, gonadotropins and gonadorelin are used in the treatment. This therapy is the prevention of infertility in the future. The use of hormones of the hypothalamic-pituitary region stimulates the development of the testicles and.

With primary hypogonadism, from the age of 14, boys are prescribed testosterone replacement therapy.

Precocious puberty in boys

Premature is considered the appearance of signs of puberty in boys under 9 years of age. This condition can lead to social maladaptation. In addition, premature sexual development is one of the causes of short stature.

The reasons

Precocious puberty is divided into:

• true (associated with the work of the hypothalamic-pituitary region);
• false (associated with autonomous secretion of hormones by the adrenal glands or tumors).

True precocious sexual development is complete (there are signs of masculinization and activation of spermatogenesis).

The reason for this condition may be:

• idiopathic;
• associated with diseases of the central nervous system;
• associated with the primary;
• arising against the background of prolonged hyperandrogenism (for example, with tumors of the adrenal glands).

False precocious puberty is usually not accompanied by activation of spermatogenesis (except in cases of familial testosterone toxicosis).

Causes of false precocious puberty:

• congenital hyperplasia of the adrenal cortex;
• , testicles;
• Cushing's syndrome;
• tumors secreting;
• Leydig cell hyperplasia (familial testosterone toxicosis);
• androgen treatment;
• isolated premature adrenarche.

Diagnostics

Examination for signs of precocious puberty includes:

• collection of anamnesis;
• general inspection;
• examination of the genitals;
• hormone tests (LH, FSH, testosterone, TSH,);
• samples with gonadoliberin;
• bone age study;
• skull x-ray, brain tomography, etc.

Treatment

For the treatment of true precocious puberty, synthetic analogues of GnRH are used. This drug suppresses the impulse secretion of LH and FSH. If the cause of the disease is the pathology of the central nervous system, then the patient is prescribed appropriate treatment (by a neurologist, neurosurgeon).

Treatment of false precocious puberty depends on the causes that caused it. If the pathology is associated with an isolated adrenarche, only observation is carried out. If a hormonally active tumor is found, radical treatment is performed (surgery, radiation therapy). In cases of congenital adrenal hyperplasia, corticosteroid therapy is selected.

Endocrinologist Tsvetkova I. G.

Until the age of 8-9 years (prepubertal period), the hypothalamus-pituitary-gonadal system is inactive: neither LH (luteinizing hormone) nor sex steroids (estradiol in girls and testosterone in boys) are detected in the blood serum. It is believed that the activity of the hypothalamus and pituitary gland at this time is under the influence of inhibitory neurons, which are still little studied.

Approximately 3 years before the first clinical signs of sexual development in children during sleep, it is already possible to determine LH in the blood serum (prepubertal period). This sleep-induced LH secretion is pulsatile and likely associated with episodic releases of hypothalamic GnRH. As puberty approaches, the amplitude and, to a lesser extent, the frequency of nocturnal LH impulses increase, which leads to an increase and maturation of the gonads and the onset of secretion of sex hormones. As a result of the joint activity of the hypothalamus, pituitary gland and gonads on early stages sexual development in children, secondary sexual characteristics appear. In the middle puberty impulse secretion of LH is recorded not only at night, but also during the day. The interval between pulses is 90-120 minutes.

In the same or more late deadline girls begin menstrual cycles and ovulation. A positive feedback loop is formed, due to which the increasing in the middle menstrual cycle the level of estrogen causes a distinct increase in the level of LH in the blood serum.

The factors that normally activate or inhibit GnRH-secreting hypothalamic neurons (the so-called GnRH pulse generator) are unknown. In experiments on monkeys, this generator is activated by a decrease in the tone of hypothalamic GABAergic neurons, accompanied by an increase in the activity of the glutamatergic system. In all likelihood, other CNS mediators also take part in this process in humans and monkeys.

It is GnRH that is the main, if not the only hormone that triggers the process of sexual development in children. Thus, by administering GnRH in a pulsed mode, it is possible to induce sexual development in immature animals and humans, as well as in cases of gonadotropin deficiency.

Many circumstances make it difficult to understand hormonal changes during sexual development:

pituitary gonadotropins are heterogeneous, their various isoforms are present in the blood. During sexual development, biologically more active isoforms may predominate in children.

the results of determining the content of immunoreactive LH depend on the method used, so the data obtained in different laboratories diverge.

gonadotropins are released into the blood in impulses, and LH and FSH synergistically act on the maturation of the gonads. Therefore, single determinations of the concentration of gonadotropins are not informative. Serial determination of their level in the blood (every 10-20 minutes for 12-24 hours) is more significant.

there are gender differences in the maturation of the hypothalamus and pituitary gland, and the concentration of LH in the blood serum during sexual development in boys increases earlier than in girls.

The study of the consequences of estrogen deficiency in boys has made more clear the effect of sex steroids (testosterone in boys and estradiol in girls) on bone growth and maturation. Both with aromatase deficiency and with defects in estrogen receptors in boys, the closure of the epiphyseal growth zones is delayed and tall stature develops. These data point to the role of estrogens, and not androgens, in skeletal maturation and growth arrest. Estrogen also stimulates the secretion of growth hormone, which, together with sex steroids, causes a rapid acceleration of growth in puberty.

The age of onset of puberty varies and is more in line with the degree of bone maturation than chronological age. The first sign of the sexual development of girls is a slight swelling of the mammary glands (at 10-11 years old), after 6-12 months. pubic hair starts. Before the first menstruation (menarche), it usually takes another 2-2.5 years, this interval can reach 6 years. In the US, 95% of girls aged 12 and 99% of girls aged 13 have at least one sign of puberty. The maximum acceleration of growth in girls begins early (usually between the ages of 11 and 12) and always precedes menarche. The mean age of menarche is 12.75 years. However, the intervals between growth acceleration, development of the mammary glands, pubic hair growth, and maturation of the internal and external genital organs vary widely.

In boys, the first sign of the onset of puberty is an increase in testicles (volume - more than 3 ml, longitudinal diameter - 2.5 cm) and thinning of the skin of the scrotum. Then there is pigmentation of the scrotum, enlargement of the penis and pubic hair. In the middle of puberty, children develop armpit hair. Growth acceleration is recorded already in the course of sexual development (at IV-V stages maturation of the genital organs, usually at the age of 13-14), i.e., about 2 years later than in girls. Growth can continue after age 18.

The age of onset of puberty depends on genetic and environmental factors. In the XX century. the age of menarche has progressively decreased, which is probably due to improved nutrition and general health of the population. However, in the last 30-40 years this age has stabilized. African American women develop secondary sexual characteristics earlier than white girls. Ballerinas, gymnasts and other athletes who have remained thin from early childhood and experience huge physical exercise, sexual development and menarche occur much later, and in adulthood oligomenorrhea or amenorrhea is often noted. Such observations confirm the idea of ​​a close relationship between energy metabolism and the activity of the GnRH pulse generator and the mechanisms of initiation and maintenance of sexual development in children. This connection is probably mediated by hormonal signals from fat cells (leptin and other peptides).

Adrenal androgens also play an important role in sexual development in children. The level of dehydroepiandrosterone (DEA) and its sulfate in the blood serum begins to increase at about 6-8 years of age, i.e., long before the increase in the content of LH or sex hormones, and, moreover, before the appearance of the earliest physical signs sexual development in children. This process is called adrenarche. Of all the adrenal C19-steroids present in the blood, the level of DEA-sulfate is the highest and practically does not fluctuate throughout the day. The result of a single determination of its concentration in the blood can serve as an indicator of the secretion of adrenal androgens. Although adrenarche precedes the activation of the gonads (gonadarche) by several years, there is apparently no causal relationship between these processes, since one can occur without the other (for example, in precocious puberty or adrenal insufficiency).

Acceleration is a ubiquitous phenomenon. The accelerated sexual development of adolescents today worries psychologists and physicians. Prematurity can lead to unintended consequences. Outwardly, a teenager may seem mature enough - this is what anatomical features puberty in boys and girls. However high growth or a magnificent bust are not evidence of the stabilization of the hormonal background and the final maturation of the brain. Let's try to figure out how the physical and sexual development of children normally goes, and what their parents should pay attention to.

What is puberty

In the body, each of us has its own The biological clock. This abstract concept explains the timeliness of the growth and development of the body, the appearance and withering of reflexes and functions. Babies suck their fingers and learn to walk, but after a few years, having acquired many skills, they go to school to explore the world. Women get wrinkles over the years, and old people get gray hair. All these and many other natural changes are a consequence of the normal functioning of the endocrine system, as well as the hypothalamus and pituitary gland. These glands are located in the brain and secrete hormonal substances necessary to start age-related biological processes.

