Muritano M, Zachmann M, Manella B, Briner J, Prader A
Department of Pediatrics, University of Zürich, Switzerland.
Horm Res. 1987;28(1):37-41. doi: 10.1159/000180923.
In 2 girls with signs of androgen overproduction, the usual causes were excluded. Patient 1 (3.6 years) presented with hypertrophy of the clitoris, patient 2 (7.8 years) with pubic and axillary hair. Urinary steroids and plasma dehydroepiandrosterone, 17-hydroxyprogesterone and estradiol were normal, but testosterone and androstenedione elevated in both cases. Echography showed polycystic ovaries. Testosterone and androstenedione returned to normal after laparotomy and removal of ovarian cysts in patient 1, and spontaneously in patient 2, in whom puberty started later appropriately for bone age.
在2名有雄激素分泌过多迹象的女孩中,排除了常见病因。患者1(3.6岁)表现为阴蒂肥大,患者2(7.8岁)表现为阴毛和腋毛生长。尿类固醇以及血浆脱氢表雄酮、17-羟孕酮和雌二醇均正常,但两名患者的睾酮和雄烯二酮均升高。超声检查显示多囊卵巢。患者1在剖腹手术并切除卵巢囊肿后,睾酮和雄烯二酮恢复正常;患者2的睾酮和雄烯二酮则自行恢复正常,其青春期开始时间根据骨龄适当延迟。