True hermaphroditism is a disease in which
the patient has both an ovary and a testicle. The disease meets
Etiology. The causes of the disease are unknown.
Pathogenesis. The pathogenesis of the disease is not fully disclosed. One thing
temporary development of male and female genitals
waving with mosaicism 46XX / 46XY. With the development of the disease
in patients with a 46XX karyotype, it is suggested that
unrecognized mosaicism in various tissues of organisms
Pathological anatomy. Histologically with egg biopsy
it is possible to detect mature follicles, and in the ovaries – the beginning
stage of spermatogenesis. Tests location combinations
Ocular and ovarian tissue can be very diverse:
bilateral ovotestis (combination on both sides of the testicular
and ovarian tissue), an egg on one side and an ovary on the other.
Clinic. In the period of sexual development are often detected pre
having the features of feminization, menstruation appears. External
The appearance of the external genitalia can be very different.
figurative: underdeveloped penis and vagina, open
separately on the perineum; penis with hypospadias and
the scrotum with the testicle descended into it, etc. Psychic and sexy
Direction depends more often on the gender of upbringing.
Laboratory data. Hormonal studies do not give
D ia and gnostas and with to and e pp ob. In the study of lame
46XX mosaicism is most often found in the som complex
46 / XY, but there may be a karyotype 46XX, less often 46XY.
Diagnosis and differential diagnosis. Crucial
when making a diagnosis has a gonadal biopsy.
True hermaphroditism is differentiated from false wives.
hermaphroditism and false male hermaphroditism.
False female hermaphroditism is most often caused by vroz
dysfunction of the adrenal cortex. Less often, it occurs when
virilizing ovarian tumors or in a growing fetus after
intake of androgenic drugs by pregnant women. In contrast to true hermaphroditism, false female hermaphro
Pediatric adrenal genesis is characterized by the presence of only
ovaries combined with signs of virilization of external geniuses
waistline and phenotype and a significant increase in urinary excretion
False male hermaphroditism is characterized by Nali
which only the testicles in combination with the formation of the genitals
by intersex type, 46XY karyotype, absence by
lovy chromatin, etc.
Forecast. The prognosis for life is favorable. Passport floor
patients set depending on the anatomical and functional
national characteristics of the reproductive system and psychosexual
Treatment. The choice of gender depends on the functional predominance.
the female or male part of the gonad. According to this
surgical correction of the external genitalia is performed.
Testicular feminization syndrome
(false male hermaphroditism
in patients with women
Testicular feminization syndrome – a disease caused by
peripheral insensitivity of peripheral tissue
her to the action of androgens in a fetus with a genetic male
scrap, resulting in a female phenotype.
Historical data. The disease was first described by Goldberg and
Maxwell in 1948, and in 1953 – Morris.
Etiology. The cause of the disease is a gene mutation. Ill
the inheritance is transmitted (by recessive autosomal type)
Pathogenesis. The basis of the pathogenesis of testicular syndrome
feminization lies the genetic insensitivity of the periphery
tissues to the action of androgens with preserved sensation
to estrogen. As a result, already in the period of emb.
developmental activity does not show masculinizing action.
VIE testicular androgens, and external genitalia preserve
neutral (female) structure. The phenomena of feminization clearly you
are in puberty, due to several
increased secretion of estrogen by the testes due to enhanced
secretion of gonadotropic hormones. Insensitivity to andro
genes in puberty is manifested by the absence of secondary
Pathological anatomy. Histologically with testicular biopsy
usually marked atrophy and hyalinization of the seminiferous tubules.
Spermatogenesis is absent in them. Often found significant
409 proliferation of testicular glandulocytes. In the bustling cells often mark
There are numerous adenomas.
Clinic. The main complaints are the absence of menstruation and polo.
hair growth. The constitution is female. Mammary glands develop
you well. Pubic hair and under
True hermaphroditism is a disease in which