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[6岁9个月女童麦库恩-奥尔布赖特综合征所致性早熟]

[Precocious puberty caused by McCune-Albright syndrome in a girl aged 6 years and 9 months].

作者信息

Wikiera Beata, Wawro Józef, Noczyńska Anna

机构信息

Klinika Endokrynologii i Diabetologii Wieku Rozwojowego AM we Wrocławiu, Wrocław.

出版信息

Endokrynol Diabetol Chor Przemiany Materii Wieku Rozw. 2006;12(1):63-7.

Abstract

The McCune-Albright syndrome is characterised by polyostotic fibrous dysplasia, "cafe-au-lait" spots and autonomous hyperfunction of various endocrine organs. The authors present the case of a girl at the age of 6 years 9 months with precocious puberty (thelarche III, menarche). High estradiol level (204 pg/ml) and low gonadoptopins concentration as well as their level after GnRH administration suggested ovarian autonomy. Ovarian cysts were found on pelvic ultrasound. Other endocrine abnormalities were excluded. Single "cafe-au-lait" spot was found on the patient skin. Scintigraphy, radiography and computed tomography scans showed fibrous dysplastic bones in the right tibia and in maxillary and sphenoid sinuses.

摘要

McCune - Albright综合征的特征为多骨性纤维发育不良、“牛奶咖啡斑”以及各种内分泌器官的自主性功能亢进。作者报告了一例6岁9个月患有性早熟(乳房发育Ⅲ期、月经初潮)的女孩病例。高雌二醇水平(204 pg/ml)、低促性腺激素浓度以及GnRH给药后的水平提示卵巢自主性。盆腔超声检查发现卵巢囊肿。排除了其他内分泌异常。在患者皮肤上发现单个“牛奶咖啡斑”。闪烁扫描、X线摄影和计算机断层扫描显示右胫骨、上颌窦和蝶窦有纤维发育不良性骨。

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