两性畸形

Methods Sixteen cases of gonadal dysgenesis were included in this study: 5 with androgen insensitivity syndrome, 1 with 17-α-hydroxylase deficiency, 4 with true hermaphrodite, 2 with 45,X/46,XY gonadal dysgenesis, 1 with 45,X gonadal dysgenesis, 1 with XY pure gonadal dysgenesis, 1 with testicular regression, and 1 XY female who gave birth to a normal baby.

病例 对16例性腺发育不全患者：其中雄激素不敏感综合症5例、17α羟化酶缺乏症1例、真两性畸形4例、45，X性腺发育不全1例、XY单纯性腺发育不全1例、睾丸退化1例、血核型为46，XY但已分娩正常儿1例以及45，X/46，XY性腺发育不全2例。

Of the 14 patients, 5 were true hermaphroditism, 5 female pseudohermaphroditism and 4 male pseudohermaphroditism.

本文报道了14例两性畸形，其中真两性畸形5例，女性假两性畸形5例，男性假两性畸形4例。

Objective This paper evaluates the importance of early diagnosis and systemic treatment of true hermaphroditism.

目的 探讨真两性畸形的早期正确诊断与合理治疗。

The strange practice of hermaphroditism is more common among species of fish than within any other group of vertebrates.

有一种奇怪的现象就是鱼类的两性畸形，比起其它种类的脊椎动物，这在鱼类物种中较为常见。

Methods Three patients with true hermaphroditism,because of inaccurate diagnosis and improper treatment at their early ages,were put in a dilemma when they grew up.

介绍3例真两性畸形早期不适当的诊断治疗给患者遗留了精神创伤和生活问题及后期的准确诊断与合理治疗。

We herein report two cases of goats with sexual deformity.They had two systems of reproductive organs and the karyotype analysis showed mosaicism for their sex chromosomes,i.e.,60,XX / XY.The proportion of XX cell line was 42% and 54%,while XY cell line was 58% and 46%,respectively,in the two abnormal goats.

本文报道2例两性畸形的山羊，它们均具雌雄两套生殖器官，染色体组型分析显示它们均为性染色体嵌合体，核型为60，XX/XY，两头畸形山羊的XX细胞系的比例分别为42%和54%，XY细胞系的比例分别为58%和46%。

Objective To evaluate the CT findings of female pseudohermaphroditism. Methods The CT findings in 4 cases of female pseudohermaphroditism were analyzed retrospectively.

目的 评价女性假两性畸形的CT表现特点及其临床价值方法回顾性分析4例经手术及临床诊断女性假两性畸形的CT表现。

Five fetuses were diagnosed with female pseudohermaphroditism (21-hydroxylase deficiency in 3 and urorectal septum malformation sequence in 2). Four fetuses were diagnosed with male pseudohermaphroditism (1 with steroid sulfatase deficiency, 1 with presumed camptomelic dysplasia, and 2 undetermined).

在这16个胎儿中有5个被诊断为女性假两性畸形（其中3个有21-羟化酶分泌不足，2个有尿道直肠隔畸形），4个被诊断为男性假两性畸形（其中1个有类固醇硫酸酯酶分泌不足，1个推测可能为弯肢性发育异常，另外2个原因未定），5个有染色体异常，2个有性逆转，核型为（46，XX+SRY）。

DNAs from a group of sex chromosome abnormal patients〔46 XX male, 45 XO Turner's syndrome, 47 XXY klinelfter syndromes, 46 XX true hermaphrodite, 46 XY hermaphrodite, 47 XYY male, 48 XXXY male〕 was hybridized to ZFT using southern bloting method.

我们对一组性染色体异常的病人(46XX男性，46XX真两性畸形，46XY两性畸形，45XO Turner's综合症，47XXY Klinelfter综合症，47XYY男性，48XXXY男性)DNA，用ZFY探针进行了Southern印迹杂交。

The results showed that all of them could detect the presence of ZFY DNA sequences except a 46 XX"male"and a 46 XX hermaphrodite, our findings confirmed that Ferguson-Smith〔1966〕 forwarded the hypothesis that the X-Y intercharge model for XX maleness can be regarded as an abnormal exchange initaited in a region proximal to TDF and extending to the telomere,and,also strongly suggested that ZFY gene palyed an important role in sex determining of human.

结果除一例46XX两性畸形，一例46XX&男性&外，余均能检测到ZFY DNA序列(ZFY DNA序列存在于46XX真两性畸形和45XO Turner's综合症，在国内外尚未见报道)。证实了1966年，Ferguson-Smith提出的理论：在父源减数分裂期间，X-Y发生不等交换，导致Y染色体DNA插入性易位。XX男性是由于Y染色体上的TDF基因转移至X染色体上所致。

The split between the two groups can hardly be papered over.

这两个团体间的分歧难以掩饰。

This approach not only encourages a greater number of responses, but minimizes the likelihood of stale groupthink.

这种做法不仅鼓励了更多的反应，而且减少跟风的可能性。