Each has its own characteristics. But the greatest stress to the body brings during puberty. There are countless changes that occur with a fragile child's body, but right now we will try to sort everything out.

The development of germ cells and organs in representatives of both sexes takes place in three stages. In girls and boys, they do not occur at the same time:

1. Prepuberty. In boys, it begins at age 8 and ends closer to eleven. In girls, it starts 1-2 years earlier.
2. puberty. The age at which children can be considered adolescents also differs. In boys, it is observed at the age of 11-17, in girls it usually ends by the age of 15.
3. Growing up. Girls are considered sexually mature at the age of 15-18 years. The formation of young men is completed by the age of 19-20.

How boys grow up

It begins with the formation of secondary sexual characteristics, the impetus for which is an increase in the concentration of testicular androgens in the body.

The process of maturation of the male reproductive system starts with a change in the size of the genitals. First, the longitudinal diameter of each testicle increases, acquiring pigmentation and folding of the scrotum. By the age of 11-12, a teenager develops pubic and axillary hair growth. AT rare cases vegetation in intimate areas occurs before the testicles increase in volume. This is not the norm, such a violation is caused by excessive production of androgens. In medicine, this phenomenon is called accelerated adrenarche.

As testicular volume increases, the size of the penis becomes larger. First, its length increases, and then the diameter. Pigmentation (darkening skin) continues to grow.

Approximately by the age of 13-14, a young man develops hair on his face, neck, chest. At this age, the genitals may well correspond to the size of adult men, but it is too early to talk about the onset of puberty. This should be evidenced by spermatogenesis. The first ejaculations and wet dreams come closer to 16 years. Puberty in boys continues until the age of 19-20, but they become fertile, that is, able to fertilize a female egg, they become earlier.

Another change that occurs under the influence of increased secretion male hormones, is the acquisition by the body of characteristic architectonics: an increase in the shoulder girdle, growth of bones and muscles.

How teenage girls are changing

In girls, it also begins with the appearance of secondary signs, and its completion is indicated by the establishment of a regular menstrual and ovulatory cycle. The first noticeable changes are expressed by breast enlargement: glandular tissue thickens under the areola area, and the peripapillary zone darkens. The growth of the mammary gland is provided by the secretion of estrogen - at the age of 10-11 years, they are produced in enough. Often girls are afraid of asymmetry (one breast seems larger), which disappears only during the formation of a mature gland.

Next secondary feature sexual development in the fairer sex is pubic and axillary hair growth. Most often, hair growth in armpits ah speaks of the approach of menarche - the first menstruation. Despite the fact that the process of formation of sexual characteristics most often occurs in this sequence, approximately 1% of girls develop hair first. Wrong flow Puberty is caused by an increased presence of male hormones in the body.

In parallel with the development of the reproductive system, the architectonics of the body changes. In girls, an increase in body weight occurs due to the buildup of predominantly adipose tissue. It begins to “accumulate” in the body from the age of six. In early puberty, adipose tissue is redistributed and deposited in the hips and pelvis.

Several stages of maturation of boys and girls

So, each of the periods of sexual development can be briefly described as follows:

• A kind of preparation for the upcoming changes is the prepubertal period. Within 2-3 years, the body accumulates enough strength to make a big leap in growth and sexual development. Then comes the teenage period, with the peculiarities of which every person has to face.
• Adolescence (pubertal) age is characterized by intensive growth, increased work of the gonads, significant changes in the figure. In both sexes, the voice also changes: in boys, due to a mutation, it becomes low and deep, in girls it loses its ringing.
• Growing up. Consolidation of acquired sexual characteristics, growth arrest, the beginning of an active sexual life and the reproduction of offspring.

Negative manifestations of puberty

In addition, with the onset of puberty, girls and boys develop similar problems with health. Often teenagers suffer from inflammation sebaceous glands, the spread of acne on the face, back, shoulders, sometimes buttocks. shiny oily skin at the age of 12-16 years is predisposed to acne.

There may be pain in the muscles and joints, dizziness and fainting, impaired coordination. In girls, the chest and lower abdomen can often hurt, and before the first menstruation, they can strongly pull the lower back.

Complex character of a teenager

Sexual development of representatives of both sexes is accompanied by another important aspect - psychological. Parents know firsthand about the difficulties of adolescence. Changes in character and rebellious behavior are predominantly hormonal in origin. a clear sign"internal breakdown", provoked by the entry into the blood of a teenager of a large amount of testosterone and estrogen, are sharp drops moods. More sensitive to hormonal changes girls. They can become violent for no particular reason, and after five minutes - compassionate and tearful.

It should be noted right away that such mental phenomena in adolescents are not treated. A well-chosen diet can help a child overcome puberty. To mitigate the hormonal "storm" of a teenager, it is important to minimize the daily amount of calories entering the body, eliminating in the first place fast carbohydrates, chocolate, caffeine, energetic drinks and fatty meals. It is better to give priority in nutrition to animal products containing calcium and protein, which are especially necessary during the period of active growth. Girls, in addition, it is important to constantly replenish iron deficiency. It is contained in beef liver, pomegranate juice, apples, red meat.

If a teenager is having a hard time going through puberty, you should contact a neurologist or psychotherapist. Boys and girls are usually recommended to drink vitamins, homeopathic sedatives and adaptogens (extract of echinacea, magnolia vine, succinic acid).

What diseases occur in adolescents

As already said, age development germ cells and the reproductive system in general is not the most favorable period for the body. Adolescence is a kind of "trigger" for the activation of a number of diseases and pathological conditions. In the pubertal period, gastritis is often exacerbated, cholecystitis is found, diabetes. The first migraine attacks in most girls suffering from this problem occurred just in adolescence. Myopia, decompensation of congenital but not diagnosed heart diseases in time, scoliosis, flat feet - in children aged 10 to 16 there are enough chances for such manifestations. AT severe cases juvenile schizophrenia or oncology develops.

On the other hand, active growth during the formation of boys and girls allows many to “outgrow” asthma, enuresis, and adenoids. It happens that the child weakens or completely disappears allergic reactions.

Violations in the work of the reproductive system

There are specific diseases that are typical only for adolescents. The most common of them:

Too early: reasons for acceleration

Speaking of violations of sexual development, they mean two options: premature ripening and its delay. Sometimes during internal system organism, which determines the rhythm of its life, a failure occurs, and natural processes arrive earlier or later than the due date.

So, acceleration is premature sexual development, which begins in boys before the age of 9, and in girls - up to 8. In such early age the body is not yet ready for pubertal changes. In the future, premature sexual development leads to early extinction reproductive functions. In addition, accelerated children often begin early sex life, which is dangerous to health, especially in the case of an unplanned unwanted pregnancy.

Pathological causes of acceleration are violations in endocrine system, benign and malignant tumors brain, pituitary gland, diseases of the adrenal glands and abnormal functioning of the sex glands. If the first signs of acceleration occur, you should consult a doctor. To prevent early offensive puberty, doctors recommend that parents do not overfeed their children, limit carbohydrate intake and give them to the sports section.

Five-year-old mother from the Guinness Book of Records

Most famous case in the story of a girl with early puberty is simply shocking. The Peruvian was only 5 years and 7 months old at the time of her son's birth. Despite the fact that the origin of the pregnancy remained unclear, in 1939 her son was born absolutely healthy with a weight of 2700 g. C-section, published a report in which they noted that Lina's menarche occurred at 2 years and 8 months, and by 4 years her mammary glands were almost formed.

Early maturation is caused either by natural or pathological causes. Today, many doctors believe that the cause of premature puberty is hormonal additives in the feed of the livestock whose meat we eat, substances released from plastic packaging and plastics. Maturation ahead of time does not pass consistently: for example, a boy may suddenly have a sexual desire and a full erection, and only then develop hair growth.

Delay secondary sexual characteristics

It occurs in adolescents and another situation - a delay in sexual development. This disorder is characterized by the absence of secondary sexual characteristics by the age of 14-15: girls have no vegetation on their bodies, their breasts are practically not developed, and the size of the penis does not increase in a boy. Sometimes such a delay in development is considered normal. It is not worth panicking ahead of time if the teenager has an asthenic physique.

Also, the reason for the delay may be the postponed serious disease, anorexia (in girls) or professional sports. But if secondary sexual characteristics do not appear even by the age of 16, it is simply necessary to consult a doctor: perhaps there are genetic abnormalities.

Girls with male-type hair, mature breasts and lack of menstruation need a detailed examination - such symptoms can be caused by an abnormal absence of the uterus with functioning ovaries.

For the health of a child in transition puberty need to be carefully monitored. It is important that a teenager eats properly and rests. FROM preventive purpose every year it is desirable to take laboratory and clinical tests of urine and blood - this simplest form survey is enough effective way identifying inflammatory processes in the body.

