肝胆胰疾病下.ppt

肝胆胰疾病下肝胆胰疾病下病毒性肝炎病毒性肝炎酒精性肝病酒精性肝病肝代谢性疾病肝代谢性疾病肝硬化肝硬化原发性肝癌原发性肝癌胆石症、胆管炎和胆囊炎，胆道肿瘤胆石症、胆管炎和胆囊炎，胆道肿瘤胰腺炎，胰腺癌胰腺炎，胰腺癌病毒性肝炎病毒性肝炎酒精性肝病酒精性肝病肝代谢性疾病肝代谢性疾病肝硬化肝硬化原发性肝癌原发性肝癌胆石症、胆管炎和胆囊炎，胆道肿瘤胆石症、胆管炎和胆囊炎，胆道肿瘤胰腺炎，胰腺癌胰腺炎，胰腺癌肝豆状核变性肝豆状核变性 Wilsons Disease13q14-21，ATP7B常染色体隐形遗传病常染色体隐形遗传病多为儿童及青少年多为儿童及青少年胆小管上铜输出的胆小管上铜输出的ATP酶失活酶失活铜离子过量铜离子过量肝、肝、CNS、肾、肾角膜角膜kayser-Fleischer 环环Micronodular cirrhosis in Wilson diseaseMicronodular cirrhosis in Wilson diseaseNearly all patients with neurologic involvement develop eye lessionKayser-Fleischer rings result from a buildup of copper in the eyes and are the most unique sign of Wilson disease.They appear in each eye as a rusty-brown ring around the edge of the iris and in the rim of the cornea.M/56.This patient died from liver failure.The cirrhotic nodules are clearly seen.The liver is a dark brown color because of the heavy iron deposition within the hepatocytesPrussian blue-stainedA Prussian blue iron stain demonstrates the blue granules of hemosiderin in hepatocytes and Kupffer cells.The Prussian blue iron stainPancreas:Microscopically,islet cells and stroma iron deposition is blue in Prussian blue stained slide.Grossly,diffuse interstitial fibrosis is present.血色病血色病(hemochromatosis)常染色体隐性遗传病常染色体隐性遗传病发病率发病率1/3001/400多见于男性，约多见于男性，约4060岁出现病症岁出现病症铁摄入正常，但铁在胃及小肠中过量吸收铁摄入正常，但铁在胃及小肠中过量吸收 致铁盐在肝和相关器官实质细胞中过量沉致铁盐在肝和相关器官实质细胞中过量沉积积肝受累最早肝受累最早肝功能异常，进展性门静脉性肝硬化肝功能异常，进展性门静脉性肝硬化肝性脑病、上消化道出血、肝性脑病、上消化道出血、15%HCC继发性含铁血黄素沉积症继发性含铁血黄素沉积症seconderay hemosiderosis)体内红细胞破坏过多体内红细胞破坏过多 溶血性贫血、重型溶血性贫血、重型海洋性贫血海洋性贫血 屡次输血屡次输血长期大量服用铁剂长期大量服用铁剂病毒性肝炎病毒性肝炎酒精性肝病酒精性肝病肝代谢性疾病肝代谢性疾病肝硬化肝硬化原发性肝癌原发性肝癌胆石症、胆管炎和胆囊炎，胆道肿瘤胆石症、胆管炎和胆囊炎，胆道肿瘤胰腺炎，胰腺癌胰腺炎，胰腺癌肝硬化肝硬化Liver cirrhosisRene LannecGreek kirrhs meaning yellowish,tawny 肝硬化肝硬化liver cirrhosis 肝细胞弥漫性变性坏死肝细胞弥漫性变性坏死纤维组织增生纤维组织增生肝细胞结节状再生肝细胞结节状再生反复交织反复交织肝脏弥漫变形、肝脏弥漫变形、变硬变硬血液循环改建血液循环改建Gross appearance basic pathologic change 纤维组织增生纤维组织增生 假小叶假小叶pseudolobule 原有肝脏病变原有肝脏病变假小叶假小叶pseudolobule 广泛增生的纤维组织分割包绕肝细胞广泛增生的纤维组织分割包绕肝细胞包括原有的肝小叶和再生的肝细胞结节包括原有的肝小叶和再生的肝细胞结节而形成的大小不等的肝细胞团。而形成的大小不等的肝细胞团。假小叶内中央静脉可缺如、偏位或两个假小叶内中央静脉可缺如、偏位或两个以上，可以出现汇管区。以上，可以出现汇管区。HistopathologyPathogenesisSchematic of stellate cell activation and liver fibrosis in comparison to the normal liver.