《儿童贫血全英》PPT课件.ppt

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1、Anemiainchildhood(小儿贫血)Diseaseofhematopoieticsysteminfantileanemia（1）nutritionalirondeficiencyanemia(IDA)（2）nutritionalmegaloblasticanemiaPrimary/immunitythrombocytopeniaPurpura(ITP)Leukemiahaematogenesisofchildrenhematopoiesis-producedbloodextramedullarybeforebirthandpostnatalmesoblasthepaticmedull

2、ary3-15w6w-6ms3msEmbryostageMesoblastichaematogenesis：3wsbegin，8wsweaken,12-15wsdisappears。liver：8wsbegin，6monthsgraduallyweaken,erythroblast、granularcellandmegakaryocyte.Embryostage3、spleen：12wsbeginerythrocyte,granule,lymphocyte4、Haematogenesisoflymphaticorgan1.thoracicgland:8ws2.lymphaticnodes:11

3、wsEmbryostage5、myelo-haematopoiesis：6monsHaematogenesisfunctionemphasis，makevariouskindsofbloodcells，uniquehematogenicorgan afterbirth.Haematopoiesispostnatal1、marrow：2、extramedullary：whenrequirementofhaemopoiesisincrease，liver、spleen、lymphadenectasis，hepatomegalyandsplenomegaly,incirculatingbloodim

4、matureerythrocytesandgranulocytes.PhysiologicalhaemolysisNormalnewbornshavehigherhemoglobin(HB)andhematocritlevelsandashortenedsurvivalperiodofthefetalRBCscontributestothedevelopmentofphysiologicanemia.Physiologicalhaemolysiserythropoiesisabruptlyceaseswithonsetofrespirationatbirth,whenthearterialox

5、ygensaturationrisestoward95%.levelsoferythropoietin(EPO)arelow.EPOhasadecreasedhalf-lifeandanincreasedvolumeofdistributioninnewborns.AshortenedsurvivalofthefetalRBCalsocontributestothedevelopmentofphysiologicanemia.thesizableexpansionofbloodvolumethatacpaniesrapidweightgainduringthefirst3mooflifeadd

6、stotheneedforincreasedRBCproduction.bloodcharacteristicsagesredbloodcells(RBC)andHbPhysiologicalhaemolysisandanemiawritebloodcells(WBC)andclassification4-6crossPlatelets150-250109/Lbloodvolume8-10%Redbloodcell(RBC)Termnewbornshavearedcellmassthatishigherthanatanyothertimeoflife.anappropriateconditio

7、nforthelowoxygenenvironmentofintrauterinelife.TheRBCcountis5.010127.01012,hemoglobinconcentrationisabout150220g/Latbirth.TheRBCandhemoglobinconcentrationinpreterminfantsareslightlylowerthanthoseinterminfants.Redbloodcell(RBC)Thewiderangeofhemoglobinconcentrationisaccountedforby:Variationinhowrapidly

8、theumbilicalcordisclamped.Aninfantspositionafterdelivery.Ifcordclampingisdelayedandthebabyisheldlowerthanplacenta,bothhemoglobinandbloodvolumeareincreasedbyaplacentaltransfusion.ChangeofHBafterbirthReticulocyteReticulocyteReticulocyteis0.04-0.06inthefirst3days.Reticulocytedecreasesto0.005-0.015after

9、4-7days.Reticulocyterisesto0.02-0.08in4-6weeks.Reticulocyteisequaltoanadultsafter5months.Whitebloodcell(WBC)ThenormalnumberofWBCishigherininfancyandearlychildhoodthanlaterinlife.WBCcountis1510920109atbirth.After612hours,itriseto2110928109andthenbeginstodecreaseto12109by1week.WBCcountmaintainsabout10

10、109atinfantperiodandapproachadultsWBCcountlevelby8years.Whitebloodcell(WBC)ThechangeinWBCclassificationistheproportionbetweenlymphocyteandgranulocyte.Lymphocyteisabout30%andgranulocyteisabout65%atbirth,butthelaterlymphocytecontrarytoneutrophilegranulocytedecreases.Theproportionbetweenlymphocyteandgr

11、anulocyteisequalat46daysafterbirth.Whitebloodcell(WBC)Lymphocyteisabout60%andgranulocyteisabout35%subsequently.Theyareequalat46years.After7yearswhitecellclassificationininfantsissimilartothatinadult.4-6DaysGranulocyteLymphocyte4-6yearsChangeofproportioninLymphocyteandGranulocytePlateletcountNormalva

12、luefortheplateletcountareabout150250109/Landvarylittlewithage.BloodvolumeBloodvolumeininfantsismorethaninadults.Thenewbornsbloodvolumeis10%ofhisweightandabout300mlonaverage.Achildsisabout8%10%ofhisweight.AnemiaDefination:Anemiaisdefinedasareductionoftheredbloodcellvolumeorhemoglobinconcentrationbelo