Parents are also responsible to their children for their sexual education. Lessons in the form of explanatory conversations about the rules of hygiene, the dangers of early sexual activity and contraception should be carried out with every teenager, no matter how adult and omniscient he may consider himself.

FLOOR FORMATION

The concept of "sex" is made up of a set of interrelated biological and socio-psychological components:

Specificity of genetic, gonadal and genital sex;

The peculiarities of the physique and proportions of the body (the ratio of the width of the shoulders and pelvis; the severity and distribution of the subcutaneous fat layer, muscle mass);

Sexual consciousness;

Appropriate stereotypes of gender-role behavior.

The formation of the genetic sex of the unborn child occurs during the fertilization of the egg and is determined by the set of sex chromosomes - the 46XX or 46XY karyotype. The genotype, in turn, determines the set of genes responsible for the formation of the type of gonads, the level of activity of enzyme systems, the synthesis of sex hormones, and the sensitivity of the tissue receptor apparatus to them. Male and female gonads develop from one undifferentiated primordium, which is morphologically the same up to 6 weeks of gestation.

The sexual differentiation of the fetus begins with the differentiation of the gonads (weeks 6-10 of gestation), identified by histological structure sex glands. The process of formation of the gamete (gonadal) sex is regulated by the genes of the sex chromosomes, among which the HY antigen is currently playing a large role. Under the influence of the latter, the development of the primary gonad into the testis is induced. The high hormonal activity of the fetal testicle (the synthesis of testosterone, the “anti-Müllerian” factor) is necessary for the further formation of the male genital tract. In the absence of the HY antigen, female gonads are formed.

Differentiation of the internal genitalia or the formation of the internal genital sex occurs at the 10-12th week of gestation from the indifferent mesonephric (Wolfian) and paramesonephric (Mullerian) ducts. The development of the female fetus proceeds by regression of the mesonephric and differentiation of the paramesonephric ducts into the uterus, oviduct, and vaginal fornix. The development of a male fetus is possible only in the presence of an actively functioning testicle, as a result of which the mesonephric ducts differentiate into the epididymis, seminal vesicles, efferent ducts, and the prostate gland.

At the same time, two primary paired duct systems develop in the male and female embryos: the Mullerian ducts and the Wolfian ducts (Fig. 7.1).

RICE. 7.1. Stages of differentiation of the internal genital organs

(A) Undifferentiated anlage at approximately 6-7 weeks of age. (B) The condition of the female genital organs in a 14-week-old fetus. (C) The condition of the male genital organs in a 14-week-old fetus. (D) The condition of the female genital organs in a 40-week-old fetus. (E) The condition of the male reproductive organs in a 40-week-old fetus.

Testicular development requires an additional step of genetic regulation. The transformation of primitive gonads into testicles begins under the influence of H-Y antigen- a chemical compound of unknown nature, the synthesis of which is determined by the Y-chromosome. In the absence of this factor, primitive gonads always develop into testicles.

Starting from this moment, sexual differentiation is carried out on three various levels: internal genital structures, external genitalia and brain, and is controlled mainly by hormones. If in right time not enough testosterone is formed, even with a chromosome set of 46, XY, anatomical development often follows a female rather than a male pattern (Jost, 1953; Jost 1972; Money, Ehrhardt, 1972; Wilson, George, Guffin, 1981).

The formation of the external genital sex is noted from the 12th to the 20th week of intrauterine development by differentiation of the urogenital sinus and genital tubercle, and in the female fetus this process occurs regardless of the state of the gonads, while in the male fetus - only with sufficient activity embryonic testicles.

In total, the phases of the formation of the inner and outer genital sex determine the state of the morphological (somatic) sex or phenotype. At the birth of a child, a civil sex is established (obstetric, passport).

Sexual differentiation in postnatal life is influenced by socio-psychological determinants that determine sexual self-awareness, the stereotype of gender-role behavior, psychosocial orientation, which in total form the child's psychosocial gender. At the same time, social gender is understood as a certain gender identity the child from the side of others, and under the psychological - the attitude of the child to himself, as to a person of a certain sex. Great importance this is allotted proper education and appropriate orientation from the surrounding people.

normal development a child and his full-fledged socio-psychological adaptation are possible only if there is a complete coincidence of the genetic, gonadal, somatic, social and psychological sex. This condition is called isosexuality. With abnormal formation of sex or the absence of this unity, the terms “heterosexuality” or “intersexuality” are used.

HORMONAL REGULATION OF SEXUAL FUNCTION

Complex state hypothalamic centers and the level regulated by them pituitary hormones called a gonadostat. Hypothalamic-pituitary regulation of sexual function is carried out according to classical pattern, which is based on the principle of direct and feedback between the main links in the chain: hypothalamic releasing hormone - pituitary tropic hormones - peripheral endocrine glands. Unique feature functioning of this system is the wave-like nature of its activity. A high level of gonadotropic and sex hormones is formed in the fetus by the middle of embryonic development and rapidly decreases towards the end of pregnancy. In the postnatal period, the level of gonadotropic secretion increases again and gradually decreases in boys by 6 months of age and in girls by 2 years. Embryonic gonadotropic activity is necessary to complete the processes of sexual differentiation. The period from 2 to 9 years in children is characterized by extremely low scores both gonadotropic and sex hormones. Inactivation of the pituitary-gonadal function during this period is the result of the inhibitory effect of the central nervous system, which maintains a long "juvenile pause", which is characteristic only for humans. In the future, the "juvenile pause" is replaced by puberty.

To date, the mechanism that “triggers” the onset of puberty has not been finally established. Obviously, this is due to accidental processes in the centers that hold back puberty in childhood. A large role in the stimulation of the gonadostat belongs to androgens of adrenal origin, the physiological concentration of which in the blood increases in children aged 6-7 years (adrenarche).

A change in tissue sensitivity to sex hormones in the pubertal period is noted at all levels of the gonadostat: the sensitivity of the hypothalamus to sex hormones decreases, the level of releasing hormones and gonadotropins increases, and the sensitivity of gonadal tissue to gonadotropic hormones also increases. The increased level of sex steroids gives rise to the formation of reproductive function.

SEXUAL DEVELOPMENT OF BOYS

The pubertal period in boys begins against the background of an increase in the concentration of androgens, mainly of testicular origin, with the formation of secondary sexual characteristics and ends with spermatogenesis. Puberty in boys covers the age range from 9 to 18 years.

In boys, the first symptom of the onset of puberty is an increase in the volume of the testicles. The criterion is an excess of a testicular volume of 4 ml according to the Prader orchidometer or an increase in the longitudinal diameter of the testis of more than 2.5 cm. The scrotum becomes slightly pigmented, acquires folding. The increase in testicular volume reflects a simultaneous increase in the mass of tubular epithelium, controlled by FSH, and interstitial Leydig cells, controlled by LH. The beginning of testicular growth in boys is accompanied by the appearance of pubic hair, although in some adolescents, hair begins to be determined only when the volume reaches 6-8 ml according to Prader. This may be due to the fact that the initial increase in the volume of the testicles occurs due to the intensive development of the tubular epithelium, while the testosterone-secreting Leydig cells are formed somewhat later. However, about 1-2% of boys have pubic hair before testicular enlargement, which is associated with increased secretion of adrenal androgens ("wrong" puberty or accelerated adrenarche). As the testicular volume increases, the size of the penis increases, first its length, then its diameter. Increased pigmentation of the external genitalia. After 1-1.5 years, axillary hair growth develops on the face. By the age of 13-14, the external genitalia, including the volume of the testicles, can fully correspond to sexually mature age. However, typical male hair growth with trapping inner surface hips and lower abdomen (rhomboid type) is formed later. The completion of puberty is evidenced by the first ejaculations and regular wet dreams, which appear on average by 15.5 years. Therefore, boys can be fertile before the development of secondary sexual characteristics is completed. Under the influence of increased secretion of androgens, changes in the architectonics of the body are formed: total muscular and bone mass, increases the growth of bones and muscles of the shoulder girdle.

The dynamics of changes in secondary sexual characteristics in children during puberty is ranked according to the J.M. Tanner, in which stage 1 corresponds to the prepubertal development of the child and the absence of secondary sexual characteristics, stage 5 corresponds to the sexually mature status.

Stages of development of the external genitalia and genital hair in boys(Marshal and Tanner)

The development of the genitals in boys begins at about 11.6 years of age, and their size and shape correspond to those of adult men at the age of 14.9 years (Marshall and Tanner, 1970) (Fig.). In some boys, the development of the genitalia is rapid (takes about a year), while in others it can take up to 5.5 years (Tanner, 1974).

Rice. Development of the male external genitalia during puberty (Marshall and Tanner, 1970).

