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肢端肥大症。识别与治疗。

Acromegaly. Recognition and treatment.

作者信息

Jaffe C A, Barkan A L

机构信息

Division of Endocrinology and Metabolism, Taubman Center, University of Michigan Medical Center, Ann Arbor.

出版信息

Drugs. 1994 Mar;47(3):425-45. doi: 10.2165/00003495-199447030-00004.

Abstract

Acromegaly is a chronic debilitating disease caused by growth hormone (GH) hypersecretion, usually from a pituitary adenoma. It is frequently diagnosed after many years of active GH hypersecretion, and causes significant morbidity and mortality due to cardiac, pulmonary and musculoskeletal changes. Local complications resulting from the pituitary tumour can also occur. The most important feature that will enable a physician to diagnosis the disease is clinical vigilance. Measurement of elevated plasma mecasermin (insulin-like growth factor I, IGF-I) is the single best test to make the diagnosis. Once the diagnosis is confirmed, a GH-secreting tumour should be sought, by performing a careful magnetic resonance imaging or computed tomography scan of the pituitary gland and hypothalamus. Therapy is directed at both preventing local complications of the tumour mass as well as normalising GH secretion. Surgical resection of the tumour is almost always the first step in treatment. If GH secretion is not normalised, which is best assessed by determining whether plasma IGF-I returns to the normal range, further treatment with radiation and/or medical therapy is required. Bromocriptine normalises GH in approximately 10% of patients and causes pituitary shrinkage in a similar fraction of patients. Octreotide is considerably more expensive than bromocriptine and is given subcutaneously, but is more effective in both normalising GH secretion and in shrinking tumours. Octreotide treatment of the pituitary tumours prior to surgical resection may be of value, but requires further investigation.

摘要

肢端肥大症是一种由生长激素(GH)分泌过多引起的慢性衰弱性疾病,通常源于垂体腺瘤。该病常在多年活跃的GH分泌过多后才得以诊断,并且由于心脏、肺部和肌肉骨骼的变化而导致显著的发病率和死亡率。垂体肿瘤还可能引发局部并发症。医生能够诊断该病的最重要特征是临床警觉性。检测血浆中升高的美卡舍明(胰岛素样生长因子I,IGF-I)是进行诊断的最佳单项检测。一旦确诊,应通过对垂体和下丘脑进行仔细的磁共振成像或计算机断层扫描来寻找分泌GH的肿瘤。治疗旨在预防肿瘤块的局部并发症以及使GH分泌正常化。肿瘤的手术切除几乎总是治疗的第一步。如果GH分泌未恢复正常(通过确定血浆IGF-I是否恢复到正常范围来最佳评估),则需要进一步进行放射治疗和/或药物治疗。溴隐亭可使约10%的患者GH分泌恢复正常,并使类似比例的患者垂体缩小。奥曲肽比溴隐亭贵得多,需皮下注射,但在使GH分泌正常化和肿瘤缩小方面更有效。在手术切除前用奥曲肽治疗垂体肿瘤可能有价值,但需要进一步研究。

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