{"title":"[6岁9个月女童mcune - albright综合征致性早熟]。","authors":"Beata Wikiera, Józef Wawro, Anna Noczyńska","doi":"","DOIUrl":null,"url":null,"abstract":"<p><p>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.</p>","PeriodicalId":11550,"journal":{"name":"Endokrynologia, diabetologia i choroby przemiany materii wieku rozwojowego : organ Polskiego Towarzystwa Endokrynologow Dzieciecych","volume":"12 1","pages":"63-7"},"PeriodicalIF":0.0000,"publicationDate":"2006-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"[Precocious puberty caused by McCune-Albright syndrome in a girl aged 6 years and 9 months].\",\"authors\":\"Beata Wikiera, Józef Wawro, Anna Noczyńska\",\"doi\":\"\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>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.</p>\",\"PeriodicalId\":11550,\"journal\":{\"name\":\"Endokrynologia, diabetologia i choroby przemiany materii wieku rozwojowego : organ Polskiego Towarzystwa Endokrynologow Dzieciecych\",\"volume\":\"12 1\",\"pages\":\"63-7\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2006-01-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Endokrynologia, diabetologia i choroby przemiany materii wieku rozwojowego : organ Polskiego Towarzystwa Endokrynologow Dzieciecych\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Endokrynologia, diabetologia i choroby przemiany materii wieku rozwojowego : organ Polskiego Towarzystwa Endokrynologow Dzieciecych","FirstCategoryId":"1085","ListUrlMain":"","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
[Precocious puberty caused by McCune-Albright syndrome in a girl aged 6 years and 9 months].
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.
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