发病机制：发病机制：病因病因 肝细胞弥漫性坏死肝细胞弥漫性坏死、炎症、炎症 网状纤维支架塌陷网状纤维支架塌陷 纤维组织增生纤维组织增生 分割包绕分割包绕肝小叶、结节状再生的肝细胞肝小叶、结节状再生的肝细胞 假小叶假小叶 肝小叶构造破坏肝小叶构造破坏肝星状细胞肝星状细胞-肌纤维母细胞肌纤维母细胞汇管区成纤维细胞等产生胶原汇管区成纤维细胞等产生胶原异常血管吻合异常血管吻合血液循环途径改建血液循环途径改建小血管堵塞炎症小血管堵塞炎症中大血管血栓中大血管血栓 临床病理联系：临床病理联系：1.门脉高压症门脉高压症 portal hypertension 2.肝功能不全肝功能不全 hepatic in-sufficiency hepatic failure门静脉高压症门静脉高压症肝肝A小叶间小叶间A门门V小叶间小叶间V汇管区汇管区血窦血窦中央静脉中央静脉终末门微终末门微V终末肝微终末肝微A小叶下小叶下V肝肝V下腔下腔V门脉压力增高原因门脉压力增高原因1、肝血窦闭塞，窦周纤维化窦性阻塞、肝血窦闭塞，窦周纤维化窦性阻塞2、假小叶压迫小叶下静脉、假小叶压迫小叶下静脉中央静脉中央静脉肝血窦肝血窦门静脉回流受阻窦后性门静脉回流受阻窦后性阻塞阻塞门脉压力增高原因门脉压力增高原因3、肝动脉与门静脉间异常吻合、肝动脉与门静脉间异常吻合动脉血流入门动脉血流入门静脉静脉门静脉压力门静脉压力(窦前性窦前性)门脉压力增高原因门脉压力增高原因Causes of portal hypertension Pre-hepatic Post-hepatic Intra-hepatic Post-hepatichepatic vein thrombosisright heart failureconstrictive pericarditisPre-hepatic stenosis of portal vein thrombosisIntra-hepatic Schistosomiasis severe diffuse fatty change sarcoidosis TB diffuse liver cirrhosis nodular regenerative hyperplasia 淤血性脾肿大淤血性脾肿大Congestive splenomagelyPortal HypertensionClinical Features1、Congestive splenomagely脾功能亢进脾功能亢进血液淤滞血液淤滞单核单核-巨噬细胞巨噬细胞 吞噬吞噬外周血细胞减少外周血细胞减少2、Ascites腹水腹水，腹膨隆，腹膨隆 淡黄色透明漏出液淡黄色透明漏出液原因：原因：1 1门静脉压力门静脉压力，肝窦流体静压，肝窦流体静压 ，管壁通，管壁通透性透性2 2肝脏合成蛋白肝脏合成蛋白，低蛋白血症，血浆胶体渗，低蛋白血症，血浆胶体渗透压透压3 3肝灭活醛固酮、抗利尿激素功能肝灭活醛固酮、抗利尿激素功能，水钠潴，水钠潴留留 Shunts 食管下段静脉丛食管下段静脉丛 脐周及腹壁静脉丛脐周及腹壁静脉丛 直肠静脉丛直肠静脉丛歪 门 邪 道奇静脉上腔静脉下腔静脉髂内静脉脐周及腹壁静脉丛曲张脐周及腹壁静脉丛曲张海蛇头海蛇头,Caput MedusaeSeen here is the anus and perianal region with prominent prolapsed true hemorrhoids.Chronic constipation,chronic diarrhea,pregnancy,and portal hypertension enhance hemorrhoid formation.Hemorrhoids can itch and bleed slowly(usually bright red blood,during defaecation).4、胃肠瘀血、水肿、胃肠瘀血、水肿消化、吸收功能消化、吸收功能 腹胀腹胀食欲不振食欲不振肝功能不全肝功能不全 hepatic insufficiencyBiochemical abnormalitiesClinical manifestationEstrogen:elevatedSpider angiomas(upper trunk),Palmar erythema,Hypogonadism(atrophic testicles),GynecomastiaSerum albumin:lowAscites Coagulative factors:decreased Prothrombin time:prolonged Tendency to hemorrhageBilirubin:elevatedJaundice,pruritusTransaminases(AST and ALT):elevatedAnorexia,nausea,general malaiseHyperammonemia Hepatic encephalopathy黄疸黄疸jaundice男子乳腺发育男子乳腺发育Gynecomastia due to alcoholic cirrhosis-A 32 year old male patient with normal secondary sex characteristics,no testicular mass,no history of drug ingestion,no other endocrine abnormalities and a normal neurological examination.Nevertheless,he had a history of more than 15 years of large amounts of alcohol intake and a liver biopsy confirm alcoholic cirrhosis.