Renal-Main肾病-71-80

RenalMain71-80Q1A35-year-oldmancomestothephysicianduetoaone-monthhistoryofweightgainandfacialedema.Thefacialedemaresolvesattheendoftheday,butankleedemadevelops.Histemperatureis37.2°C(99°F),bloodpressureis142/80mmHg:pulseis80/min，andrespirationsare16/min,Examinationshows2+ankleedema.Laboratorystudiesshow:Thispatientismostlikelyatriskfordevelopingwhichofthefollowing?A.RuptureofbrainaneurysmB.AbdominalaorticaneurysmC.HypercoagulabilityD.PulmonaryhemorrhageE.GailstonepancreatitisA1Correctanswer:CThepatientinthisclinicalvignettehasnephroticsyndrome.Nephroticsyndromeisaclinicalcomplexcharacterizedby:1.Proteinuria(3-3.5g/day-mostimportantmanifestation)2.Hypoalbuminemia3.Edema4.HyperlipidemiaandlipiduriaThebasicpathologyisalteredpermeabilityoftheglomerularmembraneforproteins.Diseasesmostcommonlycausingnephroticsyndromeareminimalchangedisease(inchildren),membranousglomerulopathy(adults),mesangialproliferativeglomerulonephritis;membranoproliferativeglomerulonephritis,andfoca!segmentalglomerulosclerosis.Nephroticsyndromeisfrequentlycomplicatedbyhypercoagulation,withaconsequentriskofthromboemboliccomplications.Theetiologyofhypercoagulationinnephroticsyndromeismultifactorialandincludes:increasedurinarylossofantithrombin3,alteredlevelsofproteinCandS,increasedplateletaggregation,hyperflbrinogenemiaduetoincreasedhepaticsynthesis,andimpairedfibrinolysis.Renalveinthrombosisisthemostcommonmanifestationofcoagulopathy(especiallywithmembranousglomerulopathy),butarterialthrombosisandpulmonaryembolismmayalsooccur.Coagulopathyislesscommonbutmoresevereinchildrenascomparedtoadultswithnephroticsyndrome.Complicationsofnephroticsyndromeinclude:proteinmalnutrition,iron-resistantmicrocytichypochromicanemiaduetotransferrinloss,vitaminDdeficiencyduetoincreasedurinaryexcretionofcholecalciferol-bindingprotein,decreasedthyroxinlevelsduetolossofthyroxine-bindingglobulin,andincreasedsusceptibilitytoinfection.(ChoicesAandB)Ruptureofabrainaneurysmandabdominalaorticaneurysmsaremorelikelytobeseeninpatientswithadultpolycystickidneydisease.Suchpatientsdonotpresentwithnephroticsyndrome.(ChoiceD)PulmonaryhemorrhageisamanifestationofGoodpasture'sdiseaseorgranulomatosiswithpolyangiitis(Wegener's).Thesecausenephritic(notnephrotic)syndrome.(ChoiceE)Gallstonepancreatitisisnotacomplicationofnephroticsyndrome.EducationalObjective:Nephroticsyndromeisahypercoagulableconditionwhichmanifestsasvenousorarterialthrombosis,andevenpulmonaryembolism.Renalveinthrombosisisthemostfrequentmanifestation.Complicationsofnephroticsyndromeinclude:proteinmalnutrition,iron-resistantmicrocytichypochromicanemia,increasedsusceptibilitytoinfection,andvitaminDdeficiency.Q2A34-year-oldwomancomestothephysician'sofficebecauseofoccasionalheadachesandpalpitations.Shehasnoothermedicalproblems.Shetakesnomedications.Shesmokesoneandahalfpacksofcigarettesdaily.Herbloodpressureis170/100mmHginbotharms,andheartrateIs80/min,Physicalexaminationshowsbilateralflankmasses。Urinalysisshows10-12redbloodcells/hpf，butotherwiseshowsnoabnormalities.Themostlikelycomplicationthatcanoccurinthispatientiswhichofthefollowing?A.LivernecrosisB.intracranialbleedingC.RestrictivecardiomyopathyD.PancreaticcancerE.AorticdissectionA2Correctanswer:BThispatientmostlikelyhasautosomaldominantpolycystickidneydisease(ADPKD).Thecluestothecorrectdiagnosisarehypertensionpalpablebilateralabdominalmassesandmicrohematuria.Intracranialberryaneurysmisacommoncomplication,andisseenin5to10%ofthecases.Althoughsuchaneurysmsarecommonanddangerouswhencoupledwithhypertension,routinescreeningforintracranialaneurysmsisnotrecommended.Theothermajorextra-renalcomplicationsofADPKDare:Hepaticcysts-mostcommonextrarenalmanifestationsofADPKD2.Valvularheartdisease-mostoftenmitralvalveprolapseandaorticregurgitation3.Colonicdiverticula4.Abdominalwallandinguinalhernia(ChoiceA)Livernecrosisisararecomplicationofthedisease.(ChoiceE)Aorticdissectioncanoccurasararecomplicationofseverehypertension,notpolycystickidneydiseaseitself.(ChoicesCandD)Restrictivecardiomyopathyandpancreaticcancerarenotassociatedwithpolycystickidneydisease.Educationalobjective:Autosomaldominantpolycystickidneydiseaseisapotentialcauseofhypertension.Hepaticcystsarethemostcommonextrarenalmanifestations.Intracranialberryaneurysmsareseenin5to10%ofthecases.Althoughsuchaneurysmsarecommonanddangerouswhencoupledwithhypertension,routinescreeningforIntracranialaneurysmsisnotrecommended.Q3A51-year-oldmanisadmittedtothehospitalbecauseofrenalfailure.Hispastmedicalhistoryissignificantforrecurrentepisodesofbilateralflankpainoverthepastseveralyearsaswellasnocturia2to3timespernightforthepast10years.Hehasnoweightloss.Onphysicalexaminationhisbloodpressureis164/100mmHg.Theliverisenlargedandamassisfeltattherightflankondeeppalpation.Whichofthefollowingisthemostlikelydiagnosis?A.HorseshoekidneyB.NephrolithiasisC.PapillarynecrosisD.PolycystickidneydiseaseE.RenalcellcarcinomaA3Correctanswer:DThismanmostlikelyhasautosomaldominantpolycystickidneydisease(ADPKD).ADPKDisoneofthemostcommonhereditarydiseasesintheUnitedState