内科学课件：肝硬化Cirrhosis

肝硬化肝硬化 Cirrhosis 肝硬化是一种以肝组织肝硬化是一种以肝组织弥漫性纤维化弥漫性纤维化、假假小叶和再生结节小叶和再生结节形成为特征的慢性肝病。是形成为特征的慢性肝病。是许多肝脏疾病晚期的共同病变。临床上有多许多肝脏疾病晚期的共同病变。临床上有多系统受累，以肝功能减退、门脉高压两大症系统受累，以肝功能减退、门脉高压两大症侯群为主要表现，晚期常出现上消化道出血、侯群为主要表现，晚期常出现上消化道出血、肝性脑病、继发感染等严重并发症而死亡。肝性脑病、继发感染等严重并发症而死亡。 Cirrhosis is a pathological diagnosis.It is characterized by widespread fibrosis with nodular regeneration. Its presence implies previous or continuing hepatic cell damage. l引起肝硬化的病因较多，同一病因可发展为引起肝硬化的病因较多，同一病因可发展为不同病理类型的肝硬化，而同一病理类型的不同病理类型的肝硬化，而同一病理类型的肝硬化亦可由不同病因引起。肝硬化亦可由不同病因引起。l常见的病因包括：常见的病因包括： 病毒性肝炎病毒性肝炎( (viral hepatitis) viral hepatitis) 酒精性肝炎酒精性肝炎( (alcoholic hepatitis) alcoholic hepatitis) 胆汁淤积胆汁淤积( (cholestasis) cholestasis) 严重心力衰竭（严重心力衰竭（severe heart failure) severe heart failure) 肝豆状核变性肝豆状核变性( (hepatolenticular disease) hepatolenticular disease) - -抗胰蛋白酶缺乏症（抗胰蛋白酶缺乏症（ - -antitrypsin antitrypsin deficiency)deficiency)。病病 因因 AetiologyAetiology一一、病毒性肝炎、病毒性肝炎( (viral hepatitis)viral hepatitis)（肝炎后肝硬（肝炎后肝硬化）化） 我国肝炎发病率达我国肝炎发病率达137/10万以上，仅乙肝病万以上，仅乙肝病毒携带者就有毒携带者就有1亿亿2千万人口。成人期感染千万人口。成人期感染HBV，10%左右演变为慢性肝炎，约左右演变为慢性肝炎，约3%发展为肝硬化。发展为肝硬化。丙肝的发病率远远低于乙肝，但感染丙肝的发病率远远低于乙肝，但感染HCV后约后约833%可演变为慢性肝炎，最后导致肝硬化。可演变为慢性肝炎，最后导致肝硬化。 在我国可能演变为肝硬化的主要是乙肝，其在我国可能演变为肝硬化的主要是乙肝，其次是丙肝，或乙肝合并丁肝感染。而甲肝和戊肝次是丙肝，或乙肝合并丁肝感染。而甲肝和戊肝一般不发展为慢性肝炎和肝硬化。一般不发展为慢性肝炎和肝硬化。 HBV感染感染免疫异常免疫异常肝细胞炎症、坏死、再生肝细胞炎症、坏死、再生纤维化纤维化假小叶假小叶病病 因因 AetiologyAetiology病病 因因 AetiologyAetiologylMan who drink over 80g of alcohol per day, or women who drink over 40g per day have a significant risk of developing cirrhosis. However, cirrhosis is not inevitable. Only 10-20 % of chronic alcoholics develop cirrhosis even though they drink the same amount of alcohol over the same period as other alcoholics. Risk factors for developing cirrhosis include genetics, gender (women develop alcoholic hepatitis and cirrhosis younger, and after less intake, than men), nutrition (alcohol is better tolerated under optimal dietary conditions), and a synergistic effect with hepatotropic viruses *.病病 因因 AetiologyAetiologylPrimary biliary cirrhosis (PBC) is a chronic cholestatic liver disease involving the interlobular and septal bile ducts. This disorder is characterized by progressive inflammatory destruction of these bile ducts with the development of portal and periportal inflammation, subsequent fibrosis, and eventually cirrhosis. Portal hypertension and the consequences of end-stage liver disease can lead to early death unless effective therapy or liver transplantation intervenes.四、循环障碍四、循环障碍（severe heart failure)severe heart failure) 病病 因因 AetiologyAetiologylOccluded hepatic veinslNormal hepatic veins 镜下可见，镜下可见，中央静脉中央静脉周围充血周围充血导致豆蔻导致豆蔻样表现。样表现。其病因一其病因一般为右心般为右心衰。