库欣综合征
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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
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肾上腺的结构
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球状带
束状带
网状带
髓质
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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
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。
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库欣综合征的病因从激素分泌分类
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。
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库欣综合征的病因
家族性库欣综合征(Familial Cushing's Syndrome)
Most cases of Cushing's syndrome are not inherited. Rarely, however, some individuals have special causes of Cushing's 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. Cushing's syndrome in MEN I may be due to pituitary, ectopic or adrenal tumors.