PSEUDOHERMAPHRODITISM

From Big Medical Encyclopedia

PSEUDOHERMAPHRODITISM (Greek pseudës false + hermaphroditism; synonym false hermaphroditism) — the term, the Crimea is designated by clinical forms of the inborn diseases which are characterized by maldevelopment of external genitals therefore genitalias have an intersexual structure or remind external genitals of an opposite sex.

The term «pseudohermaphroditism» cannot be considered successful since its wedge, contents vaguely; in practice he more and more gives way to names specific nozol. forms: a dysgenesis of testicles (see. Dysgenesis of gonads ), incomplete masculinization, testicular feminization (see), an inborn adrenogenital syndrome (see) and inborn virilescence of outside genitalias (see. Virilescence ). Unlike true hermaphroditism (see) at the diseases designated by the term «pseudohermaphroditism», gonads contain morfol, the structures characteristic only for same-gender. Thus, in the presence of testicles it is possible to speak about a men's pseudohermaphroditism, in the presence of ovaries — about women's.

At early stages of an embryogenesis internal and external genitals of an embryo are constructed on female type, i.e. the main (neutral) type of a structure of outside genitalias at the person is the female type. At 10 — a 12 weeks embryo of the person embryonal testicles synthesize so-called antimyuller a factor, under influence to-rogo there is an involution of rudiments of female internal generative organs. At a lack of an antimyullerov of a factor these rudiments remain, and at the subject with a genetic and gonadal male rudimentary vagina, a uterus, uterine tubes remain. From the 12th to the 20th week of an embryogenesis outside genitalias of an embryo with gonadal and genetic men's floor (see) under action androgens (see) undergo fiziol, a masculinization: the vulval shoot of an urogenital sine (an urinogenital bosom) atrophies, the scrotal seam is closed, the penialny urethra (a spongy part of an urethra) is formed, cavernous bodies increase, the sexual hillock turns into a penis. At a lack of androgens during this period or at disturbance of sensitivity of tissues of target organs to androgens there can be an incomplete masculinization of outside genitalias that is expressed hypospadias (see), underdevelopment of cavernous bodies and balanus, preservation of a vulval shoot of an urogenital sine (picture of a men's pseudohermaphroditism). Thus, as the basic etiol, as a factor of an incomplete masculinization serves the lack of androgens at genetically male embryo. Etiol, a factor of virilescence of external genitals of an embryo with a genetic female is surplus of androgens in the embryonal period which can be caused by inborn dysfunction of bark adrenal glands (see), existence at mother of a virilizuyushchy tumor, etc.

The dysgenesis of gonads is connected with disturbance of development of gonads in an embryogenesis. Clinically the dysgenesis of testicles is characterized by an incomplete masculinization outside and incomplete involution of female internal generative organs, and also insufficient expressiveness of secondary sexual characteristics. The dysgenesis of ovaries is clinically expressed hypogonadism (see) without manifestations of a hermaphroditism. At testicular feminization subjects to a genetic male have an incomplete masculinization of outside genitalias, on this background influence of estrogen is shown (see) on formation in the pubertal period of secondary sexual characteristics on female type.

At a virilny form of an adrenogenital syndrome under the influence of surplus of the androgens produced by adrenal glands embryos to a genetic female have a masculinization of external genitals, sometimes to such an extent that at the birth it is difficult to define a sex of the child. In such cases the scrotal seam is closed completely, the penialny urethra and a penis are formed. Internal female generative organs (a vagina, a uterus, uterine tubes) and gonads (ovaries) always correspond to female Iolu since their formation does not depend on influence of androgens. Therefore at the birth of the child with a full or incomplete masculinization of outside genitalias and lack of testicles ekstraabdominalno for sexing of the newborn genetic inspection is obligatory: definition karyotype (see) and sexual chromatin (see). Correction of a floor shall be carried out with the obligatory accounting of psychosexual orientation and anatomo-fiziol. features of the patient. One' from important criteria of the choice of a floor at a men's pseudohermaphroditism are the sizes of a penis.

P.'s treatment first of all operational. The replaceable androgenoterapiya at some forms P. is ineffective because of low sensitivity of tissues of target organs to action of androgens. A necessary component of hormonal therapy of a female pseudohermaphroditism at an adronogenitalny syndrome is therapy glucocorticoid hormones (see) for the purpose of reduction of products of androgens adrenal glands.

See also Sexual states disputable .


Bibliography: Golubeva I. V. Hermaphroditism, M., 1980, bibliogr.; Liberman L. L. Inborn disturbances of sexual development, L, L966; Savitsky G. A. Surgical treatment of congenital anomalies of sexual development in gynecologic practice, M., 1975, bibliogr.; Wilkins L. Diagnosis and treatment of endocrine disturbances at children's and youthful age, the lane with English, M., 1963; Die Intersexualität, hrsg. v. C. Overzier, Stuttgart, 1961.

I. V. Golubeva.

Яндекс.Метрика