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1、RheumatoidDiseaseinChildren小儿风湿性疾病Children’sHospitalofFudanUniversityXuHonghxu@shmu.edu.cnRheumatoidDiseaseConnectivetissuediseases（包含有120种与关节、骨骼、肌肉等相关的疾病）过敏性紫癜（anaphylactoidpurpura）类风湿性关节炎（JRA）SLE（systemiclupuserythematosis）皮肌炎（dermatomyositis）硬皮病（scleroderm
2、a）风湿热（rheumaticfever）川崎病(Kawasakidisease)RheumatoidDiseaseinChildrenThemostcommondiseases：JuvenileRheumatiodArthritisHenoch-SchonleinpurpuraKawasakiDisease(MucocutaneousLymphNodeSyndrome,MCLS)RheumaticFever冠状动脉造影CoronaryangiogramHenoch-SchonleinpurpuraAlterna
3、tivenames:AnaphylactoidpurpuraVascularpurpuraDefinitionHenoch-Schonleinpurpuraisadiseasethathasthesymptomspurplespotsontheskinjointpaingastrointestinalsymptomsglomerulonephritis(atypeofkidneydisorder)HistorystorySchonlein'sPurpura(In1837,JohanSchonlein)theski
4、nandjointsareaffectedbutthegastrointestinaltractisnotHenoch'sPurpura(Later,hisstudent,EduardHenoch)purpuraspotsontheskinandacuteabdominalproblemsnotaffectedbyjointdiseaseGeneralconditionsatypeofhypersensitivityvasculitisandinflammatoryresponsewithinthebloodve
5、sselItiscausedbyanabnormalresponseoftheimmunesystemTheexactcauseforthisdisorderisunknown.Thesyndromeisusuallyseeninchildren,butpeopleofanyagemaybeaffectedItismorecommoninboysthaningirlsManypeoplewithHenoch-Schonleinpurpurahadanupperrespiratoryillnessintheprev
6、iousweeksCausesandpathogenesisAninflammatorydiseaseofthesmallbloodvessels(capillaries)usuallyaself-limiteddiseaseItisthemostcommonformofchildhoodvascularinflammation(vasculitis)andresultsininflammatorychangesinsmallbloodvesselsCommonallergensincludeMedication
7、s(suchascertainantibiotics,antihistaminesandthiazidediuretics)Pathogens(bacterium/virus/parasite,suchasgroupAstrep,varicella,parvovirusandhepatitisB)AnacuterespiratoryinfectionCommonallergensincludefoods(suchasberries,shellfish,fish,nuts,eggs,milk,andothers)p
8、ollenanimaldander(scalesofshedskin)insectbitesCertainvaccinations,suchasformeasles,yellowfeverandcholeraPathophysiologyasmall-vesselvasculitischaracterizedbyimmunoglobulinA(IgA),C3,andimm
