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1、HepaticCirrhosis肝硬化l What is the hepatic cirrhosis? What is the hepatic cirrhosis?l Diagnosis Diagnosis DiagnosisEtiologyComplicationsprognosisevaluationl Treatment for cirrhosis(basic and complications) Treatment for cirrhosis(basic and complications)Definition and general featuresDefinition and ge
2、neral featuresl lCirrhosisisscarringCirrhosisisscarring瘢痕形成瘢痕形成 oftheliverthatinvolvestheoftheliverthatinvolvestheformationoffibrousformationoffibrous纤维纤维(scar(scar瘢瘢痕痕)tissueassociatedwiththe)tissueassociatedwiththedestructionofthenormaldestructionofthenormalarchitecturearchitecture架构架构oftheorgan.o
3、ftheorgan.l lItistheresultoflong-standingItistheresultoflong-standinginjurymostcommonlyduetoinjurymostcommonlyduetoalcohol(alcohol(酒精酒精)inexcess)inexcess过量过量ororchronichepatitis(chronichepatitis(慢性肝炎慢性肝炎).But).Buttherearemanyotherimportanttherearemanyotherimportantcauses.causes.l lItisalong-termlive
4、rdisease.Itisalong-termliverdisease.Definition and general featuresCirrhosisisaseriouscondition.Cirrhosisisaseriouscondition.Only30percentofpatientswithOnly30percentofpatientswiththisproblemwillsurvivefiveyearsthisproblemwillsurvivefiveyearsafterdiagnosis.Intheverylateafterdiagnosis.Intheverylatesta
5、gesofthedisease,seriousstagesofthedisease,plicationsmayoccur.EpidemiologyCirrhosisisaseriousandirreversiblediseaseandisthetenthleadingcauseofdeathintheUSA,withanage-adjusteddeathrateof9.2per100,000peryear.Itisoneofthemostcommonnon-neoplastic非瘤性causesofdeathamonghepatobiliary肝胆anddigestivediseasesino
6、urcountry.ThelivercontainsaboutonemillionoflobularThelivercontainsaboutonemillionoflobular叶叶unitswhichareunitswhichareapproximately2-3mmacross.Ineachlobuleareseveralapproximately2-3mmacross.Ineachlobuleareseveralthousandonemmlongsinusoids(thousandonemmlongsinusoids(肝血窦肝血窦)(capillaries).These)(capill
7、aries).Theseoveronebillionsinusoidsareallinparalleloveronebillionsinusoidsareallinparallel平行的平行的,thusthe,thusthebloodflowisveryslowandtheinterfacebloodflowisveryslowandtheinterface交界交界betweenitandthebetweenitandthehepatocytesisidealhepatocytesisideal理想的理想的fortheexchangeofmetabolites.fortheexchangeof
8、metabolites.PathophysiologyNormalNormalHepaticHepaticlobularlobularNormal Hepatic vasculature(脉管系统)l l75%ofthebloodenteringthe75%ofthebloodenteringtheliverisvenousbloodfromtheliverisvenousbloodfromtheportalvein,25%ofthebloodportalvein,25%ofthebloodsupplytotheliverisarterialsupplytotheliverisarterial
9、bloodfromthehepaticartery.bloodfromthehepaticartery.l lTerminalbranchesTerminalbranches终末支终末支oftheofthehepaticportalveinandhepatichepaticportalveinandhepaticarteryemptytogetherandmixasarteryemptytogetherandmixastheyentersinusoidsintheliver.theyentersinusoidsintheliver.l lBloodflowsthroughthesinusoid
10、sBloodflowsthroughthesinusoidsandemptiesintothecentralveinofandemptiesintothecentralveinofeachlobule.eachlobule.l lCentralveinscoalesceCentralveinscoalesce合并合并intohepaticveins,intohepaticveins,whichleavetheliverandemptyintothevenacava(whichleavetheliverandemptyintothevenacava(腔腔静脉静脉).).n ncausesscar
11、constituentsaccumulationcausesscarconstituentsaccumulation瘢痕成分积累瘢痕成分积累FibrosisandFibrosisandnodularnodular结节结节formationwithintheliversinusoidsbecomenarroworformationwithintheliversinusoidsbecomenarroworocclusiveocclusive闭塞的闭塞的n nObstructionofportalveinflowObstructionofportalveinflown narterovenoussh
12、untsarterovenousshunts动静脉分流动静脉分流withintheliverwithintheliverSinusoidsPortalveinCentralveinHepaticarteryPathophysiology Portal Portal hypertensionhypertensionl lSinusoidalcirrhosisSinusoidalcirrhosis肝血窦性肝硬化肝血窦性肝硬化(Truecirrhosis):Truecirrhosis):l lPresinusoidalcirrhosisPresinusoidalcirrhosis窦前性肝硬化窦前性肝
13、硬化: :portalveinobstructionbecauseitisnarroworocclusiveportalveinobstructionbecauseitisnarroworocclusive( (schistosomiasis血吸虫病)l lPostsinusoidalcirrhosisPostsinusoidalcirrhosis窦后性肝硬化窦后性肝硬化: :obstructionofveinsuchashepaticvein(obstructionofveinsuchashepaticvein(veno-occlusivedisease)Etiology and class
