Polycystic ovarian degeneration ( syndrome Stein - Leventhal )
Endocrinology / / August 12, 2017
name of the disease comes from the work of Stein and Leventhal.The authors describe seven patients with secondary amenorrhea, infertility and hypertrichosis, who with wedge resection of the ovaries was detected Sclerotiniose cystic them.Test excision ovarian tissue produced for diagnostic purposes, led to the restoration of the menstrual cycle and of fertility.
However, even before the work of Stein and Leventhal in Russian literature has been described as a pathological condition of the ovaries during the menstrual cycle, such as amenorrhea and oligomenorrhea.
Etiology and pathogenesis
pathogenesis of the disease is still not clear.Many researchers, observing seal capsules with polycystic ovarian degeneration, believed that it could be a mechanical barrier that prevents ovulation.There is also speculation that the ovarian capsule fibrosis is a congenital disease and is only beginning to emerge to the time of puberty.Wedge excision allows follicles located in the deep layers, mature and ovulate.Th
Recent studies have shown that the syndrome of Stein - Leventhal has quantitative and qualitative abnormalities of steroidogenesis in the ovaries.With this agreement and data Dokumov Dashov, who observed the greatest histochemical changes in the syndrome of Stein - Leventhal in cells producing steroids.
estrogen excretion in the urine of most patients is within the normal range, but without the characteristic peaks for ovulation, and without a significant decline before menstruation.However, in the study of follicular fluid contents therein when estrogen syndrome Stein - Leventhal often lower than in normal ovarian follicular fluid.But because of a multitude of cysts total production of estrogen by the ovaries is quite high, and is associated with what a normal level of estrogen in the urine.
Despite the frequent presence of hypertrichosis, urinary 17-keto steroids in the urine of most patients is normal or slightly above it.When fractional study 17-keto steroids appeared elevated androgen fraction.However, the 17-keto steroids urine metabolites are not only the most active androgen - testosterone and other steroids adrenal cortex.In addition, testosterone can not only be converted into 17-keto steroids (etioholanon and androsterone), but in other compounds.Production of testosterone in the body can be increased, and its virilizing effects will be noticeable to appear, and on the excretion of 17-ketosteroidsv it will affect slightly.It is a position often seen in the syndrome of Stein - Leventhal.Increased production of androgen in the disease proved in different ways.When testosterone determining its amount in urine with the syndrome Stein - Leventhal is several times higher than normal.In the study of follicular fluid in it found a significantly greater amount of androstenedione than normal.Although not androstenedione itself has a strong androgenic action, it is a direct precursor of testosterone and it can be converted in the ovaries and in other tissues.In addition, women with ovarian syndrome Stein - Leventhal detected and testosterone itself, whereas testosterone is not isolated from the ovaries of healthy women.Upon incubation with slices of ovarian estrogen and androgen precursors - progesterone and 17-hydroxyprogesterone, and - the ovaries of women with the syndrome Stein - Leventhal synthesize significantly higher amounts of androstenedione and testosterone than normal ovaries.When ovarian stimulation follicle stimulating hormone androstenedione content in the vein of the ovaries increased significantly more in the syndrome of Stein - Leventhal than normal.
Violation of steroidogenesis in the ovaries of the syndrome of Stein - Leventhal can be 2 types:
1) violation of the W-B-olsteroiddegidrogenazy - the enzyme that causes the conversion of pregnenolone to progesterone.Thus pregnenolone is converted predominantly dehydroepiandrosterone, which accumulates in significant quantities in polycystic ovary;
2) lack of an enzyme that converts androgens to estrogens.In the ovaries accumulate large amounts of androstenedione and its predecessor - the 17-a-hydroxyprogesterone.In both cases, violations of estrogen synthesis is reduced.However, according to most authors, the violation of steroidogenesis in the ovaries is not primary, but is a consequence of a breach of regulatory influences on the part of the hypothalamic-pituitary system.
total number of gonadotropins in the urine in the syndrome of Stein - Leventhal is no different from the norm.But in the study of luteinizing hormone, many authors found an increase in its allocation of more than half of patients with this disease.However, despite the relative increase in LH, it is not proper excretion cycling, no peaks of its isolation characteristic of the normal cycle and leading to ovulation.Although elevated levels of LH in the syndrome of Stein - Leventhal, ovulation does not occur, and are characteristic of the syndrome anovulatory cycles.Most researchers found low urine excretion pregnandiol, lack secretory transformation of the endometrium and corpora lutea in the ovaries.Any anovulatory cycles may eventually lead to polycystic ovarian degeneration.Increased LH is secondary - as a consequence of the lack of secretion of progesterone during anovulatory cycles.Change product LH attach paramount importance.The total increased, but the level of monotonous isolation of LH, similar to that observed in men, is not a consequence and cause of anovulatory cycles, as well as hyperplasia of theca cells and increased production of androgens by them.In turn, the androgens inhibit the cyclic release of LH, follicle development and ovulation.Thus, a vicious circle is created.Leventhal and Scommegna believe that the primary defect is the result of the production of LH violation hypothalamic regulation, as it were, "masculinization" of the hypothalamus.On the importance of violations of the hypothalamic regulation of the production and release of LH in the syndrome of Stein - Leventhal indicate data Greenblatt and others who observed the appearance of ovulatory cycles and pregnancy in patients with the syndrome Stein -. Leventhal clomiphene (according to many authors, the primary clomiphene site of action are hypothalamic centersgoverning the allocation of LH from the pituitary gland)..
