川崎病进展课件

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1、KawasakiDisease:AnUpdateBevinWeeks,MDYaleUniversitySchoolofMedicineObjectivesReviewthehistorical&clinicalfeaturesofa“common”butpoorlyunderstoodchildhooddiseaseExplorethedifferentialdiagnosisofthisillnessConsiderthemostimportantsequelaeofthisdiseaseDiscusstreatment&l

2、ong-termmanagementrecommendationsKawasakiDisease:AnUpdateHistoryofKawasakidiseaseEpidemiologyandetiologyPresentationanddiagnosisTreatmentChroniccardiovascularmanifestationsFollowupofpatientsQuestionsinthechronicmanagementKawasakiDisease:MucocutaneousLymphNodeSyndrom

3、e“Aself-limitedvasculitisofunknownetiologythatpredominantlyaffectschildrenyoungerthan5years.ItisnowthemostcommoncauseofacquiredheartdiseaseinchildrenintheUnitedStatesandJapan.”JaneBurns,MD**Burns,J.Adv.Pediatr.48:157.2001.HistoryofKawasakiDiseaseOriginalcaseobserved

4、byKawasakiJanuary19614y.o.boy,“diagnosisunknown”CAthrombosis1strecognized1965onautopsyofchildprev.dx’dw/MCOSFirstJapanesereportof50cases,1967FirstEnglishlanguagereportfromDr.Kawasaki1974,simultaneouslyrecognizedinHawaiiWhatisKawasakiDisease?Idiopathicmultisystemdise

5、asecharacterizedbyvasculitisofsmall&mediumbloodvessels,includingcoronaryarteriesEpidemiologyMedianageofaffectedchildren=2.3years80%ofcasesinchildren<4yrs,5%ofcasesinchildren>10yrsMales:females=1.5-1.7:1Recursin3%Positivefamilyhistoryin1%but13%riskofoccurrenceintwins

6、OverallU.S.in-hospitalmortality≈0.17%EpidemiologyAnnualincidenceof4-15/100,000childrenunder5yearsofageOver-representedinAsian-Americans,African-AmericansnextmostprevalentSeasonalvariationMorecasesinwinterandspringbutoccursthroughouttheyearTheBiggestQuestion:Whatis

7、theEtiologyofKawasakiDisease?EtiologyInfectiousagentmostlikelyAge-restrictedsusceptiblepopulationSeasonalvariationWell-definedepidemicsAcuteself-limitedillnesssimilartoknowninfectionsNocausativeagentidentifiedBacterial,retroviral,superantigenicbacterialtoxinImmunolo

8、gicresponsetriggeredbyoneofseveralmicrobialagentsNewHavenCoronavirusIdentifiedanovelhumancoronavirusinrespiratorysecretionsfroma6-month-ol