14、ification1 1 Presinusoidal fibrosisPresinusoidal fibrosisnschistosomiasis(血吸虫病)nidiopathic(先天性)portalfibrosis2 2 parenchymal(parenchymal(实质实质) (sinusoidal) fibrosis (true cirrhosis) (sinusoidal) fibrosis (true cirrhosis)ndrugsandtoxins:e.g.alcohol,methotrexate(甲氨蝶呤),isoniazid(异烟肼)ninfections:chronic
15、hepatitisBorC,brucellosis(布鲁氏菌病)nautoimmune自身免疫的nvascularabnormalities:e.g.right-sidedheartfailurenmetabolic/geneticdiseases:e.g.Wilsonsdiseasenbiliaryobstruction胆道梗阻nidiopathic(先天)/miscellaneous(各种各样的)3 3 postsinusoidal fibrosispostsinusoidal fibrosisnveno-occlusivedisease静脉闭塞症ChronichepatitisChron
16、ichepatitisBvirus(HBV)ChronichepatitisBvirus(HBV)andhepatitisCvirus(HCV)andhepatitisCvirus(HCV)infectionsareamongtheleadinginfectionsareamongtheleadingcausesofcirrhosisinthecausesofcirrhosisinthewesternworldandtheleadingwesternworldandtheleadingcauseinAsiaandAfrica.causeinAsiaandAfrica.InchronicHBVi
17、nfection,therateofprogressiontocirrhosisisinfluencedbythereplicative复制的activityofthevirusandwhethertherehasbeensuperinfection二重感染byhepatitisdeltavirus(HDV).Concurrent同时的liverinjuryfromothercauses(e.g.alcohol)mayalsohasten加速theonsetofcirrhosis.Alcoholicliverdisease Risk factors:Risk factors:alcoholin
18、gestion摄入genderhepatitisBorCinfectiongeneticfactorsnutritionalstatusVascular disordersnCongestiveheartfailureCongestiveheartfailure(chronicright-sidedheartfailure)(chronicright-sidedheartfailure)nHepaticveno-occlusivediseaseHepaticveno-occlusivedisease(Budd-chiarisyndrome)(Budd-chiarisyndrome)Biliar
19、yobstructionnPrimarybiliarycirrhosis原发性胆汁性肝硬化nSecondary(mechanical)biliarycirrhosisPrimarysclerosingcholangitis原发性硬化性胆管炎Neoplasm瘤ofbileducts胆管orpancreas胰腺Iatrogenic（医源性）orinflammatorybiliarystricture狭窄nCystic(胆囊的)fibrosisnBiliaryatresia(闭塞，不通)/neonatal(新生的)hepatitisnCongenital(先天的)biliarycysts(囊肿)Sc
20、histosomiasis血吸虫病HepatosplenicschistosomiasisHepatosplenicschistosomiasis肝脾型血吸虫病肝脾型血吸虫病 isaconsequenceofaisaconsequenceofafibroticreactionaroundegggranulomasfibroticreactionaroundegggranulomas虫卵肉芽虫卵肉芽肿肿 intheliver,andintheliver,andanassociatedenlargementofthespleenanassociatedenlargementofthespleen脾
21、脾(splenomegaly(splenomegaly脾脾肿肿大大).).Thismayoccurassoonas18monthsafteraheavyinfection,orasThismayoccurassoonas18monthsafteraheavyinfection,oraslongas20yearsinlightinfections.longas20yearsinlightinfections.Inourcountry,theschistosomiasisInourcountry,theschistosomiasisistheschistosomiasisjaponicum.ist
22、heschistosomiasisjaponicum.( japonicum)(japonicum)(日本的日本的) )Other miscellaneous(各种各种) diseases associated with cirrhosis:lmetabolic/geneticdisease:e.g.Wilsonsdisease(inwhichcopper铜buildupinorgan),hemochromatosis血色沉着病(Ageneticdisorderinvolvingincreasedabsorptionofiron铁),porphyria(卟啉症),lipid(脂)disorde
23、rs,aminoaciddisorderslChronicimmunehepatitislmanydrugsandtoxins:e.g.methotrexate(氨甲喋呤）,isoniazid(异烟肼),vitaminA,alpha-methyldopa左旋甲基多巴lcryptogenic(隐源性)cirrhosis:Indianchildhoodcirrhosis,polycystic(多囊)liverdiseasePathological alterations 病理病理变化变化NormalliverCirrhoticliver正常肝脏组织切片肝硬化组织切片Histological cla
24、ssificationMicronodularcirrhosis小结节性肝硬化Macronodularcirrhosis大结节性肝硬化MixedformcirrhosisClinical manifestationsLate cirrhosisLate cirrhosisSymptoms of Symptoms of cirrhosiscirrhosisEarly cirrhosisEarly cirrhosisl lThescartissuethatformsimpairstheliversabilitytofunctionThescartissuethatformsimpairstheli
25、versabilitytofunctionandobstructstheflowofbloodthroughtheportalvein.Asaresultandobstructstheflowofbloodthroughtheportalvein.Asaresultofthisobstruction,portalhypertensionofthisobstruction,portalhypertension门脉高压门脉高压occurs.Theseoccurs.Thesetwoaspectsinducetheclinicalmanifestationsofcirrhosis.twoaspects
26、inducetheclinicalmanifestationsofcirrhosis. Early cirrhosis Fatigue(tired)andweak.Fatigue(tired)andweak.Liverbeginstogrowlarger.Liverbeginstogrowlarger.Lossofappetite(Lossofappetite(食欲食欲)forfood)forfoodNausea(Nausea(恶心恶心)andvomiting()andvomiting(呕吐呕吐).).Weightloss.Weightloss.n In the early stage the
27、re 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 develop: Late cirrhosis nresultfromhepaticcelldysfunctionnresultfromportalhypertension食管静脉曲张胃底静脉曲张腹水男性乳房发育肝掌1.Clinical features result from Cl
