1、Anemia in childhood(小儿贫血)儿童贫血全英文1Disease of Disease of hematopoietic systemhematopoietic system infantile anemia（1）nutritional iron deficiency anemia(IDA)（2）nutritional megaloblastic anemia Primary/immunity thrombocytopenia Purpura(ITP)Leukemia 儿童贫血全英文2haematogenesis of childrenhaematogenesis of chi
2、ldrenhematopoiesis-produced blood extramedullary before birth and postnatal mesoblast hepatic medullary 3-15w 6w-6ms 3ms 儿童贫血全英文3Embryo stageEmbryo stage Mesoblastic haematogenesis：3ws begin，8ws weaken,12-15ws disappears。liver：8ws begin，6months gradually weaken,erythroblast、granular cell and megakar
3、yocyte.儿童贫血全英文4Embryo stageEmbryo stage3、spleen：12ws begin erythrocyte,granule,lymphocyte4、Haematogenesis of lymphatic organ 1.thoracic gland:8ws 2.lymphatic nodes:11ws儿童贫血全英文5Embryo stageEmbryo stage5、myelo-haematopoiesis：6mons Haematogenesis function emphasis，make various kinds of blood cells，uniq
4、ue hematogenic organ after birth.儿童贫血全英文6儿童贫血全英文7Haematopoiesis postnatalHaematopoiesis postnatal 1、marrow：2、extramedullary：when requirement of haemopoiesis increase，liver、spleen、lymphadenectasis，hepatomegaly and splenomegaly,in circulating blood immature erythrocytes and granulocytes.儿童贫血全英文8Physio
5、logical haemolysis Normal newborns have higher hemoglobin(HB)and hematocrit levels and a shortened survival period of the fetal RBCs contributes to the development of physiologic anemia.儿童贫血全英文9Physiological haemolysis erythropoiesis abruptly ceases with onset of respiration at birth,when the arteri
6、al 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 shortened survival of the fetal RBC also contributes to the development of physiologic anemia.the sizable expansion of blood volume that acco
7、mpanies rapid weight gain during the first 3 mo of life adds to the need for increased RBC production.儿童贫血全英文10blood characteristics agesblood characteristics ages red blood cells(RBC)and HbPhysiological haemolysis and anemia w r i t e b l o o d c e l l s(W B C)a n d classification 4-6 cross Platele
8、ts 150-250109/L blood volume 8-10%儿童贫血全英文11Red 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.The RBC count is 5.010127.01012,hemoglobin concentration is about 150220g/L at b
9、irth.The RBC and hemoglobin concentration in preterm infants are slightly lower than those in term infants.儿童贫血全英文12Red blood cell(RBC)The wide range of hemoglobin concentration is accounted for by:Variation in how rapidly the umbilical cord is clamped.An infants position after delivery.If cord clam
10、ping is delayed and the baby is held lower than placenta,both hemoglobin and blood volume are increased by a placental transfusion.儿童贫血全英文13Change of HB after birthChange of HB after birth儿童贫血全英文14ReticulocyteReticulocyte儿童贫血全英文15ReticulocyteReticulocyte 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.儿童贫血全英文16White 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.Af
12、ter 612 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.儿童贫血全英文17White blood cell(WBC)The change in WBC classification is the proportion between lymphocyte and granulocyte.Lymp
13、hocyte is about 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.儿童贫血全英文18White blood cell(WBC)Lymphocyte is about 60%and granulocyte is about 35%subsequent
14、ly.They are equal at 46 years.After 7 years white cell classification in infants is similar to that in adult.儿童贫血全英文194-6 DaysGranulocyteLymphocyte4-6 yearsChange of proportion in Lymphocyte and Granulocyte儿童贫血全英文20Platelet count Normal value for the platelet count are about 150250109/L and vary lit
15、tle with age.儿童贫血全英文21Blood volume Blood 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.儿童贫血全英文22AnemiaAnemiaDefination:Defination:Anemia is defined as a reduction of the red blood cell volume or
16、hemoglobin concentration below the range of values occurring in healthy 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.儿童贫血全英文23The criteria of anemiaAgeHb concentration 28 days 145 g/L14 month
