《儿童贫血全英》PPT课件.ppt

Anemiainchildhood(小儿贫血)Diseaseofhematopoieticsysteminfantileanemia（1）nutritionalirondeficiencyanemia(IDA)（2）nutritionalmegaloblasticanemiaPrimary/immunitythrombocytopeniaPurpura(ITP)Leukemiahaematogenesisofchildrenhematopoiesis-producedbloodextramedullarybeforebirthandpostnatalmesoblasthepaticmedullary3-15w6w-6ms3msEmbryostageMesoblastichaematogenesis：3wsbegin，8wsweaken,12-15wsdisappears。liver：8wsbegin，6monthsgraduallyweaken,erythroblast、granularcellandmegakaryocyte.Embryostage3、spleen：12wsbeginerythrocyte,granule,lymphocyte4、Haematogenesisoflymphaticorgan1.thoracicgland:8ws2.lymphaticnodes:11wsEmbryostage5、myelo-haematopoiesis：6monsHaematogenesisfunctionemphasis，makevariouskindsofbloodcells，uniquehematogenicorgan afterbirth.Haematopoiesispostnatal1、marrow：2、extramedullary：whenrequirementofhaemopoiesisincrease，liver、spleen、lymphadenectasis，hepatomegalyandsplenomegaly,incirculatingbloodimmatureerythrocytesandgranulocytes.PhysiologicalhaemolysisNormalnewbornshavehigherhemoglobin(HB)andhematocritlevelsandashortenedsurvivalperiodofthefetalRBCscontributestothedevelopmentofphysiologicanemia.Physiologicalhaemolysiserythropoiesisabruptlyceaseswithonsetofrespirationatbirth,whenthearterialoxygensaturationrisestoward95%.levelsoferythropoietin(EPO)arelow.EPOhasadecreasedhalf-lifeandanincreasedvolumeofdistributioninnewborns.AshortenedsurvivalofthefetalRBCalsocontributestothedevelopmentofphysiologicanemia.thesizableexpansionofbloodvolumethatacpaniesrapidweightgainduringthefirst3mooflifeaddstotheneedforincreasedRBCproduction.bloodcharacteristicsagesredbloodcells(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.ChangeofHBafterbirthReticulocyteReticulocyteReticulocyteis0.04-0.06inthefirst3days.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.4-6DaysGranulocyteLymphocyte4-6yearsChangeofproportioninLymphocyteandGranulocytePlateletcountNormalvaluefortheplateletcountareabout150250109/Landvarylittlewithage.BloodvolumeBloodvolumeininfantsismorethaninadults.Thenewbornsbloodvolumeis10%ofhisweightandabout300mlonaverage.Achildsisabout8%10%ofhisweight.AnemiaDefination:Anemiaisdefinedasareductionoftheredbloodcellvolumeorhemoglobinconcentrationbelowtherangeofvaluesoccurringinhealthypersons.Anemiaisanabsolutedecreaseinhematocrit,hemoglobinconcentration,ortheRBCcount.Anemiaisnotadiagnosis,butasignofunderlyingdisease.ThecriteriaofanemiaAgeHbconcentration28days145g/L14months90g/L46months100g/L6months6years110g/L614years120g/LAnemia1.Classification1）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 normalcytosis AnemiaMoreanemiaMCVMCHMCHCNormal80-9428-3232-38Micro-hypochromia8028943232-38microcytosis802832-38meancorpuscularvolume(MCV),meanscorpuscularhemoglobin(MCH),meancorpuscularhemoglobinconcentration(MCHC)Causes1.lostblood:acutechronic2.hemolysisIntrinsicmembranehereditaryspherocytosisGlycolysispyruvatekinasehemoglobinsicklecell,unstableHboxidationG6PDextrinsic:immune,infection,DICCauses3.deficiencyofformingHbandRBCdeficiencyofhematopoiesissubstancemedullaryhematopoiesisdisorder(Aplasticanemia)Theinhibitionofhaematopoiesisinducedby:InflamationChronicnephritisToxicityCancercellsinvasionbonemarrowSymptomsofanemiaAsymptomatic:particularlyiftheanemiadevelopsoveralongtime.Generalmanifestation:palloroftheskinandmucousmembranes,lethargy,malnutrition,growthretardation.liver,spleenandlymphnodesexpansion.Digestionsystem:anorexia,nauseaandconstipation.SymptomsofanemiaCardiovascularandrespiratorysystem:tachycardias,increasedarterypressure,wheezeandincreasedpulse.severeanemiamaycauseheartexpansionandcongestivecardiacfailure.Nerversystem:vertigo,tinnitus,irritability,anddisordersofattention.2.DiagnosisHistorypositivemanifestationlaboratorytestsBloodsmearBMHbananysisGrowth