The sequence of appearance of secondary sexual characteristics in boys(Zhukovsky M.A., 1982)

* - pubic hair in boys under 16-17 years old has female type

SEXUAL DEVELOPMENT OF GIRLS

Puberty in girls begins with the appearance of secondary sexual characteristics and ends with ovulation. Initial outward manifestation Puberty in girls is an increase in the mammary glands: the glandular tissue under the areola area becomes denser, the coloration of the areola area changes, the contour of the areola rises above the compacted glandular tissue. The development of the mammary glands in girls is provided mainly by estrogens, secreted by this age already in sufficient quantities. The glandular tissue of the mammary glands may initially appear only on one side, and the asymmetry of the development of the mammary glands persists during the first 1.5-2 years of puberty, disappearing only during the formation of a mature mammary gland. The development of secondary pubic and axillary hair growth is controlled by androgens of adrenal and ovarian origin. Pubic hair begins to appear 3-6 months after the appearance of the mammary glands, axillary hair appears 1-1.5 years later and usually immediately precedes the arrival of the first menstruation - menarche. This sequence of appearance of secondary sexual characteristics is inherent in most girls, however, in 1% of them, secondary hair growth precedes the development of the mammary glands. Such a change in the sequence of the appearance of secondary sexual characteristics is referred to as "irregular puberty" or "accelerated adrenarche" - a term indicating the maximum contribution of androgens to the process of accelerated appearance of secondary hair growth.

In parallel with the increase in the level of sex steroids and the development of secondary sexual characteristics, the architectonics of the body also changes. An increase in body weight and the amount of adipose tissue in girls begins even in the prepubertal period - from 6-7 years. In early puberty, there is a further accumulation of adipose tissue and its redistribution with a maximum deposition in the pelvis and thighs: a feminine (gynoid) type of body architectonics.

The progressive formation of secondary sexual characteristics is accompanied by an intense change in the external and internal genitalia. The small and large labia are enlarged, the nature of the mucous membrane of the vagina and the hymenial ring changes. Increases immediately before menarche vaginal discharge, they become thicker and more colored. The first menstruation - appears in girls who have reached the 4th stage of sexual development according to the Tanner scale. After the onset of menarche, the activity of the sebaceous and sweat glands in girls increases, acne vulgaris appears on the skin. The first ovulatory cycles are usually recorded 9-12 months after menarche. The final closure of growth zones and cessation of growth in girls occurs 1.5-2 years after menarche.

The development of the main secondary sexual characteristics in girls is provided by the hormonal production of the ovaries. An increase in the size of the ovaries correlates well with the stage of sexual development.

By the time of birth, the girl's ovaries contain 6-7 million primordial follicles, which are primary oocytes surrounded by one row of spindle-shaped cells, granulosa precursors, and a basement membrane, which subsequently develop into thecal cells. From birth to the onset of puberty, some of the follicles develop to the antral follicle stage and undergo atresia, which indicates the processes of estrogen secretion in girls before puberty. An increase in the level of gonadotropic hormones by puberty causes active growth of follicles whose diameter exceeds 4 mm, however, a high rate of atresia persists, and the ovaries may have a multicystic structure, which is physiological for the age preceding menarche. A further increase in the level of gonadotropins and a decrease in the FSH / LH ratio leads to morphological changes in the follicle, the maturation of granulosa and theca cells, capable of secreting sufficient estrogen and progesterone. The ability of granulosa to secrete a large number of estrogen is necessary condition for the formation of ovulatory cycles.

Stages of sexual development(Marshal and Tanner)

Stages of development of pubic hair in girls

Stages of development of the mammary glands in girls

Picture. Schematic representation of the stages of development of the mammary gland and pubic hair according to Tanner.

The sequence of appearance of secondary sexual characteristics in girls(Zhukovsky M.A., 1982)

METHODOLOGY FOR STUDYING SEXUAL DEVELOPMENT

Features of the anamnesis in assessing the state of sexual development include:

1. Collection of information about the nature of puberty from the child's parents, other children in the family or close relatives (advance or delay in terms).

2. Information about the course of both previous (presence of stillbirths, miscarriages) and present pregnancies with an emphasis on all possible adverse factors and diseases of the mother. Information about taking pregnant drugs, especially hormonal ones, is extremely important.

3. Information about the growth and development of a real child, the presence of acute and chronic diseases in the past that affected the overall development of the child.

On examination, deviations in physical development, physique features are revealed. In the presence of secondary sexual characteristics, the latter are documented in the form of a sexual formula, which respectively indicates the stages of maturation of each characteristic and the age of the onset of the first menstruation, for example, A0, P1, Ma2 or A2, P3, Ma3.

Examination of the genital organs must be carried out in the presence of either the mother or a nurse, in girls - in the supine position with the hips brought to the stomach. In boys, in addition to the severity of secondary sexual characteristics provided for by the formula, the degree of development of the external genital organs is assessed. For an objective assessment of the condition of the testicles and control of their increase in the process of maturation, a standard set of Prader-type orchidometers is used.

When examining the external genitalia, it is possible to reveal the presence of anomalies in the structure, an indefinite (intersex) condition. In the latter case, the child must undergo a mandatory endocrinological examination.

The severity of secondary sexual characteristics in boys(Mazurin A.V., Vorontsov I.M., 1985)

The severity of the development of secondary sexual characteristics in girls

(Mazurin A.V., Vorontsov I.M., 1985)

SEXUAL DEVELOPMENT ASSESSMENT

To assess sexual development in our country, standard tables of puberty are used, according to which the data of the child's sex formula, taking into account the presence and severity of secondary sexual characteristics, are compared with average age indicators.

Girls' sexual development standards

(Maksimova M.V.)

Boys Puberty Standards

(Maksimova M.V.)

It should be noted that the assessment of the puberty of boys using standardized tables, without taking into account the state of the genitals, is indicative and not entirely correct, since in this case they do not focus on the main androgen-dependent signs that are decisive in the development of reproductive function.

Currently, the system for assessing the stage of puberty recommended by J. Tanner (1985) has become widespread throughout

An example of an assessment of sexual development:

1. Ivanov N., 12 years old. Sex formula V0 P0 L0 Ax0 F0

Conclusion: sexual development corresponds to age.

2. Sonina K., 13 years old. Sex formula Ma3 P3 Ax3 Me3

Conclusion: sexual development is accelerated.

SEMIOTICS OF DISORDERS OF SEXUAL DEVELOPMENT

PREMATURE SEXUAL DEVELOPMENT

The appearance of secondary sexual characteristics in girls under 8 years of age and in boys under 9 years of age is considered as precocious sexual development.

PPR is a heterogeneous condition in its etiology and pathogenesis. In girls, the activation of the gonadal function is often of a short-term functional nature and is due to the instability of the processes of suppression of the hypothalamic-pituitary activity during childhood. Less commonly, the process of puberty has a progressive course and is the result of severe disorders of the hypothalamic-gonadal and adrenal systems.

The classification of the PPR syndrome is based on the pathogenetic principle, which takes into account the primary localization of the process in the hypothalamus-pituitary-gonadal-adrenal system. Allocate true, or central, forms of the disease, the pathogenesis of which is due to premature activity of the central part of the gonadostat: the hypothalamic-pituitary system. An increase in the secretion of sex steroids by the gonads in this case is a consequence of stimulation of the gonads by gonadotropins. False, or peripheral, forms of PPR are due to premature secretion of sex hormones by tumors of the gonads or adrenal glands, regardless of the secretion of gonadotropins. In an independent group, the so-called gonadotropin-independent forms of PPR are distinguished, in which the autonomous activation of the activity of the gonads is due to genetic disorders. In all the listed forms of the disease, sexual development has all the main characteristics of progressive puberty: in addition to the appearance of secondary sexual characteristics, the volume of the gonads increases, the rate of growth and bone maturation accelerates, reflecting the systemic effect of sex steroids on the child's body. Clinical variants of PPR with this set of features are defined as the full form of PPR. In addition, the so-called partial (incomplete) forms of PPR are distinguished, characterized by an isolated development of secondary hair growth (premature pubarche) and an isolated increase in the mammary glands (premature thelarche). There are also variants of PPR that do not fit unequivocally into any of the listed forms of the disease: PPR against the background of decompensated hypothyroidism.

It should be noted that there is no clear line between true and false forms of PPR. False forms of the disease due to the presence of hormone-producing tumors of the gonads, congenital dysfunction of the adrenal cortex can spontaneously transform into true forms of the disease, which is associated with secondary activation of the hypothalamic-pituitary axis.

PPR classification

1. True (central) gonadotropin-dependent PPR

1.1. idiopathic

1.2. Cerebral (CNS tumors, arachnoid cysts, encephalitis, meningitis, toxoplasmosis, surgery, congenital syndromes: Russell-Silver syndrome, Van Wyck-Grombach syndrome, etc.)

