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肢端肥大症

Acromegaly.

作者信息

Ben-Shlomo Anat, Melmed Shlomo

机构信息

Cedars-Sinai Medical Center, 8700 Beverly Boulevard, Los Angeles, CA, 90048, USA.

出版信息

Endocrinol Metab Clin North Am. 2008 Mar;37(1):101-22, viii. doi: 10.1016/j.ecl.2007.10.002.

Abstract

Acromegaly is caused by growth hormone hypersecretion, mostly from a pituitary adenoma, driving insulin-like growth factor 1 overproduction. Manifestations include skeletal and soft tissue growth and deformities; and cardiac, respiratory, neuromuscular, endocrine, and metabolic complications. Increased morbidity and mortality require early and tight disease control. Surgery is the treatment of choice for microadenomas and well-defined intrasellar macroadenomas. Complete resection of large and invasive macroadenomas rarely is achieved; hence, their low rate of disease remission. Pharmacologic treatments, including long-acting somatostatin analogs, dopamine agonists, and growth hormone receptor antagonists, have assumed more importance in achieving biochemical and symptomatic disease control.

摘要

肢端肥大症由生长激素分泌过多引起,主要源于垂体腺瘤,导致胰岛素样生长因子1过度产生。其表现包括骨骼和软组织生长及畸形,以及心脏、呼吸、神经肌肉、内分泌和代谢并发症。发病率和死亡率增加需要早期且严格的疾病控制。手术是微腺瘤和界限清楚的鞍内大腺瘤的首选治疗方法。大型侵袭性大腺瘤很少能实现完全切除,因此疾病缓解率较低。药物治疗,包括长效生长抑素类似物、多巴胺激动剂和生长激素受体拮抗剂,在实现生化和症状性疾病控制方面变得更为重要。

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