衰。五、化学毒物或药物五、化学毒物或药物病病 因因 AetiologyAetiology病病 因因 AetiologyAetiology目目 录录NOTE:Schistosoma induced hepatopathy is strictedly called hepatic fibrosis,for there is no obvious regenerative nodules .Characteristics:lHepatomegaly , splenomegaly is extremely obvious.lPortal Hypertension is serious .lHepatocellular dysfunction is less common.l 绿水青山枉自多，华佗无奈小虫何！l千村薜荔人遗矢，万户萧疏鬼唱歌。l坐地日行八万里，巡天遥看一千河。l牛郎欲问瘟神事，一样悲欢逐逝波。l春风杨柳万千条，六亿神州尽舜尧。l红雨随心翻作浪，青山着意化为桥。l天连五岭银锄落，地动三河铁臂摇。l借问瘟君欲何往，纸船明烛照天烧。病病 因因 AetiologyAetiologyEye close viewStaining of Copper九、营养障碍九、营养障碍病病 因因 AetiologyAetiology病病 因因 AetiologyAetiology目目 录录 发病机理发病机理 Pathogenesis1234发病机理发病机理pathogenesis发病机理发病机理 Pathogenesis病病 理理Pathology 病病 理理Pathology 1.1.小结节性肝硬化：最常见。小结节性肝硬化：最常见。Micronodular (portal cirrhosis) is characterised by regular thick fibrotic bands joining the portal tracts to hepatic veins, and with small regenerative nodules. 2.2.大结节性肝硬化：大结节性肝硬化：Macronodular (post-necrotic cirrhosis) is less common and is chracterised by course, irregular bands of fibrosis and loss of normal architecture and large regenerative nodules. It is believed usually to follow viral hepatitis with widespread necrosis. 一、假小叶形成的病理基础 1肝细胞持续不断的坏死，肝小叶纤维支架塌陷破坏。 2 再生的肝细胞不沿原支架排列，形成不规则的再生结节。 3 汇管区、肝包膜下大量纤维结缔组织增生，形成纤维间隔，向小叶内延伸，将残存的肝小叶重新分割，改建成假小叶，纤维收缩，肝脏变硬变小。假小叶的结构特征：假小叶的结构特征： 由几个不完整的肝由几个不完整的肝小叶构成，内含多个中小叶构成，内含多个中央静脉或无中央静脉。央静脉或无中央静脉。肝细胞排列和血窦分布肝细胞排列和血窦分布不规则，假小叶内肝细不规则，假小叶内肝细胞变性坏死，脂肪浸润胞变性坏死，脂肪浸润，甚至坏死再生，汇管，甚至坏死再生，汇管区纤维结缔组织增生增区纤维结缔组织增生增宽，伴有炎症细胞浸润宽，伴有炎症细胞浸润及假胆管形成。及假胆管形成。病病 理理Pathology病病 理理Pathology 临床表现临床表现 Clinical presentationlClinical presentationlThere may be no abnormal clinical or biochemical features of liver disease. Later, features of hepatocellular failure, portal hypertension, or both may appear. 临床表现临床表现 Clinical presentation 临床表现临床表现 Clinical presentation临床表现临床表现 Clinical presentation 3.3.黄疸 约约505060%60%的病人出现黄疸，一般轻中的病人出现黄疸，一般轻中度，与肝细胞损害程度度，与肝细胞损害程度有关，黄疸进行性加重有关，黄疸进行性加重说明有肝细胞持续坏死，说明有肝细胞持续坏死，或有胆汁郁积，往往提或有胆汁郁积，往往提示预后不良。示预后不良。临床表现临床表现 Clinical presentation临床表现临床表现 Clinical presentation临床表现临床表现 Clinical presentation肝肝功功能能减减退退雌激素增多雌激素增多醛固酮增多醛固酮增多抗利尿激素增多抗利尿激素增多垂体性腺轴垂体性腺轴垂体肾上腺轴垂体肾上腺轴雄激素减少雄激素减少糖皮质激素减少糖皮质激素减少蜘蛛痣、肝掌蜘蛛痣、肝掌男男 性欲降低性欲降低,睾丸萎缩睾丸萎缩女女 月经失调月经失调,闭经闭经,不孕不孕少尿少尿,浮肿浮肿,腹水腹水水吸收增加水吸收增加色素沉着色素沉着钠重吸收增加钠重吸收增加 男性乳房发育男性乳房发育 肝掌肝掌(liver palms)临床表现临床表现 Clinical presentation蜘蛛痣（spider nevi) 蜘蛛痣沿上腔静脉引流的区域分布，乳头以上胸、颈、脸、上肢。由小动脉为中心，向四周呈辐射状的毛细血管组成，形似蜘蛛。 蜘蛛痣的大小，多少以及肝掌与肝功有关，随着肝功改善，雌激素水平下降而减少、减轻。 