46,XX男性综合征病例,20多年来文献约有100多例报道,而46,XX/45,X的嵌合型男性极为罕见,本文报道1例如下。患者男,36岁,已婚,身高156cm,皮肤细腻,无胡须、腋毛及喉结,乳房不发育,外阴阴毛呈女性分布,无阴道及尿道下裂。阴茎短小,长4.5cm,双侧睾丸都小于正常人。26岁左右时曾有精液但无精子,直肠肛检前列腺较小,无子宫,B超探查无卵巢、子宫和输 46, XX male syndrome cases, more than 20 years of literature about 100 cases reported, and 46, XX / 45, X chimeric male is extremely rare, 1 reported in this article below. Male, 36 years old, married, height 156cm, delicate skin, no beard, armpit hair and Adam’s apple, undeveloped breasts, female genital pubic hair distribution, no vaginal and hypospadias. Penis short, long 4.5cm, bilateral testis are smaller than normal. 26-year-old had had sperm but no sperm, rectal anal prostate smaller, no uterus, B ultrasound probe ovary, uterus and lose