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1、To understand features of hematopoiesis and blood in children.To comprehend clinical features,diagnosis and therapy of anemia.To understand the definition,grade division and classification of anemia in children.To master etiology,pathogeny,diagnosis,therapy and prevention of nutritional iron deficie
2、ncy anemia and nutritional megaloblastic anemia.,Disease of hematopoietic system,infantile anemia（1）nutritional iron deficiency anemia(IDA)（2）nutritional megaloblastic anemiaPrimary/immunity thrombocytopenia Purpura(ITP)Leukemia,haematogenesis of children,hematopoiesis-produced blood extramedullaryb
3、efore birth and postnatal mesoblast hepatic medullary 3-15w 6w-6ms 3ms,Embryo stage,Mesoblastic haematogenesis：3ws begin，8ws weaken,12-15ws disappears。liver：8ws begin，6months gradually weaken,erythroblast、granular cell and megakaryocyte.,Embryo stage,3、spleen：12ws begin erythrocyte,granule,lymphocyt
4、e4、Haematogenesis of lymphatic organ1.thoracic gland:8ws 2.lymphatic nodes:11ws,Embryo stage,5、myelo-haematopoiesis：6mons Haematogenesis function emphasis，make various kinds of blood cells，unique hematogenic organ after birth.,Haematopoiesis postnatal,1、marrow：2、extramedullary：when requirement of ha
5、emopoiesis increase，liver、spleen、lymphadenectasis，hepatomegaly and splenomegaly,in circulating blood immature erythrocytes and granulocytes.,Physiological haemolysis,Normal newborns have higher hemoglobin(HB)and hematocrit levels and a shortened survival period of the fetal RBCs contributesto the de
6、velopment of physiologic anemia.,Physiological haemolysis,erythropoiesis abruptly ceases with onset of respiration at birth,when the arterial oxygen saturation rises toward 95%.levels of erythropoietin(EPO)are low.EPO has a decreased half-life and an increased volume of distribution in newborns.A sh
7、ortened survival of the fetal RBC also contributes to the development of physiologic anemia.the sizable expansion of blood volume that accompanies rapid weight gain during the first 3 mo of life adds to the need for increased RBC production.,blood characteristics ages,red blood cells(RBC)and HbPhysi
8、ological haemolysis and anemiawrite blood cells(WBC)and classification 4-6 crossPlatelets 150-250109/Lblood volume 8-10%,Red blood cell(RBC),Term newborns have a red cell mass that is higher than at any other time of life.an appropriate condition for the low oxygen environment of intrauterine life.T
9、he RBC count is 5.010127.01012,hemoglobin concentration is about 150220g/L at birth.The RBC and hemoglobin concentration in preterm infants are slightly lower than those in term infants.,Red blood cell(RBC),The wide range of hemoglobin concentration is accounted for by:Variation in how rapidly the u
10、mbilical cord is clamped.An infants position after delivery.If cord clamping is delayed and the baby is held lower than placenta,both hemoglobin and blood volume are increased by a placental transfusion.,Change of HB after birth,Reticulocyte,Reticulocyte,Reticulocyte is 0.04-0.06 in the first 3 days
11、.Reticulocyte decreases to 0.005-0.015 after 4-7 days.Reticulocyte rises to 0.02-0.08 in 4-6 weeks.Reticulocyte is equal to an adults after 5 months.,White blood cell(WBC),The normal number of WBC is higher in infancy and early childhood than later in life.WBC count is 15109 20109 at birth.After 612
12、 hours,it rise to 21109 28109 and then begins to decrease to 12109 by 1 week.WBC count maintains about 10109 at infant period and approach adults WBC count level by 8 years.,White blood cell(WBC),The change in WBC classification is the proportion between lymphocyte and granulocyte.Lymphocyte is abou
13、t 30%and granulocyte is about 65%at birth,but the later lymphocyte contrary to neutrophile granulocyte decreases.The proportion between lymphocyte and granulocyte is equal at 46 days after birth.,White blood cell(WBC),Lymphocyte is about 60%and granulocyte is about 35%subsequently.They are equal at
14、46 years.After 7 years white cell classification in infants is similar to that in adult.,4-6 Days,Granulocyte,Lymphocyte,4-6 years,Change of proportion in Lymphocyte and Granulocyte,Platelet count,Normal value for the platelet count are about 150250109/L and vary little with age.,Blood volume,Blood
15、volume in infants is more than in adults.The newborns blood volume is 10%of his weight and about 300ml on average.A childs is about 8%10%of his weight.,Anemia,Defination:Anemia is defined as a reduction of the red blood cell volume or hemoglobin concentration below the range of values occurring in h
16、ealthy persons.Anemia is an absolute decrease in hematocrit,hemoglobin concentration,or the RBC count.Anemia is not a diagnosis,but a sign of underlying disease.,The criteria of anemia,Anemia,1.Classification 1）degree:mild moderate severe Very severe 2）Morphology of RBC3）Causes:lost blood,hemolytic,