17、s 90 g/L46 months 100g/L6 months6 years 110g/L614 years 120g/L儿童贫血全英文24 AnemiaAnemia1.Classification 1）degree:mild moderate severe Very severe 2）Morphology of RBC3）Causes:lost blood,hemolytic,deficiency of forming Hb and RBC儿童贫血全英文25 degree RBC(van/mm3)Hb(g/L)Mild 300-400 90-110 Moderate 200-300 60-
18、90 Severe 100-200 30-60 Very severe 100 30 儿童贫血全英文26Morphologynanemia with microcytosis and hypochromianAnemia with macrocytosisnAnemia with normalcytosis Anemia儿童贫血全英文27More anemia MCV MCH MCHCNormal 80-94 28-32 32-38Micro-hypochromia 80 28 94 32 32-38microcytosis 80 28 32-38 mean corpuscular volum
19、e(MCV),means corpuscular hemoglobin(MCH),mean corpuscular hemoglobin concentration(MCHC)儿童贫血全英文28Causes1.lost blood:acute chronic2.hemolysis Intrinsic membrane hereditary spherocytosis Glycolysis pyruvate kinase hemoglobin sickle cell,unstable Hb oxidation G6PD extrinsic:immune,infection,DIC儿童贫血全英文2
20、9Causes 3.deficiency of forming Hb and RBC deficiency of hematopoiesis substance medullary hematopoiesis disorder(Aplastic anemia)The inhibition of haematopoiesis induced by:Inflamation Chronic nephritis Toxicity Cancer cells invasion bone marrow 儿童贫血全英文30Symptoms of anemia Asymptomatic:particularly
21、 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.儿童贫血全英文31Symptoms of anemia Cardiovascular and respiratory system:t
22、achycardias,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.儿童贫血全英文322.DiagnosisHistory positive manifestation laboratory tests Blood smear BM Hb ananysi
23、s 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儿童贫血全英文333.Treatment Elimination etiology General Medic
24、ine Intravenous blood Transplantations:BM ,stem cells Other儿童贫血全英文34 nutritional anemia with nutritional anemia with microcytosis and hypochromiamicrocytosis and hypochromiaDefinition Definition nutritional iron deficiency anemia(IDA)Hb、most common、6-24ms、special prevention 儿童贫血全英文35Iron metabolism
25、Iron content and distribution:2/3 of the iron is present in HB and 1/3 in tissue and transport form.Content of elemental iron(mg/kg)Adult females40Adult males50newborn75儿童贫血全英文36Iron metabolismIron absorption:The primary regulator of iron homeostasis is intestinal iron absorption.Iron absorption tak
26、es place primarily in the primarily in the duodenumduodenum by the enterocytes at the tip of the intestinal villa.Iron must pass though the apical and the then the basolateral membranes of these cells to reach the circulation.儿童贫血全英文37Iron metabolismIron storage:Most body iron is contained in HB,wit
27、h 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.The major iron stores are in the form of ferritin.As iron continues to accumulate in the cell,a second storage form,hemosiderin app
28、ears.儿童贫血全英文38Iron metabolismIron characteristics:The fetus absorbs iron from the mother across the placenta.Term infants have adequate reserves for the first 4 months of life.Preterm infants have limited iron stores and because of their higher rate of growth,they outstrip their reserves by 8 weeks
29、of age.儿童贫血全英文39Iron metabolismIron characteristics:At birth,because of“physiological haemolysis”,much iron is released to plasma and little iron is absorbed from food,During the second stage(about 2 months old),hematopoiesis is increased and more iron is absorbed from food,so iron deficiency is rar
30、e 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.儿童贫血全英文40causes1.inadequate iron stores:preterm infant,twin2.intake iron deficiency3.growth and development increased
31、 iron requirement4.iron absorb abnormal5.a amount of iron loss:hookworm infestation,repeated venesection,Meckels diverticulum,recurrent epistaxis(反复鼻出血).儿童贫血全英文41pathogenesis IRON Hb IRON Hb microcytosis and microcytosis and hypochromia RBC hypochromia RBC 儿童贫血全英文42Three stage of iron deficiencyDefi
32、ciency of iron progresses in stagesDeficiency of iron progresses in stages iron 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 circulating ir
33、on.Low serum iron less than 30ug/dl,low transferring saturation and/or elevated total iron binding capacity.儿童贫血全英文43Three stage of iron deficiency iron deficiency anemia(IDA):iron deficiency following depletion of both marrow store and circulating iron.IDIDEIDA儿童贫血全英文44clinical manifestation1.gener
