内分泌高血压幻灯中文.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,*,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,*,*,内分泌高血压幻灯中文,激素,/,血管活性物质与高血压,容量和,/,或血压,胰岛素抵抗,生长激素,(GH),催乳素,(PRL),血管加压素,(AVP),甲状旁腺高血压因子,(PHF),神经肽,Y(NPY),内源性洋地黄素（,Ouabain,）,口服避孕药,甘草,激素,/,血管活性物质与高血压,容量和,/,或血压,降钙素基因相关肽,(CGRP),心钠素,(ANP),脑钠素,(BNP)C-,型利钠肽,(CNP),肾上腺髓质素,(AM),缓激肽（,Bradykinin,）,降钙素,P,物质,(Substance P),激素,/,血管活性物质与高血压,容量和,/,或血压,前列环素,(PGI,2),一氧化氮,(NO),一氧化碳,(CO),内皮舒张因子,(EDRF),内皮超极化因子,(EDHF),激素,/,血管活性物质与高血压,Increased,Renin,Angiotensin,Aldosterone Cortisol Catecholamine Endothelin EDCF GH PHF NPY Vasopressin,Decreased,prostacyclin CGRP Adrenomedullin ANP,BNP,CNP EDRF Bradykinin NO CO PTH Substance P EDHF,BP,心血管激素与高血压,ANP,ET-1 BNP,eNOS HO-2,CNP,AM,NP,ET,NO,CO,VASOCONSTRICTION,VASODILATATION,心血管激素通过内分泌,/,旁分泌,/,自分泌机制,调节心血管的功能和结构,ANP,BNP,Endothelin(ET),CNP/Adrenomedullin,Nitric oxide(NO),Carbon monoxide(CO),心血管内分泌代谢学,Cardiovascular Endocrinology&Metabolism,Heart-传统的循环器官&重要的内分泌腺,Endothelium-做为体内最大的内分泌腺体具有分泌功能,第一届国际心血管内分泌代谢学大会,The First International Conference of Cardiovascular Endocrinology and Metabolism(CVEM)已于1998年在 举行.,Rethink Treatment,Hypertension is a Disease of Blood Vessels,Vascular Biology is Altered,(Structural and Functional),Target,the,Vasculature,Risk Factors(Traditional),Risk Factors(Nontraditional-Vascular Biology),Target Organs-Optimal Treatment,The Blood Vessel is an Organ,Largest Organ in the Body,5 x Heart in Mass,6 Tennis Courts in Area,The Vascular System RegulatesVascular Health and,Tone ThroughChronic Active Balance Between:,Vasoconstrictors Vasodilators,Growth Promoters Growth Inhibitors,_,Vascular Tone,Vascular Health(Damage),A new type of secondary hypertension,Endothelin-secreting malignant hemangioendothelioma,(Yokokawa et al,1991),Case 1.80 yr.F,Headache,nodule on scalp,HT(180/90),Case 2.74 yr.F,ibid.HT(160/106),Plasma ir-ET 10 fold,Plasma ir-ET and BP after surgery,immunostaining of ET(,+,)ir-ET in tissue extract(,+,)ET-mRNA expression(,+,),内分泌性高血压,-,非肾上腺性高血压,肾素 肾血管性高血压,肾素分泌瘤,内皮素 恶性血管内皮瘤,生长激素、生长介素 肢端肥大症、巨人症,T3,T4,甲状腺功能亢进症,PTH,PHF,甲状旁腺功能亢进症,口服避孕药 女性高血压,内皮功能受损 糖尿病,血管损伤 甲状腺功能减退症,糖尿病,激素,疾病,内分泌性高血压,-,肾上腺性高血压,醛固酮 原发性醛固酮增多症,皮质醇 库欣综合症,皮质酮 皮质酮瘤,去氧皮质酮 去氧皮质酮瘤,17,-,羟化酶缺乏症,11,-,羟化酶缺乏症,儿茶酚胺 嗜铬细胞瘤,激素,疾病,内分泌性高血压,激素 机制 疾病,NE PR CO HR,嗜铬细胞瘤,ALDO PV CO PR,原发性醛固酮增多症,F CO PR,库欣综合症,AT II CO PR,肾素分泌瘤,ET PR para/endo/autocrine,恶性血管内皮瘤,PR,:,peripheral resistance,PV,:,plasma volume,CO,:,cardiac output,原发性醛固酮增多症,醛固酮瘤,aldosterone-producing