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Anemiainchildhood

(小兒(xiǎoér)貧血)

第一頁,共九十四頁。編輯課件第二頁,共九十四頁。編輯課件Diseaseof

hematopoieticsysteminfantileanemia(1)nutritionalirondeficiencyanemia(IDA)(2)nutritionalmegaloblasticanemiaPrimary/immunitythrombocytopeniaPurpura(ITP)Leukemia第三頁,共九十四頁。編輯課件haematogenesisofchildrenhematopoiesis--producedblood

extramedullary

beforebirthandpostnatal

mesoblasthepaticmedullary3-15w6w-6ms3ms

第四頁,共九十四頁。編輯課件Embryostage

Mesoblastichaematogenesis:3wsbegin,8wsweaken,12-15wsdisappears。liver:8wsbegin,6monthsgraduallyweaken,erythroblast、granularcellandmegakaryocyte.第五頁,共九十四頁。編輯課件Embryostage3、spleen:12wsbeginerythrocyte,granule,lymphocyte4、Haematogenesisoflymphaticorgan1.thoracicgland:8ws2.lymphaticnodes:11ws第六頁,共九十四頁。編輯課件Embryostage5、myelo-haematopoiesis:6monsHaematogenesisfunctionemphasis,makevariouskindsofbloodcells,uniquehematogenicorgan

afterbirth.第七頁,共九十四頁。編輯課件第八頁,共九十四頁。編輯課件Haematopoiesispostnatal

1、marrow:2、extramedullary:when

requirementofhaemopoiesisincrease,liver、spleen、lymphadenectasis,hepatomegalyandsplenomegaly,

incirculatingbloodimmature

erythrocytesandgranulocytes.

第九頁,共九十四頁。編輯課件PhysiologicalhaemolysisNormalnewbornshavehigherhemoglobin(HB)andhematocritlevelsandashortenedsurvivalperiodofthefetalRBCscontributes

tothedevelopmentofphysiologicanemia.第十頁,共九十四頁。編輯課件Physiologicalhaemolysiserythropoiesisabruptlyceaseswithonsetofrespirationatbirth,whenthearterialoxygensaturationrisestoward95%.levelsoferythropoietin(EPO)arelow.EPOhasadecreasedhalf-lifeandanincreasedvolumeofdistributioninnewborns.AshortenedsurvivalofthefetalRBCalsocontributestothedevelopmentofphysiologicanemia.thesizableexpansionofbloodvolumethataccompaniesrapidweightgainduringthefirst3mooflifeaddstotheneedforincreasedRBCproduction.第十一頁,共九十四頁。編輯課件bloodcharacteristics–ages

redbloodcells(RBC)andHbPhysiologicalhaemolysisandanemiawritebloodcells(WBC)andclassification4-6crossPlatelets150-250×109/Lbloodvolume8-10%第十二頁,共九十四頁。編輯課件Redbloodcell(RBC)Termnewbornshavearedcellmassthatishigherthanatanyothertimeoflife.anappropriateconditionforthelowoxygenenvironmentofintrauterinelife.TheRBCcountis5.0×1012~7.0×1012,hemoglobinconcentrationisabout150~220g/Latbirth.TheRBCandhemoglobinconcentrationinpreterminfantsareslightlylowerthanthoseinterminfants.第十三頁,共九十四頁。編輯課件Redbloodcell(RBC)Thewiderangeofhemoglobinconcentrationisaccountedforby:Variationinhowrapidlytheumbilicalcordisclamped.Aninfant’spositionafterdelivery.Ifcordclampingisdelayedandthebabyisheldlowerthanplacenta,bothhemoglobinandbloodvolumeareincreasedbyaplacentaltransfusion.第十四頁,共九十四頁。編輯課件ChangeofHBafterbirth第十五頁,共九十四頁。編輯課件Reticulocyte第十六頁,共九十四頁。編輯課件ReticulocyteReticulocyteis0.04-0.06inthefirst3days.Reticulocytedecreasesto0.005-0.015after4-7days.Reticulocyterisesto0.02-0.08in4-6weeks.Reticulocyteisequaltoanadult’safter5months.第十七頁,共九十四頁。編輯課件Whitebloodcell(WBC)ThenormalnumberofWBCishigherininfancyandearlychildhoodthanlaterinlife.WBCcountis15×109~20×109atbirth.After6~12hours,itriseto21×109~28×109andthenbeginstodecreaseto12×109by1week.WBCcountmaintainsabout10×109atinfantperiodandapproachadult’sWBCcountlevelby8years.第十八頁,共九十四頁。編輯課件Whitebloodcell(WBC)