34、al 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 儿童贫血全英文45clinical manifestation 4.car
35、diovascular 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.儿童贫血全英文46clinical manifestation Main signs may be pallor of the skin and m
36、ucous membranes.Severe anemia may cause congestive cardiac failure.IDA in infancy and early childhood is associated with developmental delay and poor growth.儿童贫血全英文47laboratory test1.blood smear2.bone marrow3.iron metabolism 儿童贫血全英文48Inequality of size of erythrocytes，small cell，Central olistherozon
37、e obviously 儿童贫血全英文49hypercellular,erythroid hyperplasia,the development of cytoplasm falls behind nucleus.leukocytes and megakaryocytes are normal.儿童贫血全英文50Bone marrow iron stain：ferrugination grains in the erythocytes.Normal bone marrow iron stain正常骨髓铁染色正常骨髓铁染色IDA iron stain铁缺乏骨髓铁染色铁缺乏骨髓铁染色儿童贫血全英文
38、51laboratory test The decrease of HB concentration is more than the decrease 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.儿童贫血全英文52Blood count in iron deficiency
39、HB75g/L120g/LRBC3.541012/L4.241012/LMCV64fl86flMCHC18.5pg32pgreticulocyte1.3%1.4%WBC7.54109/L7.64109/Lproportionnormalnormalplatelet254109/L257109/L儿童贫血全英文53laboratory test Bone marrow reveals increased basophilic normoblast and polychromatic normoblast.Granulocyte system and megakaryocyte system ar
40、e normal.儿童贫血全英文54Iron metabolisms Serum ferritin(SF)(血清铁蛋白)Free erythrocyte protoporphyrin(FEP)Serum iron,total iron binding capacity Iron in bone marrow 儿童贫血全英文55Iron metabolismsIron study ID IDEIDASerum ferritin(SF)Iron store Red blood cell protoporphyrin(FEP)N Percent sideroblasts N Serum iron N
41、N/儿童贫血全英文56diagnosis first considerfirst consider-history+clinical manifestation+blood smear Decide diagnosisDecide diagnosis-bone marrow+iron metabolism May be see treatment with ironMay be see treatment with iron (The bone marrow is hypercellular,with erythroid hyperplasia,the normoblasts may have
42、 scanty,and the development of cytoplasm falls behind one of nucleus.leukocytes and megakaryocytes are normal.)儿童贫血全英文57 treatment 1.nursing feeding 2.get rid of etiology 3.iron medicine 4.interfusions blood儿童贫血全英文58Oral administration of simple ferrous salts ferrous sulfate(硫酸亚铁)ferrous gluconate（葡
43、萄糖酸亚铁）ferrous fumaratepolysaccharide iron Dosage:4-6mg/kg elemental iron per day Oral iron preparation儿童贫血全英文59 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 reco
44、ver to normal level or when SF(Serum ferritin)and FEP(Free erythrocyte protoporphyrin)is normal.Oral iron preparation儿童贫血全英文60Parenteral ironParenteral iron preparationpreparation To be administered only for gastrointestinal malabsorption or severe intolerance prevents effective oral iron therapy.儿童
45、贫血全英文61Parenteral ironParenteral iron preparationpreparation 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 more rapid or complete than that obtained with proper oral admin
46、istration of iron,unless malabsorption is a factor.儿童贫血全英文62Blood 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 controlled well.We may transfuse,severely anemia childre
47、n 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.儿童贫血全英文63Iron therapyNotice:3 points1.Injection iron in danger 2.Reaction:12-24h(irritability,appetite)-36-48h
48、(erythroid hyperplasia)-48-72h(reticulocytosis)-5-7ds(peaking)2-3ws to reticulocytes3.Times:6-8ws儿童贫血全英文64Prevention4 points mother milk feeding specter food with iron preterm infant儿童贫血全英文65 Nutritional megaloblastic Nutritional megaloblastic anemiaanemia Folic acid and vitamin B12 deficiency are p
49、rimary causes of megaloblastic anemia.儿童贫血全英文66 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.儿童贫血全英文67Causes 1.less intake 2.absorb abnormal 3.drug interactions 4.requirement increased 儿童贫血全英文68Pathogenesis folic a
50、cid folic acid with 4 folic acid folic acid with 4 hydratehydrate vitamin Bvitamin B1212 DNA DNA HbHb very large RBCvery large RBC Megaloblastic withMegaloblastic with Lot of HbLot of Hb dihydrofolate reductase(THFA)儿童贫血全英文69VitaminB12 is importance in synthesis of nerve.deficiency of vitaminB12 can
