新生儿疾病-Neonatal Jaundice lwz英文最终篇

HemolyticDiseaseoftheNewbornLiweizhongIntroductionHemolyticdiseaseofthenewbornItisanisoimmunityhemolysisassociatedwithABOorRhincompatibility.ItresultsfromtransplacentalpassageofmaternalantiboddyactiveagainstRBCantigensoftheinfant,leadingtoanincreasedrateofRBCdestruction.Itisanimportantcauseofanemiaandjaundiceinnewborninfant.EtiologyandPathogenesisABOhemolyticdiseaseABOincompatibilityTypeOmothersTypeAorBfetusesPresenceofIgGanti-AorAnti-BantibodiesintypeOmotherFrequentlyoccurringduringthefirstpregnancywithoutpriorsensitizationEtiologyandPathogenesisRhhemolyticdiseaseRhbloodgroupantigens(C,c,D,d,E,e)DECcePathophysiologyofalloimmunehemolysisresultingfromRhincompatibilityAnRh-negativemotherAnRh-positivefetusLeakageoffetalRBCintomaternalcirculationMaternalsensitizationtoDantigenonfetalRBCEtiologyandPathogenesisProductionandtransplacentalpassageofmaternalanti-DantibodiesintofetalcirculationAttachmentofmaternalantibodiestoRh-positivefetalRBCDestructionofantibody-coatedfetalRBCEtiologyandPathogenesisRhhemolyticdiseasewasrareduringthefirstpregnancyinvolvinganRh-positivefetus.Oncesensitizationhasoccurred,re-exposuretoRhDRBCinsubsequentpregnanciesleadstoananamnesticresponse,withanincreaseinthematernalanti-RhDantibodytiter.Thelikelihoodofaninfantbeingaffectedincreasedsignificantlywitheachsubsequentpregnancy.EtiologyandPathogenesisSignificanthemolysisoccurringinthefirstpregnancyindicatespriormaternalexposuretoRh-positiveRBC.FetalbleedingassociatedwithapreviousspontaneousortherapeuticabortionEctopicpregnancyAvarietyofdifferentprenatalproceduresTransfusionofsomeotherbloodproductcontainingRhDRBCinanRh-negativemotherClinicalManifestationsJaundiceAnemiaHydropsMassiveenlargementoftheliverandspleenBilirubinencephalopathy(Kernicterus)ClinicalManifestationsClinicalFeaturesOfHemolyticDiseaseClinicalFeaturesRhABOFrequencyUnusualCommonAnemiaMarkedMinimalJaundiceMarkedMinimaltomoderateHydropsCommonRareHepatosplenomegalyMarkedMinimalKernicterusCommonRareLaboratoryDiagnosisLaboratoryFeaturesOfHemolyticDiseaseLaboratoryFeaturesRhABObloodtypeofMotherRhnegativeObloodtypeofInfantRhpositiveAorBAnemiaMarkedMinimalDirectCommb’stestPositiveNegativeIndirectCommb’stestPositiveUsuallypositiveHyperbilirubinemiamarkedVariableRBCmorphologyNucleatedRBCSpherocytesDiagnosisThedefinitivediagnosisrequiresdemonstrationofbloodgroupincompatibilityandofcorrespondingantibodyboundtotheinfant’sRBC.DiagnosisAntenatalDiagnosisHistoryExpectantparents’bloodtypesMaternaltiterofIgGantibodiestoDorE(1:32)At12～16wkAt28～32wkAt36wkFetalRhandABOstatusFetaljaundicelevelDiagnosisPostnataldiagnosisJaundiceat24hrAnemia(Hematocritandhemoglobinexamination)RhorABOincompatibilityCoomb’stestpositiveExaminationforRBCantibodiesinthemother’sserumDifferentialDiagnosisCongenitalnephrosisNeonatalanemiaPhysiologicaljaundiceTreatmentMaingoalsTopreventintrauterineorextrauterinedeathoffetalorinfantformsevereanemiaandhypoxicToavoidneurotoxicityfromhyperbilirubinemiaTreatmentTreatmentoftheunborninfantUterotransfusionIndicationHydropsAnemia(Hematocrit30%)MethodPackedRBCmatchingwiththemother’sserumUmbilicalveintransfusionTreatmentDeliveryinadvanceIndicationPulmonarymaturityFetaldistressMaternaltiterofRhantibodies1:3235～37wkofgestationTreatmentTreatmentoftheliveborninfantImmediateresuscitationandsupportivetherapyTemperaturestabilizationCorrectionofacidosis:1-2mEq/kgofsodiumbicarbonateAsmalltransfusioncompatiblepackedRBCVolumeexpansionforhypotensionProvisionofassistedventilationforrespiratoryfailureTreatmentPhototherapyBluespectrumof427-475nm(orWhiteorGreen)Irradiance:10-12μW/cm2ProtectionofeyesandgenitalIndicationBilirubin≥10mg/dlat＜12hrBilirubin≥12-14mg/dlat＜18hrBilirubin≥15mg/dlat≥24hrTreatmentSideeffectofphototherapyDiarrheaDehydrationRiboflavindestructionHypocalcemiaBronze-babysyndromeTreatmentExchangetransfusionIndicationHemoglobin＜120g/LHydrops,hepatosplenomegalyandheartfailureBilirubininthe1st12oflife0.75mg/dl/hrBilirubinconcentration20mg/dlFactorssupportingearlyexchangetransfusion:Previouskernicterusinasibling,reticulocytecountsgreaterthan15%,asphyxiaofneonateandprematureinfantTreatmentBloodvolumeofexchangetransfusionDouble-volumeexchangetransfusion:150-180ml/kgBloodchooseofRhincompatibilityRhinaccordancewithmotherABOinaccordancewithneonateBloodchooseofABOincompatibilityPlasmofABtypeRBCofOtypeTreatmentDrugtreatmentIntravenousimmuneglobulin(IVIG)HumanalbuminProtoporphyrins:Sn-PP;Zn-PPGlucocorticoids:DexamethasoneInducerofliverenzyme:LuminalPreventionIntramuscularinjectionof300ugofhumananti-DglobulintoanRh-negativemotherWithin72hrofdeliveryofanectopicpregnancyAbdominaltraumainpregnancyAmniocentesisChorionicvillusbiopsyAbortion