ThechangeinWBCclassificationistheproportionbetweenlymphocyteandgranulocyte.Lymphocyteisabout30%andgranulocyteisabout65%atbirth,butthelaterlymphocytecontrarytoneutrophilegranulocytedecreases.Theproportionbetweenlymphocyteandgranulocyteisequalat4~6daysafterbirth..第十九頁,共九十四頁。編輯課件Whitebloodcell(WBC)Lymphocyteisabout60%andgranulocyteisabout35%subsequently.Theyareequalat4~6years.After7yearswhitecellclassificationininfantsissimilartothatinadult.第二十頁,共九十四頁。編輯課件4-6DaysGranulocyteLymphocyte4-6yearsChangeofproportioninLymphocyteandGranulocyte第二十一頁,共九十四頁。編輯課件Plateletcount

Normalvaluefortheplateletcountareabout150~250×109/Landvarylittlewithage.第二十二頁,共九十四頁。編輯課件BloodvolumeBloodvolumeininfantsismorethaninadults.Thenewborn’sbloodvolumeis10%ofhisweightandabout300mlonaverage.Achild’sisabout8%~10%ofhisweight.第二十三頁,共九十四頁。編輯課件AnemiaDefination:

Anemiaisdefinedasareductionoftheredbloodcellvolumeorhemoglobinconcentrationbelowtherangeofvaluesoccurringinhealthypersons.Anemiaisanabsolutedecreaseinhematocrit,hemoglobinconcentration,ortheRBCcount.

Anemiaisnotadiagnosis,butasignofunderlyingdisease.第二十四頁,共九十四頁。編輯課件ThecriteriaofanemiaAgeHbconcentration<28days<145g/L1~4months<90g/L4~6months<100g/L6months~6years<110g/L6~14years<120g/L第二十五頁,共九十四頁。編輯課件

Anemia1.

Classification1)

degree:mildmoderatesevere

Verysevere

2)

MorphologyofRBC3)Causes:lostblood,hemolytic,deficiencyofformingHbandRBC第二十六頁,共九十四頁。編輯課件degreeRBC(van/mm3)Hb(g/L)Mild300-40090-110Moderate200-30060-90Severe100-20030-60Verysevere<100<30第二十七頁,共九十四頁。編輯課件MorphologyanemiawithmicrocytosisandhypochromiaAnemiawithmacrocytosisAnemiawithnormalcytosisAnemia第二十八頁,共九十四頁。編輯課件Moreanemia

MCVMCHMCHCNormal80-9428-3232-38Micro-hypochromia<80<28<32Macrocytosis>94>3232-38microcytosis<80<2832-38

meancorpuscularvolume(MCV),meanscorpuscularhemoglobin(MCH),meancorpuscularhemoglobinconcentration(MCHC)第二十九頁,共九十四頁。編輯課件Causes1.lostblood:acutechronic2.hemolysis

IntrinsicmembranehereditaryspherocytosisGlycolysispyruvatekinasehemoglobinsicklecell,unstableHboxidationG6PDextrinsic:immune,infection,DIC第三十頁,共九十四頁。編輯課件Causes3.deficiencyofformingHbandRBCdeficiencyofhematopoiesissubstancemedullaryhematopoiesisdisorder(Aplasticanemia)Theinhibitionofhaematopoiesisinducedby:InflamationChronicnephritisToxicityCancercellsinvasionbonemarrow

第三十一頁,共九十四頁。編輯課件SymptomsofanemiaAsymptomatic:particularlyiftheanemiadevelopsoveralongtime.Generalmanifestation:palloroftheskinandmucousmembranes,lethargy,malnutrition,growthretardation.liver,spleenandlymphnodesexpansion.Digestionsystem:anorexia,nauseaandconstipation.第三十二頁,共九十四頁。編輯課件SymptomsofanemiaCardiovascularandrespiratorysystem:tachycardias,increasedarterypressure,wheezeandincreasedpulse.

severeanemiamaycauseheartexpansionandcongestivecardiacfailure.Nerversystem:vertigo,tinnitus,irritability,anddisordersofattention.

第三十三頁,共九十四頁。編輯課件2.

