Cushing综合症.pptx

Cushing syndromeCushing 综合症,第一页，共七十页。,第二页，共七十页。,Definition,Cushing syndrome,the constellation of clinical signs and symptoms resulting form chronic glucocorticoid excess,was probably first described in 1899,第三页，共七十页。,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,第四页，共七十页。,第五页，共七十页。,第六页，共七十页。,Aetiology Clinical presentationlaboratory examination Diagnosis and differential diagnosisTreatment(principle)Prognosis,第七页，共七十页。,病因分类临床表现实验室检查诊断及鉴别诊断治疗(原那么)预后,第八页，共七十页。,Aetiology病因分类,第九页，共七十页。,Hypothalamus 下丘脑CRHpituitary gland 垂体ACTHadrenal cortex肾上腺皮质zona glomerulosa zona fasciculata zona reticularisAldosteronecortisolgonadal hormone,+,+,-,-,第十页，共七十页。,Aetiology,ACTH-dependent Cushing syndromeCushing diseaseEctopic ACTH syndrome ACTH-independent Cushing syndrome Adrenocortical adenomaAdrenocortical adenocarcinomaMicronodular adrenal diseaseMassive macronodular adrenal disease,第十一页，共七十页。,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,第十二页，共七十页。,Clinical feature各型临床特征,第十三页，共七十页。,Cushing Disease,It is the most common 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,第十四页，共七十页。,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,第十五页，共七十页。,Ectopic ACTH syndrome,The 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,第十六页，共七十页。,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,第十七页，共七十页。,Adrenocortical adenoma,Patients 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,第十八页，共七十页。,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,第十九页，共七十页。,Adrenocortical adenocarcinoma,It occurs in lower than 5%patients of cushing syndromePatients with adenocarcinoma tend to have a more acute and progressive course and virilizing effects may predominateThe bulk of tumor is larger,第二十页，共七十页。,肾上腺皮质腺癌,占cushing综合症的5以下瘤体积大直径5-6cm)，肿瘤可浸润包膜病情重，进展快，重度cushing综合症的表现(低钾性碱中毒、女性男性化,第二十一页，共七十页。,Micronodular adrenal disease,The disease is also known as Meadors syndrome About half of patients with this disorder have no distinctive clinical presentation other than being young-always younger than 30 years of age,half younger than 15 years and some infants,第二十二页，共七十页。,原发性色素结节性肾上腺病,此病又称这为Meador综合症或原发性色素 性结节性肾上腺疾病患者多为儿童和青少年临床表现：一局部同一般的临床表现 一局部为家族性，呈显性遗传(Carney综合症,第二十三页，共七十页。,Massive macronodular adrenal disease大结节性巨大肾上腺病,第二十四页，共七十页。,Clinical presentation临床表现,第二十五页，共七十页。,典型病例重型cushing综合症早期病例以并发症为主者周期性或间歇性,第二十六页，共七十页。,Typical cases典 型 病 例,第二十七页，共七十页。,Clinical presentation1,Progressive obesity is the most common sign.It is usually central,involving the face,neck trunk and abdomenAll over the body and nervous system:weakness,change of emotion and so onThe skin is atrophic,the stratum corneum is thinned,and there is loss of subcutaneous fat allowing subcutaneous blood vessels to been seen,第二十八页，共七十页。,Clinical presentation2,Cardiovascular complication is a major cause of morbidity and death in untreated Cushing syndrome Moderate hypertension is commonGlucocorticoid suppress immune functionInflammatory and febrile responses to bacterial infection are suppressedDysbolism and sexual disturbance sometimes occur,第二十九页，共七十页。,临床表现1,满月脸、多血质外貌、向心性肥胖,第三十页，共七十页。,第三十一页，共七十页。,临床表现2,全身和神经系统病症：肌无力，常有不同程度的精神、情绪变化皮肤表现：皮肤薄，微血管脆性增加，易出现淤斑、紫纹,易合并真菌感染,有时可出现皮肤色素沉着,第三十二页，共七十页。,第三十三页，共七十页。,第三十四页，共七十页。,临床表现2,心血管表现：高血压心衰、脑血管意外凝血功能异常(血小板，纤维蛋白原，脂代谢紊乱，动静脉血栓对感染的抵抗力减弱使到达炎症病灶的单核细胞减少巨噬细胞对抗原的固定、吞噬和杀伤力减弱中性粒细胞运动能力，吞噬作用减弱抗体的形成受到阻抑,第三十五页，共七十页。,临床表现3,性功能障碍：月经减少、不规那么、停经、痤疮等明显男性化警惕肾上腺癌代谢障碍：脂、蛋白、糖代谢低钾性碱中毒，低钙 骨质疏松，伤口愈合不良，生长发育延迟,第三十六页，共七十页。,重型cushing综合症,多由癌肿所致，病情严重，进展迅速体重减轻高血压、浮肿、低钾性碱中毒,第三十七页，共七十页。,早期病例,全身情况较好以高血压为主肥胖、向心性的特点不够显著,第三十八页，共七十页。,以并发症为主者,Cushing综合症本身不明显多以并发症就诊：心衰、脑卒中病理性骨折、精神病症、肺部感染等等,第三十九页，共七十页。,周期性或间歇性,第四十页，共七十页。,Laboratory examination实验室检查,第四十一页，共七十页。,Laboratory examination,The laboratory examination of Cushing syndrome is cortisol hypersecretion.Increased urinary excretion of cortisol and urinary 17-OHLoss of rhythm of plasma cortisol,ACTHLoss of normal suppressibility of cortisol by low-dose dexamethasone,第四十二页，共七十页。,实验室检查,皮质醇分泌增多，失去昼夜分泌节律，且不能被小剂量地塞米松抑制试验所抑制尿17羟皮质类固醇、游离皮质醇增高血浆皮质醇浓度增高小剂量地塞米松抑制试验,第四十三页，共七十页。,皮质醇分泌增多，失去昼夜节律，不能被小剂量地塞米松抑制,第四十四页，共七十页。,Diagnosis and Differential diagnosis诊断和鉴别诊断,第四十五页，共七十页。,Diagnosis,The clinical diagnosis of Cushings syndrome is confirmed by clinical presentation,cortisol hypersecretion,loss of normal rhythm in cortisol and loss of normal suppressibility of cortisol by low-dose dexamet