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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.thesizableexpansionofbloodvolumethataccompaniesrapidweightgainduringthefirst3mooflife
6、addstotheneedforincreasedRBCproduction.bloodcharacteristicsagesredbloodcells(RBC)andHbPhysiologicalhaemolysisandanemiawritebloodcells(WBC)andclassification4-6crossPlatelets150-250109/Lbloodvolume8-10%Redbloodcell(RBC)Termnewbornshavearedcellmassthatishigherthanatanyothertimeoflife.anappropriatecondi
7、tionforthelowoxygenenvironmentofintrauterinelife.TheRBCcountis5.010127.01012,hemoglobinconcentrationisabout150220g/Latbirth.TheRBCandhemoglobinconcentrationinpreterminfantsareslightlylowerthanthoseinterminfants.Redbloodcell(RBC)Thewiderangeofhemoglobinconcentrationisaccountedforby:Variationinhowrapi
8、dlytheumbilicalcordisclamped.Aninfantspositionafterdelivery.Ifcordclampingisdelayedandthebabyisheldlowerthanplacenta,bothhemoglobinandbloodvolumeareincreasedbyaplacentaltransfusion.ChangeofHBafterbirthReticulocyteReticulocyteReticulocyteis0.04-0.06inthefirst3days.Reticulocytedecreasesto0.005-0.015af
9、ter4-7days.Reticulocyterisesto0.02-0.08in4-6weeks.Reticulocyteisequaltoanadultsafter5months.Whitebloodcell(WBC)ThenormalnumberofWBCishigherininfancyandearlychildhoodthanlaterinlife.WBCcountis1510920109atbirth.After612hours,itriseto2110928109andthenbeginstodecreaseto12109by1week.WBCcountmaintainsabou
10、t10109atinfantperiodandapproachadultsWBCcountlevelby8years.Whitebloodcell(WBC)ThechangeinWBCclassificationistheproportionbetweenlymphocyteandgranulocyte.Lymphocyteisabout30%andgranulocyteisabout65%atbirth,butthelaterlymphocytecontrarytoneutrophilegranulocytedecreases.Theproportionbetweenlymphocytean
11、dgranulocyteisequalat46daysafterbirth.Whitebloodcell(WBC)Lymphocyteisabout60%andgranulocyteisabout35%subsequently.Theyareequalat46years.After7yearswhitecellclassificationininfantsissimilartothatinadult.4-6DaysGranulocyteLymphocyte4-6yearsChangeofproportioninLymphocyteandGranulocytePlateletcountNorma
12、lvaluefortheplateletcountareabout150250109/Landvarylittlewithage.BloodvolumeBloodvolumeininfantsismorethaninadults.Thenewbornsbloodvolumeis10%ofhisweightandabout300mlonaverage.Achildsisabout8%10%ofhisweight.AnemiaDefination:Anemiaisdefinedasareductionoftheredbloodcellvolumeorhemoglobinconcentrationb
13、elowtherangeofvaluesoccurringinhealthypersons.Anemiaisanabsolutedecreaseinhematocrit,hemoglobinconcentration,ortheRBCcount.Anemiaisnotadiagnosis,butasignofunderlyingdisease.ThecriteriaofanemiaAgeHbconcentration28days145g/L14months90g/L46months100g/L6months6years110g/L614years120g/LAnemia1.Classifica
14、tion1）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 w
15、ith normalcytosis AnemiaMoreanemiaMCVMCHMCHCNormal80-9428-3232-38Micro-hypochromia8028943232-38microcytosis802832-38meancorpuscularvolume(MCV),meanscorpuscularhemoglobin(MCH),meancorpuscularhemoglobinconcentration(MCHC)Causes1.lostblood:acutechronic2.hemolysisIntrinsicmembranehereditaryspherocytosis
16、Glycolysispyruvatekinasehemoglobinsicklecell,unstableHboxidationG6PDextrinsic:immune,infection,DICCauses3.deficiencyofformingHbandRBCdeficiencyofhematopoiesissubstancemedullaryhematopoiesisdisorder(Aplasticanemia)Theinhibitionofhaematopoiesisinducedby:InflamationChronicnephritisToxicityCancercellsin
17、vasionbonemarrowSymptomsofanemiaAsymptomatic:particularlyiftheanemiadevelopsoveralongtime.Generalmanifestation:palloroftheskinandmucousmembranes,lethargy,malnutrition,growthretardation.liver,spleenandlymphnodesexpansion.Digestionsystem:anorexia,nauseaandconstipation.SymptomsofanemiaCardiovascularand
18、respiratorysystem:tachycardias,increasedarterypressure,wheezeandincreasedpulse.severeanemiamaycauseheartexpansionandcongestivecardiacfailure.Nerversystem:vertigo,tinnitus,irritability,anddisordersofattention.2.DiagnosisHistorypositivemanifestationlaboratorytestsBloodsmearBMHbananysisGrowth developme
19、nt nutrition nails fairs liverspleenandlymphnotes5 points: age, course, symptoms, feeding, pastmedicalhistory,familyhistoryMorphologyofRBC,reticulocytecount,WBC,plateletcount,bonemarrowcellsmear,HB,specialexamination3.TreatmentEliminationetiologyGeneralMedicineIntravenousbloodTransplantations:BM,ste