DiagnosisHistory–positivemanifestation–laboratorytests

BloodsmearBMHbananysis

Growthdevelopmentnutritionnailsfairsliverspleenandlymphnotes

5points:

age,course,symptoms,feeding,pastmedicalhistory,familyhistory

MorphologyofRBC,reticulocytecount,WBC,plateletcount,bonemarrowcellsmear,HB,specialexamination第三十四頁,共九十四頁。編輯課件

3.TreatmentEliminationetiologyGeneralMedicineIntravenousbloodTransplantations:BM,stemcellsOther第三十五頁,共九十四頁。編輯課件

nutritionalanemiawithmicrocytosisandhypochromia

Definitionnutritionalirondeficiencyanemia(IDA)Hb、mostcommon、6-24ms、specialprevention第三十六頁,共九十四頁。編輯課件IronmetabolismIroncontentanddistribution:

2/3oftheironispresentinHBand1/3intissueandtransportform.Contentofelementaliron(mg/kg)Adultfemales40Adultmales50newborn75第三十七頁,共九十四頁。編輯課件IronmetabolismIronabsorption:Theprimaryregulatorofironhomeostasisisintestinalironabsorption.Ironabsorptiontakesplaceprimarilyintheduodenumbytheenterocytesatthetipoftheintestinalvilla.Ironmustpassthoughtheapicalandthethenthebasolateralmembranesofthesecellstoreachthecirculation.第三十八頁,共九十四頁。編輯課件IronmetabolismIronstorage:MostbodyironiscontainedinHB,withsmalleramountsboundtoferritin(鐵蛋白)andhemosiderin(含鐵血(tiěxuè)黃素)

inthereticuloendothelialsystem,myoglobininmuscle,circulatingtransferring,andiron-containingenzymes.Themajorironstoresareintheformofferritin.Asironcontinuestoaccumulateinthecell,asecondstorageform,hemosiderinappears.第三十九頁,共九十四頁。編輯課件IronmetabolismIroncharacteristics:Thefetusabsorbsironfromthemotheracrosstheplacenta.Terminfantshaveadequatereservesforthefirst4monthsoflife.Preterminfantshavelimitedironstoresandbecauseoftheirhigherrateofgrowth,theyoutstriptheirreservesby8weeksofage.第四十頁,共九十四頁。編輯課件IronmetabolismIroncharacteristics:Atbirth,becauseof“physiologicalhaemolysis”,muchironisreleasedtoplasmaandlittleironisabsorbedfromfood,Duringthesecondstage(about2monthsold),hematopoiesisisincreasedandmoreironisabsorbedfromfood,soirondeficiencyisrareinthisstage.After4months,developmentincrease,ironinfoodisdeficientandironstoresexhaust,somostirondeficiencyanemiaoccursin6monthsto2yearsor3yearsoldchild.第四十一頁,共九十四頁。編輯課件causes1.inadequateironstores:preterminfant,akeirondeficiency3.growthanddevelopmentincreasedironrequirement4.ironabsorbabnormal5.aamountofironloss:hookworminfestation,repeatedvenesection,Meckel’sdiverticulum,recurrentepistaxis(反復(fǎnfù)鼻出血).第四十二頁,共九十四頁。編輯課件pathogenesisIRONHb

microcytosisandhypochromiaRBC

第四十三頁,共九十四頁。編輯課件ThreestageofirondeficiencyDeficiencyofironprogressesinstagesirondepletion(ID):tissueironstoresaredeleted,undernormalcondition,thiscorrelates

directlywithdecreaseintheferritinlever,reticulocytepercentagedecreases.

Irondeficienterythropoiesis(IDE):lossofcirculatingiron.Lowserumironlessthan30ug/dl,lowtransferringsaturationand/orelevatedtotalironbindingcapacity.第四十四頁,共九十四頁。編輯課件Threestageofirondeficiencyirondeficiencyanemia(IDA):irondeficiencyfollowingdepletionofbothmarrowstoreandcirculatingiron.IDIDEIDA第四十五頁,共九十四頁。編輯課件clinicalmanifestation1.

generalmanifestation:mildirondeficiencyisAsymptomatic,palloroftheskinandmucousmebranesaremostevidentandlethargy,malnutrition,growthretardation.2.

liverspleenandlymphnodesenlarge3.digestionsystem:

anorexia(食欲差),nausea(惡心(ěxīn)),constipation(便秘).diarrhea

第四十六頁,共九十四頁。編輯課件clinicalmanifestation4.cardiovascularandrespiratorymanifestation:tachycardia,increasedarterypressure,wheeze,increasedpulse.Severeanemiamaycauseheartexpansionandcongestivecardiacfailure.

