英文 肝硬化

HepaticCirrhosisCirrhosis---definitionchronic,progressed,diffusehepatocellularinjuryfibrosisnodularregenerationIncidence:17/100000/yAge:20-50yr.Hepaticcirrhosis》EtiologyLiverfunctionInjury,PortalhypertensionDiffuse,chronicliverinjuryHepato-cellularnecrosis,collapseofhepaticlobulesregenerativenodulesformationFormationofdiffusefibrousseptaComplations:UpperGIBleeding,Hepaticcoma,infections,primarylivercancer,Functionalrenalfailure1.Chronicviralhepatitis(慢性病毒性肝炎):[HBV,HCV,HBV+HDV]2.Long-termalcoholism(慢性酒精中毒)[80g/d,10yr.]3.Prolongedcholestasis(长期胆汁郁积),intra-andextra-hepatic:[primarybiliarycirrhosis,PBC]/[secondarybiliarycirrhosis]4.Drugsandtoxins(药物和毒物)[toxichepatitis---chronicactivehepatitis---cirrhosis]5.Nonalcoholicsteatohepatitis(NASH)(非酒精性脂肪性肝炎)Etiologyofcirrhosis(I)Etiologyofcirrhosis(II)6.Hepaticvenousoutflowobstruction(肝血液循环障碍)veno-occlusivedisease,Budd-Chiarisyndrome,constrictivepericarditis7.Metabolicdisorders(遗传代谢性疾病)hemochromatosis(血色病);Wilson‘sdisease(肝豆状核变性);8.Autoimmunehepatitis(AIH)(自身免疫性肝炎)9.Schistosomiasis(血吸虫病)10.Cryptogenic(隐原性)11.Mixed:alcohol+virus,HBV+HCV,HBV+schistosomiasisHepaticstellatecellactivationLiverfibrosisaccumulationofextracellularmatrixinliversynthesisofmatrixproteinsdegradationofmatrixproteinsCollagenstypeIandIIIconstitutemorethan95%ofthetotalcontentofincreasedcollageninfibroticliverPathogenesis:chronic,progressed,diffuse•Hepatocyteinjuryleadingtonecrosis.•Chronicinflammation-(hepatitis).•Capillarization(肝窦毛细血管化)ofthespaceofDisseisakeyevent.•Bridgingfibrosis.•Regenerationofremaininghepatocytesproliferateasroundnodulessurroundedbyfibroussepta.•Lossofvasculararrangementresultsinregeneratinghepatocytesineffective.•Cirrhosismayleadtoliverfailure,portalhypertension,ordevelopmentofhepatocellularcarcinomaHistopathologicclassification•micronodularuniformlysmallnodules(3mmindiameter)andregularbandsofconnectivetissue•macronodularnodulesthatvaryinsize(3mmto5cmindiameter)•mixedmacroandmicronodular(incompleteseptalcirrhosis)combineselementsofmicronodularandmacronodularcirrhosis.Consequencesofportalhypertension[I]1.Splenomegaly(脾肿大)2.Formationandopenofportal-systemiccollateral’s(门体侧支循环开放)--Esophageal/gastricvarices(食管/胃静脉曲张)(shortgastric/coronaryveins)--Rectalcollateral‘s(痔静脉丛)(Suphemorrhoidal/middle&inf.hemorrhoidal)--Caputmedusae(水母头)(umbilical/epigastric)--abdominalwallvarices(腹壁静脉曲张)--PortalsystemandleftrenalConsequencesofportalhypertension[II]3.Ascites(腹水)Theoriesofascitesformation•Underfillingtheory(灌注不足假说）•Overflowtheory(泛溢假说)•Arterialvasodilationtheory(动脉扩张假说)Ascites•Sodiumretention---Reninangiotensionaldosteronesystem(RAAS)---sympatheticnervesystem,norepinephrine---Intrarenalfactors:Kallikrein-kininsystem,Adenosine.•Waterretention---Antidiuretichormone(ADH)---ImpairedrenalsynthesisofPGs(PGE2)•Renalvasoconstriction---RAAS,AngiotensionII---SNS---ADH---ETEndocrinesystemgynecomastia(男性乳房发育),telangiectases(毛细血管扩张症),spidernevi(蜘蛛痣),palmarerythema(肝掌)testicularatrophy(睾丸萎缩)menstrualirregularities(月经失调)PulmonarymanifestationsHepatichydrothorax(肝性胸水)Hepatopulmonarysyndrome(HPS,肝肺综合征)HRSischaracterizedclinicallybythetriadofpulmonaryvasculardilatationcausingarterialhypoxemiainthesettingofadvancedliverdisease.HRS(Hepatorenalsyndrome,肝肾综合征)Occurredinthesettingof:---chronicliverdisease---advancedhepaticfailure---portalhypertensioncharacterizedby:---impairedrenalfunction---markedabnormalitiesinarterialcirculation---activationofendogenousvasoactivesystemClassifiedinto2differenttypes:---TypeI:Rapidlyprogressive---TypeII:Notrapidlyprogressive.OftenresultsinmildrenalinsufficiencycausingdiureticresistantascitesMechanismsofHRS[II]Clinicalfeatures[I]Compensatedcirrhosis(代偿期)Manypeopleexperiencefewsymptomsattheonsetofcirrhosis,symptomsaretypicallyvagueandnonspecific.---Fatigueandlossofenergy.---Lossofappetiteandnausea.---Spiderangiomas---liverfunctionisnormalDecompensatedcirrhosis(失代偿)Symptomscausedbylossoffunctioninglivercells---System：fatigue,weakness,weightloss,malnutrition---DigestiveSystem:Lossofappetite,nausea,diarrhea.Clinicalfeatures[II]---Tendencytohemorrhage(出血倾向)andanaemia(贫血):Duetoreducedsynthesisofcoagulationfactors(II,V,VII,IX,X),hypersplenism(脾亢),lowplateletcount,poorabsorption，gastrointestinalbleeding.---Hormonalabnormalitiesgynecomastia(男性乳房发育),telangiectases(毛细血管扩张症),spidernevi(蜘蛛痣),palmarerythema(肝掌)---Jaundice(黄疸)Clinicalfeatures[III]Portalhypertension(门静脉高压)1.Splenomegaly:anemia,leukopenia,thrombocytopeniaduetohypersplenism2.developmentandopenofcollateralvesselsinportalhypertensiona.Esophagealvaricesb.Rectalcollateral'sc.Caputmedusaed.Abdominalwallvaricese.Portalsystemandleftrenal3.Ascites、hepatichydrothoraxClinicalfeatures[IV]Palpationofliverfirm,hard,irregular,enlargementroundedorsharpedgebelowtherightlowerribs.Thespleenisoftenpalpable,andmaybeverylarge.Theclinicalmanifestationsfoundincirrhosis、Complications[I]Uppergastrointestinalbleeding(上消化道出血):Hematemesis(呕血)/melena(黑粪).Esophageal/gastricvaricealbleeding(食管/胃静脉出血);portalhypertensivegastropathy(门脉高压性胃病);pepti