医学精品课件：（参考翻译）14.肝硬化.ppt

1、HepaticCirrhosis肝硬化Dr.Liping TangDepartment of Gastroenterology,the first affiliated hospital of Chongqing University of Medical SciencesDiagnosisEtiologyComplicationsprognosis evaluationCirrhosis is a serious and irreversible disease and is the tenth leading cause of death in the USA,with an age-ad

2、justed death rate of 9.2 per 100,000 per year.It is one of the most common non-neoplastic 非瘤性 causes of death among hepatobiliary 肝胆 and digestive diseases in our country.SinusoidsPortal veinCentral veinHepatic arteryschistosomiasis 血吸虫病)veno-occlusive disease)n schistosomiasis(血吸虫病)n idiopathic(先天性

3、)portal fibrosisn drugs and toxins:e.g.alcohol,methotrexate(甲氨蝶呤),isoniazid(异烟肼)n infections:chronic hepatitis B or C,brucellosis(布鲁氏菌病)n autoimmune 自身免疫的n vascular abnormalities:e.g.right-sided heart failuren metabolic/genetic diseases:e.g.Wilsons diseasen biliary obstruction 胆道梗阻n idiopathic(先天)/m

4、iscellaneous(各种各样的)n veno-occlusive disease 静脉闭塞症In chronic HBV infection,the rate of progression to cirrhosis is influenced by the replicative 复制的 activity of the virus and whether there has been superinfection 二重感染 by hepatitis delta virus(HDV).Concurrent 同时的 liver injury from other causes(e.g.alc

5、ohol)may also hasten 加速 the onset of cirrhosis.alcohol ingestion 摄入 gender hepatitis B or C infection genetic factors nutritional statusnPrimary biliary cirrhosis 原发性胆汁性肝硬化nSecondary(mechanical)biliary cirrhosisPrimary sclerosing cholangitis原发性硬化性胆管炎Neoplasm 瘤 of bile ducts 胆管 or pancreas 胰腺Iatrogen

6、ic（医源性）or inflammatory biliary stricture 狭窄nCystic(胆囊的)fibrosisnBiliary atresia(闭塞，不通)/neonatal(新生的)hepatitisnCongenital(先天的)biliary cysts(囊肿)Schistosomiasis(l metabolic/genetic disease:e.g.Wilsons disease(in which copper 铜 build up in organ),hemochromatosis 血色沉着病(A genetic disorder involving increa

7、sed absorption of iron铁),porphyria(卟啉症),lipid(脂)disorders,amino acid disorderslChronic immune hepatitisl many drugs and toxins:e.g.methotrexate(氨甲喋呤）,isoniazid(异烟肼),vitamin A,alpha-methyldopa 左旋甲基多巴l cryptogenic(隐源性)cirrhosis:Indian childhood cirrhosis,polycystic(多囊)liver disease Normal liver Cirrho

8、tic liver正常肝脏组织切片肝硬化组织切片Micronodular cirrhosis 小结节性肝硬化Macronodular cirrhosis 大结节性肝硬化Mixed form cirrhosis Early cirrhosis n In the early stage there may be no symptoms at all and the disease may be discovered as a result of an investigation for another illness.When disease progresses,the symptoms may

9、 develop:食管静脉曲张胃底静脉曲张腹水男性乳房发育肝掌l General symptoms:Fatigue or even exhaustion 力竭WeaknessWeight loss and muscle wastingFever Itching 痒 Abdominal painIncrease sensitivity to drugs Frequent infectionEdema l Digestive disorders:Nausea(恶心)Loss of appetite 食欲不振 VomitingDiarrheaJaundicel bleeding and anemia

10、(贫血)Bruising(撞伤)easilySkin bleedingBleeding from gums(齿龈)or noseGastrointestinal bleeding 胃肠道出血Anemia 贫血l Endocrine(hormone)disorders:Loss of sexual desire 性欲.In men:a fall in testosterone(睾丸激素)levels,reduced testicle(睾丸)size,gynaecomastia(男子女性乳房),impotence(阳萎).In women:menstrual(月经)irregularities,i

11、ncreased menstrual pain 痛经 and menopausal(更年期)symptoms.Spider veins(蜘蛛痣)Palmar erythema(肝掌)Hyperpigmentation(色素沉着）Fluid retention 液体潴留蜘蛛痣色素沉着及黄疸男性乳房发育l Splenomegaly（脾大）n Hepatic cirrhosis resulting in congestive splenomegaly 充血性脾肿大 is a cause of hypersplenism(脾亢）.It is a type of disorder which cause

12、s the spleen to rapidly and prematurely 过早的 destroy blood cells.splenomegalyAnemia(贫血),leukopenia(白细胞减少),thrombocytopenia correction of the cytopenia 细胞减少 by splenectomy(脾切除术).l Varicesl 静脉曲张:1.gastroesophageal varices 胃食管静脉曲张2.Abdominal Vein varices 腹壁静脉曲张3.Hemorrhoids（痔）vein varicesl Ascites 腹水:Oc

