Adrenogenital syndrome
Contents:
Description:
The adrenogenital syndrome is the hereditary pathology consisting in a hyperplasia of bark of adrenal glands and its dysfunction with an autosomal and recessive mode of inheritance. Distinguish the inborn and acquired forms of an adrenogenital syndrome. Patients with an inborn form are born with a hypertrophied clitoris. The inborn form of a syndrome is called still a pseudo-female hermaphroditism. The acquired adrenogenital syndrome depending on time of emergence is classified on prepubertatny and postpubertal.
Reasons of the Adrenogenital syndrome:
The adrenogenital syndrome is characterized by virilescence of adrenal genesis and develops against the background of deficit of enzymes owing to what synthesis of predecessors of cortisol is broken. The most widespread is hereditary deficit of C21 hydroxylase which turns 17 pregnenoldione in the predecessor of cortisol. Insufficiency of cortisol on a feedback mechanism leads to strengthening of products of AKTG which stimulates not only a puchkovy, but also mesh zone of the bark of adrenal glands making androgens. It also predetermines virilescence. The excess quantity of androgens slows down allocation of gonadotrophins at the expense of what cyclic processes in ovaries with emergence of an amenorrhea are broken.
Pathogeny of an adrenogenital syndrome
Symptoms of the Adrenogenital syndrome:
The clinical picture of an adrenogenital syndrome is caused by a giperandrogeniya which consequence the excessive hirsutism, an oligomenorrhea or an amenorrhea, an atrophy of mammary glands, a uterus hypoplasia, a myopachynsis on men's type, a voice posterization, a clitoris hypertrophy, pigmentation of external genitals is.
Development of an adrenogenital syndrome in prepubertatny leads the period to primary infertility.
In case of a syndrome in the postpubertal period pregnancy is possible, however in connection with insufficiency of function of a yellow body against the background of a giperandrogeniya it, as a rule, comes to the end with a misbirth in early terms. Frequency of not incubation of pregnancy at this pathology reaches 30%.
For diagnosis in addition to clinical signs crucial importance has definition 17-KC (metabolites of androgens) in urine before dexamethasone reception. This test allows to otdifferentsirovat a hyperplasia of bark of adrenal glands from an androgensekretiruyushchy tumor of adrenal glands or yaichnonik. Besides, determine the content of testosterone in blood before test statement. For differential diagnosis with virilescence of ovarian genesis conduct ultrasonography, define the maintenance of gonadotrophins and sexual steroids in blood, carry out a laparoscopy with a biopsy of ovaries.
With the purpose to exclude a tumor of adrenal glands carry out a computer tomography.
Clitoris hypertrophy at the newborn at an adrenogenital syndrome
Treatment of the Adrenogenital syndrome:
At the heart of treatment of an adrenogenital syndrome - purpose of glucocorticoid drugs which suppress excessive products of AKTG. Preferential apply Prednisolonum or dexamethasone.
Treatment of a hirsutism presents certain difficulties and is directed to decrease in synthesis of androgens, increase in products of the protein connecting androgens, decrease in sensitivity of hair follicles to action of androgens. For this purpose appoint tsiproteron-acetate (Diane-35) which slows down education to dihydrotestosterone from testosterone in hair follicles, blocking tsitozolretseptor of the last. Drug is accepted within 4 - 6 months.
The similar mechanism of action and at a veroshpiron (Spironolactonum). Drug is used in a dose 25 mg by 2 times a day within 4 - 6 months.
Besides, synthetic progestins which oestrogenic component promotes increase in blood of steroidosvyazyvayushchy globulin therefore the content of free testosterone decreases due to increase in quantity of its connected forms are shown. The powerful clinical effect in this aspect gives марвелон.