adenoma (,APA,),特发性醛固酮增多症,idiopathic hyperaldosteronism(,IHA,),糖皮质激素可抑制性醛固酮增多症,glucocorticoid-,suppressible hyperaldosteronism (,GSH,),醛固酮癌,aldosterone-producing adrenal carcinoma,异位醛固酮瘤,ectopic aldosterone-producing adenoma,原发性肾上腺增生症,primary adrenal hyperplasia (,PAH,),肾素有反应性醛固酮腺瘤,aldosterone-producing renin,responsive adenoma (,AP-RA,),病因学,phentolamine 1+2,血清钾浓度,Heart-传统的循环器官&重要的内分泌腺,6 Tennis Courts in Area,异位醛固酮瘤 ectopic aldosterone-producing adenoma,Rethink Treatment,体位性低血压,一氧化氮(NO),(if plasma CA 1000-2000pg/ml),Carbon monoxide(CO),缓激肽（Bradykinin）,原发性醛固酮增多症,1,、高血压,缓慢发展的良性过程，少数为恶性高血压,一般降压药效果不好,眼底变化与高血压程度不平行,病程长者可出现心、脑、肾等并发症,临床表现,原发性醛固酮增多症,临床表现,2,、高尿钾、低血钾,血钾,25mmol/24h,20mmol/24h,低钾血症,软瘫、乏力、周期性麻痹,ECG-U,波、心律失常,多尿、夜尿量,日尿量,碱中毒、手足麻木、抽搐,肾功不全,原发性醛固酮增多症,临床表现,3,、糖耐量减退或糖尿病,4,、儿童生长发育障碍,5,、散发性或家族性,原发性醛固酮增多症,血清钾浓度,尿钾水平,醛固酮,/,肾素活性 比值,Captopril,试验,筛选试验,原发性醛固酮增多症,Aldosterone,Plasma Renin Activity,Cortisol-normal,18-hydroxycorticosterone(18-OHB),APA,PAH,100ng,/,dl,IHA,EH,100ng,/,dl,诊断,原发性醛固酮增多症,CT scan -,首选，薄层扫描（,2-3mm),MRI -,价格贵,不如,CT,清楚,I,131,-6-b-iodomethylnorcholesterol(NP-59),扫描,肾上腺静脉插管取血,18-OHB,18-OHF,测定,定位诊断,3.,嗜铬细胞瘤,10%肾上腺外,内皮功能受损 糖尿病,嗜铬细胞瘤（Pheochromocytoma）,Carbon monoxide(CO),Target the Vasculature,血浆儿茶酚胺(NE,E,DA),Carbon monoxide(CO),形态学:(most sensitive,less specific),醛固酮 原发性醛固酮增多症,激素/血管活性物质与高血压,肾素 肾血管性高血压,原发性醛固酮增多症,钙通道阻滞剂（,CCB,）,安体舒通（,Spironolactone,）,氯化钾制剂,阿米诺利（,Amiloride,）,手术,-APA,PAH,地塞米松,-GSH,治疗,原发性醛固酮增多症,除有严重的心、脑、肾等血管病变外，大多数原醛腺瘤病人术后病情可缓解或治愈,术后低血钾的纠正较快而血压恢复较慢,IHA,及,GSH,病人需终生服药,预后,嗜铬细胞瘤（,Pheochromocytoma,）,诊断的临床思路,病史,:,发作性症状,三联征,:,头痛、心悸、多汗,体位性低血压,难治性高血压,家族史：,嗜铬细胞瘤,Von Hippel-Lindau Syndrome,MEN,嗜铬细胞瘤（,Pheochromocytoma,）,诊断的临床思路,体格检查,:,不稳定性、难治性高血压,体位性低血压,VHLS-,或,MEN-,相关体征,IHA,EH 100ng/dl,5 x Heart in Mass,AT II CO PR 肾素分泌瘤,ACE inhibitors,T3,T4 甲状腺功能亢进症,病程长者可出现心、脑、肾等并发症,CNP/Adrenomedullin,血液动力学监测（Hemadynamic monitor）,内皮舒张因子(EDRF),内分泌性高血压-非肾上腺性高血压,1000-2000pg/ml,clonidine 0.3mg,Regitine,抑制试验,BP,170,/,110 mmHg,regitine 5mg,Glucagon,激发试验,glucagon 1mg,嗜铬细胞瘤（,Pheochromocytoma,）,药物治疗,-,adrenergic blockade,phentolamine,1,+,2,phenoxybenzamine,1,2,prazosin,1,urapidil,1,2,-,adrenergic blockade,propranolol,1,+,2,atenolol,1,2,esmolol,1,calcium channel blockades,ACE inhibitors,nitroprusside,嗜铬细胞瘤（,Pheochromocytoma,）,治疗,131-I-MIBG,-,甲基酪氨酸（,-methyl paratyrosine,）,血液动力学监测（,Hemadynamic monitor,）,血容量补充,手术,嗜铬细胞瘤,Pheochromocytoma,rule of 10s,10%,双侧,10%,肾上腺外,10%,腹腔外,10%,恶性,10%,家族性,10%,儿童,10%,血压正常,