20、mcellsOthernutritionalanemiawithmicrocytosisandhypochromiaDefinitionnutritionalirondeficiencyanemia(IDA)Hb、mostcommon、6-24ms、specialpreventionIronmetabolismIroncontentanddistribution:2/3oftheironispresentinHBand1/3intissueandtransportform.Contentofelementaliron(mg/kg)Adultfemales40Adultmales50newbor
21、n75IronmetabolismIronabsorption:Theprimaryregulatorofironhomeostasisisintestinalironabsorption.Ironabsorptiontakesplaceprimarilyintheduodenumbytheenterocytesatthetipoftheintestinalvilla.Ironmustpassthoughtheapicalandthethenthebasolateralmembranesofthesecellstoreachthecirculation.IronmetabolismIronst
22、orage:MostbodyironiscontainedinHB,withsmalleramountsboundtoferritin(铁蛋白)andhemosiderin（含铁血黄素）inthereticuloendothelialsystem,myoglobininmuscle,circulatingtransferring,andiron-containingenzymes.Themajorironstoresareintheformofferritin.Asironcontinuestoaccumulateinthecell,asecondstorageform,hemosiderin
23、appears.IronmetabolismIroncharacteristics:Thefetusabsorbsironfromthemotheracrosstheplacenta.Terminfantshaveadequatereservesforthefirst4monthsoflife.Preterminfantshavelimitedironstoresandbecauseoftheirhigherrateofgrowth,theyoutstriptheirreservesby8weeksofage.IronmetabolismIroncharacteristics:Atbirth,
24、becauseof“physiologicalhaemolysis”,muchironisreleasedtoplasmaandlittleironisabsorbedfromfood,Duringthesecondstage(about2monthsold),hematopoiesisisincreasedandmoreironisabsorbedfromfood,soirondeficiencyisrareinthisstage.After4months,developmentincrease,ironinfoodisdeficientandironstoresexhaust,somost
25、irondeficiencyanemiaoccursin6monthsto2yearsor3yearsoldchild.causes1.inadequateironstores:preterminfant,twin2.intakeirondeficiency3.growthanddevelopmentincreasedironrequirement4.ironabsorbabnormal5.a amount of iron loss: hookworm infestation,repeated venesection, Meckels diverticulum,recurrentepistax
26、is(反复鼻出血).pathogenesisIRONHbmicrocytosisandhypochromiaRBCThreestageofirondeficiencyDeficiencyofironprogressesinstagesirondepletion(ID):tissueironstoresaredeleted,undernormalcondition,thiscorrelatesdirectlywithdecreaseintheferritinlever,reticulocytepercentagedecreases.Iron deficient erythropoiesis(ID
27、E):loss of circulatingiron.Lowserumironlessthan30ug/dl,lowtransferringsaturationand/orelevatedtotalironbindingcapacity.Threestageofirondeficiencyirondeficiencyanemia(IDA):irondeficiencyfollowing depletion of both marrow store andcirculatingiron.IDIDEIDAclinicalmanifestation1.general manifestation: m
28、ild 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:tac
29、hycardia,increasedarterypressure,wheeze,increasedpulse.Severeanemiamaycauseheartexpansionandcongestivecardiacfailure.5.nervoussystemmanifestation:vertigo,irritability.clinicalmanifestationMainsignsmaybepalloroftheskinandmucousmembranes.Severeanemiamaycausecongestivecardiacfailure.IDAininfancyandearl
30、ychildhoodisassociatedwithdevelopmentaldelayandpoorgrowth.laboratorytest1.bloodsmear2.bonemarrow3.ironmetabolismInequality of size of erythrocytes，small cell，Central olistherozone obviously hypercellular,erythroidhyperplasia,thedevelopmentofcytoplasmfallsbehindnucleus.leukocytesandmegakaryocytesaren
31、ormal.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.Reticulocy
32、teisnormalorslightlydecreases.WBCandplateletsarenormal.BloodcountinirondeficiencyHB75g/L120g/LRBC3.541012/L4.241012/LMCV64fl86flMCHC18.5pg32pgreticulocyte1.3%1.4%WBC7.54109/L7.64109/Lproportionnormalnormalplatelet254109/L257109/LlaboratorytestBonemarrowrevealsincreasedbasophilicnormoblastandpolychro
33、maticnormoblast.Granulocytesystemandmegakaryocytesystemarenormal.IronmetabolismsSerumferritin(SF)(血清铁蛋白)Freeerythrocyteprotoporphyrin(FEP)Serumiron,totalironbindingcapacityIroninbonemarrowIronmetabolismsIronstudyIDIDEIDASerumferritin(SF)IronstoreRedbloodcellprotoporphyrin(FEP)NPercentsideroblastsNSe
