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The main reason for adrenogenital syndrome is considered to be a congenital deficiency of this element as C21-hydroxylase - an enzyme involved in the synthesis of androgens produced by the adrenal cortex. This enzyme is produced in sufficient quantities under the influence of the gene, which is located on the short arm of autosomes - pairs of chromosome 6 . As a rule, the inheritance of the disease characterized by an autosomal recessive character. If there is only one body of pathologically altered gene may not develop the disease, and only when abnormal genes are in different pairs of chromosomes, can develop congenital adrenal hyperplasia.
Symptoms adrenogenital syndrome
In congenital form of adrenogenital syndrome even in utero during the formation of hormonal adrenal androgens produced an excessive amount. Excess androgens results in a sexual differentiation Relating to female fetuses. By 9-11 weeks of fetal development already has a structure and sexual organs, characteristic of the female body, although the external genitalia at this stage is only beginning to emerge. This female phenotype is formed from the initial type.
In the female fetus and external genitalia affects excessive production Testosterone In the end there is a significant increase in genital tubercle, which then takes the form of penisoobraznogo clitoris labiosakralnye folds merge and resemble the scrotum. Urogenital sinus opens deformed under the clitoris, are not distributed initially to the vagina and urethra. Therefore, when a child is born is often wrongly determine its gender. Since the gonads become ovaries of the female form, congenital adrenogenital syndrome often referred to false female hermaphroditism In utero leads to overproduction of androgens adrenal hyperplasia.
Children suffering from this disease need constant monitoring by pediatric endocrinologists. Modern medical techniques provide timely surgical treatment of adrenogenital syndrome to make a correction by operating the floor and later the child developed by the female type. Now to the gynecologist-endocrinologist frequently treated patients who have congenital adrenal hyperplasia is characterized by a form of late.
When puberty congenital form C21-hydroxylase deficiency manifests in utero even during puberty, when only manifested hormonal function of the adrenal cortex. However, the violations are particularly noticeable just before the girl's first menstruation. And if the population usually occurs in the first period of 12-13 years, girls with congenital adrenal syndrome characterized by a form of puberty, first menstruation occurs much later, only in 15-16 years.
The menstrual cycle in this disease quite unstable, irregular menstrual flow, there is a tendency for girls to oligomenoree . The interval between periods is significant, as a rule, it is 30-45 days.
Hirsutism quite pronounced, quite often it manifests itself in the growth of hair rod, located on the white line of the abdomen, in the growth of hair on the upper lip of the male principle, on the thighs and around the nipples. On the body there is a large number of sebaceous glands is often observed festering hair follicles, the skin on your face becomes oily, the pores grow and expand. Girls suffering from a form of pubertal adrenogenital syndrome, characterized by relatively high growth and masculine physique, broad shoulders and narrow hips, observed hypoplastic breast. As a rule, the main complaints of patients when referring to getting medical occurrence of acne or complaints of irregular menstrual cycle.
With this form of the disease symptoms of post-pubertal adrenogenital syndrome in girls visible only after puberty. Quite often, the symptoms become pronounced after a medical abortion, while developing pregnancy or after a miscarriage. Expressed as a violation of the menstrual cycle, the intervals between periods is significantly increased, menses become scarce, often there are delays.
In this case, hyperandrogenism is characterized by relatively mild symptoms manifestations of hirsutism hardly expressed and manifested only in small pilosis on the white line on the abdomen, may be a little hair on the legs, around the nipple, or above the upper lip. Mammary glands develop at the girl in the same manner as that of her peers, build forms of female type, metabolic disorders not manifested.
Diagnosis adrenogenital syndrome
Congenital adrenal hyperplasia can be detected using modern hormonal studies And by visual inspection. This takes into account the phenotypic and medical history, such as body hair for women in atypical locations, Athletic male principle, the development of the mammary glands, and the general condition of the skin, enlarged pores and the presence of acne. Congenital adrenal hyperplasia is characterized by a substantial violation of the synthesis of steroids at the 17 SNPs, so the presence of the disease shows an increase in hormone levels in the blood and the identification of two hormones - DHEA and DHEA-S, are considered to be precursors of testosterone.
