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XY女性是指具有女性或近似女性的表型及外生殖器而性染色体为XY的个体,自幼多作为女性抚养,多因闭经或阴蒂肥大而就诊。这类患者的性腺恶性肿瘤发生率较高,有的因性别不明或在青春期后出现闭经、男性化以及不育等,给患者本人及家庭造成负担。自1981年2月至1983年5月,我们通过外周血淋巴细胞培养及G带染色体分析,共发现此类患者13例,归纳为以下五种类型:①XY性腺发育不全3例,②混合性性腺发育不全2例,③不全雄激素不敏感6例,④真两性畸型1例,⑤性腺母细胞瘤1例。现报道如下。一、体征本组13例中,患者身材1.50米以上者10例,其中4例1.70米以上,身材矮小者3例。乳房未发育或稍发育者12例,只1例真两性畴型者乳房发育好。本组5例性腺发育不全者外阴发育差,有阴道及幼稚子宫,无阴毛,阴蒂不大,手足长大,似无睾
XY female refers to the female or similar female phenotype and genitalia and sex chromosome XY individuals, since childhood more as a female support, mostly due to amenorrhea or clitoral hypertrophy and treatment. Such patients have a high incidence of gonadal malignancies, some because of unknown gender or after amenorrhea, masculine and infertility, etc., to patients and their families burden. From February 1981 to May 1983, we found 13 cases of these patients by peripheral blood lymphocyte culture and G-band chromosome analysis, which were summarized as the following five types: ①XY gonadal dysplasia in 3 cases, ② mixed gonadal 2 cases of hypoplasia, ③ incomplete androgen insensitivity in 6 cases, ④ true genital abnormalities in 1 case, ⑤ gonadoblastoma in 1 case. Report as follows now. First, the signs of this group of 13 cases, 1.50 meters in patients with more than 10 cases, of which 4 cases 1.70 meters above, short stature in 3 cases. Twelve cases of breast undeveloped or slightly developed, only one case of true gender domains breast development good. This group of 5 cases of gonadal dysplasia, poor development of the vulva, vaginal and immature uterus, no pubic hair, clitoris is not large, hand-foot grows like no testis