肝硬化英文課件

HepaticCirrhosis肝硬化英文Cirrhosis---definition

chronic,progressed,diffuse

hepatocellularinjury

fibrosis

nodularregenerationIncidence:17/100000/yAge:20-50yr.肝硬化英文Hepaticcirrhosis》EtiologyLiverfunctionInjury,PortalhypertensionDiffuse,chronicliverinjuryHepato-cellularnecrosis,collapseofhepaticlobulesregenerativenodules

formationFormationofdiffusefibrousseptaComplations:UpperGIBleeding,Hepaticcoma,infections,primarylivercancer,Functionalrenalfailure肝硬化英文1.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(長(zhǎng)期膽汁郁積),intra-andextra-hepatic:[primarybiliarycirrhosis,PBC]/[secondarybiliarycirrhosis]4.Drugsandtoxins(藥物和毒物)[toxichepatitis---chronicactivehepatitis---cirrhosis]5.Nonalcoholicsteatohepatitis(NASH)(非酒精性脂肪性肝炎)Etiologyofcirrhosis(I)肝硬化英文Etiologyofcirrhosis(II)6.Hepaticvenousoutflowobstruction(肝血液循環(huán)障礙)

veno-occlusivedisease,Budd-Chiarisyndrome,constrictivepericarditis7.Metabolicdisorders

(遺傳代謝性疾病)

hemochromatosis(血色病);Wilson‘sdisease(肝豆?fàn)詈俗冃?;8.Autoimmunehepatitis(AIH)(自身免疫性肝炎)9.Schistosomiasis(血吸蟲(chóng)病)10.Cryptogenic

(隱原性)11.Mixed:alcohol+virus,HBV+HCV,HBV+schistosomiasis肝硬化英文Hepaticstellatecellactivation肝硬化英文Liverfibrosisaccumulationofextracellularmatrixinliver

synthesisofmatrixproteinsdegradationofmatrixproteinsCollagenstypeIandIIIconstitutemorethan95%ofthetotalcontentofincreasedcollageninfibroticliver

肝硬化英文肝硬化英文肝硬化英文Pathogenesis:chronic,progressed,diffuseHepatocyteinjuryleadingtonecrosis.Chronicinflammation-(hepatitis).Capillarization(肝竇毛細(xì)血管化)ofthespaceofDisseisakeyevent.Bridgingfibrosis.Regenerationofremaininghepatocytesproliferateasroundnodulessurroundedbyfibroussepta.Lossofvasculararrangementresultsinregeneratinghepatocytesineffective.Cirrhosismayleadtoliverfailure,portalhypertension,ordevelopmentofhepatocellularcarcinoma

肝硬化英文Histopathologic

classificationmicronodular

uniformlysmallnodules(<3mmindiameter)andregularbandsofconnectivetissuemacronodular

nodulesthatvaryinsize(3mmto5cmindiameter)mixedmacroandmicronodular

(incompleteseptalcirrhosis)combineselementsofmicronodularandmacronodularcirrhosis.

肝硬化英文肝硬化英文肝硬化英文Consequencesofportalhypertension[I]1.Splenomegaly(脾腫大)2.Formationandopenofportal-systemiccollateral’s(門(mén)體側(cè)支循環(huán)開(kāi)放)

--Esophageal/gastricvarices

(食管/胃靜脈曲張)

(shortgastric/coronaryveins)

--Rectalcollateral‘s(痔靜脈叢)

(Suphemorrhoidal/middle&inf.hemorrhoidal)

--Caputmedusae(水母頭)(

umbilical/epigastric)

--abdominalwallvarices(腹壁靜脈曲張)

--Portalsystemandleftrenal肝硬化英文肝硬化英文Consequencesofportalhypertension[II]3.Ascites(腹水)

Theoriesofascitesformation

Underfillingtheory(灌注不足假說(shuō)）

Overflowtheory(泛溢假說(shuō))Arterialvasodilationtheory(動(dòng)脈擴(kuò)張假說(shuō))肝硬化英文肝硬化英文AscitesSodiumretention

---Reninangiotensionaldosteronesystem(RAAS)

