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1、Anemia in childhood (小儿贫血),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 pre
2、vention of nutritional iron deficiency anemia and nutritional megaloblastic anemia.,Disease of hematopoietic system,infantile anemia （1）nutritional iron deficiency anemia(IDA) （2）nutritional megaloblastic anemia Primary/immunity thrombocytopenia Purpura(ITP) Leukemia,haematogenesis of children,hemat
3、opoiesis -produced blood extramedullary before 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、spl
4、een：12ws begin erythrocyte, granule , lymphocyte 4、Haematogenesis of lymphatic organ 1.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
5、,1、marrow： 2、extramedullary：when requirement of haemopoiesis 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 s
6、urvival period of the fetal RBCs contributes to the development 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-li
7、fe 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 accompanies rapid weight gain during the first 3 mo of life adds to the need for increased RBC productio
8、n.,blood characteristics ages,red blood cells(RBC) and Hb Physiological haemolysis and anemia write blood cells(WBC) and classification 4-6 cross Platelets 150-250109/L blood 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 approp
9、riate condition for the low oxygen environment of intrauterine life. The 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 he
10、moglobin concentration is accounted for by: Variation in how rapidly the umbilical 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
11、birth,Reticulocyte,Reticulocyte,Reticulocyte is 0.04-0.06 in the first 3 days. 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 an
12、d early childhood than later in life. WBC count is 15109 20109 at birth. After 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.,White blood cell(WBC),The change in WBC cl
13、assification is the proportion between lymphocyte and granulocyte. Lymphocyte 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,White blood cell
14、(WBC),Lymphocyte is about 60% and granulocyte is about 35% subsequently . They are equal at 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 val
15、ue for the platelet count are about 150250109/L and vary little with age.,Blood 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.,Anemia,Defination : Anemia is defined as a reductio
16、n of the red blood cell volume or 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.,The criteria of anemia,Anemia,1.
17、Classification 1） degree : mild moderate severe Very severe 2） Morphology of RBC 3）Causes: lost blood , hemolytic , deficiency of forming Hb and RBC,degree,RBC (van /mm3 ) Hb (g/L) Mild 300-400 90-110 Moderate 200-300 60-90 Severe 100-200 30-60 Very severe 100 30,Morphology,anemia with microcytosis
18、and hypochromia Anemia with macrocytosis Anemia with normalcytosis Anemia,More anemia,MCV MCH MCHC Normal 80-94 28-32 32-38 Micro-hypochromia 94 32 32-38 microcytosis 80 28 32-38 mean corpuscular volume(MCV), means corpuscular hemoglobin (MCH), mean corpuscular hemoglobin concentration(MCHC),Causes,
19、1.lost blood :acute chronic 2. hemolysis Intrinsic membrane hereditary spherocytosis Glycolysis pyruvate kinase hemoglobin sickle cell,unstable Hb oxidation G6PD extrinsic : immune, infection, DIC,Causes,3.deficiency of forming Hb and RBC deficiency of hematopoiesis substance medullary hematopoiesis
20、 disorder (Aplastic anemia) The inhibition of haematopoiesis induced by: Inflamation Chronic nephritis Toxicity Cancer cells invasion bone marrow,Symptoms of anemia,Asymptomatic: particularly if the anemia develops over a long time. General manifestation: pallor of the skin and mucous membranes, let
21、hargy, malnutrition, growth retardation. liver, spleen and lymph nodes expansion. Digestion system: anorexia, nausea and constipation.,Symptoms of anemia,Cardiovascular and respiratory system: tachycardias, increased artery pressure, wheeze and increased pulse. severe anemia may cause heart expansio
22、n and congestive cardiac failure. Nerver system: vertigo, tinnitus, irritability, and disorders of attention.,2. Diagnosis,History positive manifestation laboratory tests Blood smear BM Hb ananysis Growth development nutrition nails fairs liver spleen and lymph notes 5 points: age, course, symptoms,
23、 feeding, past medical history ,family history Morphology of RBC, reticulocyte count, WBC, platelet count, bone marrow cell smear, HB ,special examination,3.Treatment,Elimination etiology General Medicine Intravenous blood Transplantations : BM , stem cells Other,nutritional anemia with microcytosis
24、 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 HB and 1/3 in tissue and transport form.,Iron metabolism,Iron absorption: The primary regulator of iron homeo
25、stasis 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 the basolateral membranes of these cells to reach the circulation.,Iron metabolism,Iron storage: Most body
26、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. The major iron stores are in the form of ferritin. As iron continues to accumulate in the cell, a
27、 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. Preterm infants have limited iron stores and because of their higher rate of growth, they o
28、utstrip 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 second stage (about 2 months old), hematopoiesis is increased and more iron is absorbed f
29、rom 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.,causes,1.inadequate iron stores: preterm infant, twin 2.intake iron deficienc
30、y 3.growth and development increased iron requirement 4.iron absorb abnormal 5.a amount of iron loss: hookworm infestation, repeated venesection, Meckels diverticulum, recurrent epistaxis(反复鼻出血).,pathogenesis,IRON Hb microcytosis and hypochromia RBC,Three stage of iron deficiency,Deficiency of iron
31、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 iron. Low serum iron less than 30ug/dl, low transf
32、erring 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. general manifestation: mild iron deficiency is Asymptomatic
33、, pallor of the skin and mucous mebranes are most evident and lethargy, malnutrition, growth retardation. 2. liver spleen and lymph nodes enlarge 3. digestion system: anorexia（食欲差）, nausea（恶心）, constipation（便秘）. diarrhea,clinical manifestation,4. cardiovascular and respiratory manifestation: tachyca
34、rdia, 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. Severe anemia may cause congesti
35、ve cardiac failure. IDA in infancy and early childhood is associated with developmental delay and poor growth.,laboratory test,1.blood smear 2.bone marrow 3.iron metabolism,Inequality of size of erythrocytes，small cell， Central olistherozone obviously,hypercellular , erythroid hyperplasia , the deve
36、lopment 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 decrease of red cells cou
37、nt. 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 polychromatic normob
38、last. 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 Decid
39、e diagnosis-bone marrow + iron metabolism May 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 feedi
40、ng 2. get 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 fumarate polysaccharide iron Dosage: 4-6mg/kg elemental iron per day,Oral iron preparation,Administration the iron prior to meals /betw
41、een 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 preparation,Parenteral iron preparation,To
42、be administered only 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
43、parenteral iron is no 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 spe
44、ed 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 considered.,Iron therapy,Notice : 3
45、 points 1.Injection iron in danger 2.Reaction : 12-24h(irritability ,appetite )- 36-48h(erythroid hyperplasia )- 48-72h(reticulocytosis)-5-7ds(peaking ) 2-3ws to reticulocytes 3.Times: 6-8ws,Prevention,4 points mother milk feeding specter food with iron preterm infant,Nutritional megaloblastic anemi
46、a,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.,Causes,1.less intake 2.absorb abnormal 3.drug interactions 4.requirement increased
47、,Pathogenesis,folic acid folic acid with 4 hydrate vitamin B12 DNA Hb very large RBC Megaloblastic with Lot 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
48、deficiency of vitamin B12, the symptoms and signs include 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, uns
49、teadiness of gait, and eventually memory loss and personality 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