2. False, gonadotropin-independent PPR

2.1. In boys (testicular tumors, adrenal tumors, congenital adrenal dysfunction)

2.2. Girls (ovarian tumors, adrenal tumors, ovarian follicular cysts)

3. Gonadotropin-independent forms (McCune-Albright-Braytsev syndrome, testotoxicosis)

4. Incomplete forms of PPR (accelerated pubarche, accelerated thelarche)

True precocious puberty

Sexual development may be completed quickly or slowly; the maturation process may stabilize or even regress, only to start again later. The first sign in girls is the development of the mammary glands, possibly simultaneous pubic hair growth, but more often it appears later. Then the vulva develops, hair appears in the armpits and menstruation begins. Early menstrual cycles may not be as regular as with early puberty.

In boys with PPR, the penis and testicles increase, pubic hair appears, and frequent erections occur. The voice becomes lower, growth accelerates. Spermatogenesis occurs already at the age of 5-6 years, and nocturnal emissions are possible.

In both boys and girls, PPR is accompanied by an increase in body length and weight. The differentiation of the bones of the skeleton is accelerated and corresponds to the degree of sexual development. This leads to early closure of growth zones, as a result, the final growth is lower than it could be with timely sexual development. Growth in approximately 1/3 of patients does not reach 152 cm. The development of teeth and intelligence corresponds to chronological age.

Plasma levels of FSH and LH may be high in relation to the age of the patient. However, in 50% of patients, the indicators are within the normal range. Elevated hormone levels may alternate with normal levels. Plasma levels of testosterone (in boys) and estradiol (in girls) are usually elevated in line with puberty and bone age. Changes in the EEG are possible, indicating a primary pathology of the nervous system.

The cause of PPR can be a variety of CNS lesions. All of them are associated with scarring, invasion or compression of the hypothalamic region. The most common are pinealomas, gliomas of the optic nerves, teratomas located above the Turkish saddle, neurofibromas, astrocytomas, and epindymomas. PPR is accompanied by hypothalamic hamartomas.

Hamartoma is a benign ectopia of brain tissue, in 70% of cases it contains neurosecretory granules of lulebirin (LH-releasing hormone). Ectopia is caused by impaired migration of luliberin-secreting neurons during embryogenesis, which leads to their localization outside the hypothalamus. They can function autonomously by secreting luliberin, which in turn stimulates the secretion of gonadotropins. Children with hamartomas have a very early onset of PPR. Girls are characterized by an early (up to 3 years) onset of menstruation, which is regular. Patients have pronounced neurological symptoms, there may be convulsive absences in the form of violent laughter. The hormonal status of children with hypothalamic hamartoma is characterized by high LH and FSH values ​​corresponding to mature puberty, as well as a pronounced rise in LH to stimulation with LH-releasing hormone, corresponding to the nature of the response in adults.

intracranial tumors ( germinomas) cause precocious puberty in boys by secreting human chorionic gonadotropin, which stimulates Leydig cells in the testicles. Chorionic gonadotropin-secreting germinoma in girls does not cause PPR, since there is no FSH.

At liver tumors(hepatoblastoma, hepatoma) PPR occurs as a result of the production of chorionic gonadotropin by tumor cells. Other tumors (chorio- and teratocarcinomas or teratoma) can also secrete chorionic gonadotropin and provoke PPR. Tumors are localized in the central nervous system, mediastinum or gonads. They are more common in boys (21 per 100) than in girls (2 per 100). Tumors of the mediastinum are common in boys with Klinefelter's syndrome. The serum of patients contains human chorionic gonadotropin and alpha-fetoprotein in large quantities, the level of FSH is reduced, and LH is elevated due to cross-reaction with chorionic gonadotropin.

At untreated hypothyroidism puberty in children is usually delayed and does not begin until the moment when the bone age corresponds to 12-13 years. However, premature isosexual development is also possible ( Van Wyck-Grombach syndrome), leading the process of ossification. Sexual development is usually manifested by the growth of the mammary glands in girls and the enlargement of the testicles in boys. At the same time, changes in the secretion of androgens by the adrenal cortex, characteristic of the pubertal period, are weakly expressed, as evidenced by poor pubic and axillary hair growth or its complete absence. Menstrual bleeding can be even with minimally developed mammary glands. The plasma level of TSH is sharply elevated; for some unknown reason, prolactin, LH and FSH are also secreted in excess.

With the syndrome Russell-Silver precocious puberty may also occur.

Albright syndrome- a combination of fibrous skeletal dysplasia with patchy skin pigmentation and endocrine disorders. Of the endocrine disorders, precocious puberty is most often observed, but hyperthyroidism and cushingoid syndrome are also possible. Most of the patients are girls. Previously, it was believed that endocrine disorders in this syndrome are associated with pathology of the hypothalamic-pituitary region, but autonomic hyperfunction of peripheral target glands has now been proven. Girls have low pre-pubertal LH and FSH values, both basal and luliberin-stimulated, with extremely high levels of estradiol. Also, the LH rise characteristic of puberty at night is not detected. Many sick girls have ovarian cysts on ultrasound; the level of estradiol at the same time correlates with the size of the cysts. At a later age, some patients may have signs of true puberty; this is confirmed by the fact that early pseudopuberty contributes to the activation of the hypothalamic-pituitary system.

In some children who are late treated for congenital adrenal dysfunction, a clinic of true precocious puberty develops. More often this happens if the bone age at the beginning of therapy corresponds to puberty - 12-14 years.

In the familial form of the male type of PPR (testotoxicosis), hyperplasia of the interstitial Leydig cells, sometimes in the form of adenomatous nodules, and maturation of the spermatogenic epithelium are observed. The disease is genetically determined and is transmitted from sick men and healthy women in an autosomal dominant manner with manifestation only in males, although sporadic forms also occur. The disease begins early (mean age 1.3±1.2 years) and is accompanied by a rapid rate of masculinization and bone maturation. A hormonal study reveals low basal and stimulated (LH-releasing hormone) levels of gonadotropins, their low daily fluctuations against the background of high testosterone levels corresponding to sexually mature age. As the child grows, it is possible to restore the hypothalamic-pituitary control of gonadal function, that is, the transition of the gonadotropin-independent form of PPR to gonadotropin-dependent.

False precocious puberty

The main cause of false precocious puberty in children of both sexes are hormonally active tumors of the gonads or adrenal glands. In addition, viril forms of congenital dysfunction of the adrenal cortex should also be attributed to false precocious sexual development.

The ovaries and testicles are capable of producing both male and female sex hormones, and in the same way, hormonally active gonadal tumors are capable of producing both types of hormones in children of both sexes. Depending on the predominance of certain sex steroids, a hormonally active tumor can cause a clinic of precocious puberty according to the isosexual (peculiar to the sex of the child) or heterosexual (peculiar to the opposite sex) type.

Hormonally active ovarian tumors- estrogen-producing, mainly from granulosa cell tissue, less often - highly differentiated forms of teratoma, secreting a large amount of estrogens - more common in girls under the age of 4 years. A characteristic and often the first clinical symptom is acyclic menstrual-like discharge. Secondary hair growth is poorly developed. The external genitalia are sharply estrogenized. In the blood and urine, high levels of estrogen are detected.

Hormonally active androgen-producing ovarian tumors(arrenoblastomas) occur in older girls. Their clinical manifestation is due to an excess amount of androgens in the body. A picture of the viril syndrome develops. At puberty in girls, menstruation stops or does not occur, the mammary glands atrophy, male-type hair growth develops, the voice coarsens, the clitoris hypertrophies and virilizes. The examination reveals a high level of testosterone, increased urinary excretion of 17-ketosteroids, although not to the same extent as with hormone-producing tumors of the adrenal glands. The main diagnostic method is an ultrasound examination of the pelvis.

Hormonally active testicular tumors(androblastomas and interstitial cell tumors) are relatively rare. Androblastoma is more often benign, but its malignant degeneration has also been described. The diffuse type of tumor has the highest hormonal (androgenic) activity. It is characterized by distinct endocrine manifestations: significant masculinization, sometimes true gynecomastia. With the development of a tumor mainly from tubular epithelial elements, an estrogenic effect can be expected, especially since androgens and estrogens can transform into each other.

Congenital hyperplasia of the adrenal cortex (adrenogenital syndrome) is most often caused by a deficiency of 21-hydroxylase. Two classical forms of the disease are known: salt-losing and simple virilizing. In girls, congenital adrenal hyperplasia leads to female pseudohermaphroditism. Violation of steroidogenesis manifests itself in the early stages of fetal development, therefore, signs of masculinization are already expressed to one degree or another at birth: an increase in the clitoris, a more or less pronounced fusion of the labia, and an urogenital sinus. The internal genital organs do not differ from those in healthy girls. After birth, masculinization progresses. Hair grows prematurely on the pubis, in the armpits, the voice becomes coarser, the sick girls are taller than their peers, the bone age is ahead of the chronological one, their muscles are well developed. If appropriate treatment is not carried out, the mammary glands do not develop and there is no menstruation. In the salt-losing form, virilization is more pronounced than in the variant without salt loss.