13、wtherangeofvaluesoccurringinhealthypersons.Anemiaisanabsolutedecreaseinhematocrit,hemoglobinconcentration,ortheRBCcount.Anemiaisnotadiagnosis,butasignofunderlyingdisease.ThecriteriaofanemiaAgeHbconcentration28days145g/L14months90g/L46months100g/L6months6years110g/L614years120g/LAnemia1.Classificatio

14、n1）degree:mildmoderatesevereVerysevere2）MorphologyofRBC3）Causes:lost blood,hemolytic,deficiencyofformingHbandRBCdegreeRBC(van/mm3)Hb(g/L)Mild300-40090-110Moderate200-30060-90Severe100-20030-60Verysevere10030Morphologynanemia with microcytosis and hypochromianAnemia with macrocytosisnAnemia with norm

15、alcytosis AnemiaMoreanemiaMCVMCHMCHCNormal80-9428-3232-38Micro-hypochromia8028943232-38microcytosis802832-38meancorpuscularvolume(MCV),meanscorpuscularhemoglobin(MCH),meancorpuscularhemoglobinconcentration(MCHC)Causes1.lostblood:acutechronic2.hemolysisIntrinsicmembranehereditaryspherocytosisGlycolys

16、ispyruvatekinasehemoglobinsicklecell,unstableHboxidationG6PDextrinsic:immune,infection,DICCauses3.deficiencyofformingHbandRBCdeficiencyofhematopoiesissubstancemedullaryhematopoiesisdisorder(Aplasticanemia)Theinhibitionofhaematopoiesisinducedby:InflamationChronicnephritisToxicityCancercellsinvasionbo

17、nemarrowSymptomsofanemiaAsymptomatic:particularlyiftheanemiadevelopsoveralongtime.Generalmanifestation:palloroftheskinandmucousmembranes,lethargy,malnutrition,growthretardation.liver,spleenandlymphnodesexpansion.Digestionsystem:anorexia,nauseaandconstipation.SymptomsofanemiaCardiovascularandrespirat

18、orysystem:tachycardias,increasedarterypressure,wheezeandincreasedpulse.severeanemiamaycauseheartexpansionandcongestivecardiacfailure.Nerversystem:vertigo,tinnitus,irritability,anddisordersofattention.2.DiagnosisHistorypositivemanifestationlaboratorytestsBloodsmearBMHbananysisGrowth development nutri

19、tion nails fairs liverspleenandlymphnotes5 points:age,course,symptoms,feeding,pastmedicalhistory,familyhistoryMorphologyofRBC,reticulocytecount,WBC,plateletcount,bonemarrowcellsmear,HB,specialexamination3.TreatmentEliminationetiologyGeneralMedicineIntravenousbloodTransplantations:BM,stemcellsOthernu

20、tritionalanemiawithmicrocytosisandhypochromiaDefinitionnutritionalirondeficiencyanemia(IDA)Hb、mostmon、6-24ms、specialpreventionIronmetabolismIroncontentanddistribution:2/3oftheironispresentinHBand1/3intissueandtransportform.Contentofelementaliron(mg/kg)Adultfemales40Adultmales50newborn75Ironmetabolis

21、mIronabsorption:Theprimaryregulatorofironhomeostasisisintestinalironabsorption.Ironabsorptiontakesplaceprimarilyintheduodenumbytheenterocytesatthetipoftheintestinalvilla.Ironmustpassthoughtheapicalandthethenthebasolateralmembranesofthesecellstoreachthecirculation.IronmetabolismIronstorage:Mostbodyir

22、oniscontainedinHB,withsmalleramountsboundtoferritin(铁蛋白)andhemosiderin（含铁血黄素）inthereticuloendothelialsystem,myoglobininmuscle,circulatingtransferring,andiron-containingenzymes.Themajorironstoresareintheformofferritin.Asironcontinuestoaccumulateinthecell,asecondstorageform,hemosiderinappears.Ironmeta

23、bolismIroncharacteristics:Thefetusabsorbsironfromthemotheracrosstheplacenta.Terminfantshaveadequatereservesforthefirst4monthsoflife.Preterminfantshavelimitedironstoresandbecauseoftheirhigherrateofgrowth,theyoutstriptheirreservesby8weeksofage.IronmetabolismIroncharacteristics:Atbirth,becauseof“physio

24、logicalhaemolysis”,muchironisreleasedtoplasmaandlittleironisabsorbedfromfood,Duringthesecondstage(about2monthsold),hematopoiesisisincreasedandmoreironisabsorbedfromfood,soirondeficiencyisrareinthisstage.After4months,developmentincrease,ironinfoodisdeficientandironstoresexhaust,somostirondeficiencyan

