Ogata T, Hasegawa T, Tamai S, Sato S, Hasegawa Y, Matsuo N
Department of Paediatrics, Keio University School of Medicine, Tokyo.
Horm Res. 1998 Sep;50(3):190-2. doi: 10.1159/000023272.
We report on ovarian dysfunction in a 3-year-old girl with blepharophimosis, ptosis, and epicanthus inversus syndrome (BPES). A gonadotropin releasing hormone test showed hyperresponses of luteinizing hormone (<0.2-->7.2 mIU/ml) and follicle-stimulating hormone (7.1-->44.8 mIU/ml), and a human menopause gonadotropin test yielded no estradiol response (13-->11 pg/ml). The results suggest that primary ovarian failure in type I BPES can take place in early childhood.
我们报告了一名患有睑裂狭小、上睑下垂和内眦赘皮综合征（BPES）的3岁女孩的卵巢功能障碍。促性腺激素释放激素试验显示促黄体生成素（<0.2-->7.2 mIU/ml）和促卵泡生成素（7.1-->44.8 mIU/ml）反应过度，人绝经期促性腺激素试验未产生雌二醇反应（13-->11 pg/ml）。结果表明，I型BPES的原发性卵巢功能衰竭可发生在儿童早期。
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