What are the symptoms of hermaphroditism? Who decides the gender of a "yin-yang person"?

Medically, hermaphroditism is called hermaphroditism, which is caused by androgen stimulation during pregnancy. Hermaphroditism can be divided into the following three categories: female pseudohermaphroditism, male pseudohermaphroditism, and true hermaphroditism. What are the symptoms of hermaphroditism? Who decides the gender of a "hermaphrodite"!

(1) Female pseudohermaphroditism

The chromosome karyotype is 46, XX, the gonads are ovaries, the uterus, cervix, and vagina are all present, but the external genitalia are masculine, the clitoris is thick, and the labia are united into a scrotum. The cause of excessive androgen may be congenital adrenal cortical hyperplasia or non-adrenal sources.

Congenital adrenal hyperplasia is an autosomal recessive genetic disease. Due to some enzyme defects in the fetal adrenal glands that synthesize cortisol, cortisol synthesis disorders and increased androgen synthesis are caused. Insufficient cortisol can feedback increase ACTH, causing hyperplasia and hypertrophy of the zona reticularis of the adrenal cortex; excessive androgens cause masculinization of female infants, and as the infant grows up after birth, masculinization characteristics become more obvious. Pregnant women who take androgenic drugs during pregnancy, such as synthetic progesterone, danazol or methyltestosterone, all have different degrees of androgenic effects. Taking them during pregnancy or conceiving during medication can cause masculinization of the external genitalia of female fetuses, but the degree is milder than congenital adrenal hyperplasia.

(ii) Male pseudohermaphroditism

It is the feminization of males, which is actually congenital androgen insensitivity syndrome, also known as testicular feminization syndrome. The patient's chromosome karyotype is 46, XY, the gonads are testes, the external genitalia are female, and there is no uterus. It can be divided into two categories.

1. Complete androgen insensitivity syndrome is also called testicular feminization syndrome. It is the most common male pseudohermaphroditism, which is an X-linked recessive inheritance with a karyotype of 46.XY. The gonads are testes and can secrete testosterone, but due to the disorder of target cell androgen receptor synthesis, the target organ uterus does not respond to androgens, which affects the development of the genital primordia in the male direction and the external genitalia turns to female type. Breast development occurs after puberty, and pubic hair and axillary hair are sparse or absent. There is no uterus and fallopian tube. The vagina is blind, and the gonads are often located in the inguinal groove or abdominal cavity, and sometimes can descend to the labia majora. The testicles are still normal in appearance and are juvenile.

2. Incomplete androgen insensitivity syndrome is less common. The androgen receptors of the cells are partially deficient, resulting in incomplete masculinization, making it difficult to distinguish the sex. Most of the body shape and phenotype are female, with a karyotype of 46, XY. There are testicles, which are often located in the abdominal cavity or inguinal area. The external genitalia often have hermaphroditism, manifested as varying degrees of clitoral enlargement or a short penis, a short and shallow vagina with a blind end, and after the onset of puberty, there may be masculinization such as increased pubic hair, axillary hair, and continued enlargement of the clitoris.

(III) True hermaphroditism

Patients with true hermaphroditism have both male and female gonads in their bodies, but they are underdeveloped, with the majority being oocytes and testes. The external genitalia can be male, female, or mixed, with the male type being more common, accounting for about 2/3. The most common chromosome karyotype is 46:XX, and other karyotypes include 46XX/46XY mosaics, and 46,XY is rare. Those with male external genitalia often have cryptorchidism and hypospadias, while those with female external genitalia often have clitoral hypertrophy. Breasts develop after puberty, and most have an underdeveloped uterus, while a few may have no uterus, and more than half have menstruation or cyclical hematuria.