In boys with a salt-losing form of adrenogenital syndrome, vomiting, shock and electrolyte imbalance appear at the age of 7-10 days. In males without signs of excessive excretion of salt from the body, the violation manifests itself as signs of premature isosexual development. At birth, the child looks normal, but signs of premature sexual and somatic development may appear already in the first half of life or develop more slowly and become apparent only at the age of 4-5 years and later. These signs include: an increase in the penis, scrotum, the appearance of pubic hair, acne, the smell of sweat, a decrease in the timbre of the voice. The testicles are of normal size, but appear small compared to the enlarged penis. The muscular system is well developed, bone age is ahead of chronological age. Mental development does not suffer, but due to the peculiarities of physical development, behavioral anomalies are possible. Premature closure of the epiphyses leads to early closure of the growth zones and, as a result, short stature.

There are incomplete forms of precocious puberty. Premature thelarche is an isolated development of the mammary glands in girls under the age of 8 years without other signs of puberty. Most often begins in the first 2 years. Sometimes only one gland is enlarged, or one enlarges more than the other. In 50% of children, the glands regress within 2 years, in the rest they persist until the age of 5 years and older. Premature thelarche is usually a benign process; in some cases, this is a familial trait and may be the result of increased sensitivity of breast tissues to normally low levels of estradiol in prepubertal age. Growth and ossification of the skeleton are not impaired, menstruation occurs at normal times. Plasma FSH and LH levels are usually normal, however, the reaction on the introduction of luliberin may be increased, the level of estradiol is within the normal range or slightly increased.Premature thelarche may be a sign of the onset of true puberty or pseudopuberty.It may be due to drug treatment or other exogenous exposure to estrogens.

Premature adrenarche - isolated growth of pubic and axillary hair in the absence of other signs of puberty in girls under the age of 8 years and in boys under 9 years of age. It occurs much more frequently in girls than in boys. Hair first appears on the labia, then on the pubis, and finally in the armpits. Then the smell of sweat, characteristic of adults, appears. When examining children, some acceleration of linear growth and differentiation of the bone skeleton (within 1-2 years) can be noted. The levels of gonadotropic hormones and major sex steroids do not exceed the age norm.

The appearance of secondary sexual characteristics, like the onset of premature puberty, can be caused by a variety of medications (estrogen intake, the introduction of anabolic steroids, impurities of sex hormones in foods, vitamin preparations). Estrogens contained in cosmetics can be absorbed through the skin. Exogenous estrogens cause an intense dark brown coloration of the areola of the breast, which is not usually found in endogenous types of prematurity. Prematurely appeared signs disappear with the cessation of the introduction of exogenous hormones.

DELAYED SEXUAL DEVELOPMENT

Delayed puberty is the absence of any signs of puberty in an adolescent who has reached the upper age limit of normal puberty. This means no increase in testicular volume (<4мл) у мальчиков к 14 годам и отсутствие увеличения молочных желез у девочек к 13 годам. Полное обследование необходимо проводить девочкам при отсутствии развития грудных желез в возрасте 13 лет и отсутствии менструаций в возрасте 15 лет. Мальчиков нужно обследовать в тех случаях, если у них длина яичек не достигает 2,5 см в возрасте 15 лет.

The causes of delayed puberty in children of both sexes can be divided into three main groups. The first, most frequent, is a temporary functional, or constitutional, delay in the maturation of the hypothalamic-pituitary region. The second reason is organic lesions of the hypothalamic-pituitary region, leading to a decrease in the secretion of gonadotropins (hypogonadotropic hypogonadism). The third reason is primary gonadal insufficiency, leading to disinhibition of gonadotropic secretion (hypergonadotropic hypogonadism).

Delayed sexual development of a constitutional nature- the most common cause of puberty disorders in children, it can be considered as an extreme version of the norm. However, functional disorders in the maturation of the hypothalamic-pituitary region may be due to the influence of adverse exogenous factors (chronic diseases, stress, physical and emotional overload, etc.). There are reports of the impact on the course of puberty of mutant forms of PH with a shorter life span. In the general population, homozygous carriage of abnormal forms of PH is 3%, and heterozygous - 26%. The presence of abnormal PH leads to a delayed course of puberty and may further lead to impaired reproductive function. Boys are more likely to complain of delayed puberty (9:1), although the incidence of this condition is the same for both sexes. This is due to the greater psychological maladjustment of boys. The main reason that makes a teenager suffer is growth retardation, since the growth spurt in boys is significantly remote in time from the first appearance of secondary sexual characteristics.

Hypogonadotropic hypogonadism can occur both in isolation and in combination with other variants of pituitary insufficiency or disorders in the formation of brain tissue.

Cullman's syndrome- a congenital disease with an autosomal dominant or X-linked autosomal recessive type of inheritance with varying degrees of expressiveness, more common in boys. The main characteristic of the syndrome, in addition to hypogonadism, is anosmia due to agenesis of the olfactory lobes. The olfactory lobes are the site of prenatal laying of neurons that secrete luliberin, which then migrate to the hypothalamus. Thus, agenesis of the olfactory zones leads not only to anosmia, but also to the hypothalamic form of hypogonadism.

Panhypopituitary insufficiency, in which a deficiency of gonadotropins is combined with a loss of secretion of somatotropic hormone (STH), TSH, ACTH, is often caused by tumors of the central nervous system that destroy pituitary tissue. Craniopharyngioma is the most common cause of decreased pituitary function in pubertal children. The clinical manifestation of the disease is primarily associated with a sharp decrease in the growth rate of the child due to a decrease in the secretion of growth hormone. Symptoms of diabetes insipidus and hypothyroidism are detected early enough. With a pronounced tumor volume, visual impairment develops, including bilateral narrowing of the fields associated with tumor pressure on the optic chiasm. Hypogonadism is detected in the vast majority of cases, but is not the leading symptom of craniopharyngioma.

Hypogonadotropic hypogonadism in combination with other manifestations of hypopituitarism can develop as a result of radiation therapy of head and neck tumors, accompany such genetic diseases as Prader-Willi syndrome, Lawrence-Moon-Bill syndrome, severe somatic and endocrine diseases.

Hypergonadotropic hypogonadism can develop as a result of congenital, genetically determined lesions of the gonads (Shereshevsky-Turner syndrome in girls, Klinefelter syndrome in boys, testicular dysgenesis, enzymatic disorders of testosterone synthesis). Acquired primary hypogonadism can be the result of traumatic damage to the gonads, exposure to radiation therapy, infections, and an autoimmune process.

The greatest difficulty for diagnosis is presented by two forms of delayed puberty - delayed puberty of a constitutional nature and isolated hypogonadotropic hypogonadism, while gonadal forms of hypogonadism are easily diagnosed by a sharp increase in LH and FSH already at early puberty (10-11 years). Constitutional delay in puberty and hypogonadotropic hypogonadism are characterized by equally reduced levels of gonadotropins and sex steroids. One of the most reliable diagnostic tests to separate these two conditions is the daytime and nighttime LH secretion test. During sleep in children with constitutionally delayed puberty, LH levels are significantly higher even in the absence of any signs of puberty. In children with central hypogonadism, there was no difference between nocturnal and daytime secretion of LH. The test with analogues of luliberin with 24-hour action (nafarelin, buserelin, diferelin) also has a high diagnostic value. The introduction of the analog stimulates a significant rise in LH after 6-8 hours in children with a constitutional delay and does not affect the level of LH in children with hypogonadotropic hypogonadism. The algorithm for monitoring children with puberty disorders is shown in fig.

Conclusion

Childhood is a period when the human body grows, develops and improves. It includes the period of life from birth to puberty. Periodization issues are highly controversial due to the lack of consensus on the criteria for the boundaries between age stages. A growing organism develops strictly individually, passing through its own unique way of life. Often physical and mental maturation, the functional organization of the motor apparatus and internal organs, i.e. everything that characterizes the so-called biological age is not consistent with the calendar age, ahead of it, or, conversely, lagging behind. Approximately 30% of children are ahead, and about 15-20% lag behind their peers in their development. The term biological age refers to the level of physical development and other life processes achieved by an individual. Science has a large amount of factual material about the discrepancy between calendar and biological age, and therefore it is understandable that children of the same calendar age react differently to physical and mental stress, the influence of environmental factors.