17、deficiency of forming Hb and RBC,degree,RBC(van/mm3)Hb(g/L)Mild 300-400 90-110Moderate 200-300 60-90Severe 100-200 30-60Very severe 100 30,Morphology,anemia with microcytosis and hypochromiaAnemia with macrocytosisAnemia with normalcytosis Anemia,More anemia,MCV MCH MCHCNormal 80-94 28-32 32-38Micro
18、-hypochromia 94 32 32-38microcytosis 80 28 32-38 mean corpuscular volume(MCV),means corpuscular hemoglobin(MCH),mean corpuscular hemoglobin concentration(MCHC),Causes,1.lost blood:acute chronic2.hemolysis Intrinsic membrane hereditary spherocytosis Glycolysis pyruvate kinase hemoglobin sickle cell,u
19、nstable Hb oxidation G6PD extrinsic:immune,infection,DIC,Causes,3.deficiency of forming Hb and RBCdeficiency of hematopoiesis substance medullary hematopoiesis disorder(Aplastic anemia)The inhibition of haematopoiesis induced by:Inflamation Chronic nephritis Toxicity Cancer cells invasion bone marro
20、w,Symptoms of anemia,Asymptomatic:particularly if the anemia develops over a long time.General manifestation:pallor of the skin and mucous membranes,lethargy,malnutrition,growth retardation.liver,spleen and lymph nodes expansion.Digestion system:anorexia,nausea and constipation.,Symptoms of anemia,C
21、ardiovascular and respiratory system:tachycardias,increased artery pressure,wheeze and increased pulse.severe anemia may cause heart expansion and congestive cardiac failure.Nerver system:vertigo,tinnitus,irritability,and disorders of attention.,2.Diagnosis,History positive manifestation laboratory
22、tests Blood smear BM Hb ananysis Growth development nutrition nails fairs liver spleen and lymph notes 5 points:age,course,symptoms,feeding,past medical history,family historyMorphology of RBC,reticulocyte count,WBC,platelet count,bone marrow cell smear,HB,special examination,3.Treatment,Elimination
23、 etiologyGeneral Medicine Intravenous bloodTransplantations:BM,stem cellsOther,nutritional anemia with microcytosis and hypochromia,Definition nutritional iron deficiency anemia(IDA)Hb、most common、6-24ms、special prevention,Iron metabolism,Iron content and distribution:2/3 of the iron is present in H
24、B and 1/3 in tissue and transport form.,Iron metabolism,Iron absorption:The primary regulator of iron homeostasis is intestinal iron absorption.Iron absorption takes place primarily in the duodenum by the enterocytes at the tip of the intestinal villa.Iron must pass though the apical and the then th
25、e basolateral membranes of these cells to reach the circulation.,Iron metabolism,Iron storage:Most body iron is contained in HB,with smaller amounts bound to ferritin(铁蛋白)and hemosiderin（含铁血黄素）in the reticuloendothelial system,myoglobin in muscle,circulating transferring,and iron-containing enzymes.
26、The major iron stores are in the form of ferritin.As iron continues to accumulate in the cell,a second storage form,hemosiderin appears.,Iron metabolism,Iron characteristics:The fetus absorbs iron from the mother across the placenta.Term infants have adequate reserves for the first 4 months of life.
27、Preterm infants have limited iron stores and because of their higher rate of growth,they outstrip their reserves by 8 weeks of age.,Iron metabolism,Iron characteristics:At birth,because of“physiological haemolysis”,much iron is released to plasma and little iron is absorbed from food,During the seco
28、nd stage(about 2 months old),hematopoiesis is increased and more iron is absorbed from food,so iron deficiency is rare in this stage.After 4months,development increase,iron in food is deficient and iron stores exhaust,so most iron deficiency anemia occurs in 6 months to 2 years or 3 years old child.
29、,causes,1.inadequate iron stores:preterm infant,twin2.intake iron deficiency3.growth and development increased iron requirement4.iron absorb abnormal5.a amount of iron loss:hookworm infestation,repeated venesection,Meckels diverticulum,recurrent epistaxis(反复鼻出血).,pathogenesis,IRON Hb microcytosis an
30、d hypochromia RBC,Three stage of iron deficiency,Deficiency of iron progresses in stagesiron depletion(ID):tissue iron stores are deleted,under normal condition,this correlates directly with decrease in the ferritin lever,reticulocyte percentage decreases.Iron deficient erythropoiesis(IDE):loss of c
31、irculating iron.Low serum iron less than 30ug/dl,low transferring saturation and/or elevated total iron binding capacity.,Three stage of iron deficiency,iron deficiency anemia(IDA):iron deficiency following depletion of both marrow store and circulating iron.,ID,IDE,IDA,clinical manifestation,1.gene
32、ral manifestation:mild iron deficiency is Asymptomatic,pallor of the skin and mucous mebranes are most evident and lethargy,malnutrition,growth retardation.2.liver spleen and lymph nodes enlarge3.digestion system:anorexia（食欲差）,nausea（恶心）,constipation（便秘）.diarrhea,clinical manifestation,4.cardiovascu
33、lar and respiratory manifestation:tachycardia,increased artery pressure,wheeze,increased pulse.Severe anemia may cause heart expansion and congestive cardiac failure.5.nervous system manifestation:vertigo,irritability.,clinical manifestation,Main signs may be pallor of the skin and mucous membranes.
