Immunodeficiency diseases.ppt

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1、ImmunodeficiencydiseasesWang,XiaochuanDepartmentofClinicalImmunology,Children’sHospitalofFudanUniversityPathogensEnvironmentMaterialImmunityInfectiousImmunityAllergyTumorImmunityAutoimmuneDiseasePIDImmunologicdisordersHumanImmunocompromisedHostIndividualwhohasoneormo

2、redefectsinthebody’snaturaldefensemechanismswhicharesufficientlyseveretopredisposethehosttolife-threateninginfectionandhighriskofautoimmunediseases,allergyandmalignancyPrimary-PID,damagetoanatomicalbarriersSecondary-infection,tumor,drug,othersPhysiology-Infants,agedC

3、haracteristicsofImmunoglobulinesIgGIgMIgAIgDIgESerumCon.(mg/dl)120012020030.01Percentage70-805-1010-15<1<0.01Halflife(days)21572.82.3Distribution(%)4580457550Quantity(mg/kg)1150492301.50.04Synthesis(mg/kg/D)357250.40.02Transportthroughplacenta+----DevelopmentofImmuno

4、globulinIgGfrommotherIgGofinfant6Mbirth100%IgGlevelofInfantAgedependentchangesofserumIgslevels(g/L)AgesIgGIgAIgMNeonate6.46-17.740.004-0.0170.05-0.271m-2.75-7.500.05-0.600.10-0.704m-3.70-8.300.14-0.500.33-1.257m-3.50-8.900.06-0.540.36-1.201y-5.52-11.460.06-0.740.60-2

5、.123y-4.95-12.740.33-0.890.65-2.017y-6.09-12.850.52-2.160.67-2.4612y-6.98-14.260.92-2.500.56-2.1815-18y7.54-16.020.89-3.240.72-2.28SchematicdiagramoftheexposureofmicroorganismduringearlylifefetusFulltern6MDaycarepathogensprobioticsPeriodofsusceptiblechildrenpremature

6、Fullterm6MDaycareManagementExclusionofPIDClinicalfeaturesFrequencyofinfectionSeverityComplicationsFollowupManagementGeneralmedicalcare(prevention、nutrition、exercise)ComplicationTreatmentMedicationAntibioticsImmuneregulatorImmunizations(PrimaryImmunodeficiencyDisease,

7、)PrimaryImmunodeficiencyDiseaseAgroupofdisorderscharacterizedbyanimpairedabilitytoproducenormalimmuneresponse.Mostofthesedisordersarecasedbymutationsingenesinvolvedinthedevelopmentandfunctionofimmuneorgans,cells,andmolecules.Clinicalfeatures：Recurrentinfection,highri

8、skofautoimmunediseases,allergyandmalignancy50%20%10%18%2%AntibodyComplementPhagocyteCellmediatedCombinedPredictivepatientsnumberofP