28、inical features result from hepatic cell dysfunctionhepatic cell dysfunctionlGeneralsymptoms:Fatigueorevenexhaustion力竭WeaknessWeightlossandmusclewastingFeverItching痒AbdominalpainIncreasesensitivitytodrugsFrequentinfectionEdemalDigestivedisorders:Nausea(恶心)Lossofappetite食欲不振VomitingDiarrheaJaundicelb
29、leedingandanemia(贫血)Bruising(撞伤)easilySkinbleedingBleedingfromgums(齿龈)ornoseGastrointestinalbleeding胃肠道出血Anemia贫血lEndocrine(hormone)disorders:Lossofsexualdesire性欲.Inmen:afallintestosterone(睾丸激素)levels,reducedtesticle(睾丸)size,gynaecomastia(男子女性乳房),impotence(阳萎).Inwomen:menstrual(月经)irregularities,inc
30、reasedmenstrualpain痛经andmenopausal(更年期)symptoms.Spiderveins(蜘蛛痣)Palmarerythema(肝掌)Hyperpigmentation(色素沉着）Fluidretention液体潴留蜘蛛痣色素沉着及黄疸男性乳房发育Clinical features result from portal hypertensionPortalhypertensionisastatePortalhypertensionisastateinwhichthepressurewithininwhichthepressurewithinthehepaticpo
31、rtalveinisthehepaticportalveinisexceedingexceeding超过超过thepressurethepressureinnon-portalabdominalveinsinnon-portalabdominalveins(e.g.inferiorvenacava)byat(e.g.inferiorvenacava)byatleast5mmHgleast5mmHgItcancauseenlargementofspleenandportosystemic(Itcancauseenlargementofspleenandportosystemic(门体静脉的门体静
32、脉的)collateral()collateral(附属的附属的) )vesselsdevelopinanefforttoequalizepressurebetweenthesetwovenousvesselsdevelopinanefforttoequalizepressurebetweenthesetwovenoussystems;alteredportalhemodynamics(systems;alteredportalhemodynamics(血液动力学血液动力学)canalsoleadtothe)canalsoleadtothedevelopmentofascitesdevelop
33、mentofascites腹水腹水. .lSplenomegaly（脾大）nHepaticcirrhosisresultingincongestivesplenomegaly充血性脾肿大isacauseofhypersplenism(脾亢）.Itisatypeofdisorderwhichcausesthespleentorapidlyandprematurely过早的destroybloodcells.hypersplenismhypersplenismsplenomegalyAnemia(贫血),leukopenia(白细胞减少),thrombocytopeniacorrectionoft
34、hecytopenia细胞减少bysplenectomy(脾切除术).lVaricesl静脉曲张:1.gastroesophagealvarices胃食管静脉曲张2.AbdominalVeinvarices腹壁静脉曲张3.Hemorrhoids（痔）veinvaricesPortosystemicPortosystemic（门体静脉）（门体静脉） collateralvesselscollateralvessels侧支血管侧支血管 developinanefforttoequalizepressuredevelopinanefforttoequalizepressurebetweenthese
35、twovenoussystemsbetweenthesetwovenoussystemslAscites腹水:Occurringinadvancedcirrhosis,theaccumulationoffluidintheabdominalcavity,orascites,isrelatedtoportalhypertension,significantreductioninserumalbumin血清白蛋白,andrenalretention保留ofsodium.Pathogenesis of ascites:Ascites:SinusoidalhypertensionHypoalbumin
36、emia(低白蛋白血症)FixedcapacitytoresorbascitesIncreasedsodiumandwaterreabsorptionbytheKidneys(duetohumoralfactors体液因子)Splanchnic(内脏)arteriolarvasodilation(血管舒张)increasingsympathetictone（交感紧张）lApalpable,firmliverwithablunt（钝，硬）edgeistypical,butattimestheliverissmallanddifficulttopalpate.lRegenerating再生的nod
37、ulesareonlyoccasionallypalpable.3Examine liver by palpationcomplicationslUppergastrointestinaltractbleedinglhepaticencephalopathylinfectionslHepatorenalsyndromelHepatopulmonarysyndromelHepatocellularcarcinomalfluidandelectrolyte（电解质）disorderslPortalveinthrombosisPortalveinthrombosis血栓形成血栓形成lUppergas
38、trointestinaltractbleedingUppergastrointestinaltractbleedingmayoccurfromgastroesophagealvarices将脉曲张,portalhypertensivegastropathy,orgastroduodenalulcer胃十二指肠溃疡.Hemorrhage（出血）maybemassive,resultinginfatalexsanguination致死性大出血orportosystemicencephalopathy.门体肝性脑病lhepaticencephalopathy(脑病)n n HepaticHepat
39、ic encephalopathyencephalopathy isis aa statestate ofof disordereddisordered centralcentralnervousnervoussystemsystemfunctionfunctionresultingresultingfromfromfailurefailureofofthetheliverlivertotodetoxifydetoxify noxiousnoxious agentsagents ofof gutgut originorigin 肠肠源源性性 becausebecause ofofhepatoc
40、ellularhepatocellulardysfunctiondysfunctionandandportosystemicportosystemicshuntingshunting门门体体分分流流. . (ammonia(ammonia氨氨 isisthethemostmostreadilyreadilyidentifiedidentifiedtoxin,toxin,butbutisisnotnot solelysolely 唯唯 一一 的的 responsibleresponsible forfor thethe disturbeddisturbed mentalmentalstatus.
