湘雅儿科精品课件kawasaki disease

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1、KawasakiDiseaseDepartmentofPediatricWangXiu-ying中南大学儿科学教研室第九章第八节■UnderstandtheetiologyandpathologyofKawasakiDisease(KD)■MastertheclinicalmanifestationsanddiagnosticcriterionofKD.■MasterthetreatmentofKD.PurposeandRequirementKawasakidiseaseformerlyknownasmucocutaneouslymphnodesyndrome（MCLS皮肤粘膜淋巴结综合征）

2、.80%ofpatientsareyoungerthan5yr,Approximately20%ofuntreatedpatientsdevelopcoronaryarteryabnormalitiesincludinganeurysms（动脉瘤）.kawasakidiseasehasreplacedacuterheumaticfeverastheleadingcauseofacquiredheartdiseaseinchildreninourcountry.ConceptionEtiologyandPathogenesis※infectiousfactors(感染因素)※immunerea

3、ction（免疫反应）※otherfactors(其他因素)Aseverevasculitis（血管炎）ofallbloodvesselsArteria(动脉)Vena(静脉)Capillary(毛细血管)PATHOLOGYFeverRashofvariousforms（多形红斑）Mucosavary（粘膜变化）Cervicallymphnodeswelling（颈淋巴结肿大）Changesoftheperipheralextremities（周围四肢的变化）CLINICALMANIFESTATIONRashinvolvingthetrunkandtheextremitesRashofvar

4、iousformsSwellingofthehandsSwellingofthefeetPeriungualdesquamationtipsofthefingersandtoesStrawberrytongue,drycrackedlipserythemaConjunctivitiserythemaoftheoralandpharyngealmucosaUsuallyunilateralwithanodesizeof1.5cmorgreaterindiameterCervicallymphnodeswellingMildanemiaWBC↑,N↑,Ptnormal-↑ESR↑,CRP↑Hem

5、oglobinelectrophoresisα2↑CD4Tc↑,CD8Tc↓IgA↑,IgM↑,C3normal-↑LaboratoryexaminationTwo-dimensionalechocardiographyisthemostusefultesttomonitorthepotentialdevelopmentofcoronaryabnormalities※Feverlastingforatleast5daysPresenceofatleasefourofthefollowingfivesigns:☆Bilateralbulbarconjunctivalinjection,gene

6、rallynonpurulent☆changesinthemucosaoftheoropharynx,includinginjectedpharynx,injectedand/ordryfissuredlips,strawberrytongueDiagnosiscriteriaDiagnosiscriteria☆Changesoftheperipheralextremities,suchasedemaand/orerythemaofthehandsorfeetintheacutephase;orperiungualdesquamationinthesubacutephase☆Rash,pri

7、marilytruncal;poiymorphousbutnonvesicular☆Cervicaladenopathy,≥1.5cm,usuallyunilaterallymphadenopathyIllnessnotexplainedbyotherknowndiseaseprocess必备条件：原因不明的发热至少持续5天以上其他：应具备下列5项体征中的4项非渗出性球结膜充血口腔咽部改变四肢末端变化多形性皮