25、emiaoccursin6monthsto2yearsor3yearsoldchild.causes1.inadequateironstores:preterminfant,twin2.intakeirondeficiency3.growthanddevelopmentincreasedironrequirement4.ironabsorbabnormal5.a amount of iron loss:hookworm infestation,repeated venesection,Meckels diverticulum,recurrentepistaxis(反复鼻出血).pathogen

26、esisIRONHbmicrocytosisandhypochromiaRBCThreestageofirondeficiencyDeficiencyofironprogressesinstagesirondepletion(ID):tissueironstoresaredeleted,undernormalcondition,thiscorrelatesdirectlywithdecreaseintheferritinlever,reticulocytepercentagedecreases.Iron deficient erythropoiesis(IDE):loss of circula

27、tingiron.Lowserumironlessthan30ug/dl,lowtransferringsaturationand/orelevatedtotalironbindingcapacity.Threestageofirondeficiencyirondeficiencyanemia(IDA):irondeficiencyfollowing depletion of both marrow store andcirculatingiron.IDIDEIDAclinicalmanifestation1.general manifestation:mild iron deficiency

28、 isAsymptomatic,pallor of the skin and mucousmebranesaremostevidentandlethargy,malnutrition,growthretardation.2.liverspleenandlymphnodesenlarge3.digestionsystem:anorexia（食欲差）,nausea（恶心）,constipation（便秘）.diarrheaclinicalmanifestation4.cardiovascularandrespiratorymanifestation:tachycardia,increasedart

29、erypressure,wheeze,increasedpulse.Severeanemiamaycauseheartexpansionandcongestivecardiacfailure.5.nervoussystemmanifestation:vertigo,irritability.clinicalmanifestationMainsignsmaybepalloroftheskinandmucousmembranes.Severeanemiamaycausecongestivecardiacfailure.IDAininfancyandearlychildhoodisassociate

30、dwithdevelopmentaldelayandpoorgrowth.laboratorytest1.bloodsmear2.bonemarrow3.ironmetabolismInequality of size of erythrocytes，small cell，Central olistherozone obviously hypercellular,erythroidhyperplasia,thedevelopmentofcytoplasmfallsbehindnucleus.leukocytesandmegakaryocytesarenormal.Bonemarrowirons

31、tain：ferruginationgrainsintheerythocytes.Normalbonemarrowironstain正常骨髓铁染色正常骨髓铁染色IDAironstain铁缺乏骨髓铁染色铁缺乏骨髓铁染色laboratorytestThedecreaseofHBconcentrationismorethanthedecreaseofredcellscount.Bloodsmearrevealsthemorefeatureofmicrocyteandhypochromia.MCV80fl,MCH26pg,MCHC0.31.Reticulocyteisnormalorslightlyd

32、ecreases.WBCandplateletsarenormal.BloodcountinirondeficiencyHB75g/L120g/LRBC3.541012/L4.241012/LMCV64fl86flMCHC18.5pg32pgreticulocyte1.3%1.4%WBC7.54109/L7.64109/Lproportionnormalnormalplatelet254109/L257109/LlaboratorytestBonemarrowrevealsincreasedbasophilicnormoblastandpolychromaticnormoblast.Granu

33、locytesystemandmegakaryocytesystemarenormal.IronmetabolismsSerumferritin(SF)(血清铁蛋白)Freeerythrocyteprotoporphyrin(FEP)Serumiron,totalironbindingcapacityIroninbonemarrowIronmetabolismsIronstudyIDIDEIDASerumferritin(SF)IronstoreRedbloodcellprotoporphyrin(FEP)NPercentsideroblastsNSerumironNN/diagnosisfi

34、rst consider-history+clinicalmanifestation+bloodsmearDecidediagnosis-bonemarrow+ironmetabolismMaybeseetreatmentwithiron(Thebonemarrowishypercellular,witherythroidhyperplasia,the normoblasts may have scanty,and thedevelopment of cytoplasm falls behind one of nucleus.leukocytesandmegakaryocytesarenorm

35、al.)treatment1.nursingfeeding2.getridofetiology3.ironmedicine4.interfusionsbloodOral administration of simple ferrous salts ferrous sulfate(硫酸亚铁)ferrous gluconate（葡萄糖酸亚铁）ferrous fumaratepolysaccharide iron Dosage:4-6mg/kg elemental iron per day Oral iron preparation Administration the iron prior to

36、meals/between to meals.Administration ascorbic acid with iron preparation.Therapeutic course:withdrawal of iron preparation 6-8 weeks after hemoglobin recover to normal level or when SF(Serum ferritin)and FEP(Free erythrocyte protoporphyrin)is normal.Oral iron preparationParenteral iron preparation