41、)status.)n nAA patientpatient withwith chronicchronic hepatichepatic encephalopathyencephalopathy maymaydevelopdevelopprogressiveprogressivelosslossofofmemory,memory,disorientationdisorientation定定向向障障碍碍, ,untidiness,untidiness, andand muscularmuscular tremorstremors 肌肌肉肉萎萎缩缩, , leadingleading toto a
42、aformformofofchronicchronicdementiadementia痴痴呆呆. .TheTheingestioningestion摄摄取取 ofofproteinproteininvariablyaggravatesinvariablyaggravates加重加重thesesymptoms.thesesymptoms.linfectionsCirrhosiscancauseimmunesystemdysfunction,leadingtoinfection:Spontaneousbacterialperitonitis(SBP),pneumonia,urinaryinfect
43、ionorbileductinfection.nSpontaneousbacterialperitonitis自发性细菌性腹膜炎(SBP)isanominous（不详的）complicationoflate-stageliverdisease,itisoftenheralded不宣布byAbdominalpain,increasingascites,fever.nItisbelievedtoreflectalteredgutwallpermeabilitytobacteria,impairedcapacityofhepaticandsplenicmacrophages脾巨噬细胞toclearp
44、ortalbacteremias,and/orthepresenceofalargevolumeofperitonealfluidconductivetobacterialgrowth.lHepatorenalsyndromeHepatorenalsyndrome,alsoknownasfunctionalrenalfailure,isdefinedasrenalfailureassociatedwithsevereliverdiseasewithoutanintrinsicabnormalityofthekidney.l l Hepatorenalsyndromeischaracterize
45、dbyazotemiaHepatorenalsyndromeischaracterizedbyazotemia（氮（氮质血症）质血症）,oliguria,oliguria（少尿）（少尿）,hyponatremia,hyponatremia（低血钠）（低血钠）,low,lowurinarysodiumurinarysodium（低尿钠）（低尿钠）inapatientwithend-stageliverinapatientwithend-stageliverdisease.disease.l lItsprognosisisverypoor,inpartbecauseoneoftheItsprogn
46、osisisverypoor,inpartbecauseoneofthesyndromedenotesend-stageliverdiseasesyndromedenotesend-stageliverdisease. .lHepatorenalsyndromeType1HRSischaracterizedbyrapidlyprogressivekidneyfailure,withadoublingofserumcreatinine肌酐toalevelgreaterthan226mol/L(2.5mg/dL)orahalvingofthecreatinineclearancetolesstha
47、n20mL/minoveraperiodoflessthantwoweeks.Theprognosisofindividualswithtype1HRSisparticularlygrim糟糕的,withamortalityrateexceeding50%afteronemonth.Type2HRSisslowerinonsetandprogression.Itisdefinedbyanincreaseinserumcreatininelevelto133mol/L(1.5mg/dL)oracreatinineclearanceoflessthan40mL/min,andaurinesodiu
48、m10mol/L。lHepatopulmonarysyndromeHepatopulmonaryHepatopulmonarysyndromesyndromeliverdiseasepulmonaryvasculardilationreducedarterialoxygenationn nAffectedpatientscomplainofexertional(Affectedpatientscomplainofexertional(劳累）劳累）dyspneadyspnea（呼吸困难）（呼吸困难）,withpulmonaryfunctiontestsdemonstratingnormallun
49、gvolumesbut,withpulmonaryfunctiontestsdemonstratingnormallungvolumesbutmarkedlyreduceddiffusingcapacity.markedlyreduceddiffusingcapacity.n nHypoxemiaHypoxemia（血氧不足）（血氧不足）anddyspneaareusuallyworseintheanddyspneaareusuallyworseinthestandingthanthesupinestandingthanthesupine（仰卧）（仰卧）position.Oxygencanim
50、proveposition.Oxygencanimprovesymptomsbutdoesnotreversesymptomsbutdoesnotreverse逆转逆转thedefects.thedefects.lHepatocellularcarcinomalHepatocellularcarcinomaisanepithelialtumorarisingfrommalignanttransformationofthehepatocyte.Itisobservedcharacteristicallyasacomplicationofcirrhosis.especiallyrelatedtoc
51、hronicviralinfections(HBVorHCV).nItpresentsaspersistingabdominalpain,apalpableabdominalmass,and/orconstitutionalsymptomsinpatientswithcirrhosis.nThediagnosismustbeconsideredincirrhosiswithsuddendecompensation失代偿orinthosewhodevelopbloodyascites.ldisordersoffluid,electrolyteandacidbaseregulationLowing
52、estionofsodiumforalongtimeLosssodiumbydiuretics(利尿剂)andparacentesis(放液)Elevatedantidiuretichormone抗利尿激素toconservebodywaterHyponatremiaHyponatremia( (低钠血症低钠血症) )HypokalemiaHypokalemia( (低钾血症低钾血症) )metabolicalkalosismetabolicalkalosis( (碱中毒碱中毒) )InadequateintakeandvomitingordiarrheaRenallosses:drugs(d
53、iuretics),hyperaldosteronism(高醛固酮血症)Hypokalemiamayinducemetabolicalkalosis代谢性碱中毒orencephalopathyClotting凝结orobstructionofbloodflowalongtheveinsfromtheintestinesandspleenandintotheliver.Thiscausesbloodtobackupandresultsvariousproblemssuchasenlargedspleenandabdominalpain.Theobstructioncanoccuracutely(
54、overashortspaceoftime)orchronically(overalongerperiodoftime)lPortalveinthrombosislEnlargedspleenlEnlargedabdomenlAbdominalpainlDiarrhealNausealBloodinstoollVomitinglPortalhypertensionsymptomsaformofvenousthrombosisaffectingthehepaticportalvein,whichcanleadtoportalhypertensionandreductioninthebloodsu
55、pplytotheliver.LaboratoryfindingslLaboratoryfindingsandotherexaminationsAnemia,causesincludesuppressionoferythropoiesis红细胞生成byalcoholaswellasfolate叶酸deficiency,hemolysis溶血,hypersplenism脾亢,andinsidious潜在的orovert明显的bloodlossfromthegastrointestinaltract.Whitebloodcellcountmaybelow,elevated,ornormal,ref
