库欣综合征-曾正陪ppt课件.ppt

库欣综合征 曾正陪 北京协和医院内分泌科 In 1912, a physician by the name of Harvey Cushing described eight patients with central body obesity, glucose intolerance, hypertension, excess hair growth, osteoporosis, kidney stones, menstrual irregularity, and emotional liability.It is now known that these symptoms are the result of excess production of cortisol by the adrenal glands. 库欣综合征（Cushing’s syndrome）又称皮质醇增多症，或柯兴综合征，是由于肾上腺皮质长期分泌过量皮质醇引起的症候群，这称为自发性库欣综合症。长期应用外源性糖皮质激素或饮用酒精饮料也可引起类似库欣综合征临床表现，称为类库欣综合征。 库欣综合征的发现 肾上腺的结构 球状带 束状带 网状带 髓质 ACTH依赖性 ACTH Dependent (80%) 垂体或垂体以外的某些肿瘤组织分泌过量ACTH，使双侧肾上腺皮质增生并分泌过量皮质醇，皮质醇的分泌过多是继发的。   ACTH非依赖性 ACTH Independent (20%) 肾上腺皮质自主地分泌过量皮质醇，其原因可以是肾上腺皮质肿瘤，也可以是大结节增生。 ? 库欣综合征的病因从激素分泌分类 库欣综合征的病因从激素分泌分类 库欣综合征(Cushing syndrome,皮质醇增多症) Since cortisol production by the adrenal glands is normally under the control of the pituitary (like the thyroid gland), overproduction can be caused by a tumor in the pituitary or within the adrenal glands themselves. 因垂体或肾上腺肿瘤分泌过多皮质醇所致的一组症候群 库欣病(Cushing disease) When a pituitary tumor secretes too much ACTH (Adrenal Cortical Tropic Hormone), it simply causes the otherwise normal adrenal glands to produce too much cortisol. This type of Cushings syndrome is termed Cushings Disease“. In this case, serum cortisol will be elevated, and, serum ACTH will be elevated at the same time.因垂体瘤分泌过多促肾上腺皮质激素(ACTH),刺激肾上腺分泌过多皮质醇所致的库欣综合征 库欣综合征的病因从病变部位分类 垂体瘤（Pituitary Adenomas）Pituitary adenomas cause most cases of Cushings syndrome. They are benign, or non-cancerous, tumors of the pituitary gland which secrete increased amounts of ACTH. Most patients have a single adenoma. This form of the syndrome, known as Cushings disease, affects women five times more frequently than men. 异位ACTH综合征(Ectopic ACTH Syndrome) Some benign or malignant (cancerous) tumors that arise outside the pituitary can produce ACTH. This condition is known as ectopic ACTH syndrome. Lung tumors cause over 50 percent of these cases. Men are