34、rumironNN/diagnosisfirst consider - history + clinicalmanifestation+bloodsmearDecidediagnosis-bonemarrow+ironmetabolismMaybeseetreatmentwithiron(Thebonemarrowishypercellular,witherythroidhyperplasia, the normoblasts may have scanty, and thedevelopment of cytoplasm falls behind one of nucleus.leukocy
35、tesandmegakaryocytesarenormal.)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 Admi
36、nistration 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 prep
37、arationParenteral 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 calculat
38、ed dose, but the response to parenteral iron is no more rapid or complete 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 inf
39、ection, 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 considere
40、d. 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 infantNutritionalmeg
41、aloblasticanemia 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.requirementinc
42、reasedPathogenesisfolicacidfolicacidwith4hydratevitaminB12DNAHbverylargeRBCMegaloblasticwithLotofHbdihydrofolatereductase(THFA)VitaminB12isimportanceinsynthesisofnerve.deficiencyofvitaminB12canleadtodiscordofneurologypsychology.InthemacrocyticanemiaproducedbydeficiencyofvitaminB12,thesymptomsandsign
43、sincludethoseofanemiaandneuropathy.nVitaminB12deficiencyneurologypsychologysymptomPatientsdevelopademyelinatinglesionofneuronsofthespinalcolumnandcerebralcortex.Thisconditionresultsinparesthesiasofthehandsandfeet,unsteadinessofgait,andeventuallymemorylossandpersonalitychanges.Thereisretardofintellec
44、tiveandphysicaldevelopment.TremblingofExtremitiesorhead,hypertensionofmuscle,tendonreflexreinforcement,positiveBabinskissignmayappear.Clinicalmanifestation1.General features: puffiness, poor nutrition,hair yellowed, mild edema, petechia (plt),mucocutaneoushemorrhage.2.featureofanemia:lethargy,extram
45、edullary3.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
46、seen , thrombocytopenia Hypercellular , Megaloblastic changes, hypersegmentation LaboratorytestsBloodroutineexamination:macrocyticanemia,thedecreaseofredcellcountismorethanthedecreaseofHB.MCV94fl,MCH32pg.Rreticulocyteisdecrease.WBCandplateletsarealsodecreased.Bonemarrow:increasedbasophilicnormoblast
47、andpolychromaticnormoblastic.Granulocyticsystemandmegakaryocytesystem:normal/lessthannormal.LaboratorytestsVitamineB12:normalserumvitaminB12levelsrangefrom200-800ng/L,B1212ng/LrevealsB12deficiency.Folate:normalserumfolatelevelsrangefrom5-6ug/L,folate3ug/Lrevealsdeficiency.others:LDH:serumlacticdehyd
48、rogenase(LDH)isincreaseDiagnosisfirst consider - history + clinicalmanifestationMarked symptoms and signs ofcentralnervoussystem.(itsupportsdefiencyofvitaminB12.)+.bloodsmeardecidediagnosis-.bonemarrow+metabolism(TodistinguishthedeficiencyoffolicacidwiththedeficiencyofvitaminB12.)maybeseetreatmentwi
49、thmedicineTreatment1.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
50、 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
51、vit B12 is sufficient.Folicacidpreparation.Folic acid may be administered orally in a dose of 15 mg/24 hr. If the specific diagnosis is in doubt, 125 mg/24 hr of folate may be used for a week as a diagnostic test. vitamine C will help absorption of folate.IDAmegaloblasticanemiaage6m-2y6m-2yCauseiron
52、VtB12/folicacidClinicalmanifestationpallorpuffinessextramedullary NervoussystemslightstrikingIDAmegaloblasticanemiaBloodsmearmicro-hypochromiamacrocytosisBonemarrowcytoplasmfallsbehindnucleusMegaloblasticchangesIronmetabolismabnormalNVtB12/folicacidN In this case, macrocyticanemiaiscausedbydeficienc
53、yofvitaminB12.Why? In the macroblastic anemia produced by deficiency of vitamin B12, the symptoms and signs include those of anemia and neuropathy. Patients develop a demyelinating lesion of neurons of the spinal column and cerebral cortex. This condition results in paresthesias(感觉异常) of the hands a
54、nd feet, unsteadiness of gait, and eventually memory loss and personality（智力） changes. There is retard of intellective and physical development. Trembling（震颤） of Extremities or head, hypertension of muscle, tendon reflex reinforcement, positive Babinskis sign may appear. Final diagnosisDiagnosis TreatmentTherapy
