Jaundice and hepatomegaly.docx
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Jaundiceandhepatomegaly
Jaundiceandhepatomegaly
3.6Jaundiceandhepatomegaly3.6.1Distinguishbetweenprehepatic,hepaticandpost-hepaticjaundiceonclinicalandbiochemicalgroundsThebasiccauseofjaundiceisproductionofbilirubinataratefasterthathelivercanconjugateit.PrehepaticFree(unconjugated)bilirubinispresentinthebloodatmuchhigherconcentrations.Theliverworksmuchharderthannormalbutcannotkeepupenoughtofullyconjugateallthebilirubintheamountofunconjugatedbilirubinremainhigh.Theexcess(abovenormal)amountsofconjugatedbilirubinpassalongthebilecanaliculiandendsupasurobiligeninthegut.Fromhereitischangedintowatersolubleurobilinogen(bybacteria)whichisreabsorbedintotheplasmaandpassedthroughthekidnetandintourine.Urobilinogenhasnocolour.Unconjugatedbilirubinisnotwatersolubleandsocannotbeexcretedinurine.Post-hepaticthereisafaultorblockagesomewheredownstreamofconjugation.Conjugatedbilirubinfromthelivercan’tgettothegut(forexample,becausethebileductisblocked),sospillsoverintothebloodstream.Conjugatedbilirubiniswatersoluble,andcanhencepassviatheglomerulusintotheurine,givingitacharacteristicdarkcolour.Meanwhile,thedropingutbilirubinleaveslesssubstrateforbacteriasolittleornourobilinogencanbeformed;consequentlynoneisfoundintheurine,andlittlestercobilincanbemade.Cholestasisthereforeleadstopalestoolsanddarkurine.HepaticDamagetothehepatocytesthemselvesresultsinamixedpicture.Earlyoninthediseasebothtypesofbilirubincontributetothejaundice,butasthediseaseprogressescholestasisoftensupervenesandconjugatedhyperbilirubinaemiabecomesthedominatingfeature.InsummaryPre-hepatic(Haemolytic)DarkStoolHaemoglobinReticulocytosisNNNNoneN/Hepatic(toxins,infections)Post-hepatic(Cholestatic)DarkurinePalestoolNN//NoneClinicalFBCLFTUnconjugatedbilirubinConjugatedbilirubinALPALTUrineBilirubinUrobiligenTests:
urine,FBC,UE,LFT,Serology(HAV,HBV,HCV,EBV,CMV),clotting,bloodfilm,reticulocytecount,Coombstest(usedintheinvestigationofhaemolyticanaemia.ApositiveCoombs’testisfoundincasesofautoimmunehaemolysisduetothepresenceofIgG,complementorboth,onthesurfaceofthepatient’sredcells).Specifictests:
serumcopperceruloplasmin(inWilsons),serum1antitrypsin,serumironandTIBC(inhaemochromatosis)Imaging:
USS,ERCP,MRCP,CT,Liverbiopsy/N/N/N3.6.2Distinguishbetweeninfectiousandmechanicalcausesofbiliaryobstruction;3.6.3InitiateappropriateinvestigationsforbiliaryobstructionMechanicalGallstonesCancerheadofpancreasCancerhepaticCancercholecysticStricturesPSCStricturesPBSInfectiousCirrhosis-viralCT/MRI3.6.4DistinguishthecommoncausesofhepatomegalyonclinicalgroundsCirrhosisCardiacfailureEarlyGenerallyregularsurfaceNojaundiceLateGenerallyirregularsurfaceWithjaundiceOthercauses:
hepatitis,otherinfection,sarcoid,haemochromatosis,fattyliver,Riedelslobe,biliarytractdisease3.6.5InitiateappropriateinvestigationsforhepatomegalyCT/MRIFBC(withbloodfilm)CXR/AXRESR,UE,LFT,Ca2+InvestigationsLFTs:
ALPinPSCCRP/ESR:
inPSC/PBHWCC:
ininfectionSerology:
HAV,HBV,HCV,EBV,CMVUSS:
gallstones,cancer,cirrhosisERCP/MRCP:
stricturesSecondarymetastasesGenerallyirregularNojaundiceGenerallyregularNojaundiceTense,tenderliverPeripheraloedemaIncreasedJVPBiopsy3.6.6RecognisethemanifestationsofchronicliverdiseaseincludingencephalopathyandportalhypertensionLiverFunctionalbumincoagulationproteinsBileComplementoestrogenAmmoniaureaGluconeogenesis(aminoacidsglucose)StorageofglucoseasglycogenGlycogenolysisPortalhypertensionAriseinpressurewithintheportalveinanditstributaries.Resultantfromincreasedresistancetoportalbloodflowcausedbycirrhosis:
perisinusoidalcollagendeposition,perivenularfibrosis,expansionofnodules.Consequencesincludeporto-sytemicshuntinginwhichsomebloodbypassestheliverthroughanastomoseswithsystemiccirculationvesselsthisoccursatthreesites:
Betweensystemicandportalveinsoesophagealveinandleftgastricveinrectal/inferiorrectalveinsandsuperiorrectalveinsmallepigastricvofanteriorabdowallandparaumbilical*Bleedingfromthissitemayoccurthroughtheruptureofdelicateengorgedvesselsbyeatingthismayresultinhaematemesis,melaenaordeath.Otherresultsofportalhypertension:
Ascites(canalsobecausedbycongestiveheartfailure,malignancyortrauma)TransudatevsExudate:
Atransudateisafluidcontaining25mgofproteinpermlofwater.Anexudatecontainsmorethanthis.Transudatesaretypicallycausedby