37、Tobeadministeredonlyforgastrointestinal malabsorption or severeintolerance prevents effective oral irontherapy.Parenteral iron preparationA parenteral iron preparation(iron dextran)is an effective form of iron and is usually safe when given in a properly calculated dose,but the response to parentera

38、l iron is no more rapid or plete than that obtained with proper oral administration of iron,unless malabsorption is a factor.BloodTransfusionWith a severe anemia,immediate red blood cell transfusion may advisable,especially in cardiac failure or severe infection,but volume and speed of transfusion m

39、ust be controlled well.We may transfuse,severely anemia children should be given only 2-3ml/kg of packed cells at any one time.If there is evidence of frank congestive failure,a modified exchange transfusion using fresh-packed RBCs should be considered.IrontherapyNotice:3 points1.Injection iron in d

40、anger 2.Reaction:12-24h(irritability,appetite)-36-48h(erythroid hyperplasia)-48-72h(reticulocytosis)-5-7ds(peaking)2-3ws to reticulocytes3.Times:6-8wsPrevention4 pointsmother milk feeding specter food with iron preterm infantNutritionalmegaloblasticanemia Folic acid and vitamin B12 deficiency are pr

41、imary causes of megaloblastic anemia.The clinical features include anemia，the decrease of red cell is more than that of HB，the volume of red cell is larger than normal.Causes1.lessintake2.absorbabnormal3.druginteractions4.requirementincreasedPathogenesisfolicacidfolicacidwith4hydratevitaminB12DNAHbv

42、erylargeRBCMegaloblasticwithLotofHbdihydrofolatereductase(THFA)VitaminB12isimportanceinsynthesisofnerve.deficiencyofvitaminB12canleadtodiscordofneurologypsychology.InthemacrocyticanemiaproducedbydeficiencyofvitaminB12,thesymptomsandsignsincludethoseofanemiaandneuropathy.nVitaminB12deficiencyneurolog

43、ypsychologysymptomPatientsdevelopademyelinatinglesionofneuronsofthespinalcolumnandcerebralcortex.Thisconditionresultsinparesthesiasofthehandsandfeet,unsteadinessofgait,andeventuallymemorylossandpersonalitychanges.Thereisretardofintellectiveandphysicaldevelopment.TremblingofExtremitiesorhead,hyperten

44、sionofmuscle,tendonreflexreinforcement,positiveBabinskissignmayappear.Clinicalmanifestation1.General features:puffiness,poor nutrition,hair yellowed,mild edema,petechia(plt),mucocutaneoushemorrhage.2.featureofanemia:lethargy,extramedullary3.neurologypsychology:irritability,vertigo.4.digestive sympto

45、ms:anorexia,nausea,diarrhea.Laboratorytests1.bloodsmear2.bonemarrow3.bloodbiochemistrytests4.othersvariation in BRC shape and size,macrocytosis,reticulocyte count is low,nucleated RBCs and megaloblastic morphology are often seen,thrombocytopenia Hypercellular,Megaloblastic changes,hypersegmentation

46、LaboratorytestsBloodroutineexamination:macrocyticanemia,thedecreaseofredcellcountismorethanthedecreaseofHB.MCV94fl,MCH32pg.Rreticulocyteisdecrease.WBCandplateletsarealsodecreased.Bonemarrow:increasedbasophilicnormoblastandpolychromaticnormoblastic.Granulocyticsystemandmegakaryocytesystem:normal/less

47、thannormal.LaboratorytestsVitamineB12:normalserumvitaminB12levelsrangefrom200-800ng/L,B1212ng/LrevealsB12deficiency.Folate:normalserumfolatelevelsrangefrom5-6ug/L,folate3ug/Lrevealsdeficiency.others:LDH:serumlacticdehydrogenase(LDH)isincreaseDiagnosisfirst consider-history+clinicalmanifestationMarke

48、d symptoms and signs ofcentralnervoussystem.(itsupportsdefiencyofvitaminB12.)+.bloodsmeardecidediagnosis-.bonemarrow+metabolism(TodistinguishthedeficiencyoffolicacidwiththedeficiencyofvitaminB12.)maybeseetreatmentwithmedicineTreatment1.nursingfeeding2.getridofetiology3.medicinevitB12,folicacidVitami

49、nB12preparationVit B12 preparation to treat vit B12 deficiency.Not to use folic acid preparation in patients with vitB12 deficiency only.Intramuscular administration of vit B12 0.5-1 mg，QW or 100g，BiW，usually with reticulocytosis in 2-4 days,unless there is concurrent inflammatory disease.If there i

50、s evidence of neurologic involvement,1 mg should be injected intramuscularly daily for at least 2 wk.Maintenance therapy is necessary throughout a patients life；monthly intramuscular administration of 1 mg of vit B12 is sufficient.Folicacidpreparation.Folic acid may be administered orally in a dose