development nutrition nails fairs liverspleenandlymphnotes5 points:age,course,symptoms,feeding,pastmedicalhistory,familyhistoryMorphologyofRBC,reticulocytecount,WBC,plateletcount,bonemarrowcellsmear,HB,specialexamination3.TreatmentEliminationetiologyGeneralMedicineIntravenousbloodTransplantations:BM,stemcellsOthernutritionalanemiawithmicrocytosisandhypochromiaDefinitionnutritionalirondeficiencyanemia(IDA)Hb、mostmon、6-24ms、specialpreventionIronmetabolismIroncontentanddistribution:2/3oftheironispresentinHBand1/3intissueandtransportform.Contentofelementaliron(mg/kg)Adultfemales40Adultmales50newborn75IronmetabolismIronabsorption:Theprimaryregulatorofironhomeostasisisintestinalironabsorption.Ironabsorptiontakesplaceprimarilyintheduodenumbytheenterocytesatthetipoftheintestinalvilla.Ironmustpassthoughtheapicalandthethenthebasolateralmembranesofthesecellstoreachthecirculation.IronmetabolismIronstorage:MostbodyironiscontainedinHB,withsmalleramountsboundtoferritin(铁蛋白)andhemosiderin（含铁血黄素）inthereticuloendothelialsystem,myoglobininmuscle,circulatingtransferring,andiron-containingenzymes.Themajorironstoresareintheformofferritin.Asironcontinuestoaccumulateinthecell,asecondstorageform,hemosiderinappears.IronmetabolismIroncharacteristics:Thefetusabsorbsironfromthemotheracrosstheplacenta.Terminfantshaveadequatereservesforthefirst4monthsoflife.Preterminfantshavelimitedironstoresandbecauseoftheirhigherrateofgrowth,theyoutstriptheirreservesby8weeksofage.IronmetabolismIroncharacteristics:Atbirth,becauseof“physiologicalhaemolysis”,muchironisreleasedtoplasmaandlittleironisabsorbedfromfood,Duringthesecondstage(about2monthsold),hematopoiesisisincreasedandmoreironisabsorbedfromfood,soirondeficiencyisrareinthisstage.After4months,developmentincrease,ironinfoodisdeficientandironstoresexhaust,somostirondeficiencyanemiaoccursin6monthsto2yearsor3yearsoldchild.causes1.inadequateironstores:preterminfant,twin2.intakeirondeficiency3.growthanddevelopmentincreasedironrequirement4.ironabsorbabnormal5.a amount of iron loss:hookworm infestation,repeated venesection,Meckels diverticulum,recurrentepistaxis(反复鼻出血).pathogenesisIRONHbmicrocytosisandhypochromiaRBCThreestageofirondeficiencyDeficiencyofironprogressesinstagesirondepletion(ID):tissueironstoresaredeleted,undernormalcondition,thiscorrelatesdirectlywithdecreaseintheferritinlever,reticulocytepercentagedecreases.Iron deficient erythropoiesis(IDE):loss of circulatingiron.Lowserumironlessthan30ug/dl,lowtransferringsaturationand/orelevatedtotalironbindingcapacity.Threestageofirondeficiencyirondeficiencyanemia(IDA):irondeficiencyfollowing depletion of both marrow store andcirculatingiron.IDIDEIDAclinicalmanifestation1.general manifestation:mild iron deficiency 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,increasedarterypressure,wheeze,increasedpulse.Severeanemiamaycauseheartexpansionandcongestivecardiacfailure.5.nervoussystemmanifestation:vertigo,irritability.clinicalmanifestationMainsignsmaybepalloroftheskinandmucousmembranes.Severeanemiamaycausecongestivecardiacfailure.IDAininfancyandearlychildhoodisassociatedwithdevelopmentaldelayandpoorgrowth.laboratorytest1.bloodsmear2.bonemarrow3.ironmetabolismInequality of size of erythrocytes，small cell，Central olistherozone obviously