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Male congenital testicular dysplasia syndrome, is a kind of sex chromosome number distortion syndrome, common karyotype of 47, XXY. Which patients than normal-weight men an X chromosome. About every 1,000 in men with uncommon one. In infertile men for l / 10. Main features: suffering from congenital high-testosterone nine dysplasia syndrome boy at birth, developing normally. Often at puberty or adult rear symptoms. Taller, refers to the distance between two hands from (the middle finger) > height, slender, skin delicate, limbs hypodermic and adipose plump, voice tapering, frequently have no laryngeal tuberculosis, lack or sparse beard, DaoSanJiao type distribution in pubic hair, 25% of patients appear feminine breast, the penis small or normal, testicular small and hard, sperm fewer or lack, low sexual desire. In developing give androgen therapy, can promote sex organ and secondary sex characteristics growth. Patients can be married, most infertility. Testicular are male sex organ, its main function is to produce sperm and egg and sperm combined to form a new individual, thus ensuring the continuation of the human race. Additional, still secrete hormone androgen, can stimulate sperm happen and male organ and the development of FuXingZheng attached. Therefore, whether the innate developmental disorders or nurture caused by many factors of the testis lesions, all can cause infertility. Congenital testicular dysplasia syndrome in the incidence of a new born baby for 1/700 ~ 1/800, in men ShengShiXian dysplasia and barren patients as high as 30%. Most patients karyotype of 47, XXY, 80%, and several other types. Because of chromosomes at meiosis of separation, accounting for approximately caused chimeras 1/3, karyotype 46, 47, XY/XXY or 45, X / 47 XXY, patients may, on one side testicular developing normally. Other XXXY etc, XXYY polyploid relatively rare. Gram levy its individual representation for men, and in childhood and young period signs is not apparent, and to youth development period gradually appeared breast enlargement, beards and pubic hair and YeMao exiguous, shoulder is narrow, the buttock width and so on women posture. A minority of patients with female temperament, external genital organs still have male characteristic, but testicular small and soft, sexual function lowly, semen no sperm. Testicular biopsy shows interstitial hyperplasia, born fine tubular glass sample change, sperm happen to a complete stop or severe decrease. About 1 in 4 patients experienced developmental obstacles, and the more XX chromosome developmental obstacles the more obvious. According to statistics, this kind of patient breast cancer higher chance to pituitary gonadotropin, particularly shortness of follicle-stimulating hormone FSH, in plasma and urine concentration were higher, and plasma testosterone concentrations are below normal to human chorionic gonadotropin and testosterone response was very poor. Treatment to supplement the male hormone deficiencies give priority to, can be generally every 3 ~ 4 weeks for males of 200mg, injection has helped, but cannot affect women type breast. In order to appearance or psychological factors, can sometimes be breast molding resection. 2011-03-15
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