True hermaphroditism ( true intersexuality )
Endocrinology / / May 14, 2016
Diseases associated with impaired sexual differentiation
People of true hermaphroditism, t. E. Ripening in one organism germ cells of both sexes, does not happen.Under this condition realize a condition in which an organism present in both ovarian and testicular elements.
Most patients found karyotype XX, but described karyotype XY, XX / XY, XO / XY, XX / XXY, and others. It is possible that in many cases there was unrecognized mosaic or a carry of the Y-hromosomy on any other chromosome, which made possible the development of testicular tissue.In the embryonic period an equilibrium of cortical and medullary gonads, which allows to exist and ovarian and testicular tissue.Depending on the activity of testicular tissue develop external and internal sex organs.In general, in the pathogenesis of true intersexuality is very much unclear.In some cases, it is proven double fertilization.
incidence of the disease is unknown.It describes a number of families where several children in one g
Clinic is extremely diverse.Normally, the left ovary or ovotestis there, and on the right - the egg or ovotestis;other options are possible.Egg may be in the scrotum, the inguinal canal or abdomen and in most cases contains hyalinized atrophic seminiferous tubules, but in some cases there were stored tubules with spermatogenesis.The ovaries are often atrophic, with the growth of the stroma, but sometimes they are stored follicles.
From the eggs usually develop epididymis and vas deferens, and on the other hand - the uterus and tube.However, even with one or both sides may be stored as Mullerian and Wolffian derivatives.Extremely diverse views of the external genitalia - is almost normal in size penis with hypospadias, or his sharp underdevelopment.Perhaps half of the scrotum with the testicles.The vagina is opened to the crotch midline scrotal cleaved, either in the urogenital sinus.Accordingly, referring to the external genitalia of the female patients is considered, and some - men.In puberty may occur or the feminization or virilization, or both effects (a significant increase in breast along with pronounced facial hairiness).Given the variability of the clinical picture and a variety of pathogenic mechanisms, laboratory findings are not typical.Isolation of gonadotropins may be normal or increased, depending on the endocrine function of the gonads.The presence or absence of a sexual chromatin depends on the karyotype.
- Congenital disorders of sexual differentiation.Normal sexual differentiation
- male gonads.Embryology, anatomy and physiology
- gonadal dysgenesis (Shereshevskii syndrome - Turner)
- dysgenesis seminiferous tubules (Klinefelter
- syndrome Early puberty
- Tumors Leydigovyh cells hilyusa ovarian
Suspected true intersexualityarises because of nondescript external genitalia. However, the diagnosis can be established only on the basis of histological study of biopsies of material obtained during the operation of each of the gonads.
The choice of tactics of treatment depends on the psychosexual orientation of patients (almost always coincides with their passport floors) and, consequently, on as people what sex they will live. If it is a female, then completely remove the entire testicular tissue (including ovotestis), resecting the clitoris, if necessary dissect urogenital sinus produce epilation.In the men's field, remove all ovarian elements for gynecomastia produce a mastectomy, as well as a series of plastic surgery of the external genital organs for the purpose of straightening the penis and create a penile urethra.If the operation had completely removed the gonads, should be assigned the appropriate hormone treatment - cyclic estrogen for women and testosterone for men continuous reception.Infertility patients, of course, is incurable.