肝掌肝掌Palmar erythema蜘蛛状痣蜘蛛状痣Spider angiomas(upper trunk)出血倾向出血倾向 (Spontaneous bleeding)Clinical manifestationThe classification of cirrhosisAccording to the gross morphologyMicronodular 3mmMixed nodularIncompleted separatedThe descriptive terms should not be used as primary classification The classification of cirrhosisAccording to the causesAlcoholic Viral hepatitisCardiacBiliaryParasiticPigmentalCryptogenicM/39.with hepatitis B infection.The liver is greatly enlarged and weighted 1720 g.Its surface is coarsely nodular.Here is an example of alcoholic cirrhosis.Note that the liver also has a yellowish hue,indicating that fatty change is present.Schistosomiasis Cirrhosis胆汁性肝硬化胆汁性肝硬化Biliary cirrhosis原发性原发性(少见少见 90%女性女性自身免疫性损伤自身免疫性损伤 肝内小胆管慢性非化肝内小胆管慢性非化 脓性破坏性胆管炎脓性破坏性胆管炎慢性进展性胆汁淤积慢性进展性胆汁淤积不全分割型不全分割型继发性继发性 肝外胆管堵塞、胆道上行性感染肝外胆管堵塞、胆道上行性感染Feathery degenerationBile lakesNutmeg liverCardiac cirrhosisThe classification of cirrhosisIntegrated classificatonPortal(micronodular Postnecrotic(macronodular or mixedBiliary(Incompleted separated)CongestiveParasitic 1、病毒性肝炎：、病毒性肝炎：HBV HCV 我国最常我国最常见见的原因的原因 2、慢性酒精中毒：、慢性酒精中毒：欧、美国家最常欧、美国家最常见见原因原因 3、营营养缺乏：养缺乏：胆碱、蛋氨酸；合成磷脂障碍胆碱、蛋氨酸；合成磷脂障碍 脂肪肝脂肪肝4、化学毒物：、化学毒物：杀杀虫虫剂剂、CCl4等等病因病因门脉性肝硬化门脉性肝硬化portal cirrhosis坏死后性肝硬化坏死后性肝硬化postnecrotic cirrhosis肝实质发生大片坏死的根底上形成的肝实质发生大片坏死的根底上形成的较严重的病毒性肝炎较严重的病毒性肝炎 亚急性重型肝炎亚急性重型肝炎 重度慢性肝炎重度慢性肝炎+坏死严重坏死严重药物及化学物质中毒药物及化学物质中毒病理特点：病理特点：大体：体积小，重量轻，质硬，外表大体：体积小，重量轻，质硬，外表及切面结节状及切面结节状(1cm)，纤维间隔，纤维间隔厚，厚薄不均厚，厚薄不均镜下：假小叶形态悬殊镜下：假小叶形态悬殊 肝细胞不同程度变性、坏死肝细胞不同程度变性、坏死 宽大的纤维间隔宽大的纤维间隔 胆管增生和炎细胞浸润显著胆管增生和炎细胞浸润显著postnecrotic cirrhosis临床临床肝功能障碍肝功能障碍 出现早，重出现早，重 门脉高压门脉高压 出现晚，轻出现晚，轻恶变率恶变率 高高postnecrotic cirrhosisSummaryIn cirrhosis the liver is diffusely replaced by nodules of hepatocytes separated by fibrosisThe most common causes of cirrhosis are diseases of alcohol abuse and viral hepatitis Cirrhosis is best classified according to the cause of liver damageSummary The most common and dangerous complications of cirrhosis Fatal bleeding of lower esophageal plexusHepatic coma(hepatic encephalopathy)Hepatocellular carcinoma,especially for the case from chronic hepatitis B and C病毒性肝炎病毒性肝炎酒精性肝病酒精性肝病肝代谢性疾病肝代谢性疾病肝硬化肝硬化原发性肝癌原发性肝癌胆石症、胆管炎和胆囊炎，胆道肿瘤胆石症、胆管炎和胆囊炎，胆道肿瘤胰腺炎，胰腺癌胰腺炎，胰腺癌原发性肝癌原发性肝癌(primary carcinoma of the liver)原发于肝细胞或肝内胆管上皮细胞原发于肝细胞或肝内胆管上皮细胞的恶性肿瘤。