儿童贫血全英文课件

1、儿童贫血全英文,Anemia in childhood (小儿贫血),儿童贫血全英文,儿童贫血全英文,Disease of hematopoietic 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,haematogenesis of children,hematopoiesis -produced blood

2、extramedullary before birth and postnatal mesoblast hepatic medullary 3-15w 6w-6ms 3ms,儿童贫血全英文,Embryo stage,Mesoblastic haematogenesis：3ws begin，8ws weaken, 12-15ws disappears。 liver：8ws begin，6months gradually weaken ,erythroblast、granular cell and megakaryocyte.,儿童贫血全英文,Embryo stage,3、spleen：12ws

3、begin erythrocyte, granule , lymphocyte 4、Haematogenesis of lymphatic organ 1.thoracic gland:8ws 2.lymphatic nodes:11ws,儿童贫血全英文,Embryo stage,5、myelo-haematopoiesis：6mons Haematogenesis function emphasis，make various kinds of blood cells，unique hematogenic organ after birth.,儿童贫血全英文,儿童贫血全英文,Haematopo

4、iesis 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,Physiological haemolysis,Normal newborns have higher hemoglobin(HB) and hematocrit l

5、evels and a shortened survival period of the fetal RBCs contributesto the development of physiologic anemia.,儿童贫血全英文,Physiological haemolysis,erythropoiesis abruptly ceases with onset of respiration at birth, when the arterial oxygen saturation rises toward 95%. levels of erythropoietin (EPO) are lo

6、w. 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 accompanies rapid weight gain during the first 3 mo of life adds to the ne

7、ed for increased RBC production.,儿童贫血全英文,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%,儿童贫血全英文,Red blood cell (RBC),Term newborns have a red cell mass that is hi

8、gher 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 birth. The RBC and hemoglobin concentration in preterm infants are slightly lower than those in term infa

9、nts.,儿童贫血全英文,Red 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 clamping is delayed and the baby is held lower than placenta, both hemoglobin and blood volume are inc

10、reased by a placental transfusion.,儿童贫血全英文,Change of HB after birth,儿童贫血全英文,Reticulocyte,儿童贫血全英文,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

11、months.,儿童贫血全英文,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 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

12、approach adults WBC count level by 8 years.,儿童贫血全英文,White blood cell(WBC),The change in WBC classification 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

13、proportion between lymphocyte and granulocyte is equal at 46 days after birth.,儿童贫血全英文,White blood cell(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.,儿童贫血全英文,4-6 Day

14、s,Granulocyte,Lymphocyte,4-6 years,Change of proportion in Lymphocyte and Granulocyte,儿童贫血全英文,Platelet count,Normal value for the platelet count are about 150250109/L and vary little with age.,儿童贫血全英文,Blood volume,Blood volume in infants is more than in adults. The newborns blood volume is 10% of hi

15、s weight and about 300ml on average. A childs is about 8%10% of his weight.,儿童贫血全英文,Anemia,Defination : Anemia is defined as a reduction 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 , hem

16、oglobin concentration, or the RBC count. Anemia is not a diagnosis, but a sign of underlying disease.,儿童贫血全英文,The criteria of anemia,儿童贫血全英文,Anemia,1. Classification 1） degree : mild moderate severe Very severe 2）Morphology of RBC 3）Causes: lost blood , hemolytic , deficiency of forming Hb and RBC,儿

17、童贫血全英文,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,儿童贫血全英文,Morphology,anemia with microcytosis and hypochromia Anemia with macrocytosis Anemia with normalcytosis Anemia,儿童贫血全英文,More anemia,MCV MCH MCHC Normal 80-94 28-32 32-38 Mi

18、cro-hypochromia 94 32 32-38 microcytosis 80 28 32-38 mean corpuscular volume(MCV), means corpuscular hemoglobin (MCH), mean corpuscular hemoglobin concentration(MCHC),儿童贫血全英文,Causes,1.lost blood :acute chronic 2. hemolysis Intrinsic membrane hereditary spherocytosis Glycolysis pyruvate kinase hemogl

19、obin sickle cell,unstable Hb oxidation G6PD extrinsic : immune, infection, DIC,儿童贫血全英文,Causes,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 Toxicit

20、y Cancer cells invasion bone marrow,儿童贫血全英文,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: an

21、orexia, nausea and constipation.,儿童贫血全英文,Symptoms of anemia,Cardiovascular 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 disorder

22、s of attention.,儿童贫血全英文,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, feeding, past medical history ,family history Morphology of RBC, reticulocyte count, W

23、BC, platelet count, bone marrow cell smear, HB ,special examination,儿童贫血全英文,3.Treatment,Elimination etiology General Medicine Intravenous blood Transplantations : BM , stem cells Other,儿童贫血全英文,nutritional anemia with microcytosis and hypochromia,Definition nutritional iron deficiency anemia (IDA) Hb

24、、 most common 、 6-24ms、 special prevention,儿童贫血全英文,Iron metabolism,Iron content and distribution: 2/3 of the iron is present in HB and 1/3 in tissue and transport form.,儿童贫血全英文,Iron metabolism,Iron absorption: The primary regulator of iron homeostasis is intestinal iron absorption. Iron absorption t

25、akes 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.,儿童贫血全英文,Iron metabolism,Iron storage: Most body iron is contained in HB, with smaller amounts b

26、ound 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 appears.,儿童贫血

27、全英文,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 outstrip their reserves by 8 weeks of ag

28、e.,儿童贫血全英文,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 from food, so iron deficiency is

29、 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.,儿童贫血全英文,causes,1.inadequate iron stores: preterm infant, twin 2.intake iron deficiency 3.growth and developm

