1. Familial abnormal testicular matching is a disease of leydig cells. There are no leydig cells in the testis, which can't synthesize and secrete testosterone, resulting in feminization of reproductive ducts and external genitalia, and the phenotype is female. Testosterone level is very low, which does not increase after HCG administration, but plasma LH level increases obviously, and the patient is infertile due to sexual dysfunction. The chromosome karyotype is 46XY. 2.20,22 carbon chain enzyme deficiency (20,22 22 22Desmolasedeficiency) These two enzymes are necessary in the initial stage of hydrocortisone and testosterone synthesis. The deficiency of these two enzymes will lead to the synthesis disorder of adrenocortical hormone in adrenal gland and testosterone in testis. The patient's phenotype is female, showing male pseudohermaphroditism, including female reproductive tract and female external genitalia. However, the gonad is a male genitalia with the testes located in the pelvic cavity, inguinal canal or labia majora. Glucocorticoid, mineralocorticoid and sex hormone are all low, while FSH, LH and ACTH are high. The chromosome karyotype is 46XY. 3.3 1- p-hydroxysteroid dehydrogenase deficiency (3- p-hydroxysteroid dehydrogenase deficiency), the synthesis of aldosterone, hydrocortisone and androsterone is blocked, which shows adrenal cortex dysfunction; In the testis, sexual function is low due to disorder of testosterone secretion; Male pseudohermaphroditism, almost all patients have cryptorchidism, and the chromosome karyotype is 46XY. Adrenal cortical hormone and aldosterone can be used as alternative therapy, and abnormal external genitalia needs plastic surgery. 4.17-a-alphabet. Hydroxylamine deficiency (17-alphabet. The deficiency of hydroxylase can hinder the simultaneous synthesis of hydrocortisone, testosterone, estradiol and testosterone in adrenal cortex, so the clinical manifestations of patients are that the testis does not descend to the scrotum, the penis is smaller, hypospadias appears, the external genitalia becomes feminine, and the male secondary sexual characteristics do not appear in adolescence. Patients generally have no residual accessory mesonephric duct, which indicates that it is normal for testis to synthesize Mullerian duct inhibitory factor (MIF) in embryonic stage. Due to the lack of this enzyme, the synthesis of adrenocortical hormones such as dehydrocorticosterone, corticosterone and aldosterone is too high, and patients may have symptoms such as hypertension, hypernatremia, hypokalemia and alkalosis. The chromosome karyotype is 46XY, which is autosomal recessive inheritance. Adrenal cortical hormone replacement therapy can restore the symptoms of hypotension and hypertension to normal. When the sex is female, the gonads should be removed and female hormone replacement therapy should be adopted. 5. 17 ketosteroid reductase deficiency (17. Ketosteroid reductase deficiency) 17 ketosteroid reductase deficiency affects the synthesis of testosterone, which can make the external genitalia completely feminine, with shallow vagina but no uterus. The level of testosterone in plasma is low and the level of estrogen is high. Female breasts often develop in adolescence, depending on the level of estrogen. The chromosome karyotype is 46XY. Diagnosis can be made according to the male genotype with abnormal external reproductive organs, and the determination of sex hormones is of diagnostic and differential significance. Testicular excision and estrogen replacement therapy are generally performed.