mcune - albright综合征所致肢端肥大症

Q4 Medicine
Martin J. Rutkowski, D. Southwell, T. Cardinal, L. Blevins
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引用次数: 1

摘要

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。
Acromegaly Due to McCune–Albright Syndrome
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.
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来源期刊
US endocrinology
US endocrinology Medicine-Endocrinology, Diabetes and Metabolism
CiteScore
1.90
自引率
0.00%
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