儿童贫血全英文.ppt

Anemiainchildhood(小儿贫血),Tounderstandfeaturesofhematopoiesisandbloodinchildren.Tocomprehendclinicalfeatures,diagnosisandtherapyofanemia.Tounderstandthedefinition,gradedivisionandclassificationofanemiainchildren.Tomasteretiology,pathogeny,diagnosis,therapyandpreventionofnutritionalirondeficiencyanemiaandnutritionalmegaloblasticanemia.,Diseaseofhematopoieticsystem,infantileanemia（1）nutritionalirondeficiencyanemia(IDA)（2）nutritionalmegaloblasticanemiaPrimary/immunitythrombocytopeniaPurpura(ITP)Leukemia,haematogenesisofchildren,hematopoiesis-producedbloodextramedullarybeforebirthandpostnatalmesoblasthepaticmedullary3-15w6w-6ms3ms,Embryostage,Mesoblastichaematogenesis：3wsbegin，8wsweaken,12-15wsdisappears。liver：8wsbegin，6monthsgraduallyweaken,erythroblast、granularcellandmegakaryocyte.,Embryostage,3、spleen：12wsbeginerythrocyte,granule,lymphocyte4、Haematogenesisoflymphaticorgan1.thoracicgland:8ws2.lymphaticnodes:11ws,Embryostage,5、myelo-haematopoiesis：6monsHaematogenesisfunctionemphasis，makevariouskindsofbloodcells，uniquehematogenicorganafterbirth.,Haematopoiesispostnatal,1、marrow：2、extramedullary：whenrequirementofhaemopoiesisincrease，liver、spleen、lymphadenectasis，hepatomegalyandsplenomegaly,incirculatingbloodimmatureerythrocytesandgranulocytes.,Physiologicalhaemolysis,Normalnewbornshavehigherhemoglobin(HB)andhematocritlevelsandashortenedsurvivalperiodofthefetalRBCscontributestothedevelopmentofphysiologicanemia.,Physiologicalhaemolysis,erythropoiesisabruptlyceaseswithonsetofrespirationatbirth,whenthearterialoxygensaturationrisestoward95%.levelsoferythropoietin(EPO)arelow.EPOhasadecreasedhalf-lifeandanincreasedvolumeofdistributioninnewborns.AshortenedsurvivalofthefetalRBCalsocontributestothedevelopmentofphysiologicanemia.thesizableexpansionofbloodvolumethataccompaniesrapidweightgainduringthefirst3mooflifeaddstotheneedforincreasedRBCproduction.,bloodcharacteristicsages,redbloodcells(RBC)andHbPhysiologicalhaemolysisandanemiawritebloodcells(WBC)andclassification4-6crossPlatelets150-250109/Lbloodvolume8-10%,Redbloodcell(RBC),Termnewbornshavearedcellmassthatishigherthanatanyothertimeoflife.anappropriateconditionforthelowoxygenenvironmentofintrauterinelife.TheRBCcountis5.010127.01012,hemoglobinconcentrationisabout150220g/Latbirth.TheRBCandhemoglobinconcentrationinpreterminfantsareslightlylowerthanthoseinterminfants.,Redbloodcell(RBC),Thewiderangeofhemoglobinconcentrationisaccountedforby:Variationinhowrapidlytheumbilicalcordisclamped.Aninfantspositionafterdelivery.Ifcordclampingisdelayedandthebabyisheldlowerthanplacenta,bothhemoglobinandbloodvolumeareincreasedbyaplacentaltransfusion.,ChangeofHBafterbirth,Reticulocyte,Reticulocyte,Reticulocyteis0.04-0.06inthefirst3days.Reticulocytedecreasesto0.005-0.015after4-7days.Reticulocyterisesto0.02-0.08in4-6weeks.Reticulocyteisequaltoanadultsafter5months.,Whitebloodcell(WBC),ThenormalnumberofWBCishigherininfancyandearlychildhoodthanlaterinlife.WBCcountis1510920109atbirth.After612hours,itriseto2110928109andthenbeginstodecreaseto12109by1week.WBCcountmaintainsabout10109atinfantperiodandapproachadultsWBCcountlevelby8years.,Whitebloodcell(WBC),ThechangeinWBCclassificationistheproportionbetweenlymphocyteandgranulocyte.Lymphocyteisabout30%andgranulocyteisabout65%atbirth,butthelaterlymphocytecontrarytoneutrophilegranulocytedecreases.Theproportionbetweenlymphocyteandgranulocyteisequalat46daysafterbirth.,Whitebloodcell(WBC),Lymphocyteisabout60%andgranulocyteisabout35%subsequently.Theyareequalat46years.After7yearswhitecellclassificationininfantsissimilartothatinadult.,4-6Days,Granulocyte,Lymphocyte,4-6years,ChangeofproportioninLymphocyteandGranulocyte,Plateletcount,Normalvaluefortheplateletcountareabout150250109/Landvarylittlewithage.,Bloodvolume,Bloodvolumeininfantsismorethaninadults.Thenewbornsbloodvolumeis10%ofhisweightandabout300mlonaverage.Achildsisabout8%10%ofhisweight.,Anemia,Defination:Anemiaisdefinedasareductionoftheredbloodcellvolumeorhemoglobinconcentrationbelowtherangeofvaluesoccurringinhealthypersons.Anemiaisanabsolutedecreaseinhematocrit,hemoglobinconcentration,ortheRBCcount.Anemiaisnotadiagnosis,butasignofunderlyingdisease.,Thecriteriaofanemia,Anemia,1.Classification1）degree:mildmoderatesevereVerysevere2）MorphologyofRBC3）Causes:lostblood,hemolytic,deficiencyofformingHbandRBC,degree,RBC(van/mm3)Hb(g/L)Mild300-40090-110Moderate200-30060-90Severe100-20030-60Verysevere1003232-38microcytosis802832-38meancorpuscularvolume(MCV),meanscorpuscularhemoglobin(MCH),meancorpuscularhemoglobinconcentration(MCHC),Causes,1.lostblood:acutechronic2.hemolysisIntrinsicmembranehereditaryspherocytosisGlycolysispyruvatekinasehemoglobinsicklecell,unstableHboxidationG6PDextrinsic:immune,infection,DIC,Causes,3.deficiencyofformingHbandRBCdeficiencyofhematopoiesissubstancemedullaryhematopoiesisdisorder(Aplasticanemia)Theinhibitionofhaematopoiesisinducedby:InflamationChronicnephritisToxicityCancercellsinvasionbonemarrow,Symptomsofanemia,Asymptomatic:particularlyiftheanemiadevelopsoveralongtime.Generalmanifestation:palloroftheskinandmucousmembranes,lethargy,malnutrition,growthretardation.liver,spleenandlymphnodesexpansion.Digestionsystem:anorexia,nauseaandconstipation.,Symptomsofanemia,Cardiovascularandrespiratorysystem:tachycardias,increasedarterypressure,wheezeandincreasedpulse.severeanemiamaycauseheartexpansionandcongestivecardiacfailure.Nerversystem:vertigo,tinnitus,irritability,anddisordersofattention.,2.Diagnosis,HistorypositivemanifestationlaboratorytestsBloodsmearBMHbananysisGrowthdevelopmentnutritionnailsfairsliverspleenandlymphnotes5points:age,course,symptoms,feeding,pastmedicalhistory,familyhistoryMorphologyofRBC,reticulocytecount,WBC,plateletcount,bonemarrowcellsmear,HB,specialexamination,3.Treatment,EliminationetiologyGeneralMedicineIntravenousbloodTransplantations:BM,stemcellsOther,nutritionalanemiawithmicrocytosisandhypochromia,Definitionnutritionalirondeficiencyanemia(IDA)Hb、mostcommon、6-24ms、specialprevention,Ironmetabolism,Ironcontentanddistribution:2/3oftheironispresentinHBand1/3intissueandtransportform.,Ironmetabolism,Ironabsorption:Theprimaryregulatorofironhomeostasisisintestinalironabsorption.Ironabsorptiontakesplaceprimarilyintheduodenumbytheenterocytesatthetipoftheintestinalvilla.Ironmustpassthoughtheapicalandthethenthebasolateralmembranesofthesecellstoreachthecirculation.,Ironmetabolism,Ironstorage:MostbodyironiscontainedinHB,withsmalleramountsboundtoferritin(铁蛋白)andhemosiderin（含铁血黄素）inthereticuloendothelialsystem,myoglobininmuscle,circulatingtransferring,andiron-containingenzymes.Themajorironstoresareintheformofferritin.Asironcontinuestoaccumulateinthecell,asecondstorageform,hemosiderinappears.,Ironmetabolism,Ironcharacteristics:Thefetusabsorbsironfromthemotheracrosstheplacenta.Terminfantshaveadequatereservesforthefirst4monthsoflife.Preterminfantshavelimitedironstoresandbecauseoftheirhigherrateofgrowth,theyoutstriptheirreservesby8weeksofage.,Ironmetabolism,Ironcharacteristics:Atbirth,becauseof“physiologicalhaemolysis”,muchironisreleasedtoplasmaandlittleironisabsorbedfromfood,Duringthesecondstage(about2monthsold),hematopoiesisisincreasedandmoreironisabsorbedfromfood,soirondeficiencyisrareinthisstage.After4months,developmentincrease,ironinfoodisdeficientandironstoresexhaust,somostirondeficiencyanemiaoccursin6monthsto2yearsor3yearsoldchild.,causes,1.inadequateironstores:preterminfant,akeirondeficiency3.growthanddevelopmentincreasedironrequirement4.ironabsorbabnormal5.aamountofironloss:hookworminfestation,repeatedvenesection,Meckelsdiverticulum,recurrentepistaxis(反复鼻出血).,pathogenesis,IRONHbmicrocytosisandhypochromiaRBC,Threestageofirondeficiency,Deficiencyofironprogressesinstagesirondepletion(ID):tissueironstoresaredeleted,undernormalcondition,thiscorrelatesdirectlywithdecreaseintheferritinlever,reticulocytepercentagedecreases.Irondeficienterythropoiesis(IDE):lossofcirculatingiron.Lowserumironlessthan30ug/dl,lowtransferringsaturationand/orelevatedtotalironbindingcapacity.,Threestageofirondeficiency,irondeficiencyanemia(IDA):irondeficiencyfollowingdepletionofbothmarrowstoreandcirculatingiron.,ID,IDE,IDA,clinicalmanifestation,1.generalmanifestation:mildirondeficiencyisAsymptomatic,palloroftheskinandmucousmebranesaremostevidentandlethargy,malnutrition,growthretardation.2.liverspleenandlymphnodesenlarge3.digestionsystem:anorexia（食欲差）,nausea（恶心）,constipation（便秘）.diarrhea,clinicalmanifestation,4.cardiovascularandrespiratorymanifestation:tachycardia,increasedarterypressure,wheeze,increasedpulse.Severeanemiamaycauseheartexpansionandcongestivecardiacfailure.5.nervoussystemmanifestation:vertigo,irritability.,clinicalmanifestation,Mainsignsmaybepalloroftheskinandmucousmembranes.Severeanemiamaycausecongestivecardiacfailure.IDAininfancyandearlychildhoodisassociatedwithdevelopmentaldelayandpoorgrowth.,laboratorytest,1.bloodsmear2.bonemarrow3.ironmetabolism,Inequalityofsizeoferythrocytes，smallcell，Centralolistherozoneobviously,hypercellular,erythroidhyperplasia,thedevelopmentofcytoplasmfallsbehindnucleus.leukocytesandmegakaryocytesarenormal.,Bonemarrowironstain：ferruginationgrainsintheerythocytes.,Normalbonemarrowironstain正常骨髓铁染色,IDAironstain铁缺乏骨髓铁染色,laboratorytest,ThedecreaseofHBconcentrationismorethanthedecreaseofredcellscount.Bloodsmearrevealsthemorefeatureofmicrocyteandhypochromia.MCV80fl,MCH26pg,MCHC0.31.Reticulocyteisnormalorslightlydecreases.WBCandplateletsarenormal.,Bloodcountinirondeficiency,laboratorytest,Bonemarrowrevealsincreasedbasophilicnormoblastandpolychromaticnormoblast.Granulocytesystemandmegakaryocytesystemarenormal.,Ironmetabolisms,Serumferritin(SF)(血清铁蛋白)Freeerythrocyteprotoporphyrin(FEP)Serumiron,totalironbindingcapacityIroninbonemarrow,Ironmetabolisms,diagnosis,firstconsider-history+clinicalmanifestation+bloodsmearDecidediagnosis-bonemarrow+ironmetabolismMaybeseetreatmentwithiron(Thebonemarrowishypercellular,witherythroidhyperplasia,thenormoblastsmayhavescanty,andthedevelopmentofcytoplasmfallsbehindoneofnucleus.leukocytesandmegakaryocytesarenormal.),treatment,1.erfusionsblood,Oraladministrationofsimpleferroussaltsferroussulfate(硫酸亚铁)ferrousgluconate（葡萄糖酸亚铁）ferrousfumaratepolysaccharideironDosage:4-6mg/kgelementalironperday,Oralironpreparation,Administrationtheironpriortomeals/betweentomeals.Administrationascorbicacidwithironpreparation.Therapeuticcourse:withdrawalofironpreparation6-8weeksafterhemoglobinrecovertonormallevelorwhenSF(Serumferritin)andFEP(Freeerythrocyteprotoporphyrin)isnormal.,Oralironpreparation,Parenteralironpreparation,Tobeadministeredonlyforgastrointestinalmalabsorptionorsevereintolerancepreventseffectiveoralirontherapy.,Parenteralironpreparation,Aparenteralironpreparation(irondextran)isaneffectiveformofironandisusuallysafewhengiveninaproperlycalculateddose,buttheresponsetoparenteralironisnomorerapidorcompletethanthatobtainedwithproperoraladministrationofiron,unlessmalabsorptionisafactor.,BloodTransfusion,Withasevereanemia,immediateredbloodcelltransfusionmayadvisable,especiallyincardiacfailureorsevereinfection,butvolumeandspeedoftransfusionmustbecontrolledwell.Wemaytransfuse,severelyanemiachildrenshouldbegivenonly2-3ml/kgofpackedcellsatanyonetime.Ifthereisevidenceoffrankcongestivefailure,amodifiedexchangetransfusionusingfresh-packedRBCsshouldbeconsidered.,Irontherapy,Notice:3points1.Injectionironindanger2.Reaction:12-24h(irritability,appetite)-36-48h(erythroidhyperplasia)-48-72h(reticulocytosis)-5-7ds(peaking)2-3wstoreticulocytes3.Times:6-8ws,Prevention,4pointsmothermilkfeedingspecterfoodwithironpreterminfant,Nutritionalmegaloblasticanemia,FolicacidandvitaminB12deficiencyareprimarycausesofmegaloblasticanemia.,Theclinicalfeaturesincludeanemia，thedecreaseofredcellismorethanthatofHB，thevolumeofredcellislargerthannormal.,Causes,1.lessintake2.absorbabnormal3.druginteractions4.requirementincreased,Pathogenesis,folicacidfolicacidwith4hydratevitaminB12DNAHbverylargeRBCMegaloblasticwithLotofHb,dihydrofolatereductase,(THFA),VitaminB12isimportanceinsynthesisofnerve.deficiencyofvitaminB12canleadtodiscordofneurologypsychology.InthemacrocyticanemiaproducedbydeficiencyofvitaminB12,thesymptomsandsignsincludethoseofanemiaandneuropathy.,VitaminB12deficiency,neurologypsychologysymptom,Patientsdevelopademyelinatinglesionofneuronsofthespinalcolumnandcerebralcortex.Thisconditionresultsinparesthesiasofthehandsandfeet,unsteadinessofgait,andeventuallymemorylossandpersonalitychanges.Thereisretardofintellectiveandphysicaldevelopment.TremblingofExtremitiesorhead,hypertensionofmuscle,tendonreflexreinforcement,positiveBabinskissignmayappear.,Clinicalmanifestation,1.Generalfeatures:puffiness,poornutrition,hairyellowed,mildedema,petechia(plt),mucocutaneoushemorrhage.2.featureofanemia:lethargy,extramedullary3.neurologypsychology:irritability,vertigo.4.digestivesymptoms:anorexia,nausea,diarrhea.,Laboratorytests,1.bloodsmear2.bonemarrow3.bloodbiochemistrytests4.others,variationinBRCshapeandsize,macrocytosis,reticulocytecountislow,nucleatedRBCsandmegaloblasticmorphologyareoftenseen,thrombocytopenia,Hypercellular,Megaloblasticchanges,hypersegmentation,Laboratorytests,Bloodroutineexamination:macrocyticanemia,thedecreaseofredcellcountismorethanthedecreaseofHB.MCV94fl,MCH32pg.Rreticulocyteisdecrease.WBCandplateletsarealsodecreased.Bonemarrow:increasedbasophilicnormoblastandpolychromaticnormoblastic.Granulocyticsystemandmegakaryocytesystem:normal/lessthannormal.,Laboratorytests,VitamineB12:normalserumvitaminB12levelsrangefrom200-800ng/L,B1212ng/LrevealsB12deficiency.Folate:normalserumfolatelevelsrangefrom5-6ug/L,folate3ug/Lrevealsdeficiency.others:LDH:serumlacticdehydrogenase(LDH)isincrease,Diagnosis,firstconsider-history+clinicalmanifestationMarkedsymptomsandsignsofcentralnervoussyste