黄疸》-09级一系-老年-麻醉-救援-心理-陶小红

Jaundice(黄疸)　DepartmentofGastroenterology,FirstAffiliatedHospitalofChongqingMedicalUniversityXIAOHONGTAO(陶小红)Jaundiceoricterus:Whatisjaundice?ClinicallyitistheyellowdiscolorationoftheSclera(巩膜)SkinMucousmembraneduetodepositionofbilepigment(bilirubin胆红素)Serumtotalbilirubin(normal1.7-17.1umol/lit)Conjugatedbilirubin:0-3.42μmol/l，Unconjugatedbilirubin:1.7-13.68μmol/l.1.DEFINITION2.BILIRUBINMETABNISMHemeDegradationHEMEBILIVERDINO2Fe+3NADPHNADP+(openstheporphyrinring)BILIRUBINNADPHNADP+BILIRUBINdiglucuronideBILETheFateofBilirubin…Alb=albuminB=bilirubinGST=glutathione-S-transferaseUDPGA=uridinediphosphoglucuronicacid;CB=conjugatedbilirubinUGT1A1=UDP-glucuronosyltransferase1A1MRP2=Multi-drugResistanceProtein2AdaptedfromHarrison’s15thEd.“PrinciplesofInternalMedicine”,2001.MRP2B+GSTCBPlasmaHepaticCellBileAlbBAlb?:GSTBsERB+UDPGAUGT1A1BilirubinExcretionIntestinesLiverBCBCBBUrobilinogenB-glucoronidasebacteriabacteriaBileEnterohepaticcirculationKidneyUrobilinoxUrobilinogenUrobilinStercobilinStercobilingogenfecesUrineoxLiverAlbuninHemeFerrohemetransferaseBilirubinConjugatedbilirubinKidneyUrobilinStercobilinogenUrobilinogenIntestinesAlbuminBilirubinreductaseHemeGlobinHemoglobinoxidaseBiliverdinMarrowInfantilityErythrocyteFerrohemeEnzyme15%~20%Bilirubin|AlbuminErythrocyte80%~85%UDPUDPGABilirubinglucuronolactoneurobilinogenEnterohepaticcirculationY/ZproteincathepsinErythrocyteBiochemicalchangeofNormalbilirubinmetabolism3.CLASSFICATION1.DependingonEtiologyHemolyticJaundice　（溶血性黄疸）　　　　HepatocellularJaundice（肝细胞性黄疸）CholestaticJaundice（胆汁淤积性黄疸）Congenitaljaundice　（先天性黄疸）2.DependingonbilirubinUnconjungatedbilirubinincreasedjaundiceConjungatedbilirubinincreasedjaundiceEtiology1.Congenitalhemolyticanemia(thalassemia(海洋性贫血),hereditaryspherocytosis(遗传性球形红细胞增多症)).2.Posteriorityacquiredhemolyticanemia(autoimmunityhemolyticanemia,hemolyticdiseaseofnewborn,posttransfusionhemolytic,Favism(蚕豆病).MechanismAlargenumberoferythrocytedestroyedrapidlyAnemia,hypoxiaandtoxityoferythocytemetabolismproducts3.1.HemolyticJaundiceHemolyticJaundicemechanismClinicalManifestationMildjaundice,lightlemon,noskinitch(痒).Acutehemolysis:fever,chill,headache,vomit,backache,anemia,hemoglobinuria(血红蛋白尿),acuterenalfailure.Chronichemolysis:anemiaandsplenomegaly(脾大).LaboratoryExamination1.SerumTB↑,UCB↑,CBnormal.2.UCB↑→intestinalCB↑→faecalcolordeepen.3.IntestinalUrobilinogen(尿胆原)↑→urinaryUrobilinogen↑.4.Acutehemolytis,occultbloodtest(+).5.Bloodtest:anemia,reticulocyte(网织红细胞)↑,erythrocyteproliferation↑.3.2.HepatocellularJaundiceEtiologyHepatocytedamage.MechanismImpairhepatocyteuptakeing,conjugatingandexcretingbilirubin,UCB↑CBrefluxintoblood,serumCB↑→jaundice.HepatocellularJaundicemechanismClinicalManifestationSkinandmucosa:lighttodeepyellow,mildskinitch(痒)Tired,lossofappetitehemorrhagictendency,ascites,coma.PrimarydiseasemanifestationLaboratoryExaminationCB↑,UCB↑Icterichepatitis:CB↑↑UCB↑Urine:CB(+),urobilinogen↑Bloodtest:liverdamaged3.3.CholestaticJaundiceEtiologyObstructioninliver:virushepatitis,drugcaused,drughepatitis,primarybiliarycirrhosis.ObstructionofExtrahepaticbileduct:narrow,obstruction,stone,inflammtory,tumor.MechanismObstructioncausessmallbileductandbilecapillarybroken,conjugatedbilirubinrefluxtoblood.FailureofhepatocyteexcretingCB,bilirubindepositandbilethrombus(胆栓)formation.CholestaticJaundicemechanismClinicalManifestationSkindarkyellow,yellowgreen.Skinitch(痒),bradycardia(心动过缓),darkyellowofurine,faecallightyelloworclaycolor.Courvoisier’ssign.LaboratoryExaminationSerumCB↑Urinebilirubin(+)Urobilinogen,stercobilin(粪胆原)↓orabsenceSerumalkalinephosphataseandCholesterol(胆固醇)↑A.Hemolyticanemiaexcesshemolysisunconjugatedbilirubin(inblood)conjugatedbilirubin(releasedtobileduct)UBUrobilinogenB.Hepatitisunconjugatedbilirubin(inblood)conjugatedbilirubin(inblood)UBUrobilinogenC.Biliaryductstoneunconjugatedbilirubin(inblood)conjugatedbilirubin(inblood)UBUrobilinogen5.GeneticDisordersofBilirubinMetabolismConditionDefectBilirubinClinicalFindingsCrigler-NajjarsyndromeseverelydefectiveUDP-glucuronyltransferaseUnconjugatedbilirubinProfoundjaundiceGilbertssyndromereducedactivityofUDP-glucuronyltransferaseUnconjugatedbilirubinVerymildjaundiceduringillnessesDubin-JohnsonsyndromeabnormaltransportofconjugatedbilirubinintothebiliarysystemConjugatedbilirubinModeratejaundiceRotorssyndromedeficiencyofuptakingUCBandexcretingCB,CB↑jaundice.Crigler-NajjarsyndromeGilbertssyndromeThreekindsofjaundicelaborotoryexaminationindentificationClassHemolyticHepatocellularCholestaticTBincreasedincreasedincreasedCBnormalincreasedmarkedlyincreasedCB/TB＜15％一20％＞30％一40％＞50％一60％urinebilirubin—十十十urobilinogenincreasedslightincreaseddecreasedorabsenceALT、ASTnormalmarkedlyincreasedmayincreasedALPnormalincreasedmarkedlyincreasedr-GTnormalincreasedmarkedlyincreasedPTnormaldelayeddelayedVitKrespondnopoorgoodCholesterolnormalslightincreasedordecreasedmarkedlyincreasedSerumproteinnormalAlbdecreased,GlobincreasednormalAccessoryExamination1.Ultrosound2.Xray3.ERCP(Endoscopicretrogradecholangiopancreatography,逆行胰胆管造影)4.PTC(Percutaneoustranshepticcholangiography,经皮肝穿刺胆管造影)5.CT(Computedtomography)6.MRI(Magneticresonancecholangiopancreatography)7.LiverBiopsyandLC(Laparoscopy，腹腔镜)AccompanyingSymptomsFever:acutecholangitis,liverabscess,leptospirosis(钩端螺旋体病),septicemia(败血症),lobarpneumonia(大叶性肺炎).Feverthenjaundice:virushepatitis,acutehemolysis.Upperabdominalsharppain:biliarycalculi(胆道结石),liverabscess,biliaryascariasis(胆道蛔虫病),Rightupperabdomsharppain,chill,highfever,jaundice,Charcottriadsign:acutepyogeniccholangitis(急性化脓性胆管炎),Sustainedrightupperabdominalblurorswellpain:virushepatitis,liverabscess,primarycarcinomaofliverHepatomegaly(肝大)①Mildormoderate,softormoderatehard,smoothsurface:Virushepatitis,acuteinfectionofbiliarytract.obstructionofbiliarytract②Obviousenlargement,hard,raggedsurface:primaryorsecondarycarcinomaofliver.③Notobviousenlargement,hard,edgenottidy,noduleonthesurface:cirrhosis.Gallbladderenlargement:commonbileductobstruction,pancreaticheadcarcinoma,commonbileductcarcinoma,carcinomaofampulla.Splenomegaly:virushepatitis,leptospirosis(钩端螺旋体病),septicemia(败血症),Malaria(疟疾),cirrhosis,hemolyticanemia,lymphoma(淋巴瘤).Acites:serioushepatitis,decompensationofcirrosis(肝硬化失化偿期),carcinomaofliver.InquisitionPoints（问诊要点）1.Assurejaundice2.Etiologyandonset3.Accompanysyndrome4.Timeandfluctuationofjaundice5.InfluncetotheoverallhealthInoneword:makesurethetypeofjaundice,andthenfindthepathogenyRecognisejaundicePosition：sclera,skinandtonguesleevesPseudojaundice(假性黄疸):eatCarotene(胡罗卜素)food(Carotene,pumpkin(南瓜),tomato,orange)alsocancauseskinstainedyellow,butbilirubinisnotincreased.Oldpeoplefataccumulationatbulbarconjunctiva(球结膜),yellow,unevendistributionQuestions1)Unconjugatedhyperbilirubinemiausuallyresultsfromaintra-orextrahepaticbiliaryobstruction.A.TrueB.False2)Clinicalfeaturesofcholecystitisincludeallofthefollowingexcept:A.RUQabdominalpainB.RadiationtoRupperbackC.Nausea,VomitingD.+Murphy’ssignE.Fever3)Amarkerofacutehepatocyteinjuryis:A.PTB.ALTC.AlbuminD.DirectbilirubinE.Ammonia1.A13yearsoldmale,chiefcomplaints“Findingskinandsclerayellowandfeelingrightupperabdomenswellpainfor1week”Bloodtest:TBil:80μmol/L,DBil:50μmol/L,ALT:1200U/L.BUltrasound:normal.Whatarethepossiblediagnosesandyoursuggestedexaminations.HepatocellularandCholesteticjaundice.Hepatitisvirustest,Urinetest,orMRCP.QuestionReferencebooksCecilmecidine23rdedition.Davidson’principlesandpracticeofmedicine.Harrison'sprinciplesofinternalmedicine.16thedition.