In the Soffer and others have shown that the syndrome of Stein - Leventhal urinary no antigonadotropnym factors, which normally are one of the means of regulation of the action of gonadotropins to the ovaries.The syndrome Stein - Leventhal this regulation is violated, and the ovaries are exposed to unregulated stimulation of gonadotropins, which, in their opinion, and can cause polycystic their degeneration.
However, none of these theories reveals the mechanism before the end of ovarian cystic degeneration.It is difficult to explain as good therapeutic effect wedge resection in this disease.Most authors believe that the removal of most of the most altered of the ovaries eliminates the effect of increased androgen levels, inhibits the cyclical release of gonadotropins;thus a vicious circle is interrupted and restored normal cycle.
Typically, cortisone treatment in the syndrome of Stein - Leventhal is ineffective.This argues against a primary dysfunction of the adrenal cortex in this disease.But it is impossible to completely eliminate some involvement in this syndrome, dysfunction of the adrenal cortex, as in some cases the absence of clinical effect of ovarian wedge resection was successfully complemented the treatment with prednisone.The complex pathogenesis of the disease requires a broad examination of the ovaries and the pituitary gland, and adrenal cortex.Any reason that violates pituitary-ovarian relationships, and so leads to a disruption of the biosynthesis of steroids in the ovaries can cause a syndrome Stein - Leventhal as a consequence of the establishment of a vicious circle.These reasons may be dysfunction of the thyroid gland, adrenal glands, emotional factors, genetic factors.
The syndrome Stein - Leventhal is characterized by bilateral ovarian enlargement from small size to a value that exceeds normal size more than 2-4 times.The contours of the ovary are saved, and the characteristic crinkles disappear.Ovarian Consistency thick, gray surface, shiny, relief is smoothed due to seal tunica albuginea by collagen fibers.
on the cut in the cortical layer, seen multiple smooth-walled cyst containing opalescent liquid.Stroma dense.Cyst size can vary from 2-15 mm to 2 cm in diameter.
Microscopic study of ovarian tissue shows multiple cystic changes follicles at different stages of maturity, have undergone atresia.The number of primordial follicles sharply reduced.The walls of the cysts consist of two layers: the inner - from follicular epithelium (granulosa) with signs of degeneration and outdoor - izgiperplazirovannoy-tech fabric.Cells are exposed to the theca luteal tissue processes.Hyperplastic theca folliculi attributed to the secretion of androgens, which are responsible for the clinical picture of the disease.Yellow body resected ovaries are rare.The density of the ovaries due to the fact that the stromal cells often represent the only type of ovarian tissue, which replaces all other structural elements of the body.Uterus slightly less normal value or without any deviation from the norm.The endometrium often able to proliferation without pronounced hyperplasia, rarely - atrophic.Only in cases where the clinical picture of the disease were observed uterine bleeding, the endometrium showed pictures of glandular cystic hyperplasia or polyposis.
- ovarian hormones.Estrogens
- ovarian anatomy
- ovarian hormones.Androgens
- menstrual cycle Hormonal
- menstrual irregularities
- tests of functional diagnostics of ovarian activity
- Violation monthly rate.Amenorrhea
- Violation of the menstrual cycle with monthly rhythm disorder.Opsomenoreya
- Violation of the menstrual cycle with monthly rhythm disorder.Polimenoreya
- principles of hormone therapy menstrual disorders
disease develops in young women aged 20-30 years.One of the first manifestations of a disruption of the menstrual cycle type gipooligomenoree, often turning into persistent amenorrhea.
In more rare cases of menstrual disorders are characterized menometrorrhagias, and bleeding may be repeated for several years.
Another most common symptom is infertility, and sometimes women, previously (before the onset of the disease) had a normal pregnancy and childbirth.
In 50% of patients with the syndrome of Stein - Leventhal observed hypertrichosis, mild expression on his face and limbs, in rare cases, very extensive, but no other signs of virilization.Despite hypertrichosis, patients have a well-defined female phenotype, with well-developed secondary sexual characteristics.The general condition of the patients did not significantly change, performance and tone normal.Several authors have noted the condition of neuroticism, a tendency to depressive mood due to the presence of menstrual disorders and the inability to conceive.
Hormone therapy sex hormones are almost always not successful.The most effective is a surgical treatment that consists in ovarian wedge resection.Removal of a large part of the most altered ovarian tissue, and cut thick capsule leads to the restoration of normal function of the ovaries.
Usually the menstrual cycle is restored within the period of 2 to 6 weeks after surgery.In 60-70% of cases is reduced ability to conceive.Hypertrichosis disappears after the operation, but not progressing.
In some cases, the presence of patients with high content of 17-keto steroids is recommended to combine surgery with prednisone therapy.
Recently, there have been reports of successful treatment of this syndrome by restoring ovulation in giving the human pituitary gonadotropin or clomiphene.