---sympatheticnervesystem,norepinephrine---Intrarenalfactors:Kallikrein-kininsystem,Adenosine.Waterretention

---Antidiuretichormone(ADH)

---ImpairedrenalsynthesisofPGs(PGE2)Renalvasoconstriction

---RAAS,AngiotensionII

---SNS---ADH---ET肝硬化英文Endocrinesystem

gynecomastia(男性乳房發(fā)育),telangiectases(毛細(xì)血管擴(kuò)張癥),spidernevi(蜘蛛痣),palmarerythema(肝掌)

testicularatrophy(睪丸萎縮)menstrualirregularities(月經(jīng)失調(diào))肝硬化英文Pulmonarymanifestations

Hepatichydrothorax

(肝性胸水)

Hepatopulmonarysyndrome(HPS,肝肺綜合征)HRSischaracterizedclinicallybythetriadofpulmonaryvasculardilatationcausingarterialhypoxemiainthesettingofadvancedliverdisease.

肝硬化英文HRS(Hepatorenalsyndrome,肝腎綜合征)

Occurredinthesettingof:---chronicliverdisease---advancedhepaticfailure---portalhypertension

characterizedby:

---impairedrenalfunction---markedabnormalitiesinarterialcirculation---activationofendogenousvasoactivesystem

Classifiedinto2differenttypes:

---TypeI:Rapidlyprogressive---TypeII:Notrapidlyprogressive.Oftenresultsinmildrenalinsufficiencycausingdiureticresistantascites肝硬化英文MechanismsofHRS[II]肝硬化英文Clinicalfeatures[I]Compensatedcirrhosis(代償期)

Manypeopleexperiencefewsymptomsattheonsetofcirrhosis,symptomsaretypicallyvagueandnonspecific.

---Fatigueandlossofenergy.---Lossofappetiteandnausea.---Spiderangiomas---liverfunctionisnormalDecompensatedcirrhosis(失代償)

Symptomscausedbylossoffunctioninglivercells

---System：fatigue,weakness,weightloss,malnutrition---DigestiveSystem:Lossofappetite,nausea,diarrhea.肝硬化英文Clinicalfeatures[II]

---Tendencytohemorrhage(出血傾向)andanaemia(貧血):Duetoreducedsynthesisofcoagulationfactors(II,V,VII,IX,X),hypersplenism(脾亢),lowplateletcount,poorabsorption，gastrointestinalbleeding.---Hormonalabnormalities

gynecomastia(男性乳房發(fā)育),telangiectases(毛細(xì)血管擴(kuò)張癥),spidernevi(蜘蛛痣),palmarerythema(肝掌)

---Jaundice(黃疸)肝硬化英文Clinicalfeatures[III]Portalhypertension(門(mén)靜脈高壓)1.Splenomegaly:anemia,leukopenia,thrombocytopeniaduetohypersplenism

2.developmentandopenofcollateralvesselsinportalhypertension

a.

Esophagealvarices

b.Rectalcollateral's

c.Caputmedusae

d.Abdominalwallvarices

e.Portalsystemandleftrenal

3.Ascites、hepatichydrothorax

肝硬化英文肝硬化英文肝硬化英文Clinicalfeatures[IV]

Palpationofliverfirm,hard,irregular,enlargementroundedorsharpedgebelowtherightlowerribs.

Thespleenisoftenpalpable,andmaybeverylarge.肝硬化英文Theclinicalmanifestationsfoundincirrhosis

、肝硬化英文Complications[I]Uppergastrointestinalbleeding(上消化道出血):

Hematemesis(嘔血)/melena(黑糞).Esophageal/gastricvaricealbleeding(食管/胃靜脈出血);portalhypertensivegastropathy(門(mén)脈高壓性胃病);pepticulcer(消化性潰瘍)肝硬化英文Infections:spontaneousbacterialperitonitis(自發(fā)性細(xì)菌性腹膜炎)(4-8%)：Fever,worseningjaundiceorrenaldysfunction,abdominalpain(occurringonlyin50%ofpatients),andencephalopathyarethemostcommonclinicalfindingsinSBP.However,thepatientisfrequentlyasymptomatic.BecausecultureofascitesfluidisnegativeinalargenumberofpatientswithSBP,diagnosisshouldbebasedonthepresenceof>250neutrophils/mm3.肝硬化英文Complications[II]

