Acuteandchronichepatobiliarymanifestationsofsicklecelldisease:Areview

摘要： Sicklecelldisease(SCD)isacommonhemoglobinopathywhichcanaffectmultipleorgansystemsinthebody.Withinthedigestivetract,thehepatobiliarysystemismostcommonlyaffectedinSCD.Themanifestationsrangefrombenignhyperbilirubinemiatoovertliverfailure,withthespectrumofacuteclinicalpresentationsoftenreferredtoassicklecellhepatopathy.ThisisanumbrellatermreferringtoliverdysfunctionandhyperbilirubinemiaduetointrahepaticsicklingprocessduringSCDcrisisleadingtoischemia,sequestrationandcholestasis.Inthisreview,wedetailthepathophysiology,clinicalpresentationandbiochemicalfeaturesofvariousacuteandchronichepatobiliarymanifestationsofSCDandpresentandevaluateexistingevidencewithregardstomanagementofthisdiseaseprocess.Wealsodiscussrecentadvancesandcontroversiessuchastheroleoflivertransplantationinsicklecellhepatopathyandhighlightimportantquestionsinthisfieldwhichwouldrequirefurtherresearch.Ouraimwiththisreviewistohelpincreasetheunderstanding,aidinearlydiagnosisandimprovemanagementofthisimportantdiseaseprocess. ... （共9页）