56、lectinghypersplenismorinfection.Thrombocytopenia(血小板减少症)maybesecondarytoalcoholicmarrowsuppression酒精性骨髓抑制,sepsis败血症,folatedeficiency,orsplenicsequestration脾隔离症.blood cell tests:Urinalysis 尿分析尿分析:lInpatientswithcirrhosis,bilirubin(胆红素)andurobilinogen(尿胆原)maybemeasuredduringaurinetestwhenjaundiceispre
57、sent.sometimes,protein,casts管型orbloodcellcouldbefound.Liver function test:Modest一定程度的elevationsofAST,ALT,alkalinephosphatase(碱性磷酸酶)andprogressiveelevationofthebilirubin.Serumalbumin(白蛋白)islow,gammaglobulinisincreased.Prolongation延长oftheprothrombintime(凝血时间)mayresultfromfailureofsynthesis合成ofclotting
58、constituents凝血成分bytheliver.Immune function test:Tcellcountdecreasesinmorethan50%ofpatientswithcirrhosis,CD3,CD4andCD8cellsareallreduced.SerumIgGandIgAlevelsareelevated,especiallyforIgG,coinciding一致withtheincreasinglevelofgammaglobulinInsomepatients,theantinuclearantibodycouldbedetectedinserum.Inthos
59、epatientswhosepathogene病原体areHBV,HCV,orHDVsuperimposed叠加的onHBV,theviraldetectionmaybepositive.Ascites test:Allpatientswithnew-onsetascitesorthoserequiringhospitalizationAllpatientswithnew-onsetascitesorthoserequiringhospitalization住院治住院治疗疗shouldundergodiagnosticparacentesisshouldundergodiagnosticpar
60、acentesis诊断性穿刺诊断性穿刺withcellcount,withcellcount,ascitesalbuminascitesalbumin白蛋白白蛋白determination,Gramstain,andculture.Directlydetermination,Gramstain,andculture.Directlyinoculatinginoculating注射注射ascitesintobloodculturebrothascitesintobloodculturebroth肉汤肉汤atthebedsideisatthebedsideisessentialifinfected
61、ascitesisdoubted.essentialifinfectedascitesisdoubted.diagnosisdiagnosisRivaltRivaltatestatestBiochemicBiochemicalalanalysisanalysisWhitebloodWhitebloodcellcount(percellcount(permmmm3)3)Serum/ascitesSerum/ascitesalbuminalbumingradientgradient梯度梯度 (g/dL)(g/dL)CytologyCytologyCirrhosisCirrhosis(clear)(
62、clear)-ProteinProteinusuallyusually2.5g/dL2.5g/dL250PMN1.11.1-combinedwithcombinedwithSBPSBP(turbid)(turbid)+Albumin1Albumin250PMN250PMN1.11.1-PeritonealPeritonealcarcinomatosiscarcinomatosis腹膜癌扩散腹膜癌扩散 (maybebloody)(maybebloody)+ProteinProteinusuallyusually2.5g/dL2.5g/dLoften500often500PMNPMN1.11.1n
63、eoplasneoplasmcellmcellmaybemaybedetecteddetectedDiagnosis value of ascitesImaging:Barium(钡)studiesoftheuppergastrointestinaltractmayrevealthepresenceofesophageal食管的orgastricvarices静脉曲张,thoughendoscopy(内窥镜)ismoresensitive.UltrasoundorCTishelpfulforassessingliverorsplenicsize,detectingascitesorhepati
64、cnodules,includingsmallhepatocellularcarcinomas.TogetherwithDopplerstudies,ultrasoundisusedtoevaluatepatency开放ofthesplenic,portal,andhepaticveins.Hepaticnodulesmaybecharacterizedfurtherbyintravenouscontrast-enhancedspiralCTscan静脉增强螺旋CTorMRIalongwithserumAFPlevels.Endoscopic test 内窥镜检查:Endoscopictest
65、demonstratesthepresenceofvaricesanddetectsspecificcausesofbleedingintheesophagus食管,stomach,andproximalduodenum十二指肠近端.Liver biopsy:Liverbiopsyremainstobethegoldstandardfordocumenting（用文件）证明cirrhosis,identifyingthecause,andassessingtheextent程度ofscarformation.Laparoscopy(腹腔镜腹腔镜):Insomecenters,liverbiop
66、syisperformedbylaparoscopy,whichmaybehelpfulinjudgingthetypeofcirrhosis.diagnosis:l lPhysicalexamfindthatliverfeelshardandtubercularPhysicalexamfindthatliverfeelshardandtubercular（结节）（结节）l ltheliverfunctiontestandimagingprovidepositivefindingtheliverfunctiontestandimagingprovidepositivefindingl lliv
67、erbiopsyshowsscarorothersignsofcirrhosisliverbiopsyshowsscarorothersignsofcirrhosisl lamedicalhistoryofchronicliverinfections(especiallyHBVinfection),amedicalhistoryofchronicliverinfections(especiallyHBVinfection),Long-termalcoholismorliverinjuryfromprescriptionmedicationsLong-termalcoholismorliveri
68、njuryfromprescriptionmedications处方药处方药l lclinicalmanifestationsofhepaticdysfunctionandportalhypertensionclinicalmanifestationsofhepaticdysfunctionandportalhypertensionDifferential diagnosis:lDistinguishwiththosepatientswithDistinguishwiththosepatientswithsplenomegalysplenomegaly脾肿大脾肿大lDistinguishwit
69、hthosepatientswithascitesDistinguishwiththosepatientswithascitesordistendedabdomenordistendedabdomenlDistinguishwiththosepatientswithDistinguishwiththosepatientswithsymptomsoccurringlikethecomplicationssymptomsoccurringlikethecomplicationsofcirrhosis:ofcirrhosis:(uppergastrointestinalbleeding,(upper
70、gastrointestinalbleeding,encephalopathy,renalfailure,etal.)encephalopathy,renalfailure,etal.)DiagnosisEtiologyComplicationsprognosisevaluationprognosisltheprognosisofcirrhosisisrelatedtocause,theprognosisofcirrhosisisrelatedtocause,histologytype,hepaticfunctionandcomplications.histologytype,hepaticf
