What is the Disruption of the Sexual Development of Girls?
Violations of puberty in girls make up 3-4%. A significant role in the development of the disease is played by genetic predisposition, as well as adverse factors (radiation, hypoxia, viral infections, some drugs). Anomalies of the reproductive system are more common in children born to patients with alcoholism, drug addiction, endocrine diseases, as well as from elderly parents.
Sexual development is a genetically programmed process that begins at the age of 7-8 years and ends by the age of 17-18. The appearance of secondary sexual characteristics and menstrual-like discharge up to 7 years should be regarded as premature sexual development (PCD). Underdevelopment or lack of secondary sexual characteristics up to the age of 15–16 years refers to delayed sexual development.
Causes of Violations of the Sexual Development of Girls
Premature sexual development can be isosexual, i.e. on the female type, and heterosexual – on the male type.
An isosexual type of prostatic hyperplasia has cerebral (true prostatic hyperplasia) (may be complete and incomplete), have a constitutional and ovarian form (false prosthetic).
The cerebral form is considered true PPR because the pathological process involves the pituitary zones of the hypothalamus, where the premature secretion of HFHL leads to the production of gonadotropins in the pituitary gland, which, in turn, stimulates the secretion of estrogen in the ovaries.
The true PWD in girls is functional or organic. The causes of organic lesions are fetal hypotrophy, asphyxiation and birth trauma, meningitis, encephalitis and other neuroinfections. There are less common brain tumors – ganglioneuroma, hamartomas, astrocytomas.
A true MacRune-Ol-Bright-Braytsev syndrome, which includes not only PPR, but also fibrous dysplasia of tubular bones in combination with asymmetric pigmentation of the skin, is a true PCP. The causes of bone pathology are not entirely clear.
The constitutional form of the outage is hereditary, family nature.
The ovarian form of PPR is more often observed in case of hormone-producing ovarian tumors: granulocellular, thecellular tumors, teratoblastoma with elements of the hormone-active tissue. Follicular cysts, as a rule, small (3-4 cm) can also be the cause of PPR of ovarian genesis. At the same time, the hypothalamic-pituitary structures remain immature. The secretion of estrogen tumor autonomous.
Symptoms of Violations of the Sexual Development of Girls
Cerebral PPR manifested as incomplete (thelarche and / or adrenarche) and full form. The incomplete form of PPR is considered to be the stretched first phase of puberty. Menarche age in these girls is 10-11 years old. With full form, there are secondary sexual characteristics and menstruation. The development of secondary sexual characteristics with the full form of the true PPR is significantly ahead of that with timely puberty, although the sequence of their appearance is not disturbed. Bone age is significantly ahead of the passport, growth does not exceed 150-152 cm.
With MacKune-Albright-Braytsev syndrome, PPR can manifest itself in both incomplete and full form. In girls with an incomplete form, physical development is accelerated. The growth rate of the tubular bones and the rate of ossification of their epiphyses are the same, so they are no different from healthy ones in constitution and height. With full form, sexual development is significantly accelerated.
The constitutional form of IDP is not accompanied by any cerebral or neurological pathology. The sequence of the appearance of secondary sexual characteristics is not disturbed, and the duration of puberty is similar to physiological. It is only the age of menarche that matters (8-9 years).
In case of PPR of ovarian genesis caused by a hormone-producing tumor, there is no neurological symptoms, secondary sexual characteristics are slightly developed. The sequence of the appearance of signs of puberty is perverted: acyclic menstrual-like discharge appears first. Somatic development is not accelerated. Clinical manifestations of PPR in follicular cysts consist in scant serum secretions from the genital tract and a slight increase in the mammary glands. In follicular cysts, PPR symptoms are transient and undergo a reverse development as the follicular cyst regresses.
Diagnosis of Violations of the Sexual Development of Girls
The diagnosis of PPR is established on the basis of anamnesis, the dynamics of sexual and physical development, gynecological examination. Ultrasound of the pelvic organs, determination of the level of gonadotropins and estrogens in the blood are required, laparoscopy is performed if necessary, and bone age is determined and neurophysiological methods of investigation are used (REG, EEG). If a pituitary tumor is suspected, magnetic resonance imaging is indicated. Endocrinologists, neuropathologists, and ophthalmologists should be involved in the examination of these patients.
Treatment of Violations of the Sexual Development of Girls
Therapy of CPD includes the treatment of the underlying disease that caused CPD, and inhibition of CPD. When CPD cerebral Genesis treatment is carried out by neurologists or neurosurgeons. To inhibit puberty, drugs are used that act on hypothalamic structures that regulate the synthesis of lyuliberin or block the action of hormones on target organs (decapeptil depot, diferelin, androkur). Children with a constitutional form of PPD need only dynamic observation. Ovarian tumors should be removed, followed by histological examination. After 1.5-2 months after surgery, in these patients, all signs of SCC undergo regression. Follicular cysts are not recommended to be removed immediately, as cysts that do not exceed 3–4 cm in diameter undergo a reverse development within 2–3 months, and signs of arrhythmias disappear.
Heterosexual PPD is associated with hyperproduction of androgens and is observed in cases of congenital dysfunction of the adrenal cortex (adrenogenital syndrome) or virilizing adrenal tumors.