《儿童贫血全英》PPT课件.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 bloodcharacteristics ages redbloodcells RBC andHbPhysiologicalhaemolysisandanemiawritebloodcells WBC andclassification4 6crossPlatelets150 250 109 Lbloodvolume8 10 Redbloodcell RBC Termnewbornshavearedcellmassthatishigherthanatanyothertimeoflife anappropriateconditionforthelowoxygenenvironmentofintrauterinelife TheRBCcountis5 0 1012 7 0 1012 hemoglobinconcentrationisabout150 220g Latbirth TheRBCandhemoglobinconcentrationinpreterminfantsareslightlylowerthanthoseinterminfants Redbloodcell RBC Thewiderangeofhemoglobinconcentrationisaccountedforby Variationinhowrapidlytheumbilicalcordisclamped Aninfant spositionafterdelivery Ifcordclampingisdelayedandthebabyisheldlowerthanplacenta bothhemoglobinandbloodvolumeareincreasedbyaplacentaltransfusion ChangeofHBafterbirth Reticulocyte Reticulocyte Reticulocyteis0 04 0 06inthefirst3days Reticulocytedecreasesto0 005 0 015after4 7days Reticulocyterisesto0 02 0 08in4 6weeks Reticulocyteisequaltoanadult safter5months Whitebloodcell WBC ThenormalnumberofWBCishigherininfancyandearlychildhoodthanlaterinlife WBCcountis15 109 20 109atbirth After6 12hours itriseto21 109 28 109andthenbeginstodecreaseto12 109by1week WBCcountmaintainsabout10 109atinfantperiodandapproachadult sWBCcountlevelby8years Whitebloodcell WBC ThechangeinWBCclassificationistheproportionbetweenlymphocyteandgranulocyte Lymphocyteisabout30 andgranulocyteisabout65 atbirth butthelaterlymphocytecontrarytoneutrophilegranulocytedecreases Theproportionbetweenlymphocyteandgranulocyteisequalat4 6daysafterbirth Whitebloodcell WBC Lymphocyteisabout60 andgranulocyteisabout35 subsequently Theyareequalat4 6years After7yearswhitecellclassificationininfantsissimilartothatinadult 4 6Days Granulocyte Lymphocyte 4 6years ChangeofproportioninLymphocyteandGranulocyte Plateletcount Normalvaluefortheplateletcountareabout150 250 109 Landvarylittlewithage Bloodvolume Bloodvolumeininfantsismorethaninadults Thenewborn sbloodvolumeis10 ofhisweightandabout300mlonaverage Achild sisabout8 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 60Verysevere 100 30 Morphology anemiawithmicrocytosisandhypochromiaAnemiawithmacrocytosisAnemiawithnormalcytosisAnemia Moreanemia MCVMCHMCHCNormal80 9428 3232 38Micro hypochromia94 3232 38microcytosis 80 2832 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 History positivemanifestation laboratorytestsBloodsmearBMHbananysisGrowthdevelopmentnutritionnailsfairsliverspleenandlymphnotes5points 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 twin2 intakeirondeficiency3 growthanddevelopmentincreasedironrequirement4 ironabsorbabnormal5 aamountofironloss hookworminfestation repeatedvenesection Meckel sdiverticulum 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 MCV 80fl MCH 26pg MCHC 0 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 nursingfeeding2 getridofetiology3 ironmedicine4 interfusionsblood 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 4points mothermilkfeedingspecterfoodwithiron preterminfant 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 positiveBabinski ssignmayappear 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 MCV 94fl MCH 32pg Rreticulocyteisdecrease WBCandplateletsarealsodecreased Bonemarrow increasedbasophilicnormoblastandpolychromaticnormoblastic Granulocyticsystemandmegakaryocytesystem normal lessthannormal Laboratorytests VitamineB12 normalserumvitaminB12levelsrangefrom200 800ng L B12 12ng LrevealsB12deficiency Folate normalserumfolatelevelsrangefrom5 6ug L folate 3ug Lrevealsdeficiency others LDH serumlacticdehydrogenase LDH isincrease Diagnosis firstconsider history clinicalmanifestation Markedsymptomsandsig