Lahera Vargas Marcos, da Costa César Varela
Servicio de Endocrinología, Hospital Ramón y Cajal, Madrid, España.
Endocrinol Nutr. 2009 Jan;56(1):32-9. doi: 10.1016/S1575-0922(09)70191-3. Epub 2009 Mar 1.
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).
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)。