Cushing综合症培课件.ppt

Cushing syndromeCushing 综合症 Definition Cushing syndrome, the constellation of clinical signs and symptoms resulting form chronic glucocorticoid excess, was probably first described in 1899 定 义为各种病因造成肾上腺分泌过多的糖皮质激素〔主要是皮质醇〕所致病症的总称主要临床表现为满月脸，多血质外貌，向心性肥胖、痤疮、皮肤紫纹高血压和骨质疏松等 Aetiology Clinical presentationlaboratory examination Diagnosis and differential diagnosisTreatment 〔 principle〕 Prognosis 病因分类临床表现实验室检查诊断及鉴别诊断治疗〔原则〕预后 Aetiology病因分类 Hypothalamus 下丘脑〔CRH〕pituitary gland 垂体〔ACTH〕adrenal cortex肾上腺皮质zona glomerulosa zona fasciculata zona reticularis〔Aldosterone〕〔cortisol〕〔gonadal hormone〕++-- AetiologyACTH-dependent Cushing syndromeCushing diseaseEctopic ACTH syndrome ACTH- independent Cushing syndrome Adrenocortical adenomaAdrenocortical adenocarcinomaMicronodular adrenal diseaseMassive macronodular adrenal disease 病因分类 依赖ACTH 的cushing综合症 cushing 病异位ACTH综合症非依赖ACTH 的cushing综合症肾上腺皮质腺瘤 肾上腺皮质癌 原发性色素结节性肾上腺病大结节性巨大肾上腺病 Clinical feature各型临床特征 Cushing DiseaseIt is the most mon type of Cushing syndrome, and accounts for about 70% of the syndromePathogenesisMost patients have ACTH-secreting anterior pituitary corticortrope microadenomas〔＜10mm in diameter〕, but a small number have diffuse corticotrope hyperplasia and pituitary macroadenoma Cushing病最常见，约占cushing综合症的70%,多见于成人，女性多于男性，是由于垂体分泌过多的ACTH所致病因垂体微腺瘤－80%垂体大腺瘤－10％：压迫、侵袭等病症下丘脑功能紊乱 Ectopic ACTH syndromeThe majority of patients with ectopic ACTH syndrome have outside pituitary tumor Other sites are pulmonary carcinosis, carcinoid of bronchus, thymic carcinoma pancreatic cancer, chromaffin tumor, medullary carcinoma of thyroid and so on 异位ACTH综合症继发于垂体以外的恶性肿瘤小细胞肺癌、支气管类癌、胸腺癌、胰腺癌、嗜铬细胞瘤、神经母细胞瘤 等等临床上分两型缓慢进展型迅速进展型 Adrenocortical adenomaPatients with adrenal adenoma usually have gradual onset of sign of hypercortisolism, accounts for about 15-20% of the syndromeAdenoma is round or ellipse, diameter is 3-4cm, weight is about 40g 肾上腺皮质腺瘤多见于成人，男性多于女性－ 15%-20%腺瘤呈圆形或椭圆形，直径约3-4cm，重40g左右，包膜完整其病缓慢，病情中等，多毛及雄激素增多少见