Early puberty

Early puberty

Stein-Leventhal syndrome is characterized by menstrual disorders, infertility, bilateral ovarian enlargement, hirsutism in the female phenotype.
The syndrome occurs in 1.45–2.8% of all gynecological diseases; it occurs in women more often at the age of 20–30 years.

Etiology. The cause of the syndrome is unknown. Rather, a polycystic change in the ovaries is a stereotypical response of the organism to various pathological states. Polycystic changes in the ovaries can occur, for example, in diseases of the hypothalamic-pituitary system, in patients with Itsenko-Cushing’s disease, congenital dysfunction of the adrenal cortex, in uterine fibroids, and a number of other conditions.

Pathogenesis. The pathogenesis of the syndrome is not fully established.
Most researchers believe that it is most likely caused by a violation of steroidogenesis in the ovaries. This impairment can be either primary due to the enzymatic block of estrogen synthesis from their predecessors, androgens, or secondary due to impairment of the hypothalamic-pituitary gonadotropic regulation or adrenal function. An enzyme block leading to a primary disorder of steroidogenesis in the ovaries, some authors see as a manifestation of the genetic deficiency of enzyme systems involved in the synthesis of estrogen in the ovaries.

A secondary violation of steroidogenesis in the ovaries due to a violation of the hypothalamic-pituitary regulation is primarily associated with excessive and monotonous (non-cyclic) secretion of luteinizing hormone (LH). Lack of cyclicity in the secretion of LH leads to anovulatory cycles and further to polycystose ovarian degeneration. Violation of cyclic LH secretion can occur, on the one hand, with an excess of androgens in the body, and on the other, with primary disorders in the hypothalamic region. It is believed that a violation of steroidogenesis in the ovaries occurs due to a deficiency of enzyme systems along the path of pathological conversion of pregnenolone to dehydroepiandrosterone with accumulation of the latter in polycystic ovaries, or along the path of accumulation of androstenedione with subsequent conversion to testosterone. Violation of steroidogenesis in the ovaries leads to a decrease in the content of estrogen and an increase in the content of androgens. As a result, the mental cycle and ovulation are violated.

Excess testosterone in the body leads to the development of hirsutism.
Pathological anatomy. Both ovaries are enlarged, have a brilliant gray surface and a dense consistency due to the sharp thickening and hyalinosis of the albugine. Histologically, a multitude of follicular cysts with atresia are noted. The outer walls of the cysts consist of hyperplastic tech-tissue, which is considered as the site of the formation of androgens, and the inner walls – of the follicular epithelium, often degenerating. A sharp decrease in the number of primordial follicles is found. In the cortex and medulla of the ovaries, a marked proliferation of connective tissue and vascular sclera are noted. Yellow bodies are absent (anovulatory state).

Clinic. Patients complain of menstrual disorder, infertility. Often there are hirsutism and hypertrichosis of varying severity (Fig. 61). The constitution is female. Sometimes moderate obesity is observed. During a bimanual study, there is a bilateral significant increase in testicular.
nicknames. They are dense, mobile, painless. In some cases, the uterus is underdeveloped. The menstrual cycle is broken. Hypo-or amenorrhea occurs more often, less often menometrorrhagia. Characterized by anovulatory cycles.

Laboratory data. In the blood, the level of estrogens is often reduced, while testosterone, androsterone, 17-hydroxyprogesterone and LH are elevated. Excretion with urine 17-KS normal or slightly increased. More typically, increased secretion of testosterone and some androgen fractions: andro-stendion and ethiocholanolone. Sometimes the urinary excretion of 17-KS is significantly increased, which is associated with functional hypercorticism. Excretion with urine 17-ACS normal.

Diagnostic tests. For the diagnosis of Stein-Levental syndrome, samples with the introduction of chorionic gonadotropin or progesterone, based on the blockade of LH secretion, are used. With Stein’s syndrome – Leventhal intramuscular
The introduction of human chorionic gonadotropin (choriogonin) to 1500 IU daily for 4-5 days leads to an increase in urine 17-C, which indicates their ovarian origin
denie After administration of progesterone, urinary excretion of 17-COP of ovarian genesis is reduced. In the case of initially elevated urinary 17-CS, a cortisone (dexamethasone) test is performed. The urinary excretion of 17-COP of ovarian origin after the administration of cortisone (dexamethasone) decreases slightly.

local_offerevent_note December 1, 2018

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