The problems of studying the physical development of children are given a lot of attention in the scientific literature by both domestic and foreign scientists. Of course, without information about physical development and physical performance, it is not possible to judge the state of health, socio-hygienic and socio-economic conditions of life, abilities and preparation for work and sports. Quantitative determination of physical performance is necessary in the organization of physical education of the population of different age and sex groups, in the selection, planning and forecasting of training loads of athletes, in the organization of the motor regime of patients in clinics and rehabilitation centers, etc.

The recommended procedure for determining physical development includes the following activities in sequence: measurements and weighing according to the generally accepted methodology; assessment of constitutional features of physique and puberty; definition of the age group; recording the obtained measurements in centile intervals. A direct assessment of physical development implies an assessment of each individual indicator, as well as their combination, the marked dynamics in comparison with previous measurements and the determination of further tactics for monitoring the child.

Of course, in assessing the morphological and functional characteristics of the human body, it is necessary to apply uniform methods and approaches. Unfortunately, the often used typologies do not establish a single relationship between the growth rate of individual body sizes and the maturation time of the children's body. The correct assessment of the rate of individual development of the human body is determined only in the case when the analysis of somatic characteristics is carried out using indicators of the biological maturation of the organism.

Currently, the pediatrician begins to trace the assessment of the physical development of the child from the children's clinic, determining a comprehensive assessment of the state of health. Assessment of the state of health is carried out for all children at certain epicrisis periods of life. Epicrisis terms - this is the period of time after which a mandatory comprehensive assessment of the state of health is carried out: at 1 year of life - 1 month (1 time per month); in the 2nd year - 3 months (1 time in 3 months); in the 3rd year - 6 months in (1 time in 6 months); from 4 to 7 years and older - 1 year (1 time in 1 year).

For a comprehensive assessment of the state of health of a child in a children's clinic, the following criteria are used:

Anamnesis (genealogical, biological, social);

Level of physical development;

Level and harmony of neuropsychic development;

Functional state of organs and systems;

The degree of resistance of the organism;

The presence or absence of chronic diseases or congenital malformations.

BIBLIOGRAPHY

1. Dedov I.I., Semicheva T.V., Peterkova V.A. - Sexual development of children: norm and pathology / Moscow, 2002

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The period in the life of children when their accelerated sexual development and puberty is reached is called the period of puberty, which occurs mainly in adolescence. The puberty of girls usually outstrips the puberty of boys, as well as a significant individual variation in the timing and pace of this maturation. The course of puberty is influenced both by the hormonal status of the organism itself (the activity of the pituitary, pineal and adrenal glands), as well as by a number of external factors (hereditary characteristics, health status, diet, work and rest regimen, climate characteristics, domestic and socio-economic living conditions and etc.). Sexual development is usually inhibited under unfavorable living conditions, with excessively intensive sports or hard physical labor, with inadequate food (insufficient content of proteins, fats, carbohydrates and vitamins), with severe or repeated (chronic) diseases. In large cities, adolescent puberty usually occurs earlier than in rural areas.

Puberty is associated primarily with the development of primary and the appearance of secondary sexual characteristics. The primary sexual characteristics, as mentioned, include the development of the sex glands and genital organs in girls - the ovaries, vagina, uterus, oviducts; in boys - testicles, penis, prostate. During puberty, women develop the formation of mature eggs, and men - sperm.

Secondary sexual characteristics in women are the features of the development of the larynx, skeleton and muscles according to the female type, the appearance of hair on the pubis and under the arms, the development of the mammary (mammary) glands, the appearance of a peculiar roundness of forms, a change in the shape of the body, the emergence of interest in the other sex, a change in the psyche and behavior.

In men, the appearance of a mustache and beard, an increase in the thyroid cartilage of the larynx, the appearance of an Adam's apple, a change in voice, the appearance of hair on the pubis, under the arms and on the body, the development of the skeleton, muscles and body shape according to the male type, the appearance of interest in the other sex, and mental and behavioral changes.

The period of puberty is associated with profound morphological and functional changes in all organs and the organism as a whole. The relationship between the endocrine glands and, above all, the hypothalamic-pituitary system is changing. Under the influence of the somatotropic hormone of the pituitary gland, the growth of the body in length increases. The pituitary gland also stimulates the activity of the thyroid gland, enhances the activity of the adrenal glands and gonads. The growth of the secretion of sex hormones just contributes to the development of the so-called secondary sexual characteristics.

Puberty is not a smooth process and has certain stages, each of which is characterized by the specifics of the functioning of the endocrine glands and the whole organism as a whole. Stages are determined by the combination of primary and secondary sexual characteristics. Both boys and girls have 5 stages of puberty.

Stage I: prepubertal, or the period of childhood, covers the entire period of a child's life immediately preceding puberty: in girls - up to 8-9 years; in children, this stage lasts 1.5-2 years longer, namely up to 9-10 years. In the blood of both boys and girls of this age period, the same amount of both sex hormones (androgens and estrogens) is observed, which are derivatives only of the adrenal glands. In this regard, underdeveloped primary sexual characteristics remain in the body of children and the development of secondary sexual characteristics is completely absent.

Stage II: the beginning of puberty, or the beginning of adolescence. In girls, this lasts from 8-9 to 10-11 years and is characterized by the beginning of the growth of the internal genital organs: the uterus, fallopian tubes, ovaries and vagina; at 10 years of age, the swelling of the mammary glands begins, a slight hair growth appears along the labia. In boys, this stage lasts from 9-10 to 11-12 years and is associated with an increase in the size of the external genitalia and sex glands (an increase in the size of the testicles), a slight pubic hair also appears (however, the hair is still sparse and straight). Both in men and women during this period, the secretion of sex hormones increases, the function of the adrenal glands is activated. With the onset of puberty, the pituitary gland is sharply activated, its gonadotropic and somatotropic functions increase. The increase in the secretion of somatotropic hormone at this stage is more pronounced in girls, which leads to a more significant activation of their growth processes (girls begin to overtake boys in growth). This acceleration in the growth of the body length of children is called the "pubertal leap". For girls, the "growth spurt" occurs at 11-13 years old, for boys - at 13-15 years old. In the indicated periods of the life of children, the increase in body length increases sharply (the second period of accelerated growth) and reaches 8-10 cm per year.

Stage III: the first period of puberty (the beginning of adolescence). In girls, this is the period from 12 to 13 years and it consists in the further growth of the internal and external genital organs, mammary glands. Hair spreads towards the pubis and appears in the armpits. There is a further increase in the blood levels of gonadotropic (FSH) pituitary hormones. From C years, irregular maturation of individual eggs can be observed and the first menstruation appears. Such menstruation can last up to 7-9 days, sometimes accompanied by significant pain, and their subsequent repetition is habitually delayed for several months, and sometimes for a whole year or more.

In boys, this period lasts from 13 to 14 years of age and is associated with a subsequent increase in the testicles and penis (mainly in length). Pubic hair becomes darker, coarser, begins to spread to the perineum. The function of the sex glands is activated. Mature male sex cells (spermatozoa) begin to form in the testicles already at the age of 13-14 years, therefore, during this period, the first spontaneous ejaculation of the seed may appear, which usually occurs during sleep and is called pollution. In healthy boys who develop normally, at the age of 13-14 years, there is an increase in the nipples and even a slight swelling of the rudiments of the mammary glands. These changes are explained by the reaction of the rudiments of breast tissue to a sharp increase in the release of sex hormones, but these phenomena are transient and disappear on their own by the age of 14-15. In boys from the age of 13-14, the secretion of somatotropic hormone of the pituitary gland also increases, which causes the beginning of an accelerated growth in the length of their body ("growth spurt"), due to which they gradually begin to catch up and overtake girls in growth. From 12-13 years old, men begin intensive growth thyroid cartilage of the larynx, clearly visible on the front surface of the neck in the form of a protrusion (the so-called "Adam's apple" or Adam's apple), which causes a break in the voice.

IV stage: the second period of puberty (continuation of adolescence). In girls, this lasts from 14 to 15 years, during which the genital organs continue to develop intensively, the growth and development of the mammary glands is completed, pubic and axillary hair growth continues according to the adult type, but it remains less common. The maturation of eggs in the ovaries in most girls gradually acquires a certain periodization, contributes to the normalization of regular menstruation, but in about 10-12% of girls aged 13-14 years, menstrual cycles may still remain irregular. Only at the age of 15-16 years, the ovarian function in healthy girls usually acquires a cyclical nature, typical for an adult woman; they begin to form a sufficient amount of sex hormones and menstruation is normalized. This is the so-called physiological period of the formation of menstrual function. It should be emphasized that irregular menstruation after the age of 15 indicates deviations from normal sexual development and requires a special medical examination. From the age of 14, girls begin to change in the distribution of adipose tissue: the deposition of fat on the hips, in the abdomen and shoulder girdle increases and, thus, a female body type begins to form. Noticeable changes also occur in the structure of the skeleton, especially the bones of the pelvis, significantly increase in width. At this stage, sex hormones (estrogens) begin to be intensively produced, and the content of somatotropic hormone in the blood decreases and the growth rate of the body of girls falls.

