库欣综合征课件

,单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,2021精选ppt,*,单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,2021精选ppt,*,单击此处编辑母版标题样式,单击此处编辑母版文本样式,二级,三级,四级,五级,2021精选ppt,*,库欣综合征,1,2021精选ppt,库欣综合征12021精选ppt,内容,1,、病因病机及分类,2,、诊断及鉴别诊断,3,、治疗,4,、最新研究进展分享,2,2021精选ppt,内容1、病因病机及分类22,3,2021精选ppt,32021精选ppt,肾上腺的结构,球状带,束状带,网状带,髓质,4,2021精选ppt,肾上腺的结构球状带束状带网状带髓质42021精选ppt,下丘脑,-,垂体,-,肾上腺皮质轴（,HPA,）,下丘脑 CRH,垂体 ACTH,异位ACTH,肾上腺皮质 F,5,2021精选ppt,下丘脑-垂体-肾上腺皮质轴（HPA）,皮质醇的生理作用,1,、糖原代谢、糖异生、组织葡萄糖利用,2,、脂代谢,3,、免疫和炎症的影响,4,、骨骼肌和结缔组织的影响,5,、水和电解质的影响,6,、应激,7,、其他,6,2021精选ppt,皮质醇的生理作用1、糖原代谢、糖异生、组织葡,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,（,Cushings 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,7,2021精选ppt,In 1912,a physician by the na,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,8,2021精选ppt,库欣综合征的病因从激素分泌分类82021精选ppt,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.,因垂体肿瘤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,9,2021精选ppt,库欣综合征的病因从激素分泌分类 92021精选ppt,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 affected 3 times more frequently than women.The most common forms of ACTH-producing tumors are oat cell,or,small cell lung cancer,which accounts for about 25 percent of all lung cancer cases,and carcinoid tumors.Other less common types of tumors that can produce ACTH are,thymomas,pancreatic islet,cell tumors,and,medullary carcinomas of the thyroid,.,10,2021精选ppt,库欣综合征的病因从病变部位分类垂体瘤（Pituitary A,库欣综合征的病因,从病变部位分类,肾上腺肿瘤,(,Adrenal Tumors),Sometimes,an abnormality of the adrenal glands,most often an adrenal tumor,causes Cushings syndrome.The average age of onset is about 40 years.Most of these cases involve non-cancerous tumors of adrenal tissue,called,adrenal adenomas,which release excess,cortisol,into the blood.,Adrenocortical carcinomas,or adrenal cancers,are the least common cause of Cushings syndrome.Cancer cells secrete excess levels of several adrenal cortical hormones,including,cortisol,and,adrenal,androgens.,Adrenocortical carcinomas usually cause very high hormone levels and rapid development of symptoms.,11,2021精选ppt,库欣综合征的病因从病变部位分类肾上腺肿瘤(Adrenal T,库欣综合征的病因,家族性库欣综合征,(Familial Cushings Syndrome),Most cases of Cushings syndrome are not inherited.Rarely,however,some individuals have special causes of Cushings syndrome due to an inherited tendency to develop tumors of one or more endocrine glands.In,Primary Pigmented Micronodular Adrenal Disease,children or young adults develop small cortisol-producing tumors of the adrenal glands.In,Multiple Endocrine Neoplasia Type I(MEN I),hormone secreting tumors of the parathyroid glands,pancreas and pituitary occur.Cushings syndrome in MEN I may be due to pituitary,ectopic or adrenal tumors.,12,2021精选ppt,库欣综合征的病因家族性库欣综合征(Familial Cush,病 因 比例（,%,）,ACTH,依赖性,80,库欣病,68,异位,ACTH,综合症,12,异位,CRH,综合症,1,ACTH,非依赖性,20,肾上腺腺瘤,10,肾上腺腺癌,8,双侧肾上腺小结节增生,1,大结节增生,1,引自“,Williams Textbook of Endocrinology,第,9,版,库欣综合征病因分类和相对比例,13,2021精选ppt,病 因,Causes of Adrenal Cushings Syndrome,Cortisol,ACTH,Bilateral disease,Cortisol,ACTH,Unilateral adrenal tumor,Adrenal Carcinoma,Adrenal Adenoma,Bilateral Macronodular,Adrenal Hyperplasia(AIMAH),Primary Pigmented Nodular,Adrenocortical Disease(PPNAD),14,2021精选ppt,Causes of Adrenal Cushings S,库欣综合征的发病率,it is relatively rare and most commonly affects adults aged 20 to 50.,An estimated 10 to 15 of every million people are affected each year.,库欣病发病率在美国每百万人口每年发病约,5-25,例。我国尚无确切的流行病资料。,男女性别之比为,1:3-8,，男女差别极为显著，原因尚不明。,库欣病可发生在任何年龄，以,2