皮肤粘膜淋巴结综合征（英语）-教学课件（非AI生成）.ppt

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皮肤粘膜淋巴结综合征（英语）-教学课件（非AI生成）.ppt

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KawasakiDisease

Mucocutaneouslymphnodesyndrome,MCLS皮肤粘膜淋巴结综合征1

BackgroundKawasakidisease(KD)isafebrileillnessofchildhood.Itisaself-limitedacutevasculiticsyndromeofunknownetiology.2

BackgroundfirstdescribedbyTomisakuKawasakiin1967.Atthattime,hereported50childrenfrom1961-1967whopresentedwithadistinctiveclinicalillnesscharacterizedbyfeverandrash,whichwasthenthoughttobeabenignchildhoodillness.3

BackgroundSeveralyearslater,fatalitiesoccurredinJapanamongchildrenyoungerthan2years.Thefatalitiesoccurredwhenpatientswereimprovingorhadrecovered.completethromboticocclusionofcoronaryarteryaneurysmswithamyocardialinfarction(MI)4

Background15-25%ofuntreatedpatientsdevelopcoronaryarteryaneurysms.KawasakisyndromehasnowsurpassedrheumaticfeverastheleadingcauseofacquiredheartdiseaseintheUnitedStatesamongchildrenyoungerthan5years.

EpidemiologyMostcommoninJapan112/100,000vs17/100,000MostlikelytooccurduringthewinterandspringMaletofemaleratiois1.5：180%ofaffectedpatientsareyoungerthanage5yearsThemortalityrateisabout0.17%6

Etiologyremainunclearmutipleagents(viruses,staphylococcisuperantigens)→immuneactivation.ParvovirusB19,Yersinia,andcytomegalovirus……geneticpredispositiontogeneticpolymorphisms.7

PathogenesisAffectingmedium-sizedarteriesMultipleorgansystemareinvolvedCardiovascularinvolvementismostsignificantPericardium(pericarditis)Myocardium(myocarditis)Valves(valvulities)Coronaryarteries(coronaryarteryaneurysms)--mostimportant8

PathogenesisPathologyofcoronaryarteryabnormalities恢复期（可长达数年）28-31天10-21天1-9天动脉周围炎冠脉全层血管炎，血栓、动脉瘤。炎症消散，内膜增厚，冠脉阻塞疤痕形成，冠脉再通。9

PathogenesisCoronaryaneurysmsGreatriskofaneurysmformationMalesYoungchildren(6months)Prolongedfever(14d)Highneutrophil(WBC30)Lowhemoglobin(HCT0.35)Lowplateletcounts(20)highplateletcounts(1000)UntreatedwithIVIG