71、unctionandcomplications.lIntheclinicalsettings,prognosisisbestIntheclinicalsettings,prognosisisbestdeterminedbythePughmodificationdeterminedbythePughmodification修正修正oftheoftheChild-Turcotteclassification,whichincludestheChild-Turcotteclassification,whichincludesthevariablesofascites,encephalopathy,s
72、erumvariablesofascites,encephalopathy,serumalbumin,serumbilirubin(albumin,serumbilirubin(胆红素胆红素),andprothrombin),andprothrombin( (凝血素凝血素)time.)time.(AgradeisbestwhileCgradeispoor)(AgradeisbestwhileCgradeispoor)Measure1 point2 points3 pointsunitsBilirubin (total)34(50(3)mol/l(mg/dL)Serum albumin3528-
73、3528mg/LINR 国际化标准比值（or PT prolongation)2.20(18)no unit(seconds)AscitesNoneSuppressedwithmedicationRefractoryno unitHepaticencephalopathyNoneGradeI-II(orsuppressedwithmedication)GradeIII-IV(orrefractory)no unitChild-Pughclassification82%C12-1530%B9-1110%A5-8Perioperativemortality围手术期死亡率andprognosisCl
74、assPointspoorbest/LiverdamagefromcirrhosiscannotbeLiverdamagefromcirrhosiscannotbereversed(reversed(逆转逆转),buttreatmentcanstopor),buttreatmentcanstopordelayfurtherprogressionandreducedelayfurtherprogressionandreducecomplications(complications(并发症并发症).).TreatmentdependsonthecauseofTreatmentdependsonth
75、ecauseofcirrhosisandanycomplicationsapersoncirrhosisandanycomplicationsapersonisexperiencing.isexperiencing.Treatment:Generalmeasures:l一. far from injury factors e.g.abstaining(e.g.abstaining(禁绝禁绝)fromalcoholordrugsharmfultoliver)fromalcoholordrugsharmfultolivermedicationsusedtotreatthedifferenttype
76、sofhepatitismedicationsusedtotreatthedifferenttypesofhepatitis肝炎肝炎,suchaslamivudine(,suchaslamivudine(拉米夫定拉米夫定)orentecavir)orentecavir（恩（恩替卡韦）替卡韦）tabletforviralhepatitisandcorticosteroids(tabletforviralhepatitisandcorticosteroids(皮皮质类固醇质类固醇)forautoimmune()forautoimmune(自身免疫自身免疫)hepatitis)hepatitisCi
77、rrhosiscausedbyWilsonsdisease,inwhichcopperCirrhosiscausedbyWilsonsdisease,inwhichcopper铜铜buildsupinorgans,istreatedwithmedicationstobuildsupinorgans,istreatedwithmedicationstoremovethecopperremovethecopperl二二. Relax and an adequate bed rest. Relax and an adequate bed restl 三. dietthedietshouldbepal
78、atable(thedietshouldbepalatable(美味美味),with),withadequatecalories(adequatecalories(卡路里卡路里)andprotein(75-)andprotein(75-100g/day)and,ifthereisfluidretention100g/day)and,ifthereisfluidretention液体潴留液体潴留, ,sodium(sodium(钠）钠）restriction(restriction(限制限制).).Inthepresenceofhepaticencephalopathy,Inthepresenc
79、eofhepaticencephalopathy,proteinintakeshouldbereducedto60-proteinintakeshouldbereducedto60-80g/day.vitaminsupplementationisdesirable80g/day.vitaminsupplementationisdesirable满满足需要的足需要的. .Noalcoholanddrugsharmfultoliver,hardorNoalcoholanddrugsharmfultoliver,hardorcoarsecoarse（粗糙）（粗糙） foodareavoided(fo
80、odareavoided(避免避免). ).lTherearenodrugstoreverse逆转cirrhosiswhichhavetheespeciallygoodeffect,vitaminsordigestiveenzymemaybeprescribed规定/开药.somechineseherbal（草药）medicinetreatmentmayimprovetheliverfunctionandsymptoms.l lForthosepatientswithadvancedcirrhosis,supportiveForthosepatientswithadvancedcirrhosi
81、s,supportivetreatmentisneededbytransfusiontreatmentisneededbytransfusion（输液）（输液）,andthebalance,andthebalanceoffluidandelectrolyte(offluidandelectrolyte(电解质电解质)shouldbemaintained.)shouldbemaintained.l 四. drugAscites and edemal lBasically:Dietarysodiumrestriction(400-Basically:Dietarysodiumrestriction
82、(400-800mg/day)800mg/day)restrictionoffluidintake(800-1000ml/day)isrestrictionoffluidintake(800-1000ml/day)isrequiredespeciallyforpatientswithhyponatremiarequiredespeciallyforpatientswithhyponatremia低低钠血症（低血钠）钠血症（低血钠）(500ml/day).(500ml/day).l lDiureticsDiuretics（利尿剂）（利尿剂）: :spironolactone(100mg-400m
83、g/day)spironolactone(100mg-400mg/day)（氨体舒通）（氨体舒通）furosemide(40-160mg/day)furosemide(40-160mg/day)（速尿）（速尿）The goal of weight loss should be no more than 0.5-0.7 kg/dayl Large-volume paracentesis （穿刺放液）（穿刺放液）Inpatientswithmassiveascitesandrespiratory(呼吸）compromise让步,ascitesrefractory（难控制）todiuretics利尿
84、剂,orintolerablediureticsideeffects,large-volumeparacentesis(4-6L)iseffectivewhenthisisdone,itisoftenthepractice实践togiveintravenous(静脉内的)albuminconcomitantly（伴随）atadosageof10g/Lofascitesfluidremovedtoprotecttheintravascularvolume血管内容积.l increase plasma(血浆血浆) colloid（胶体）（胶体）（胶体）（胶体） osmotic （渗透）（渗透）（渗
85、透）（渗透）pressure（压）（压）（压）（压）InfusionsofalbuminorbloodmaybeusedtoincreaseInfusionsofalbuminorbloodmaybeusedtoincreasetheplasmacolloidosmoticpressureandcouldbehelpfultheplasmacolloidosmoticpressureandcouldbehelpfultoimprovetheliverfunctionanddiminishtoimprovetheliverfunctionanddiminish减少减少thetheascites.
