【正文】 0g左右，包膜完整 其病緩慢，病情中等，多毛及雄激素增多少見 第十九頁，共七十頁。 Aetiology ACTHdependent Cushing syndrome Cushing disease Ectopic ACTH syndrome ACTH independent Cushing syndrome Adrenocortical adenoma Adrenocortical adenocarcinoma Micronodular adrenal disease Massive macronodular adrenal disease 第十一頁，共七十頁。 Definition Cushing syndrome, the constellation of clinical signs and symptoms resulting form chronic glucocorticoid excess, was probably first described in 1899 第三頁，共七十頁。 第六頁，共七十頁。 Cushing Disease It is the most mon type of Cushing syndrome, and accounts for about 70% of the syndrome Pathogenesis Most patients have ACTHsecreting anterior pituitary corticortrope microadenomas(＜ 10mm in diameter), but a small number have diffuse corticotrope hyperplasia and pituitary macroadenoma 第十四頁，共七十頁。 Micronodular adrenal disease The disease is also known as Meador’s syndrome About half of patients with this disorder have no distinctive clinical presentation other than being youngalways younger than 30 years of age, half younger than 15 years and some infants 第二十二頁，共七十頁。 臨床表現(xiàn) 1 滿月臉、多血質(zhì)外貌、向心性肥胖 第三十頁，共七十頁。 早期病例 全身情況較好 以高血壓為主 肥胖、向心性的特點不夠顯著 第三十八頁，共七十頁。 Diagnosis The clinical diagnosis of Cushing’s syndrome is confirmed by clinical presentation , cortisol hypersecretion, loss of normal rhythm in cortisol and loss of normal suppressibility of cortisol by lowdose dexamethasone 第四十六頁，共七十頁。 Treatmentcushing disease Transsphenoidal microadenomectomy is the most rational current treatment for Cushing disease In patients who are not cured by this treatment, three options remain Reoperate on the pituitary gland Radiate the pituitary gland Perform surgical adrenalectomy and corticoid antagonist 第五十四頁，共七十頁。 手術(shù)前后的處理 患者血漿皮質(zhì)醇濃度很高，一旦切除相關(guān)腫瘤，皮質(zhì)醇分泌量銳減，有可能發(fā)生急性腎上腺皮質(zhì)功能不全的危險 手術(shù)前后均需補充糖皮質(zhì)激素 第六十二頁，共七十頁。 內(nèi)容總結(jié) Cushing syndr