湘雅儿科精品-Anemia in childhood课件

Anemiainchildhood

(小儿贫血)WuqingWanDept.ofpediatrics中南大学儿科学教研室第十五章TeachingpurposeTounderstandfeaturesofhematopoiesisandbloodinchildren.Tocomprehendclinicalfeatures,diagnosisandtherapyofanemia.Tounderstandthedefinition,gradedivisionandclassificationofanemiainchildren.Tomasteretiology,pathogenosis,diagnosis,therapyandpreventionofnutritionalirondeficiencyanemiaandnutritionalmegaloblasticanemia.Featuresofhematopoiesisinchildren

Hematopoiesisinfetalperiod（胎儿期造血）

Developmentalhematopoiesisoccursinthreeanatomicstages—mesoblastic（中胚层）,hepatic（肝）,andmyeloid（骨髓）.

FeaturesofhematopoiesisinchildrenHematopoiesisafterbirth

Hematopoesisinbonemarrow

Extramedullaryhemopoiesis

（骨髓外造血）Hematopoesisinbonemarrow

Yellowbonemarrow（黄骨髓）cancomebacktoredbonemarrow（红骨髓）whenhematopoeticneedincreases.Yellowbonemarrowisdeficiencyinchildren,especiallyininfantandtoddlerperiod.

Whenhematopoieticdemandincreases,liver,spleenandlymphnodescomebacktotheStatustoproducebloodcells,hepatomegaly（肝肿大）andsplenomegaly（脾肿大）appears,andmaybethereareimmatureerythrocytesandgranulocytesincirculatingblood.Extramedullaryhemopoiesisisthespecificphenomenaonlyappearingininfantandtoddler.

Extramedullaryhemopoiesis（髓外造血）FeaturesofbloodinchildrenHemoglobin

Atbirth1yr2yr%2040608005d5yDifferentialcountofWBC(白细胞分类计数)Lymphocyte淋巴细胞Granulocyte粒细胞

Bloodroutine

HypochromicmicrocyticMacrocyticNormocytic

（Hb↓＞RBC↓）

（Hb↓＜RBC↓)（Hb↓=RBC↓）IDA

（缺铁性贫血）

megaloblastic

acutebleedingThalassemia

VitB12↓↓

hemolyticanemiasideroblasticanemiafolicacid↓

aplasticanemiaChronicinfectionmostofsecondaryanemia

AgeHbconcentration(g/L)<28d<1451～4mon<904～6mon<1006mon～6y<1106～14y<120Thecriterionofanemia.

Nutritionalirondeficiencyanemia(IDA)

营养性缺铁性贫血

Zhou,male,10monthsold.Palenessinfaceandlipsfor4mons.Hisfacialexpressionbecamemoreandmorepalesince6monthsofage.Withoutfeverandbleeding.Hewasprematurebirthat33weeksofgestationalageandfedwithMother'smilkonly.

Case1PE:Pallor.nojaundiceandhemorrhagicspotsinskinandsclera;Bilateralsubmandibularlymphnodesare0.8×0.8cm.Breathsoundsinlungsaredistinct;therearenorhonchiandmoistrale.HR100/min.ThereisII/VIsystolicmurmurinprecordialregion.Theliverandspleenenlargeto4cmand3cmbelowlowercostamarginrespectively.Case1questions

Doananemiaexist?Whichkindofanemiadoesitbelong?

answerHypochromicmicrocyticanemia(小细胞低色素性贫血）

answer

ThecauseofanemiainthecaseaboveisdeficiencyofironbecauseheisprematureandisfedwithMother'smilkonly.etiology

Theetiologicalfactorsofnutritionalirondeficiencyanemia:

Storedirondeficiencyatbirth.Inadequatediet.Rapidlygrowth.Malabsorption.Excessivelossofiron.SmearofBloodnormalThepatient’BonemarrowBonemarrowexamination

Thebonemarrowishypercellular,witherythroidhyperplasia（过度增生）.Thenormoblasts(幼红细胞)mayhavescanty,fragmentedcytoplasmwithpoorhemoglobinization.Leukocytesandmegakaryocytesarenormal.HemosidenincannotbedemonstratedinmarrowspecimensbyPrussianbluestaining.questions

Pathogenesisofnutritionalirondeficiencyanemia.

