Mahoudeau J, Reznik Y, Nivot S
CHU de Caen.
Bull Assoc Anat (Nancy). 1991 Mar;75(228):39-42.
Gonadal dysgenesis are classified according to the aspect of external genitalia. The group with female genitalia includes Turner's syndrome, and "pure" dysgenesis; the group with male genitalia involves Klinefelter's syndrome, XX males and may be anorchia; the group with ambiguous genitalia includes "mixed" gonadal dysgenesis, true hermaphroditism and Leydig-cell agenesis. An algorythmic approach to patients with ambiguous external genitalia is presented in order to distinguish between gonadal dysgenesis and male or female pseudohermaphroditism.
性腺发育不全根据外生殖器的情况进行分类。具有女性生殖器的组包括特纳综合征和“单纯”发育不全;具有男性生殖器的组涉及克兰费尔特综合征、XX男性,也可能是无睾症;具有两性畸形生殖器的组包括“混合性”性腺发育不全、真两性畸形和莱迪希细胞发育不全。本文提出了一种针对外生殖器两性畸形患者的算法方法,以区分性腺发育不全与男性或女性假两性畸形。