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[库欣综合征的患病率、病因及临床特征]

[Prevalence, etiology and clinical findings of Cushing's syndrome].

作者信息

Lahera Vargas Marcos, da Costa César Varela

机构信息

Servicio de Endocrinología, Hospital Ramón y Cajal, Madrid, España.

出版信息

Endocrinol Nutr. 2009 Jan;56(1):32-9. doi: 10.1016/S1575-0922(09)70191-3. Epub 2009 Mar 1.

DOI:10.1016/S1575-0922(09)70191-3
PMID:19627706
Abstract

Endogenous Cushing's syndrome is a very rare entity, with an incidence of 2-4 cases per million inhabitants per year. Cases caused by ectopic ACTH secretion are under-diagnosed. Cushing's disease is the most frequent cause of endogenous Cushing's syndrome, which is 5 or 6 times more frequent than adrenal Cushing's syndrome, with an incidence of between 1.2 and 2.4 cases per million inhabitants per year. Cushing's disease is 3-8 times higher in women than in men. The frequency of adrenal tumors is 3 times higher in women, while that of Cushing's syndrome due to adrenal tumors is 3-5 times higher. Age at diagnosis of Cushing's syndrome varies according to the etiology. Most cases of Cushing's disease are due to a pituitary adenoma, although the tumor may not be visible on the available imaging techniques. ACTH-independent Cushing's syndrome is found in 20% of cases and is most frequently due to adenomas (10%) or adrenal carcinomas (8). Bilateral micronodular hyperplasia and macronodular hyperplasia are infrequent entities, representing less than 10% of all cases of ACTH-independent Cushing's syndrome. Both familial and sporadic forms exist: the familial form, or Carney complex, and ACTH-independent bilateral macronodular hyperplasia, in which the size of the adrenal glands is considerably enlarged. The signs and symptoms of Cushing's syndrome are a direct result of long-term exposure to excessive glucocorticoids. Most signs and symptoms are highly prevalent in the general population (hypertension, central obesity, diabetes mellitus or carbohydrate intolerance, osteoporosis, and characteristic phenotypical alterations).

摘要

内源性库欣综合征是一种非常罕见的病症,每年每百万居民中的发病率为2至4例。由异位促肾上腺皮质激素(ACTH)分泌引起的病例诊断不足。库欣病是内源性库欣综合征最常见的病因,其发病率比肾上腺性库欣综合征高5至6倍,每年每百万居民中的发病率在1.2至2.4例之间。库欣病在女性中的发病率比男性高3至8倍。肾上腺肿瘤在女性中的发生率高3倍,而肾上腺肿瘤所致库欣综合征的发生率高3至5倍。库欣综合征的诊断年龄因病因而异。大多数库欣病病例是由垂体腺瘤引起的,尽管在现有的成像技术上可能看不到肿瘤。20%的病例为促肾上腺皮质激素非依赖性库欣综合征,最常见的原因是腺瘤(10%)或肾上腺癌(8%)。双侧微小结节性增生和大结节性增生较少见,占促肾上腺皮质激素非依赖性库欣综合征所有病例的比例不到10%。家族性和散发性形式均存在:家族性形式即卡尼综合征,以及促肾上腺皮质激素非依赖性双侧大结节性增生,其中肾上腺大小显著增大。库欣综合征的体征和症状是长期暴露于过量糖皮质激素的直接结果。大多数体征和症状在普通人群中非常普遍(高血压、向心性肥胖、糖尿病或碳水化合物不耐受、骨质疏松症以及特征性的表型改变)。

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1
[Prevalence, etiology and clinical findings of Cushing's syndrome].[库欣综合征的患病率、病因及临床特征]
Endocrinol Nutr. 2009 Jan;56(1):32-9. doi: 10.1016/S1575-0922(09)70191-3. Epub 2009 Mar 1.
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[Cushing's syndrome: special issues].[库欣综合征:特殊问题]
Endocrinol Nutr. 2009 May;56(5):251-61. doi: 10.1016/S1575-0922(09)71408-1. Epub 2009 Jul 1.
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[Clinical and preclinical aspects of adrenal Cushing syndrome].肾上腺库欣综合征的临床与临床前方面
Minerva Endocrinol. 1995 Mar;20(1):39-47.
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[Diagnosis and differential diagnosis of Cushing's syndrome].[库欣综合征的诊断与鉴别诊断]
Endocrinol Nutr. 2009 Feb;56(2):71-84. doi: 10.1016/S1575-0922(09)70555-8. Epub 2009 May 1.
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[Morphology of the adrenal cortex in Cushing's syndrome].[库欣综合征中肾上腺皮质的形态学]
Schweiz Med Wochenschr. 1980 Sep 6;110(36):1300-6.
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Genetics of Cushing's syndrome.库欣综合征的遗传学。
Neuroendocrinology. 2010;92 Suppl 1:6-10. doi: 10.1159/000314215. Epub 2010 Sep 10.
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[Cushing's disease caused by adrenocortical nodular hyperplasia--diagnosis and surgical treatment].肾上腺皮质结节状增生所致库欣病——诊断与外科治疗
No Shinkei Geka. 1984 May;12(6):689-96.
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[Cushing's syndrome 1985: current views and possibilities].[1985年库欣综合征:当前观点与展望]
Schweiz Med Wochenschr. 1986 Mar 1;116(9):262-5.
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[Cushing syndrome: Physiopathology, etiology and principles of therapy].[库欣综合征:病理生理学、病因及治疗原则]
Presse Med. 2014 Apr;43(4 Pt 1):376-92. doi: 10.1016/j.lpm.2014.02.001. Epub 2014 Mar 20.
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Familial adrenocorticotropin-independent Cushing's syndrome with bilateral macronodular adrenal hyperplasia.家族性促肾上腺皮质激素非依赖性库欣综合征伴双侧大结节性肾上腺增生。
J Clin Endocrinol Metab. 1993 Jan;76(1):189-91. doi: 10.1210/jcem.76.1.8380604.

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Role of bilateral inferior petrosal sinus sampling (BIPSS) in the diagnosis of Cushing's disease in a patient with double superior vena cava.
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