铁缺乏

贮存铁

血清铁

血红蛋白铁↓

酶铁↓

红细胞内Ｈｂ合成↓↓

含铁酶↓

细胞分裂不受影响

铁依赖酶↓

Ｈｂ↓↓，RBC正常或↓

某些细胞功能紊乱，如

生物氧化、细胞呼吸小细胞低色素性贫血

神经递质分解与合成等

血液系统症状

非血液系统症状

一般贫血表现

行为发育异常

髓外造血表现

皮肤粘膜损害

心血管系统症状

细胞免疫功能↓缺铁性贫血发病机理

IronstudyIronstudyIDIDEIDA*serumferritin(SF)↓↓↓↓↓Ironstore↓↓↓↓↓FEP(红细胞游离原卟啉）N↑↑↑sideroblastsN↓↓↓SerumironNNor↓↓↓Therapy

goodnursingremovalofetiologicalfactors

treatmentwithironpreparation

Oraladministrationofsimpleferroussalts

ferroussulfate

ferrousgluconate

ferrorsfumarate

polysaccharideiron

Dosage:4-6mg/kgelementalironperday

Oralironpreparation

Administrationtheironpriortomeals

Administrationascorbicacidwithironpreparation.

Therapeuticcourse:withdrawalofironpreparation6-8weeksafterhemoglobinrecovertonormallevelorwhenSFandFEPisnormal.

OralironpreparationParenteralironTobeadministeredonlyforgastrointestinalmalabsorptionorsevereintolerancepreventseffectiveoralirontherapy.ReticulocytosisTransfusionPreventionNutritionalMegaloblasticAnemia

（营养性巨幼细胞性贫血）

FolicacidandvitaminB12deficiencyareprimarycausesofmegaloblasticanemia.Case2.

Zhou,male,8gressivedrowsinessandgoasleepfor20hrpreday.Hecannotsmileandtoraisehishead,whichcanbedonebefore.HewasfedwithMother'smilkonly.Case2.

PE:emotionalexpressionSluggishness（呆滞）,Pallor.nojaundice,rashesandhemorrhagicspotsinskin;pallorinhislips.Breathsoundsaredistinctandtherearenoraleinlungs.Heartrateis110timesperminute.Theliverandspleenenlargeto3centimetersand4centimetersbelowlowercostamarginrespectively.BloodroutineBeforeAfternormallevelsWBC3.18.6(4.0～10.0)109/LLYM83.066.0(50～70)%GRAN17.032(30～50)%RBC1.982.93(3.5～5.5)1012/LHb6979(110～160)g/LMCV103.388.0(82～92)flMCH34.827.0(27～34)pgMCHC337307(320～360)g/LRDW19.420.9(11.5～14.5)%RC0.0180.090(0.005～0.015)PLT80644(100～300)109/Lquestions:1.Whichkindofanemiaitbelongs?

AnswerTheclinicdatashowsamacrocyticanemia.

SmearofbloodpatientnormalSmearofpatient’bloodBM(骨髓)Bonemarrowexamination

Thebonemarrowishypercellularbecauseoferythroidhyperplasia.Megaloblasticchangesareprominent,althoughsomenormalRBCprecursorsmayalsobefound.Large,abnormalneutrophilicforms(giantmetamyelocytes)withcytoplasmicvacuolationareseen,aswellashypersegmentationofthenucleiofmegakaryocytes.questions:

2.IsitcausedbydeficiencyoffolicacidorvitaminB12?

Answer

Inthiscase,macrocyticanemiaiscausedbydeficiencyofvitaminB12.Why?questions:

3.whatisthedifferencesinclinicalmanifestationbetweendeficiencyoffolicacidanddeficiencyofvitaminB12?

叶酸↓

四氢叶酸↓

核苷酸尤其胸苷合成↓

VitB12↓DNA合成↓RNA合成正常

神经髓鞘中脂

细胞分裂↓

蛋白质,如Hb

蛋白合成不足

细胞增殖↓

合成正常

神经纤维功能

细胞巨幼变

不完整

细胞数量下降

表情呆滞,反应差RBC↓WBC↓PLT↓

其它组织

发育落后,常倒退

细胞↓

肢体震颤

可有病理反射

精神神经症状

巨幼细胞性贫血营养性巨幼细胞性贫血发病机理

Answer

InthemacroblasticanemiaproducedbydeficiencyofvitaminB12,thesymptomsandsignsincludethoseofanemiaandneuropathy.Patientsdevelopademyelinatinglesionofneuronsofthespinalcolumnandcerebralcortex.Thisconditionresultsinparesthesias(感觉异常)ofthehandsandfeet,unsteadinessofgait,andeventuallymemor