5.nervoussystemmanifestation:vertigo,irritability.第四十七頁,共九十四頁。編輯課件clinicalmanifestationMainsignsmaybepalloroftheskinandmucousmembranes.Severeanemiamaycausecongestivecardiacfailure.IDAininfancyandearlychildhoodisassociatedwithdevelopmentaldelayandpoorgrowth.第四十八頁,共九十四頁。編輯課件laboratorytest1.bloodsmear2.bonemarrow3.ironmetabolism

第四十九頁,共九十四頁。編輯課件Inequalityofsizeoferythrocytes,smallcell,Centralolistherozoneobviously第五十頁,共九十四頁。編輯課件hypercellular,erythroidhyperplasia,thedevelopmentofcytoplasmfallsbehindnucleus.leukocytesandmegakaryocytesarenormal.第五十一頁,共九十四頁。編輯課件Bonemarrowironstain:ferruginationgrainsintheerythocytes.Normalbonemarrowironstain正常(zhèngcháng)骨髓鐵染色IDAironstain鐵缺乏骨髓(ɡǔsuǐ)鐵染色第五十二頁,共九十四頁。編輯課件laboratorytestThedecreaseofHBconcentrationismorethanthedecreaseofredcellscount.Bloodsmearrevealsthemorefeatureofmicrocyteandhypochromia.MCV<80fl,MCH<26pg,MCHC<0.31.Reticulocyteisnormalorslightlydecreases.WBCandplateletsarenormal..第五十三頁,共九十四頁。編輯課件BloodcountinirondeficiencyHB75g/L120g/LRBC3.54×1012/L4.24×1012/LMCV64fl86flMCHC18.5pg32pgreticulocyte1.3%1.4%WBC7.54×109/L7.64×109/Lproportionnormalnormalplatelet254×109/L257×109/L第五十四頁,共九十四頁。編輯課件laboratorytestBonemarrowrevealsincreasedbasophilicnormoblastandpolychromaticnormoblast.Granulocytesystemandmegakaryocytesystemarenormal.第五十五頁,共九十四頁。編輯課件IronmetabolismsSerumferritin(SF)(血清(xuèqīng)鐵蛋白)Freeerythrocyteprotoporphyrin(FEP)Serumiron,totalironbindingcapacityIroninbonemarrow第五十六頁,共九十四頁。編輯課件IronmetabolismsIronstudyID

IDEIDASerumferritin(SF)↓↓↓↓↓Ironstore↓↓↓↓↓Redbloodcellprotoporphyrin(FEP)N

↑↑↑PercentsideroblastsN↓↓↓SerumironNN/↓↓↓第五十七頁,共九十四頁。編輯課件diagnosis

firstconsider---history+clinicalmanifestation+bloodsmear

Decidediagnosis---bonemarrow+ironmetabolismMaybeseetreatmentwithiron

(Thebonemarrowishypercellular,witherythroidhyperplasia,thenormoblastsmayhavescanty,andthedevelopmentofcytoplasmfallsbehindoneofnucleus.leukocytesandmegakaryocytesarenormal.)第五十八頁,共九十四頁。編輯課件

treatment1.

nursingfeeding2.

getridofetiology3.

ironmedicine4.

interfusions

blood第五十九頁,共九十四頁。編輯課件Oraladministrationofsimpleferroussalts

ferroussulfate(硫酸亞鐵(liúsuānyàtiě))

ferrousgluconate(葡萄糖酸亞鐵)

ferrousfumarate

polysaccharideiron

Dosage:4-6mg/kgelementalironperday

Oralironpreparation第六十頁,共九十四頁。編輯課件Administrationtheironpriortomeals/betweentomeals.Administrationascorbicacidwithironpreparation.

Therapeuticcourse:withdrawalofironpreparation6-8weeksafterhemoglobinrecovertonormallevelorwhenSF(Serum

ferritin)andFEP(Free

erythrocyteprotoporphyrin)isnormal.

Oralironpreparation第六十一頁,共九十四頁。編輯課件Parenteraliron

preparation

Tobeadministeredonlyforgastrointestinalmalabsorptionorsevereintolerancepreventseffectiveoralirontherapy.第六十二頁,共九十四頁。編輯課件Parenteraliron

preparation

Aparenteralironpreparation(irondextran)isaneffectiveformofironandisusuallysafewhengiveninaproperlycalculateddose,buttheresponsetoparenteralironisnomorerapidorcompletethanthatobtainedwithproperoraladministrationofiron,unlessmalabsorptionisafactor.第六十三頁,共九十四頁。編輯課件BloodTransfusion

Withasevereanemia,immediateredbloodcelltransfusionmayadvisable,especiallyincardiacfailureorsevereinfection,butvolumeandspeedoftransfusionmustbecontrolledwell.Wemaytransfuse,severelyanemiachildrenshouldbegivenonly2-3ml/kgofpackedcellsatanyonetime.Ifthereisevidenceoffrankcongestivefailure,amodifiedexchangetransfusionusingfresh-packedRBCsshouldbeconsidered.第六十四頁,共九十四頁。編輯課件IrontherapyNotice:3points1.Injectionironindanger2.Reaction:12-24h(irritability

