摘要：

Polycysticliverdisease(PLD)isararehereditarydiseasethatindependentlyexistsinisolatedPLD;orasanaccompanyingsymptomofautosomaldominantpolycystickidneydiseaseandautosomalrecessivepolycystickidneydiseasewithcomplicatedmechanisms.PLDcurrentlylacksaunifieddiagnosticstandard.ThediagnosisofPLDisusuallymadewhenthenumberofhepaticcystsismorethan20.GigotclassificationandSchnelldorferclassificationarenowcommonlyusedtodefineseverityinPLD.MostPLDpatientshavenoclinicalsymptoms;andminoritywithseverecomplicationsneedtreatments.Somatostatinanalogues;mammaliantargetofrapamycininhibitor;ursodeoxycholicacidandvasopressin-2receptorantagonistarethepotentiallyeffectivemedicaltherapies;whilecystaspirationandsclerosis;transcatheterarterialembolization;fenestration;hepaticresectionandlivertransplantationaretheoptionsofinvasiontherapies.However;theeffectivenessofthesetherapiesexceptlivertransplantationarestilluncertain.Furthermore;thereisnounifiedstrategytotreatPLDbetweenmedicalcentersatpresent.InordertobetterunderstandrecentstudyprogressesonPLDforclinicalpracticeandobtainpotentialdirectionsforfutureresearches;thisreviewmainlyfocusesontherecentprogressinPLDclassification;clinicalmanifestation;diagnosisandtreatment.Forinformation;wealsoprovidedmedicaltreatmentprocessesofPLDinourmedicalcenter.

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