Acromegaly Due to McCune–Albright Syndrome

Q4 Medicine US endocrinology Pub Date : 2020-01-01 DOI:10.17925/use.2020.16.1.47
Martin J. Rutkowski, D. Southwell, T. Cardinal, L. Blevins
{"title":"Acromegaly Due to McCune–Albright Syndrome","authors":"Martin J. Rutkowski, D. Southwell, T. Cardinal, L. Blevins","doi":"10.17925/use.2020.16.1.47","DOIUrl":null,"url":null,"abstract":"McCune–Albright syndrome (MAS) is characterized by polyostotic fibrous dysplasia, café-au-lait hyperpigmentation of the skin, and acromegaly. Ablative therapy in affected patients must target the entire pituitary gland, making medical management the preferred therapy. Targeted medical therapies, including somatostatin and growth hormone (GH) receptor blockade, have shown promise in achievement of biochemical remission. We present the case of a 15-year-old girl with MAS who was discovered to have pituitary enlargement on computed tomography scan. She presented with café-au-lait spots, fibrous dysplasia of the sphenoid bone, and elevated insulin-like growth factor 1 and GH levels that failed to suppress on oral glucose tolerance testing. Treatment with a somatostatin analog was shown to be a safe and effective long-term management strategy of acromegaly that provides advantages over surgical resection.","PeriodicalId":23490,"journal":{"name":"US endocrinology","volume":null,"pages":null},"PeriodicalIF":0.0000,"publicationDate":"2020-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"1","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"US endocrinology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.17925/use.2020.16.1.47","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"Medicine","Score":null,"Total":0}
引用次数: 1

Abstract

McCune–Albright syndrome (MAS) is characterized by polyostotic fibrous dysplasia, café-au-lait hyperpigmentation of the skin, and acromegaly. Ablative therapy in affected patients must target the entire pituitary gland, making medical management the preferred therapy. Targeted medical therapies, including somatostatin and growth hormone (GH) receptor blockade, have shown promise in achievement of biochemical remission. We present the case of a 15-year-old girl with MAS who was discovered to have pituitary enlargement on computed tomography scan. She presented with café-au-lait spots, fibrous dysplasia of the sphenoid bone, and elevated insulin-like growth factor 1 and GH levels that failed to suppress on oral glucose tolerance testing. Treatment with a somatostatin analog was shown to be a safe and effective long-term management strategy of acromegaly that provides advantages over surgical resection.
查看原文
分享 分享
微信好友 朋友圈 QQ好友 复制链接
本刊更多论文
mcune - albright综合征所致肢端肥大症
麦库恩-奥尔布赖特综合征(MAS)的特征是多骨纤维发育不良,皮肤色素沉着和肢端肥大症。消融治疗在受影响的患者必须针对整个垂体,使医疗管理的首选治疗。靶向药物治疗,包括生长抑素和生长激素(GH)受体阻断,已经显示出实现生化缓解的希望。我们提出的情况下,15岁的女孩与MAS谁被发现有垂体增大的计算机断层扫描。她表现为卡萨姆-奥莱斑,脊柱纤维发育不良,胰岛素样生长因子1和生长激素水平升高,口服葡萄糖耐量试验未能抑制。与手术切除相比,生长抑素类似物治疗是一种安全有效的肢端肥大症长期治疗策略。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
求助全文
约1分钟内获得全文 去求助
来源期刊
US endocrinology
US endocrinology Medicine-Endocrinology, Diabetes and Metabolism
CiteScore
1.90
自引率
0.00%
发文量
0
期刊最新文献
Dietary Practices During Common Religious Fasts in People with Type 2 Diabetes Mellitus Among Different Religions in North India—A Questionnaire-based Study Case Reports of Familial Non-autoimmune Hyperthyroidism in Two Malaysian Families due to Germline-activating Mutations in the Thyrotropin Receptor Gene Acromegaly Due to McCune–Albright Syndrome Effect of Genital Tuberculosis on Ovarian Reserve Controversies and Pitfalls in the Diagnosis of Extrapulmonary Tuberculosis with a Focus on Genital Tuberculosis
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
现在去查看 取消
×
提示
确定
0
微信
客服QQ
Book学术公众号 扫码关注我们
反馈
×
意见反馈
请填写您的意见或建议
请填写您的手机或邮箱
已复制链接
已复制链接
快去分享给好友吧!
我知道了
×
扫码分享
扫码分享
Book学术官方微信
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术
文献互助 智能选刊 最新文献 互助须知 联系我们:info@booksci.cn
Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。
Copyright © 2023 Book学术 All rights reserved.
ghs 京公网安备 11010802042870号 京ICP备2023020795号-1