86、ascites.l continuous ultrafiltration（超滤） and reinfusion of protein concentrate 浓缩蛋白thistechniqueisanefficientandinexpensivemethodthistechniqueisanefficientandinexpensivemethodoftreatmentofresistantascitesoftreatmentofresistantascites（顽固性腹水）（顽固性腹水）,it,itcouldincreasethealbuminconcentrationsinplasmaco
87、uldincreasethealbuminconcentrationsinplasmaandtheeffectivebloodvolume,improverenalandtheeffectivebloodvolume,improverenalcirculation,resultinginascitesdiminutioncirculation,resultinginascitesdiminution（减少）（减少）.It.Itshouldbeforbiddeninpatientswithinfections.shouldbeforbiddeninpatientswithinfections.l
88、 Peritoneovenous Le Veen shuntslThistechniqueisdesignedtoprovideonewayflowofsterile(消毒)asciticfluid腹水fromtheabdomentothevascularsystem.( (腹腔静脉分流术腹腔静脉分流术腹腔静脉分流术腹腔静脉分流术) )lTransjugular intrahepatic portosystemic shunt (TIPS)Transjugular intrahepatic portosystemic shunt (TIPS)( (经颈静脉肝内门体静脉分流术经颈静脉肝内门体静脉
89、分流术)TIPSisaprocedurethatmaybeusedtoreduceportalhypertensionanditsTIPSisaprocedurethatmaybeusedtoreduceportalhypertensionanditscomplications.ATIPSproceduremaybeperformedbyaradiologistcomplications.ATIPSproceduremaybeperformedbyaradiologist放射科医师放射科医师, ,whoplacesasmallwire-mesh(whoplacesasmallwire-mesh
90、(金属丝网金属丝网)coil()coil(盘盘, ,卷卷)(stent)intoalivervein()(stent)intoalivervein(静脉静脉)whereitisexpandedusingasmallinflatable()whereitisexpandedusingasmallinflatable(膨胀膨胀)balloon()balloon(气球气球).Thestent).Thestent支架支架 formsachannel,orshuntformsachannel,orshunt分流分流,thatbypasses,thatbypasses绕过绕过theliver.Thisch
91、annelreducestheliver.Thischannelreducespressureintheportalvein.pressureintheportalvein.Complicationsl l Hemorrhage( Hemorrhage(出血出血出血出血) from esophageal () from esophageal (食管的食管的食管的食管的) varices:) varices:Acuteresuscitation(Acuteresuscitation(复生复生) )Emergentendoscopy(Emergentendoscopy(内窥镜检查内窥镜检查) )P
92、harmacologictherapyPharmacologictherapyBalloontubeBalloontube球囊扩展导管球囊扩展导管tamponadetamponade( (填塞填塞) )Portaldecompressive(Portaldecompressive(减压减压)procedure)procedureAcute resuscitation( (复生复生) )l lPatientsareneededtotransferredtoanPatientsareneededtotransferredtoanICUwhereconstantmonitoring(ICUwhere
93、constantmonitoring(监测监测)could)couldbeprovided.Nofood,restinbedandbeprovided.Nofood,restinbedandoxygenshouldbegiven.oxygenshouldbegiven.l lrapidassessmentandresuscitationrapidassessmentandresuscitation复苏复苏 withfluidsorbloodproductsareessential.withfluidsorbloodproductsareessential.l lNasogastric(Naso
94、gastric(鼻饲鼻饲)tubeshouldbeplaced)tubeshouldbeplacedtoevacuatetoevacuate疏散疏散/ /排泄排泄thestomachandtothestomachandtomonitorforongoingbleeding.monitorforongoingbleeding.Emergent endoscopyBandingBanding(varicealligation)(varicealligation)( (套扎套扎) )SclerotherapySclerotherapy(Sclerosingagent)(Sclerosingagent
95、)( (硬化剂硬化剂) )l Pharmacologic therapySomatostatin(Somatostatin(生长抑素生长抑素) )Vasopressin(Vasopressin(垂体后叶素垂体后叶素) )H2-receptorantagonistsandprotonpumpH2-receptorantagonistsandprotonpumpinhibitorsinhibitors质子泵抑制剂质子泵抑制剂VitaminKVitaminKAntibioticprophylaxis(Antibioticprophylaxis(预防预防) )Propranolol(Propranol
96、ol(心得安心得安) )isosorbidemononitrate(isosorbidemononitrate(硝酸异山梨醇酯硝酸异山梨醇酯) )lBalloontubetamponade(填塞)Mechanicaltamponade(压塞)withspeciallydesignednasogastrictubes鼻胃管containinglargegastricandesophagealballoonsprovidesinitialcontrolofactivevaricealhemorrhage活动性静脉曲张出血in60-90%ofpatients.Portal decompressive
97、 procedureTIPS:itcancontrolacutehemorrhageinover90%ofpatientsactivelybleedingfromgastricoresophagealvarices.Emergencyportosystemicshuntsurgery紧急门静脉分流手术l hepatic encephalopathyThegoalsoftreatmentincludelifesupport,Thegoalsoftreatmentincludelifesupport,eliminationelimination消除消除ortreatmentofprecipitat
