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患男,24岁,因婚后3年不孕双方来院就诊。患者婚后性欲低下、阴茎不能勃起,“精液”量少、淡薄。查体:阳性可见,男子第二性征发育不良,阴毛稀少、阴茎短小,睾丸中等硬度,长径117cm,横径0.8cm。精液中量少,内无精子。女方在妇产科检查正常。此综合征是先天性睾丸生精上皮发育不全或不发育疾病。发病率约占男性0.1～0.2％,国内报道认为其性染色体畸变的发生机率与母体怀孕的年龄和胎次有关,性染色体畸形以47xxy多见,46XX、46xy del ey等少见。本征应与47xyyTurner氏综合征和继发性睾丸萎缩相鉴别。治疗可采用丙酸睾丸酮150mg,肌注,隔月一次;维生素E50mg,1日3次;经治疗3～5月后可使性欲增强,睾
Male, 24 years old, because of infertility 3 years after marriage to hospital. Patients with low sexual desire after marriage, the penis can not be erect, “semen” less, light. Physical examination: positive, male secondary sexual dysplasia, sparse pubic hair, short penis, testicular medium hardness, long diameter 117cm, diameter 0.8cm. Sperm in the amount of less, no sperm. Woman check in obstetrics and gynecology normal. This syndrome is congenital testicular germ cell hypoplasia or underdevelopment disease. The incidence rate of about 0.1 ~ 0.2% of men, domestic reports that the incidence of sex chromosome aberration and maternal pregnancy age and parity related to sex chromosome abnormalities to 47xxy more common, 46XX, 46xy del ey rare. Indications should be differentiated from 47xyy Turner’s syndrome and secondary testicular atrophy. Treatment can be used testosterone propionate 150mg, intramuscular injection, once every other month; vitamin E50mg, 3 times a day; after 3 to 5 months of treatment can make libido, testis