30、ent 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).,儿童贫血全英文,pathogenesis,IRON Hb microcytosis and hypochromia RBC,儿童贫血全英文,Three stage of iron deficiency,Deficiency of iron progres

31、ses 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 transferring

32、saturation and/or elevated total iron binding capacity.,儿童贫血全英文,Three stage of iron deficiency,iron deficiency anemia (IDA): iron deficiency following depletion of both marrow store and circulating iron.,ID,IDE,IDA,儿童贫血全英文,clinical manifestation,1.general manifestation: mild iron deficiency is Asymp

33、tomatic , 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,儿童贫血全英文,clinical manifestation,4. cardiovascular and respiratory manifes

34、tation: 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,clinical manifestation,Main signs may be pallor of the skin and mucous membranes. Severe an

35、emia 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,laboratory test,1.blood smear 2.bone marrow 3.iron metabolism,儿童贫血全英文,Inequality of size of erythrocytes，small cell， Central olistherozone obviously,儿童贫血全英文,h

36、ypercellular , erythroid hyperplasia , the development of cytoplasm falls behind nucleus. leukocytes and megakaryocytes are normal.,儿童贫血全英文,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,儿童贫血全英文,laboratory test,The decrease

37、 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,Blood count in iron deficiency,儿童贫血全英文,laboratory te

38、st,Bone marrow reveals increased basophilic normoblast and polychromatic normoblast. Granulocyte system and megakaryocyte system are normal.,儿童贫血全英文,Iron 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,I

39、ron metabolisms,儿童贫血全英文,diagnosis,first consider - history + clinical manifestation + blood smear Decide 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 cytop

40、lasm falls behind one of nucleus. leukocytes and megakaryocytes are normal.),儿童贫血全英文,treatment,1. nursing feeding 2. get rid of etiology 3. iron medicine 4. interfusions blood,儿童贫血全英文,Oral administration of simple ferrous salts ferrous sulfate(硫酸亚铁) ferrous gluconate（葡萄糖酸亚铁） ferrous fumarate polysac

41、charide iron Dosage: 4-6mg/kg elemental iron per day,Oral iron preparation,儿童贫血全英文,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 recover to normal level or wh

42、en SF(Serum ferritin) and FEP(Free erythrocyte protoporphyrin) is normal.,Oral iron preparation,儿童贫血全英文,Parenteral iron preparation,To be administered only for gastrointestinal malabsorption or severe intolerance prevents effective oral iron therapy.,儿童贫血全英文,Parenteral iron preparation,A parenteral

43、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 administration of iron, unless malabsorption is a factor.,儿童贫血全英文,Blood Tran

44、sfusion,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 children should be given only 2-3ml/kg of packed cells at any one time.

45、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,Iron therapy,Notice : 3 points 1.Injection iron in danger 2.Reaction : 12-24h(irritability ,appetite )- 36-48h(erythroid hyperplasia )- 48-72h(reticulocytosis)-5-7d

46、s(peaking ) 2-3ws to reticulocytes 3.Times: 6-8ws,儿童贫血全英文,Prevention,4 points mother milk feeding specter food with iron preterm infant,儿童贫血全英文,Nutritional megaloblastic anemia,Folic acid and vitamin B12 deficiency are primary causes of megaloblastic anemia.,儿童贫血全英文,The clinical features include ane

47、mia， 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,Causes,1.less intake 2.absorb abnormal 3.drug interactions 4.requirement increased,儿童贫血全英文,Pathogenesis,folic acid folic acid with 4 hydrate vitamin B12 DNA Hb very large RBC Megaloblastic wi

48、th Lot of Hb,dihydrofolate reductase,(THFA),儿童贫血全英文,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 include those of anemia and neuropathy.,Vit

49、amin B12 deficiency,neurology psychology symptom,儿童贫血全英文,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 personality changes. There is retar

50、d of intellective and physical development. Trembling of Extremities or head, hypertension of muscle, tendon reflex reinforcement, positive Babinskis sign may appear.,儿童贫血全英文,Clinical manifestation,1. General features: puffiness, poor nutrition, hair yellowed, mild edema, petechia (plt), mucocutaneo

51、us hemorrhage. 2. feature of anemia: lethargy, extramedullary 3.neurology psychology: irritability, vertigo. 4.digestive symptoms : anorexia, nausea, diarrhea.,儿童贫血全英文,Laboratory tests,1.blood smear 2.bone marrow 3.blood biochemistry tests 4. others,儿童贫血全英文,variation in BRC shape and size, macrocyto

52、sis , reticulocyte count is low , nucleated RBCs and megaloblastic morphology are often seen , thrombocytopenia,儿童贫血全英文,Hypercellular , Megaloblastic changes, hypersegmentation,儿童贫血全英文,Laboratory tests,Blood routine examination: macrocytic anemia, the decrease of red cell count is more than the decr

53、ease of HB. MCV94fl, MCH32pg. Rreticulocyte is decrease. WBC and platelets are also decreased. Bone marrow: increased basophilic normoblast and polychromatic normoblastic. Granulocytic system and megakaryocyte system : normal/less than normal.,儿童贫血全英文,Laboratory tests,Vitamine B12: normal serum vita

54、min B12 levels range from 200-800ng/L, B1212ng/L reveals B12 deficiency. Folate : normal serum folate levels range from 5-6ug/L, folate 3ug/L reveals deficiency. others :LDH : serum lactic dehydrogenase(LDH) is increase,儿童贫血全英文,Diagnosis,first consider - history + clinical manifestationMarked symptoms and signs of central nervous system.( it supports defiency of vitamin B12.) + .blood smear