KIARI-FROMMELYA SYNDROME

From Big Medical Encyclopedia

KIARI-FROMMELYA SYNDROME (J. Century of Chiari, Austrian gynecologist, 1817 — 1854; R. J. Frommel, is mute. the gynecologist, 1854 — 1912) — the symptom complex which is characterized by a pathological long lactation in combination with an amenorrhea and an atrophy of generative organs. Frommelem is described for the first time in 1882. Kiari with soavt, described a typical picture K. — T. page in two observations. More detailed description of a syndrome taking into account data of the world literature is given to Mavromati (L. Mavromati, 1950).

Etiopathogenesis To. — T. by page it is studied not up to the end. Most of researchers considers the prime cause its long secretion of prolactin a front share of a hypophysis after the delivery owing to defeat of kernels of a hypothalamus (tumoral, infectious and toxic, rheumatic, etc.), is normal responsible for braking of products prolactin (see). Excess products of this hormone lead to a hypertrophy of ferruterous tissue of mammary glands, slow down secretion of follicle-stimulating hormone. Thereof follicles in ovaries do not ripen, the lack of estrogen brings to to an amenorrhea (see), to atrophic processes in the sexual device. Atrophic processes especially are expressed, than the number of the previous childbirth are more and the lactic period is longer. Manifestations To. — T. pages are quite typical. Patients complain of headaches, an indisposition, deterioration in sight, the plentiful expiration of milk out of communication with feeding, lack of periods for a long time. Obesity or weight loss, patol, pilosis is quite often observed. Mammary glands are considerably increased, with sharply expanded network of hypodermic vessels and lack of characteristic pigmentation of peripapillary circles. At a palpation sharply hypertrophied segments of glands are defined, during the pressing — from a nipple a plentiful discharge. Ginekol, a research finds atrophic changes of a vulva, vagina, neck and body of the womb. At tsitol, a research of vulval smears the characteristic picture of sharp deficit of estrogen is observed. Gistol, a research of scraping of an endometria finds atrophic changes it. Allocation with urine of estrogen, luteinizing and follicle-stimulating hormones is sharply reduced, and sometimes completely is absent.

In the presence of long (2 — 3 years and more) the persistent lactation expressed to a hypertrophy of mammary glands and an atrophy of generative organs the diagnosis is very probable. It is necessary to exclude, however, existence of fibrous and cystous mastopathies (see). In cases of especially long persistent lactation, emergence of decrease in sight and a bitemporal hemianopsia the lactation can be one of precursory symptoms of a tumor of a brain. At diagnosis To. — T. villages carry out careful rentgenol., Ophthalmolum., nevrol, and endokrinol, inspection of patients.

Treatment hormonal. Intramuscular injections of a pituitary gonadotrophin (follicle-stimulating hormone) are appointed. The good effect gives use of oestrogenic hormones — hexestrol, Microfollinum, etc.; treatment, as a rule, long, is carried out by courses. In the absence of effect of use of the specified hormonal drugs the roentgenotherapy is appointed; radiation of a hypophysis X-ray is possible.

Forecast favorable.


Bibliography: The multivolume guide to obstetrics and gynecology, under the editorship of L. S. Persianinov, t. 3, book 2, page 587, M., 1964; The Guide to endocrinology, under the editorship of B. V. Alyoshin, etc., page 58, M., 1973; T of e-t e p E. Hormonal disturbances at men and women, the lane with polsk., page 440, Warsaw, 1968; G h i a r i J., In r an u n C. u. Spaeth J. Klinik der Geburtshilfe und Gynakologie, Erlangen, 1852 — 1855; Frommel R. t)ber puerperale Atrophie des Uterus, Z. Geburtsh. Gynak., Bd 7, S. 305, 1882.

C.H. Kopshev.

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