的恶性肿瘤。肝癌发病率大国，发病率占全球肝癌发病率大国，发病率占全球45%高发年龄高发年龄4050岁岁起病隐匿起病隐匿40%诊断时没有病症诊断时没有病症确诊时多数已到达中晚期确诊时多数已到达中晚期5年生存率年生存率2%-16%病因病因 病毒性肝炎病毒性肝炎 肝硬化肝硬化 黄曲霉素黄曲霉素高危人群高危人群 乙肝外表抗原阳性者乙肝外表抗原阳性者 慢性肝炎患者慢性肝炎患者 有肝癌家族史有肝癌家族史早期发现肝癌早期发现肝癌定期体检定期体检 每半年做一次肝功能、每半年做一次肝功能、AFP、B超超病病 变变 肉眼类型：肉眼类型：早期肝癌早期肝癌 小肝癌小肝癌 中晚期肝癌中晚期肝癌：巨块型：巨块型 多结节型多结节型 弥漫型弥漫型 多合并肝硬化多合并肝硬化Massive type of HCC in a 17-year-old patient without cirrhosisMassive type of HCCThe liver showing an unifocal massive neoplasm with cirrhosis;some satellite tumor nodules is directly adjacent.Mutifocal type of HCCDiffusely infiltrative type of HCCHCC with intrahepatic metastasistumor thrombosisAdenocarcinoma arising from the bile ducts in the porta hepatis.F/78.The patient died from the effect of obstructive jaundice.cholangiocarcinomaHCCcholangiocarcinomaCombined hepatocellular and cholangiocarcinoma肝脏其他常见肿瘤肝脏其他常见肿瘤 1.原发原发 2.继发转移更常见继发转移更常见良性良性恶性恶性海绵状血管瘤海绵状血管瘤肝细胞腺瘤肝细胞腺瘤 等等肝母细胞瘤肝母细胞瘤血管肉瘤血管肉瘤 等等Cavernous haemangioma in the liver.This was an incidental finding.Occasionally these haemangiomas may bleed,but mostly they are asymptomatic.Most liver-cell adenomas arise in women of child-bearing age,usually after prolonged use of oral contraceptives.Subcapsular adenomas are at risk for rupture.metastatic carcinomas from the stomachMetastatic carcinoma in liverThe liver may be involved by secondary tumors,presenting multiple hepatic metastases.病毒性肝炎病毒性肝炎酒精性肝病酒精性肝病肝代谢性疾病肝代谢性疾病肝硬化肝硬化原发性肝癌原发性肝癌胆石症、胆管炎和胆囊炎，胆道肿瘤胆石症、胆管炎和胆囊炎，胆道肿瘤胰腺炎，胰腺癌胰腺炎，胰腺癌正常胆囊正常胆囊normal gallbladderpigment stones are predisposed by increased hepatic secretion of bilirubin.cholesterol stones are predisposed by changes in cholesterol solubility in bile.Chronic cholecystitis.F/48.The gall bladder shows the features of chronic cholecystitis and it is packed with small multifaceted mixed gall stones.Acute superimposed on chronic cholecystitis.F/38.The gall bladder is distended.Both serosal and mucosal surfaces are reddened and inflamed.The wall of the gall bladder is thickened and there are a few mixed calculi within it.Cholesterolosis.F/36.The gall bladder showschronic cholecystitis.There are multiple yellowspots on its mucosal surface.This is due to the accumulation of lipid in thelamina propria.