It is necessary to identify and measure 17 CS at the time of diagnosis, was detected by a urine test for the presence of metabolites of androgens. During a blood test for the diagnosis of adrenogenital syndrome is determined by the level of hormones DHEA-S and 17 SNPs. When the complex examination for a full diagnosis should be considered symptoms of hyperandrogenism and other violations in the endocrine system. The indicator of the COP-17 in the urine and hormone levels of DHEA-S, T, 17 SNPs, and DHEA should be identified twice - first to test with dexamethasone and other glucocorticoids, and then, after its implementation. If the level of hormones in the analysis is reduced to 70-75%, this shows only the development of androgens in the adrenal cortex.
Accurate diagnosis involves adrenogenital syndrome Ultrasound of the ovaries During which revealed anovulation , It can be determined if there are follicles different maturity level that does not exceed preovulatory size. Typically, in such cases the ovaries will increase, but, in contrast to polycystic ovary syndrome, adrenogenital syndrome when they have not observed any increase in the stroma, nor the presence of small follicles directly beneath the capsule of the ovary. The diagnosis is often used measurement of basal body temperature, with a duration of disease indicates the characteristic phases - a long first phase of the menstrual cycle and for a brief time for the second phase.
Treatment adrenogenital syndrome
Even during pregnancy should continue the therapeutic treatment glucocorticoids up to the 13th week to avoid miscarriage. By that time already well formed placenta, which will ensure that a sufficient amount of hormones necessary for the proper formation of the fetus. Patients suffering adrenogenital syndrome Must be closely monitored by doctors at all stages of pregnancy, particularly important to proper treatment in the early stages of the fetus. Basal temperature should be measured daily until the 9th week of pregnancy, every two weeks, ultrasound diagnosis is needed to identify the the tone of the myometrium and explore the state of detachments of the ovum.
If the patient has previously occurred miscarriage estrogensoderzhaschie need to take drugs to significantly improve blood flow to the embryo in utero. As preparation for pregnant women prescribers mikrofollin A daily dose of which is 0, 25-0, 5 mg, or proginova in an amount of 1-2 mg. Status of women should be carefully monitored, especially should pay attention to complaints of pain in the abdomen, as well as the presence of bleeding from the urinary tract.
Now with the adrenogenital syndrome in the treatment of not carrying a pregnancy even during I-II trimester drug use djufaston Which is an analogue of natural progesterone . This drug is not characterized by androgenic effect, which distinguishes it from the funds norsteroidnogo series, the use of which can lead to masculinization of the fetus, especially females. This drug is used to treat cervical incompetence Which is often concomitant with the disease adrenogenital syndrome.
If pregnancy despite the therapy, does not occur, ovulation does not occur, and the duration of the phases of the menstrual cycle remains the same, in addition to therapy with glucocorticoids, it is necessary to stimulate ovulation. For this purpose clomiphene Assigned 50-100 mg at certain stages of the menstrual cycle. When a woman goes to a doctor complaining of a hirsutism male principle, irregular menses or on empyesis on the face and body, but is not interested in pregnancy, therapy has been held for other drugs.
Typically, such means comprise antiandrogens and Estrogens The most commonly used among them Diane-35 . In the presence of hirsutism in a single complex with it appointed cyproterone acetate In a dose of 25-50 mg per day. The course of treatment with this drug is designed for 12-14 days. The full medical treatment takes three to six months, only if the treatment becomes effective. But the cause of disease is not eliminated, so after treatment discontinuation symptoms adrenogenital syndrome start to show again.
The use of glucocorticoids, allowing to normalize the ovarian function, not lead to a significant reduction of hirsutism. To get rid of this problem, you should take oral contraceptives with progestins , such as gestodene . desogestrel . norgestimate . Among the non-hormonal drugs can be identified veroshpiron Which must be taken within six months at 100 mg daily, in this case the majority of patients there is a significant decrease in hirsutism .
In postpubertatnom form this disease patients who do not wish to become pregnant, hormonal treatments usually are not assigned, especially if delays are not long-term nature, acne and skin rashes are insignificant. If a woman must assign hormonal contraceptives, should give preference to drugs such as Mersilon . gestodene . desogestrel . norgestimate But taking such medications more consecutive years are not recommended.
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