34、Severe anemia may cause congestive cardiac failure.IDA in infancy and early childhood is associated with developmental delay and poor growth.,laboratory test,1.blood smear2.bone marrow3.iron metabolism,Inequality of size of erythrocytes，small cell，Central olistherozone obviously,hypercellular,erythr
35、oid hyperplasia,the development of cytoplasm falls behind nucleus.leukocytes and megakaryocytes are normal.,Bone marrow iron stain：ferrugination grains in the erythocytes.,Normal bone marrow iron stain正常骨髓铁染色,IDA iron stain铁缺乏骨髓铁染色,laboratory test,The decrease of HB concentration is more than the de
36、crease of red cells count.Blood smear reveals the more feature of microcyte and hypochromia.MCV80fl,MCH26pg,MCHC0.31.Reticulocyte is normal or slightly decreases.WBC and platelets are normal.,Blood count in iron deficiency,laboratory test,Bone marrow reveals increased basophilic normoblast and polyc
37、hromatic normoblast.Granulocyte system and megakaryocyte system are normal.,Iron metabolisms,Serum ferritin(SF)(血清铁蛋白)Free erythrocyte protoporphyrin(FEP)Serum iron,total iron binding capacity Iron in bone marrow,Iron metabolisms,diagnosis,first consider-history+clinical manifestation+blood smear De
38、cide diagnosis-bone marrow+iron metabolismMay be see treatment with iron(The bone marrow is hypercellular,with erythroid hyperplasia,the normoblasts may have scanty,and the development of cytoplasm falls behind one of nucleus.leukocytes and megakaryocytes are normal.),treatment,1.nursing feeding 2.g
39、et rid of etiology 3.iron medicine 4.interfusions blood,Oral 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.A
40、dministration 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 preparation,Parenteral iron preparation,To be administered onl
41、y for gastrointestinal malabsorption or severe intolerance prevents effective oral iron therapy.,Parenteral iron preparation,A 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
42、more rapid or complete than that obtained with proper oral administration of iron,unless malabsorption is a factor.,Blood Transfusion,With 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
43、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.,Iron therapy,Notice:3 points1.Injection iron in dange
44、r 2.Reaction:12-24h(irritability,appetite)-36-48h(erythroid hyperplasia)-48-72h(reticulocytosis)-5-7ds(peaking)2-3ws to reticulocytes3.Times:6-8ws,Prevention,4 pointsmother milk feeding specter food with iron preterm infant,Nutritional megaloblastic anemia,Folic acid and vitamin B12 deficiency are p
45、rimary 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.,Causes,1.less intake2.absorb abnormal3.drug interactions4.requirement increased,Pathogenesis,folic acid folic acid with 4 hydrate
46、 vitamin B12 DNA Hb very large RBCMegaloblastic withLot of Hb,dihydrofolate reductase,(THFA),VitaminB12 is importance in synthesis of nerve.deficiency of vitaminB12 can lead to discord of neurology psychology.In the macrocytic anemia produced by deficiency of vitamin B12,the symptoms and signs inclu
47、de those of anemia and neuropathy.,Vitamin B12 deficiency,neurology psychology symptom,Patients develop a demyelinating lesion of neurons of the spinal column and cerebral cortex.This condition results in paresthesias of the hands and feet,unsteadiness of gait,and eventually memory loss and personal
48、ity changes.There is retard of intellective and physical development.Trembling of Extremities or head,hypertension of muscle,tendon reflex reinforcement,positive Babinskis sign may appear.,Clinical manifestation,1.General features:puffiness,poor nutrition,hair yellowed,mild edema,petechia(plt),mucoc
49、utaneous hemorrhage.2.feature of anemia:lethargy,extramedullary3.neurology psychology:irritability,vertigo.4.digestive symptoms:anorexia,nausea,diarrhea.,Laboratory tests,1.blood smear 2.bone marrow3.blood biochemistry tests4.others,variation in BRC shape and size,macrocytosis,reticulocyte count is
50、low,nucleated RBCs and megaloblastic morphology are often seen,thrombocytopenia,Hypercellular,Megaloblastic changes,hypersegmentation,Laboratory tests,Blood routine examination:macrocytic anemia,the decrease of red cell count is more than the decrease of HB.MCV94fl,MCH32pg.Rreticulocyte is decrease.
