Naffah J
Bull Acad Natl Med. 1989 Jun;173(6):709-14; discussion 714-5.
The rare syndrome of embryonic testicular regression or agonadism with XY karyotype is described in this paper in a sibship of 7 including 3 cases of regression at a earlier time (in three amenorrheic sisters and on case of tardive and incomplete regression in a brother with bilateral testicular hypotrophy. The antigen H-Y is present in the three sisters. The pathogenesis of this syndrome, compared with that of pure gonadal dysgenesis is discussed.
本文描述了一个有7名成员的家系中的罕见综合征——胚胎睾丸退化或XY核型性腺发育不全，其中包括3例早期退化病例（3名闭经姐妹），以及1例晚期不完全退化病例（1名双侧睾丸发育不全的兄弟）。三名姐妹中存在H-Y抗原。文中还讨论了该综合征与单纯性腺发育不全相比的发病机制。