临床表现临床表现 Clinical presentation临床表现临床表现Clinical presentation1、临床表现临床表现 Clinical presentationlPortal vascular resistance is increased due to collagen deposition and fibrosis seen in liver cirrhosis and hence formation of varices in the gastro-oesophageal junction. In addition, sodium retention and vasoactive substances such as nitric oxide will increase plasma volume and splanchnic vasodilatation,respectively, and thus maintain portal hypertension.1门门V胃冠状胃冠状V 胃底、食胃底、食管下段管下段V 奇奇V 上腔上腔V 常因食管粘膜炎症，进食粗糙，常因食管粘膜炎症，进食粗糙，刺激性食物，或腹内压突然增高而破刺激性食物，或腹内压突然增高而破裂出血，发生呕血，黑便，甚至休克裂出血，发生呕血，黑便，甚至休克等症状。等症状。2 门门V 附脐附脐V 脐周脐周V 丛丛 胸腹壁胸腹壁V胸廓胸廓V上腔上腔V 腹壁浅腹壁浅V腹壁下腹壁下V下腔下腔V3 门门V 肠系膜下肠系膜下V 直肠上直肠上V 直肠直肠V丛丛 直肠中下直肠中下V 下腔下腔V * 临床表现临床表现 Clinical presentationgastroesophageal VaricesCirrhosis abdominal collateral circulation临床表现临床表现 Clinical presentation临床表现临床表现并发症并发症 Complications并发症并发症 Complications并发症并发症 Complications l原因：原因：l表现：表现：并发症并发症 Complications并发症并发症 Complications并发症并发症 Complications并发症并发症 ComplicationslThe renal abnormality is thought to be functional because transplanted kidneys from a donor patient with hepatorenal syndrome to a recipient will result in a normal functioning kidney. However, extreme cases will cause tubular necrosis and renal damage.并发症并发症 Complications并发症并发症 Complications并发症并发症 Complications 实验及其他检查实验及其他检查 laboratory tests and investigations实验及其他检查实验及其他检查laboratory tests and investigations实验及其他检查实验及其他检查laboratory tests and investigations实验及其他检查实验及其他检查laboratory tests and investigationsUltrasound demonstrates fatty change, size, and fibrosis as well as hepatocellular carcinoma. 实验及其他检查实验及其他检查laboratory tests and investigations实验及其他检查实验及其他检查laboratory tests and investigations诊诊 断断 DiagnosisDiagnosis鉴别诊断鉴别诊断 Differential diagnosis鉴别诊断鉴别诊断 Differential diagnosis治治 疗疗 TreatmentTreatment *门静脉高压治疗门静脉高压治疗 及并发症治疗及并发症治疗（一）保肝或改善肝功（一）保肝或改善肝功（ Transjugular Intrahepatic Portosystemic Shunt ， l一级预防：未出血者一级预防：未出血者l二级预防：防治再出血二级预防：防治再出血l*预防再出血：预防再出血：l70%病人会再出血，且死亡率高病人会再出血，且死亡率高l控制活动性出血后，内镜下对曲张静脉套扎或硬化控制活动性出血后，内镜下对曲张静脉套扎或硬化l胃底曲张静脉注射组织胶胃底曲张静脉注射组织胶l药物首选普萘洛尔，药物首选普萘洛尔，10mg/d，逐日加，逐日加10mg至静息心率为至静息心率为基础心率基础心率75%，或，或55次次/分分l合用合用5-单硝酸异山梨醇酯单硝酸异山梨醇酯l*预防首次出血：首选普萘洛尔，目的将肝静脉压力小于预防首次出血：首选普萘洛尔，目的将肝静脉压力小于12mmHg。无效者可谨慎套扎或硬化。无效者可谨慎套扎或硬化lUrgent endoscopy is required, during which sclerosant is injected in or around the varices to cause inflammatory obliteration. Alternatively, elastic band ligation of the varices at endoscopy produces thrombotc obliteration. Repeat sclerotherapy or banding is usually needed to prevent further bleeds. （三）其他并发症治疗（三）其他并发症治疗（三）手术（三）手术项目项目 Child-Pugh one year survival rate A 95% B 75%95% C 50%75%lWhat are the major clinical manifestations of cirrhosis?lWhat are the complications of cirrhosis?lWhat are the mechanisms of ascites?lSBP？lWhat are the major clinical manifestations of cirrhosis?lWhat are the complications of cirrhosis?lWhat are the mechanisms of acsites?lSBP？谢谢