13、curring in advanced cirrhosis,the accumulation of fluid in the abdominal cavity,or ascites,is related to portal hypertension,significant reduction in serum albumin 血清白蛋白,and renal retention 保留 of sodium.Pathogenesis of ascites:Ascites:Sinusoidal hypertensionHypoalbuminemia(低白蛋白血症)Fixed capacity to r

14、esorb ascitesIncreased sodium and water reabsorption by the Kidneys(due to humoral factors 体液因子)Splanchnic(内脏)arteriolar vasodilation(血管舒张)increasing sympathetic tone（交感紧张）lA palpable,firm liver with a blunt（钝，硬）edge is typical,but at times the liver is small and difficult to palpate.lRegenerating 再

15、生的 nodules are only occasionally palpable.l Upper gastrointestinal tract bleeding l hepatic encephalopathy l infectionsl Hepatorenal syndromel Hepatopulmonary syndromel Hepatocellular carcinomal fluid and electrolyte（电解质）disordersl Upper gastrointestinal tract bleeding may occur from gastroesophagea

16、l varices 将脉曲张,portal hypertensive gastropathy,or gastroduodenal ulcer 胃十二指肠溃疡.Hemorrhage（出血）may be massive,resulting in fatal exsanguination 致死性大出血 or portosystemic encephalopathy.门体肝性脑病 Cirrhosis can cause immune system dysfunction,leading to infection:Spontaneous bacterial peritonitis(SBP),pneumo

17、nia,urinary infection or bile duct infection.nSpontaneous bacterial peritonitis 自发性细菌性腹膜炎(SBP)is an ominous（不详的）complication of late-stage liver disease,it is often heralded 不宣布 by Abdominal pain,increasing ascites,fever.nIt is believed to reflect altered gut wall permeability to bacteria,impaired c

18、apacity of hepatic and splenic macrophages 脾巨噬细胞 to clear portal bacteremias,and/or the presence of a large volume of peritoneal fluid conductive to bacterial growth.l Hepatorenal syndromeHepatorenal syndrome,also known as functional renal failure,is defined as renal failure associated with severe l

19、iver disease without an intrinsic abnormality of the kidney.l Hepatorenal syndromeType 1 HRS is characterized by rapidly progressive kidney failure,with a doubling of serum creatinine 肌酐 to a level greater than 226 mol/L(2.5 mg/dL)or a halving of the creatinine clearance to less than 20 mL/min over

20、a period of less than two weeks.The prognosis of individuals with type 1 HRS is particularly grim 糟糕的,with a mortality rate exceeding 50%after one month.Type 2 HRS is slower in onset and progression.It is defined by an increase in serum creatinine level to 133 mol/L(1.5 mg/dL)or a creatinine clearan

21、ce of less than 40 mL/min,and a urine sodium 10 mol/L。l Hepatopulmonary syndromeliver diseasepulmonary vascular dilation reduced arterial oxygenationl Hepatocellular carcinoma is an epithelial tumor arising from malignant transformation of the hepatocyte.It is observed characteristically as a compli

22、cation of cirrhosis.especially related to chronic viral infections(HBV or HCV).nIt presents as persisting abdominal pain,a palpable abdominal mass,and/or constitutional symptoms in patients with cirrhosis.nThe diagnosis must be considered in cirrhosis with sudden decompensation 失代偿 or in those who d

23、evelop bloody ascites.of Low ingestion of sodium for a long timeLoss sodium by diuretics(利尿剂)and paracentesis(放液)Elevated antidiuretic hormone 抗利尿激素 to conserve body waterInadequate intake and vomiting or diarrheaRenal losses:drugs(diuretics),hyperaldosteronism(高醛固酮血症)Hypokalemia may induce metaboli

24、c alkalosis 代谢性碱中毒 or encephalopathyClotting 凝结 or obstruction of blood flow along the veins from the intestines and spleen and into the liver.This causes blood to back up and results various problems such as enlarged spleen and abdominal pain.The obstruction can occur acutely(over a short space of

25、time)or chronically(over a longer period of time)l Enlarged spleen l Enlarged abdomenl Abdominal painl Diarrhea l Nauseal Blood in stooll Vomitingl Portal hypertensionsymptomsa form of venous thrombosis affecting the hepatic portal vein,which can lead to portal hypertension and reduction in the bloo

26、d supply to the liver.Laboratory findingsl Laboratory findings and other examinationsAnemia,causes include suppression of erythropoiesis红细胞生成 by alcohol as well as folate 叶酸 deficiency,hemolysis溶血,hypersplenism 脾亢,and insidious 潜在的 or overt 明显的 blood loss from the gastrointestinal tract.White blood