肝内胆管结石肝内胆管结石胆道肿瘤胆道肿瘤胆囊和肝外胆管癌胆囊和肝外胆管癌 (carcinoma of the gallbladder and extrahepatic bile ducts)胆囊和肝外胆管上皮细胞发生的恶性肿瘤胆囊和肝外胆管上皮细胞发生的恶性肿瘤 Carcinoma of the gallbladder.Adenocarcinoma of the gallbladder is seen as a raised area in fundus.Most tumors have invaded through the wall at the time of diagnosis.胰腺外分泌部胰腺外分泌部Exocrine pancreas胰腺实质由外分泌部和内分泌部组成胰腺实质由外分泌部和内分泌部组成外分泌部占外分泌部占90%，分泌胰液，经导管排入十，分泌胰液，经导管排入十二指肠，有重要的化学性消化作用二指肠，有重要的化学性消化作用 急性胰腺炎急性胰腺炎Acute pancreatitis慢性胰腺炎慢性胰腺炎Chronic pancreatitis 胰腺假性囊肿胰腺假性囊肿Pseudocyst of the pancreas 肿瘤肿瘤TumorsNormal common bile duct and pancreatic duct急性水肿性间质性胰腺炎急性水肿性间质性胰腺炎acute edemous pancreatitis暴饮暴食酗酒；胆道结石暴饮暴食酗酒；胆道结石间质充血水肿间质充血水肿炎细胞浸润炎细胞浸润中性粒细胞和单核细胞中性粒细胞和单核细胞可局灶性脂肪坏死可局灶性脂肪坏死预后好预后好Acute hemorrhagic pancreatitisHemorrhage and necrosisLipase released from the pancreatic acini causes the development of foci of fat necrosis.This is seen as white spots.Histologically these spots are composed of necrotic adipose tissue,with adjacent reactive inflammation.特点：特点：发病急，病情重发病急，病情重病理：胰腺肿大，构造模糊；病理：胰腺肿大，构造模糊；广泛坏死，出血广泛坏死，出血休克和肾功能衰竭，休克和肾功能衰竭，20%死亡死亡急性出血性胰腺炎急性出血性胰腺炎Acute hemorrhagic pancreatitisPathogenesis胰腺坏死胰腺坏死胰液外溢胰液外溢大量出血大量出血剧烈腹痛剧烈腹痛休克、急性肾衰休克、急性肾衰腹膜炎腹膜炎淀粉酶、脂酶淀粉酶、脂酶入血、尿入血、尿血尿淀粉酶、脂酶升高血尿淀粉酶、脂酶升高胰高血糖素胰高血糖素降钙素降钙素骨钙释放骨钙释放 血钙血钙炎症刺激炎症刺激脂肪坏死脂肪坏死吸收钙吸收钙持续呕吐持续呕吐血钾、钠血钾、钠急性急性胰腺炎反复发作胰腺炎反复发作胆道胆道系统疾病，慢性酒精中毒系统疾病，慢性酒精中毒轻轻-中度的腹部疼痛中度的腹部疼痛慢性胰腺炎慢性胰腺炎Chronic pancreatitisChronic pancreatitis.F/43.The pancreas is atrophic and fibrotic.The main pancreatic duct is dilated and there are calculi in the ducts of the pancreas.The patient was an alcoholic.Chronic pancreatitis.The exocrine pancreas has been replaced by fibrous tissue.Pseudocyst in the body and tail of the pancreas.F/67.This is a well-recognized complication of pancreatitis.胰腺癌胰腺癌(carcinoma of pancreas)15%5%Carcinoma of the head of pancreasTumor appears as gritty,grey,hard nodules,irregularlyinvading the adjacentstructures.Histologically they areusually moderatedifferentiated adeno-carcinomas.Summary Key PiontsViral hepatitisAlcoholic liver diseaseCirrhosisKey Terms liver cirrhosispseudolobuleportal hypertensionSmall(early)hepatocellular carcinomaThank you!医学资料仅供参考,用药方面谨遵医嘱