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McCune-Albright综合征与肢端肥大症:使用卡麦角林和长效生长抑素进行激素控制——病例报告

McCune-Albright syndrome and acromegaly: hormonal control with use of cabergoline and long-acting somatostatin--case report.

作者信息

Almeida João Paulo C, Albuquerque Lucas Alverne F, Ferraz Camila L H, Mota Italo, Gondim Jackson, Ferraz Tânia M B L

机构信息

Department of Neuroendocrinology, Hospital Geral de Fortaleza,Universidade Federal do Ceará (UFCE), Medical School, Fortaleza, CE, Brazil.

出版信息

Arq Bras Endocrinol Metabol. 2009 Feb;53(1):102-6. doi: 10.1590/s0004-27302009000100015.

Abstract

OBJECTIVES

The use of drug therapy based on cabergoline, octreotide and long-acting release (LAR) octreotide has presented varying results in the treatment of GH excessive production in patients with McCune-Albright Syndrome.

METHODS

We report the case of a 29 year-old female patient presenting McCune-Albright Syndrome and complaint of excessive bone growth.

RESULTS

The patient presented a pituitary adenoma involving the right internal carotid artery and excessive secretion of growth hormone (no GH suppression was observed after the oral glucose tolerance test). Due to the presence of diffuse thickness in skull base bones, surgical approach was not considered effective and the patient was submitted to drug therapy with octreotide LAR and cabergoline. At the one year follow-up, GH and IGF-1 levels were normal and no adverse effects were present.

CONCLUSION

The use of drug therapy based on the association of cabergoline and octreotide is safe and able to achieve complete hormonal control in the treatment of acromegaly for McCune-Albright patients.

摘要

目的

基于卡麦角林、奥曲肽和长效释放(LAR)奥曲肽的药物治疗在麦库综合征患者生长激素过度分泌的治疗中呈现出不同的结果。

方法

我们报告了一例29岁患有麦库综合征且主诉骨生长过度的女性患者。

结果

该患者出现垂体腺瘤累及右侧颈内动脉,生长激素分泌过多(口服葡萄糖耐量试验后未观察到生长激素抑制)。由于颅底骨存在弥漫性增厚,手术治疗被认为无效,患者接受了长效奥曲肽和卡麦角林的药物治疗。在一年的随访中,生长激素和胰岛素样生长因子-1水平正常,且未出现不良反应。

结论

在治疗麦库综合征患者的肢端肥大症时,联合使用卡麦角林和奥曲肽的药物治疗是安全的,并且能够实现完全的激素控制。

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