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库欣综合征的流行病学。

Epidemiology of Cushing's syndrome.

机构信息

Medical Department M, Aarhus University Hospital, Aarhus, Denmark.

出版信息

Neuroendocrinology. 2010;92 Suppl 1:1-5. doi: 10.1159/000314297. Epub 2010 Sep 10.

DOI:10.1159/000314297
PMID:20829610
Abstract

Overt Cushing's syndrome is a rare disorder with an annual incidence of 2-3/million of which benign adrenal adenomas account for 0.6/million. The female:male ratio is 3:1. Preliminary data indicate a high proportion of subclinical Cushing's syndrome in certain risk populations such as patients with type 2 diabetes or osteoporosis. The clinical implications of these observations are presently unclear. Surgery remains first line treatment for overt disease and initial cure or remission is obtained in 65-85% of patients with Cushing's disease. Late recurrences, however, occur in up to 20% and the risk does not seem to plateau even after 20 years of follow-up. A 2- to 3-fold increase in mortality is observed in most studies, and this excess mortality seems confined to patients in whom initial cure was not obtained. Cushing's syndrome continues to pose diagnostic and therapeutic challenges and life-long follow-up is mandatory.

摘要

库欣综合征(Cushing's syndrome)是一种罕见的疾病,其年发病率为 2-3/百万人,其中良性肾上腺腺瘤占 0.6/百万人。女性与男性的比例为 3:1。初步数据表明,某些高危人群(如 2 型糖尿病或骨质疏松症患者)中存在亚临床库欣综合征的高比例。这些观察结果的临床意义目前尚不清楚。手术仍然是显性疾病的一线治疗方法,库欣病患者中 65-85%的患者可获得初始治愈或缓解。然而,迟发性复发高达 20%,并且即使在 20 年的随访后,风险似乎也不会趋于平稳。大多数研究都观察到死亡率增加 2-3 倍,这种超额死亡率似乎仅限于初始治疗未获得治愈的患者。库欣综合征仍然存在诊断和治疗方面的挑战,需要终身随访。

相似文献

1
Epidemiology of Cushing's syndrome.库欣综合征的流行病学。
Neuroendocrinology. 2010;92 Suppl 1:1-5. doi: 10.1159/000314297. Epub 2010 Sep 10.
2
Long-term remission rates after pituitary surgery for Cushing's disease: the need for long-term surveillance.库欣病垂体手术后的长期缓解率:长期监测的必要性。
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[Subclinical Cushing's syndrome:analysis of diagnosis and surgical effect].[亚临床库欣综合征:诊断与手术效果分析]
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Acquired prolactin deficiency (APD) after treatment for Cushing's disease is a reliable marker of irreversible severe GHD but does not reflect disease status.库欣病治疗后获得性催乳素缺乏(APD)是不可逆性严重生长激素缺乏(GHD)的可靠标志物,但不能反映疾病状态。
Clin Endocrinol (Oxf). 2004 Apr;60(4):476-83. doi: 10.1111/j.1365-2265.2004.02004.x.
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Epidemiology of Cushing's syndrome and subclinical disease.库欣综合征及亚临床疾病的流行病学
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7
Cushing's syndrome in children and adolescents. Presentation, diagnosis, and therapy.儿童及青少年库欣综合征。临床表现、诊断与治疗。
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The use of postoperative ACTH levels as a marker for successful transsphenoidal microsurgery in Cushing's disease.使用术后促肾上腺皮质激素(ACTH)水平作为库欣病经蝶窦显微手术成功的标志物。
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The long-term outcome of pituitary irradiation after unsuccessful transsphenoidal surgery in Cushing's disease.库欣病经蝶窦手术失败后垂体放疗的长期结果。
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10
[Spontaneous remission of corticosteroid osteopenia after successful surgical treatment of Cushing's syndrome. A cross-sectional study].[库欣综合征成功手术治疗后皮质类固醇性骨质减少的自发缓解。一项横断面研究]
Cas Lek Cesk. 1997 Jul 30;136(15):464-7.

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