Premature sexual development

Precocious puberty is characterized by early development of the mammary glands, early onset of the menstrual cycle and early pubic hair in girls, early enlargement of the external genitalia in boys. Hair in the armpits is not clearly expressed or absent. The body is proportionally developed, children of both sexes do not differ in height from their peers, they do not lag behind in mental development, sexual desire and masturbation are not typical.

True precocious puberty is usually accompanied by infertility.

A thorough neurological and ophthalmological examination is necessary, which in the early stages may indicate the presence of a space-occupying process in the hypothalamus.

Some individuals experience puberty at age 8, which may be normal. If puberty begins before this age, the patient should be examined.

The first place among the causes of the disease is occupied by tumor lesions of the central nervous system with an impact on the hypothalamic region. Significantly sharply observed after encephalitis, meningitis, severe craniocerebral trauma. In some cases, premature puberty of a constitutional nature is possible.

Biological aspect. Each of the fiscal signs of puberty can be thought of as a kind of biochemical study for a certain hormone. Enlargement of the testicles in boys is the first sign of the onset of sexual development and is associated with the entry of shock doses of pituitary gonadotropin into the blood. Enlargement of the mammary glands in girls and the penis in boys is associated with increased secretion of gonadal sex steroids. The appearance of pubic hair is a manifestation of androgen production in the adrenal glands. Boys' growth accelerates when the volume of the testicles reaches 10-12 ml (when measured by the principle of comparison with orchidometer balls). Girls begin to grow more quickly as their mammary glands develop. The fourth stage of mammary gland development is characteristic of the onset of menstruation (in most girls). But this coordinated development of puberty signs can sometimes be disrupted. For example, with Cushing's syndrome, the intensity of pubic hair growth can disproportionately outpace the increase in testicular volume; In hypothyroidism, the testicles are quite large [the FSH level is elevated due to an even more significant increase in the TSH level], but the rate of growth increase is reduced.

Premature puberty in boys is manifested by rapid growth of the penis and testicles, increasing frequency of erections, masturbation, pubic hair, specific body odor and fumes. Accordingly, secondary sexual characteristics in girls also change. The most important and far-reaching "complication" is growth retardation caused by the fusion of the epiphyses with the diaphyses. When examining such children, parents should also be asked about some general endocrine symptoms characterizing hypothalamic dysfunction: polyuria, polydipsia, obesity, sleep disorders and temperature regulation. Signs of increased intracranial pressure and certain visual disorders may be observed.

Premature sexual development in girls occurs approximately 4 times more often than in boys. In girls, the cause is often not identified, while in 80-90% of boys it can be determined. If the onset of the disease occurs before the age of 2, the cause can often be a hamartoma developing in the hypothalamus. On a CT scan, it looks like a rounded "non-enhancing" formation.

Other (mostly rare) causes:

• CNS tumors and hydrocephalus.
• Condition after encephalitis or meningitis.
• McCune-Albright syndrome.
• Craniopharyngioma.
• Tuberous sclerosis.
• Hepatoblastoma.
• Choriocarcinoma.
• Hypothyroidism.

Examination of the patient. General X-ray of the skull, determination of bone age based on X-ray examination, CT scanning of the skull (head), study of urinary excretion of 17-ketosteroids, ultrasound of the pelvis (in girls), determination of T4 content in the blood.

Differential diagnosis. First of all, it is necessary to exclude tumors of the testicles or ovaries. A thorough gynecological examination should be the first stage of diagnosis. In case of tumors of the adrenal cortex, premature pseudomaturation occurs, in which hirsutism is significantly expressed, premature ossification of the epiphyses and, accordingly, short stature, obesity and arterial hypertension are noted. True menstrual cycles are not observed. As a rule, early menstruation quickly turns into persistent amenorrhea. It should be differentiated from Albright's disease, which develops only in girls, and from Recklinghausen's neurofibromatosis, which is often the cause of premature puberty in girls.

Physiology and treatment of precocious puberty

Treatment should be aimed at eliminating the underlying cause of the disease. In case of premature puberty of a constitutional nature, special treatment is not required.

The onset of puberty depends on the cessation of neuronal inhibition in the medial-basal hypothalamus, where gonadotropin-releasing hormone (GnRH) is secreted, and on a decrease in hypothalamic-pituitary sensitivity to negative feedback from gonadal steroids. These changes are accompanied by a significant increase in the frequency and power of sharp "flushes" (into the blood) of luteinizing hormone (LH) and, to a lesser extent, follicle-stimulating hormone (FSH). And it is the ability to secrete "pulse" amounts (shock amounts) of GnRH at high speed that determines normal gonadal function. A constantly high concentration of GnRH in the blood paradoxically suppresses the secretion of pituitary gonadotropins, which creates the basis for the treatment of premature puberty with synthetic analogues of GnRH.

After subcutaneous administration or after nasal insufflation of the drugs, there is a reversal of gonadal maturity and all clinical correlates of puberty (except pubic hair growth, since there is no change in the secretion of androgens by the adrenal cortex). The rate of skeletal maturation also decreases. Treatment is continued until mid-puberty or until the onset of menstruation (approximately 11 years). In families of such patients, the physician should instill hope that the sick child will develop normally in the future.

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