27、cell count may be low,elevated,or normal,reflecting hypersplenism or infection.Thrombocytopenia(血小板减少症)may be secondary to alcoholic marrow suppression 酒精性骨髓抑制,sepsis 败血症,folate deficiency,or splenic sequestration 脾隔离症.l In patients with cirrhosis,bilirubin(胆红素)and urobilinogen(尿胆原)may be measured d

28、uring a urine test when jaundice is present.sometimes,protein,casts 管型 or blood cell could be found.Modest 一定程度的 elevations of AST,ALT,alkaline phosphatase(碱性磷酸酶)and progressive elevation of the bilirubin.Serum albumin(白蛋白)is low,gamma globulin is increased.Prolongation 延长 of the prothrombin time(凝血

29、时间)may result from failure of synthesis 合成 of clotting constituents 凝血成分 by the liver.T cell count decreases in more than 50%of patients with cirrhosis,CD3,CD4 and CD8 cells are all reduced.Serum IgG and IgA levels are elevated,especially for IgG,coinciding 一致 with the increasing level of gamma glob

30、ulin In some patients,the antinuclear antibody could be detected in serum.In those patients whose pathogene 病原体 are HBV,HCV,or HDV superimposed 叠加的 on HBV,the viral detection may be positive.Barium(钡)studies of the upper gastrointestinal tract may reveal the presence of esophageal 食管的 or gastric var

31、ices 静脉曲张,though endoscopy(内窥镜)is more sensitive.Ultrasound or CT is helpful for assessing liver or splenic size,detecting ascites or hepatic nodules,including small hepatocellular carcinomas.Together with Doppler studies,ultrasound is used to evaluate patency 开放 of the splenic,portal,and hepatic ve

32、ins.Hepatic nodules may be characterized further by intravenous contrast-enhanced spiral CT scan 静脉增强螺旋CT or MRI along with serum AFP levels.Endoscopic test demonstrates the presence of varices and detects specific causes of bleeding in the esophagus 食管,stomach,and proximal duodenum 十二指肠近端.Liver bio

33、psy remains to be the gold standard for documenting（用文件）证明 cirrhosis,identifying the cause,and assessing the extent 程度 of scar formation.In some centers,liver biopsy is performed by laparoscopy,which may be helpful in judging the type of cirrhosis.DiagnosisEtiologyComplicationsprognosis evaluationMe

34、asure1 point2 points3 pointsunitsBilirubin(total)34(50(3)mol/l(mg/dL)Serum albumin3528-3528mg/LINR 国际化标准比值（or PT prolongation)2.20(18)no unit(seconds)AscitesNoneSuppressed with medicationRefractoryno unitHepatic encephalopathyNoneGrade I-II(or suppressed with medication)Grade III-IV(or refractory)no

35、 unit82%C12-1530%B9-1110%A5-8Perioperative mortality 围手术期死亡率 and prognosisClassPointspoorbest/General measures:l l There are no drugs to reverse 逆转 cirrhosis which have the especially good effect,vitamins or digestive enzyme may be prescribed 规定/开药.some chinese herbal（草药）medicine treatment may impro

36、ve the liver function and symptoms.The goal of weight loss should be no more than 0.5-0.7 kg/dayIn patients with massive ascites and respiratory(呼吸）compromise 让步,ascites refractory（难控制）to diuretics 利尿剂,or intolerable diuretic side effects,large-volume paracentesis(4-6 L)is effective when this is don

37、e,it is often the practice 实践 to give intravenous(静脉内的)albumin concomitantly（伴随）at a dosage of 10g/L of ascites fluid removed to protect the intravascular volume 血管内容积.l This technique is designed to provide one way flow of sterile(消毒)ascitic fluid 腹水 from the abdomen to the vascular system.)Mechani

38、cal tamponade(压塞)with specially designed nasogastric tubes 鼻胃管 containing large gastric and esophageal balloons provides initial control of active variceal hemorrhage 活动性静脉曲张出血 in 60-90%of patients.lPortal decompressive procedure TIPS:it can control acute hemorrhage in over 90%of patients actively b

39、leeding from gastric or esophageal varices.Emergency portosystemic shunt surgery 紧急门静脉分流手术 lSpontaneous bacterial peritonitis(Hepatorenal syndrome is characterized by azotemia（氮质血症）（氮质血症）,oliguria（少尿）（少尿）,hyponatremia（低血钠）（低血钠）,low urinary sodium（低尿钠）（低尿钠）Complication of Hepatic Cirrhosis are _，_，_，

40、_，_，_，_。How to explain the Hepatic Cirrhosis?What are the most common causes of cirrhosis in our country and in western country?What are the clinical manifestations of late cirrhosis?_，_，_，_.The clinical manifestations of Portal hypertension include _，_，_.What are the pathogenesis of ascites of cirrhosis?How to treat the cirrhosis patients with ascites or gastroesophageal varices bleeding?How to diagnose the hepatic cirrhosis correctly?