hypercellular,erythroidhyperplasia,thedevelopmentofcytoplasmfallsbehindnucleus.leukocytesandmegakaryocytesarenormal.Bonemarrowironstain：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.Reticulocyteisnormalorslightlydecreases.WBCandplateletsarenormal.BloodcountinirondeficiencyHB75g/L120g/LRBC3.541012/L4.241012/LMCV64fl86flMCHC18.5pg32pgreticulocyte1.3%1.4%WBC7.54109/L7.64109/Lproportionnormalnormalplatelet254109/L257109/LlaboratorytestBonemarrowrevealsincreasedbasophilicnormoblastandpolychromaticnormoblast.Granulocytesystemandmegakaryocytesystemarenormal.IronmetabolismsSerumferritin(SF)(血清铁蛋白)Freeerythrocyteprotoporphyrin(FEP)Serumiron,totalironbindingcapacityIroninbonemarrowIronmetabolismsIronstudyIDIDEIDASerumferritin(SF)IronstoreRedbloodcellprotoporphyrin(FEP)NPercentsideroblastsNSerumironNN/diagnosisfirst consider-history+clinicalmanifestation+bloodsmearDecidediagnosis-bonemarrow+ironmetabolismMaybeseetreatmentwithiron(Thebonemarrowishypercellular,witherythroidhyperplasia,the normoblasts may have scanty,and thedevelopment of cytoplasm falls behind one of nucleus.leukocytesandmegakaryocytesarenormal.)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 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 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 parenteral 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 must 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 danger 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 primary 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.requirementincreasedPathogenesisfolicacidfolicacidwith4hydratevitaminB12DNAHbverylargeRBCMegaloblasticwithLotofHbdihydrofolatereductase(THFA)VitaminB12isimportanceinsynthesisofnerve.deficiencyofvitaminB12canleadtodiscordofneurologypsychology.InthemacrocyticanemiaproducedbydeficiencyofvitaminB12,thesymptomsandsignsincludethoseofanemiaandneuropathy.nVitaminB12deficiencyneurologypsychologysymptomPatientsdevelopademyelinatinglesionofneuronsofthespinalcolumnandcerebralcortex.Thisconditionresultsinparesthesiasofthehandsandfeet,unsteadinessofgait,andeventuallymemorylossandpersonalitychanges.Thereisretardofintellectiveandphysicaldevelopment.TremblingofExtremitiesorhead,hypertensionofmuscle,tendonreflexreinforcement,positiveBabinskissignmayappear.Clinicalmanifestation1.General features:puffiness,poor nutrition,hair yellowed,mild edema,petechia(plt),mucocutaneoushemorrhage.2.featureofanemia:lethargy,extramedullary3.neurologypsychology:irritability,vertigo.4.digestive symptoms: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 LaboratorytestsBloodroutineexamination:macrocyticanemia,thedecreaseofredcellcountismorethanthedecreaseofHB.MCV94fl,MCH32pg.Rreticulocyteisdecrease.WBCandplateletsarealsodecreased.Bonemarrow:increasedbasophilicnormoblastandpolychromaticnormoblastic.Granulocyticsystemandmegakaryocytesystem:normal/lessthannormal.LaboratorytestsVitamineB12:normalserumvitaminB12levelsrangefrom200-800ng/L,B1212ng/LrevealsB12deficiency.Folate:normalserumfolatelevelsrangefrom5-6ug/L,folate3ug/Lrevealsdeficiency.others:LDH:serumlacticdehydrogenase(LDH)isincreaseDiagnosisfirst consider-history+clinicalmanifestationMarked symptoms and signs ofcentralnervoussystem.(itsupportsdefiencyofvitaminB12.)+.bloodsmeardecidediagnosis-.bonemarrow+metabolism(TodistinguishthedeficiencyoffolicacidwiththedeficiencyofvitaminB12.)maybeseetreatmentwithmedicineTreatment1.nursingfeeding2.getridofetiology3.medicinevitB12,folicacidVitaminB12preparationVit 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 is 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