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库欣综合征:诊断与治疗观念的转变

The Cushing syndromes: changing views of diagnosis and treatment.

作者信息

Gold E M

出版信息

Ann Intern Med. 1979 May;90(5):829-44. doi: 10.7326/0003-4819-90-5-829.

Abstract

Cushing's syndrome is the common clinical presentation of three unique disorders that give rise to hypercortisolism. In most cases neoplasms underly each of these disorders. Clinical features are highly variable and not accounted for by cortisol alone; indeed, the multihormonal basis for much of the clinical syndrome remains uncertain. Demonstration of sustained, excessive cortisol production is essential and depends on a pattern of repeated measurements and several different procedures. Plasma adrenocorticotropin, although not helpful in establishing the diagnosis, has proved valuable in differentiating the three major entities that cause hypercortisolism. The renewed significance of pituitary microadenomas and their improved detection by sella tomography has accompanied recent, impressive advances in transsphenoidal microsurgery. This may become the preferred treatment for pituitary Cushing's syndrome in the adult, particularly where the appropriate equipment and skills are available; for children, external pituitary irradiation seems to offer safe and effective therapy.

摘要

库欣综合征是三种导致皮质醇增多症的独特病症的常见临床表现。在大多数情况下,这些病症均由肿瘤引起。临床特征变化很大,并非仅由皮质醇所致;实际上,该临床综合征的多激素基础仍不明确。持续、过量的皮质醇生成的证明至关重要,这取决于重复测量的模式和几种不同的检测方法。血浆促肾上腺皮质激素虽对确诊无帮助,但已证明在区分导致皮质醇增多症的三种主要病因方面具有重要价值。垂体微腺瘤的重新发现及其通过蝶鞍断层扫描技术的更好检测,伴随着经蝶窦显微手术近期令人瞩目的进展。这可能成为成人垂体性库欣综合征的首选治疗方法,尤其是在具备适当设备和技术的情况下;对于儿童,垂体外部照射似乎提供了安全有效的治疗。

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