Hepatocellularcarcinoma(肝細(xì)胞肝癌)

Hepaticencephalopathy

(肝性腦病)Asterixis(撲翼樣振顫)Disoriented(定向障礙)Coma(昏迷)

肝硬化英文Complications[III]

Hepatorenalsyndrome(HRS):

Oliguria(少尿),azotemia(氮質(zhì)血癥),hypotension(低血壓),

dilutionalhyponatremia(稀釋性低鈉血癥),lowurinarysodium(低鈉尿)肝硬化英文Complications[IV]Electrolyteandacid-baseimbalance(電介質(zhì)酸鹼平衡失調(diào))

hyponatremia,hypokalemiaAndhypochloremicalkalosis

肝硬化英文Laboratoryfindings[I]BloodandurineroutinesLiverfunctiontests

---toestimatetheseverityofliverdysfunction：ALT,AST,AKP,GGT,serumtotalbilirubin,serumalbumin,prothrombintime,globulin,cholesterol.

---todifferentialdiagnosis:Alcoholic:AST/ALT>=2;PBC:AKP,GGT>>ALT,AST

---torefecthepaticfibrosis:PIIIP、HA、laminin---toquanlityliverfunction肝硬化英文Immunology

Cellularimmune

,hormonalimmune

autoimmuneliverdisease:IgG,globulinANA(+),SMA(+)PBC:IgM,AMA(+)MarkerofvirusAFP肝硬化英文Laboratoryfindings[II]

Ascitesparacentesis:

routine,culture,ADA,LDH,

SAAG(serumascitesalbumingradient)

(血清腹水白蛋白梯度)>11g/L

Ultrasonography,CTscanning：

biliaryobstruction,livermasses,splenomegaly,ascites.

Endoscopy:

thenumber,appearance,andsizeofanyesophageal/gastricvarix,portalhypertensivegastropathy(PHG)肝硬化英文Laboratoryfindings[III]

Radionuclide:99mTC-MIBI,H/L

liverbiopsy:toconfirmthediagnosis

Laparoscopy

HVPG(hepaticveinpressuregradient)(肝靜脈壓力梯度)(wedged-free)hepaticvenouspressureNormal:5-6mmHg,>10mmHg:varices;>12mmHg:rupture

肝硬化英文肝硬化英文肝硬化英文肝硬化英文肝硬化英文Diagnosis[I]

Etiologyofcirrhosis

Pathologyofcirrhosis

Evaluatingofliverfunction:Child-Pughclassification

Searchingforcomplications肝硬化英文Diagnosis[II]

thehistoryofdiseasecontributestoidentifyingthecauseofcirrhosis.

historyofviralhepatitis,bloodtransfusions,medicationuse,alcoholuse,sexualpracticesshouldbecarefullyreviewed.

signsandsymptomsconfirmtoexistenceofportalhypertensionandimparedliverfunction.

liverfunctiontests:hypoalbuminemia,hyperbilirubinemia，theprolongedprothrombintimesuggesthepaticdecompensation.

Imagingstudy:UltrasoundandCTreadilyidentifythelesion,buthavenocharacteristicfindings.肝硬化英文

Child-Pughclassification

Scorea

variable

123Encephalopathy(degree)NilSlight-ModerateModerate-SevereAscites(degree)NilSlightModerate-SevereBilirubin(umol/L)<3434-51>51Albumin(g/L)

3528-34<28ProthrombinIndex(%)>7040-70<40ProthrombinTime(s)<1415-17>18ProthrombinTime(INR)

<1.31.3～1.5

>1.5*PBC:SB(μmol/L)17～6868～170>170

aScoresaresummedtodetermineChild’sclass:classA=5-6classB=7-9classC=10-15Diagnosis[III]肝硬化英文DifferentialDiagnosis

Otherconditionofhepatomegalyorsplenomegaly：

chronicvirushepatitis,Gaucher’sdisease,lymphomasandleukaemias,congestivesplenomegaly

Differebtialdiagnosisofcirrhoticascitesandothertypesofascites：

malignantascites,constrictivepericarditis,tuberculousperitonitis,etal.