In boys, the youthful stage of puberty occurs at the age of 15-16 and is characterized by the preservation of a high level of somatotropic hormone and androgens in the blood, which determines the accelerated rate of their growth. From this moment, the guys begin to overtake the girls in terms of growth in body length. The size of the external genital organs continues to increase, the voice finally changes (becomes lower, rougher), juvenile acne appears, the hair growth of the armpits and pubis is basically completed and the hair growth of the body begins. Facial hair appears first on the upper lip, then on the cheeks and chin. In children of this period, the ability to perform sexual intercourse is gradually formed, then the ability to ejaculate (semen eruption), and then the ability to fertilize.

Stage V: completion of puberty (the onset of biological puberty - adolescence). During this stage, for girls, at 16-17 years old, and for men at 17-18 years old, all anatomical and functional changes associated with puberty are completed. In healthy girls, they develop normally, a regular normal sexual cycle and characteristic female features of body shapes are established. The sexual cycle is considered normal when menstruation occurs at regular intervals, the same number of days continue with the same intensity. Normal menstruation lasts on average, as indicated, from C to 5 days, and during this time about 50-250 cm3 of blood is released. If menstruation is established, then they are repeated every 24-28 days.

In boys, at the stage of completion of puberty, the gonads and genital organs finally develop, the formation of sperm is stabilized, the development of secondary sexual characteristics according to the type of the male body is basically completed, a specific male type of pubic hair is formed (hair spreads cone-shaped in the navel). At the end of puberty, hair appears on the anterior surface of the chest. It should be pointed out that the intensity of the development of the hairline in men is largely determined by hereditary, genetic factors, on which the prevalence of the hairline also depends. During puberty in boys, in addition to the above changes, there is an intensive development of muscles, which subsequently determines greater muscle strength than in girls.

By the end of sexual development at the age of 15 years in girls and 16 years in men, the formation of somatotropic hormone decreases and, as a result, the annual increase in body length first decreases and can be only 0.5-2 cm per year, and from the age of 19-20 years in girls and 21-24 years old in men, it usually stops completely.

Due to the intensive growth of the bone skeleton and muscular system in adolescents, the development of internal organs (heart, lungs, gastrointestinal tract) does not always have time, which can cause various temporary functional disorders in the body of children. This must be taken into account when organizing both educational and physical (including sports) work of adolescents. So, for example, the growth of the heart usually outstrips the growth of blood vessels, as a result of which blood pressure can rise (the so-called teenage hypertension manifests itself), which in turn makes it difficult for the heart itself to work. At the same time, the rapid restructuring of the whole organism, which occurs during puberty, makes increased demands on the work of the heart. As a result, heart failure ("youthful heart") may occur, which often leads to dizziness and even to short-term unconsciousness due to spasms of the cerebral vessels. There may also be headaches, fatigue, periodic bouts of lethargy, cold extremities. With the end of puberty, these disorders usually disappear without a trace.

At the stage of puberty, due to the general activation of the hypothalamus, undergo significant changes in the function of the central nervous system. The emotional sphere is changing significantly: the emotions of adolescents become mobile, changeable, and contradictory. The increased sensitivity of the character of children is often combined with callousness, shyness, and deliberate swagger. Usually there is excessive criticism and intolerance towards parental care. During this period, sometimes there is a decrease in mental and physical performance, neurotic reactions, irritability, tearfulness are observed (especially in girls during the first menstruation).

In adolescence (transitional) age, the personality of a teenager is intensively formed, a feeling of adulthood arises, attitudes towards members of the opposite sex change. Children, during this period of their life, need a particularly sensitive attitude of parents and teachers. You should not specifically draw the attention of adolescents to complex changes in their body, psyche, but it is important to explain the regularity and biological meaning of these changes. The art of the educator is to find such forms and methods of work that would switch the attention of adolescents to various types of socially useful activities, distract them from sexual experiences (for example, during this period it is advisable to increase the requirements for the quality of education, work, behavior, activities sports, etc.).

At the same time, a tactful, respectful attitude of adults to the initiative and independence of adolescents, the ability to direct their energy in the right direction is very important. During puberty, it is important to create conditions for the normal physical development of the youthful organism. You need a varied, adequate diet with plenty of vitamins, as well as long stays in the fresh air, sports, and the like.

The period of onset of the biological puberty of girls and boys requires special attention of teachers.

In girls, the first menstruation is sometimes accompanied by poor general condition, weakness, pain, or significant blood loss. There may also be a slight increase in temperature, vomiting, diarrhea or constipation, dizziness. It is not true that during menstruation it is necessary to lie down. With good health, you need to lead a normal lifestyle, continue to do morning exercises and simple physical exercises. For this time, exercises related to jumping, cycling, lifting heavy things are prohibited. It is also not recommended to skate, ski, make long walks, take hot baths, swim and sunbathe. Various nervous shocks, severe physical pain, moving from north to south, from the lowlands to the mountains can disrupt the menstrual cycle, and long, exhausting work, chronic overwork can even cause the cessation of menstruation. If menstruation passes with significant pain, too heavy for bleeding, then you should consult a doctor. During menstruation, accompanied by a deterioration in the general condition of the body, girls need to be released from work or work. During menstruation, girls should be protected from overcooling, especially the legs and lower abdomen. Do not sit on cold stones and other chilled objects.

From the diet during menstruation, strongly stimulating substances such as vinegar, mustard, pepper, horseradish should be excluded. You can not drink beer, wine and other alcoholic beverages, because due to increased blood flow, this can lead to an increase in menstrual bleeding. It is especially necessary to monitor the timely emptying of the bladder and intestines, since their overflow leads to displacement of the uterus, which can cause pain and delay discharge. During menstruation, it is necessary to carefully monitor the cleanliness of your body, since the inner surface of the uterus bleeds at the same time, turns into a kind of surface of an open wound, where pathogenic microbes can find favorable conditions for their development.

In boys during puberty, as mentioned above, involuntary eruptions of the seed - wet dreams (from the Latin Pollucio - pollution) can occur, which most often occurs during sleep. The appearance of the first wet dream indicates that the boy began to produce spermatozoa. Mixing with the secretions of the seminal vesicles and pidmihurov gland, they accumulate in the form of sperm in the genital tract and are naturally removed after the tension of the penis in the form of nocturnal involuntary eruptions. The first wet dreams usually occur around the age of 15-16. Since then, wet dreams can even occur in an adult male with prolonged sexual abstinence. With the help of wet dreams, the body is freed from excess sperm and sexual tension. This is quite expedient and the body's natural reaction creates the physiological conditions for sexual abstinence. Thus, the fact of wet dreams is an absolutely normal, physiological phenomenon, so they should not be afraid or ashamed, and after them there are no disorders of sexual function. Pollutions usually occur from 1-3 times a month to 1 time in 1.5-2 months. On average, wet dreams appear intermittently from 10 to 60 days. If wet dreams are observed every night or even several times a night, then in this case you should consult a doctor. To prevent wet dreams from recurring very often, children are not recommended to eat spicy foods at night, drink plenty of fluids, cover themselves with too warm a blanket, sleep in swimming trunks or tight panties. The bed should not be too soft. In addition, it is necessary to keep the foreskin of the penis clean.

In adolescents of both sexes, onanism is often observed. Teenagers with an unstable mentality are especially susceptible to masturbation, as well as those who have physical developmental disabilities that prevent them from taking an active part in age-appropriate activities, work and entertainment. It is wrong to consider masturbation as the "disease of the century." However, onanism can also be a consequence of inflammatory changes in the genital organs in girls and boys. Itching in the vulva due to infection with pinworms can be one of the causes of onanism in children. According to neuropsychiatric observations, persistent onanism is often observed in children with certain mental illnesses. Only after making sure that masturbation is not a symptom of a certain disease, appropriate individual explanatory and educational work should be carried out.

It must be borne in mind that biological puberty cannot be equated with social maturity. Although a girl can become pregnant with the onset of menstruation, her body is not yet ready for a normal sexual life. This applies equally to adolescents - children who may have mature spermatozoa in the seminal fluid. Puberty of adolescent boys, even in physiological terms, occurs throughout adolescence. Social puberty can only be considered the age of full puberty (girls after 17-18 years old, and guys after 19-20 years old), when personality formation is completed and physical, spiritual and civic maturity begins. Social puberty provides the opportunity not only to conceive a child, but also the ability of parents to provide the best conditions for bearing and feeding a child and further normal all-round development.