98、ingortreatmentofprecipitatingfactorsfactors诱发因素诱发因素,andremovalor,andremovalorneutralization(neutralization(中和中和)ofammonia()ofammonia(氨氨)and)andothertoxins.othertoxins.Precipitatingfactorsmustbeidentifiedandtreated:GastrointestinalPrecipitatingfactorsmustbeidentifiedandtreated:Gastrointestinalbleedin
99、gmustbestopped.Theintestinesmustbeemptiedofblood.bleedingmustbestopped.Theintestinesmustbeemptiedofblood.BloodbreaksdownintoproteincomponentsBloodbreaksdownintoproteincomponents组分组分thatareconvertedthatareconvertedtoammonia.Treatmentofinfections,renalfailure,andelectrolytetoammonia.Treatmentofinfecti
100、ons,renalfailure,andelectrolyteabnormalitiesabnormalities电解质异常电解质异常(especially(especiallyHypokalemiaandmetabolicHypokalemiaandmetabolicalkalosis)alkalosis)isimportant.isimportant.lSpontaneous bacterial peritonitis (SBP)Intravenousantibiotictherapy(Intravenousantibiotictherapy(抗生素疗法抗生素疗法)shouldbe)sho
101、uldbecarriedfornotlessthan2weeks.(early,adequateandcarriedfornotlessthan2weeks.(early,adequateandcombination)combination)supplementalsupplementaladministrationofadministrationofintravenousalbuminintravenousalbuminmayreducemortality.mayreducemortality.CulturesofascitesgivesthehighestyieldCulturesofas
102、citesgivesthehighestyield收益收益ofof80-90%positivewhenbloodculturebottlesare80-90%positivewhenbloodculturebottlesareinoculatedinoculated接种接种atthebedside.Commonisolatesatthebedside.Commonisolates隔离群隔离群areEcoli(areEcoli(大肠杆菌）大肠杆菌） andpneumococciandpneumococci（肺炎球菌）（肺炎球菌）,butanaerobes,butanaerobes（厌氧菌）（厌氧
103、菌） arerare.arerare.l Hepatorenal syndromeTreatmentisgenerallyineffective.Treatmentisgenerallyineffective.themosteffectivetreatmentistocorrecttheunderlyingliverthemosteffectivetreatmentistocorrecttheunderlyingliverdisease,bylivertransplantation.disease,bylivertransplantation.Treatinganyunderlyinginfe
104、ctionsandoptimizingTreatinganyunderlyinginfectionsandoptimizing优化优化volumevolumestatusareimportantadjunctivestatusareimportantadjunctive（附属）（附属）measures.measures.Potentiallynephrotoxic(Potentiallynephrotoxic(对肾脏有害的对肾脏有害的)drugs,especially)drugs,especiallyNSAIDSNSAIDS（非甾体类抗炎药）（非甾体类抗炎药）,shouldbewithdraw
105、n.,shouldbewithdrawn.TIPSTIPSHepatorenal 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）LiverplantationLivertransplantationisusuallydonewhenothermedicaltreatmentLivertransplantationisusuallydonewhenothermedicaltreatmentcannotk
106、eepadamagedliverfunctioning.Itisindicatedinselectedcannotkeepadamagedliverfunctioning.Itisindicatedinselectedcasesofirreversiblecasesofirreversible不可逆的不可逆的,progressivechronicliverdisease(e.g.,progressivechronicliverdisease(e.g.advancedcirrhosiswithseverecomplications),fulminantadvancedcirrhosiswiths
107、everecomplications),fulminant（爆发性）（爆发性） hepaticfailure,andcertainmetabolicdiseasesinwhichthemetabolichepaticfailure,andcertainmetabolicdiseasesinwhichthemetabolicdefectdefect代谢缺陷代谢缺陷isintheliver.isintheliver.ComplicationofHepaticCirrhosisare_，_，_，_，_，_，_。HowtoexplaintheHepaticCirrhosis?Whatarethemos
108、tcommoncausesofcirrhosisinourcountryandinwesterncountry?Whataretheclinicalmanifestationsoflatecirrhosis?HepatorenalsyndromeischaracterizedbyHepatorenalsyndromeischaracterizedby_，_，_，_.HowtoexplaintheHepatopulmonarysyndrome?HowtojudgeapatientiswithSBP?HowtoexplaintheHepatopulmonarysyndrome?HowtojudgeapatientiswithSBP?TheclinicalmanifestationsofPortalhypertensioninclude_，_，_.Whatarethepathogenesisofascitesofcirrhosis?Howtotreatthecirrhosispatientswithascitesorgastroesophagealvaricesbleeding?Howtodiagnosethehepaticcirrhosiscorrectly?