Portalhypertension:肝硬化英文Treatmentofcirrhosis[I]

specifictreatmentfortheunderlyingetiologyoftheliverdisease

antivirustherapy--viralhepatitisabstinencefromalcohol--alcoholicUrsodeoxycholicacid(UDCA)(熊去氧膽酸)--PBCPenicillamine(青霉胺)—Wilson’sdisease

GeneralTreatments:

Highcalories(40kcal/kg·d)、adequateprotein(1-1.5g/kg·d)、vitamin、Herbalcompounds.肝硬化英文TreatmentofAscitesa.Bedrest,sodium

andwaterrestriction.

1.Fluidintake:800-1000ml/d(hyponatremia,serumsodium<130meq/L)2.Dietarysodiumintake

：88mmol/d(2.0gNacl)Mildpatients：restonbed，withdietarysaltrestriction,lossofascitesoccursin10%to15%ofpatients.肝硬化英文TreatmentofAscites[II]

b.Increasingrenalsodiumandwaterexcretion:

--Diuretics:

urinarysodium/urinarypotassium>1Spironolactone(安體舒通)+furosemide(速尿)urinarysodium/urinarypotassium<1higherdosesspironolactone，

肝硬化英文TreatmentofAscites(III)c.Large-volumeparacentesis

associatedwithplasmavolumeexpansiond.Ascitesultrafiltrationandre-infusione.Peritoneo-venous(LeVeen)shuntsf.

TIPS(transjugularintrahepaticporto-systemicstent)(經(jīng)頸靜脈門(mén)體分流術(shù))

g.Livertransplantation(肝移植)肝硬化英文肝硬化英文TIPS---stentpositionedbetweenthehepaticandportalveins肝硬化英文Treatmentofcirrhosis[IV]

surgicaltreatmentofportalhypertension

portacavalshuntsurgery：

portacavalmesocaval

distalsplenorenalshunts

Choiceofpatients：

Child-Pugh:A,Bbleedingfromgastroesophagealvarices,hypersplenism.肝硬化英文Treatmentofcirrhosis[V]

TreatmentcomplationsTreatmentofacutevaricealhaemorrhage：

----Generalmanagement:abstainfood,intensivecare,volumeandbloodreplacement,specificmeasurestostopthebleeding

----Pharmacologicaltherapy:

vasopressin(垂體后葉素)somatostatin(生長(zhǎng)抑素)

Octreotide(奧曲肽)肝硬化英文Treatmentofacutevaricealhaemorrhage：

___Emergentendoscopy:afterPatient’shemodynamicstatusstabilized(usuallywithin2-12hours)

----Balloontubetamponade(if

bleedingcontinues)

----Endoscopicvaricealsclerotherapyandbandligation

----Prophylactictherapytopreventrebleeding:Beta-adrenergicantagonists(普奈洛爾),endoscopicsclerotherapy(硬化劑)/banding(套扎)(usually3-6sessions),portacavalshunting,TIPS肝硬化英文肝硬化英文肝硬化英文肝硬化英文肝硬化英文肝硬化英文TIPS---stentpositionedbetweenthehepaticandportalveins肝硬化英文Treatmentportalhypertension(<12mmHg)EradicatevaricesLivertransplantationShuntSurgicalshuntsTIPSPharmacotherapyQ

Endoscopy:EVS,EVLDevascularizationR肝硬化英文TreatmentofSBP1.AscitesPMN>250/mm3:antibiotictherapyshouldbeinitiated.2.AscitesPMN<250/mm3andasciticfluidculturecontinuestobepositive:initiationofantibiotictreatment.3.Follow-updiagnosticparacentesisperformed48hoursafterstartingtherapyallowsassessmentofresponsetotreatmentandtheneedtomodifyantibioticcoverage.4.Long-termprophylaxis---Patientswhohaverecoveredfromanepisodeof