↓,appetite↑)---36-48h(erythroidhyperplasia)---48-72h(reticulocytosis↑)---5-7ds(peaking)2-3wstoreticulocytes3.Times:6-8ws第六十五頁,共九十四頁。編輯課件Prevention4points—mothermilkfeedingspecterfoodwithiron–preterminfant第六十六頁,共九十四頁。編輯課件

Nutritionalmegaloblasticanemia

FolicacidandvitaminB12deficiencyareprimarycausesofmegaloblasticanemia.第六十七頁,共九十四頁。編輯課件Theclinicalfeaturesincludeanemia,thedecreaseofredcellismorethanthatofHB,thevolumeofredcellislargerthannormal.第六十八頁,共九十四頁。編輯課件Causes1.lessintake2.absorbabnormal3.druginteractions4.requirementincreased第六十九頁,共九十四頁。編輯課件Pathogenesis

folicacidfolicacidwith4hydrate

vitaminB12

DNA

Hb

verylargeRBCMegaloblasticwithLotofHb

dihydrofolatereductase

(THFA)第七十頁,共九十四頁。編輯課件VitaminB12isimportanceinsynthesisofnerve.deficiencyofvitaminB12canleadtodiscordofneurologypsychology.InthemacrocyticanemiaproducedbydeficiencyofvitaminB12,thesymptomsandsignsincludethoseofanemiaandneuropathy.VitaminB12deficiency

neurologypsychologysymptom第七十一頁,共九十四頁。編輯課件Patientsdevelopademyelinatinglesionofneuronsofthespinalcolumnandcerebralcortex.Thisconditionresultsinparesthesiasofthehandsandfeet,unsteadinessofgait,andeventuallymemorylossandpersonalitychanges.Thereisretardofintellectiveandphysicaldevelopment.TremblingofExtremitiesorhead,hypertensionofmuscle,tendonreflexreinforcement,positiveBabinski'ssignmayappear.第七十二頁,共九十四頁。編輯課件Clinicalmanifestation1.Generalfeatures:puffiness,poornutrition,hairyellowed,mildedema,petechia(plt↓),mucocutaneoushemorrhage.2.featureofanemia:lethargy,extramedullary3.

neurologypsychology:irritability,vertigo.4.

digestivesymptoms:anorexia,nausea,diarrhea.第七十三頁,共九十四頁。編輯課件Laboratorytests

1.bloodsmear2.bonemarrow3.bloodbiochemistrytests4.others第七十四頁,共九十四頁。編輯課件variationinBRCshapeandsize,macrocytosis,reticulocytecountislow,nucleatedRBCsandmegaloblasticmorphologyareoftenseen,thrombocytopenia

第七十五頁,共九十四頁。編輯課件Hypercellular,Megaloblasticchanges,hypersegmentation第七十六頁,共九十四頁。編輯課件LaboratorytestsBloodroutineexamination:macrocyticanemia,thedecreaseofredcellcountismorethanthedecreaseofHB.MCV>94fl,MCH>32pg.Rreticulocyteisdecrease.WBCandplateletsarealsodecreased.Bonemarrow:increasedbasophilicnormoblastandpolychromaticnormoblastic.Granulocyticsystemandmegakaryocytesystem:normal/lessthannormal.第七十七頁,共九十四頁。編輯課件LaboratorytestsVitamineB12:

normalserumvitaminB12levelsrangefrom200-800ng/L,B12<12ng/LrevealsB12deficiency.Folate:normalserumfolatelevelsrangefrom5-6ug/L,folate<3ug/Lrevealsdeficiency.others:LDH:

serumlacticdehydrogenase(LDH)isincrease第七十八頁,共九十四頁。編輯課件Diagnosisfirstconsider---history+clinicalmanifestation[Markedsymptomsandsignsofcentralnervoussystem.(itsupportsdefiencyofvitaminB12.)]+.bloodsmeardecidediagnosis---.bonemarrow+metabolism(TodistinguishthedeficiencyoffolicacidwiththedeficiencyofvitaminB12.)maybeseetreatmentwithmedicine

第七十九頁,共九十四頁。編輯課件Treatment1.nursingfeeding2.getridofetiology3.medicinevitB12,folicacid

第八十頁,共九十四頁。編輯課件VitaminB12preparation

VitB12preparationtotreatvitB12deficiency.Nottousefolicacidpreparatio

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