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1、精选pptAnemia in childhood (小儿贫血) 精选ppt精选pptDisease of hematopoietic system infantile anemia(1)nutritional iron deficiency anemia(IDA)(2)nutritional megaloblastic anemia Primary/immunity thrombocytopenia Purpura(ITP) Leukemia 精选ppthaematogenesis of childrenhematopoiesis -produced blood extramedullary

2、before birth and postnatal mesoblast hepatic medullary 3-15w 6w-6ms 3ms 精选pptEmbryo stage Mesoblastic haematogenesis:3ws begin,8ws weaken, 12-15ws disappears。 liver:8ws begin,6months gradually weaken ,erythroblast、granular cell and megakaryocyte.精选pptEmbryo stage3、spleen:12ws begin erythrocyte, gran

3、ule ,lymphocyte4、Haematogenesis of lymphatic organ 1.thoracic gland:8ws 2.lymphatic nodes:11ws精选pptEmbryo stage5、myelo-haematopoiesis:6mons Haematogenesis function emphasis,make various kinds of blood cells,unique hematogenic organ after birth.精选ppt精选pptHaematopoiesis postnatal 1、marrow: 2、extramedu

4、llary:when requirement of haemopoiesis increase,liver、spleen、lymphadenectasis,hepatomegaly and splenomegaly, in circulating blood immature erythrocytes and granulocytes . 精选pptPhysiological haemolysis Normal newborns have higher hemoglobin(HB) and hematocrit levels and a shortened survival period of

5、 the fetal RBCs contributes to the development of physiologic anemia. 精选pptPhysiological 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 in

6、creased 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 production. 精选pptbloo

7、d characteristics ages red blood cells(RBC) and HbPhysiological haemolysis and anemia write blood cells(WBC) and classification 4-6 cross Platelets 150-250109/L blood volume 8-10% 精选pptRed blood cell (RBC) Term newborns have a red cell mass that is higher than at any other time of life. an appropria

8、te 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.精选pptRed blood cell (RBC)The wide range of he

9、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.精选pptChange of HB af

10、ter birth精选pptReticulocyte精选pptReticulocyte 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. 精选pptWhite blood cell(WBC) The normal number of WBC is hig

11、her 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 approach adults WBC count level by 8 years.精选pptWhite blood cell(WBC)

12、 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 proportion between lymphocyte and granulocyte is equal at 46 days after b

13、irth.精选pptWhite 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.精选ppt4-6 DaysGranulocyteLymphocyte4-6 yearsChange of proportion in Lymphocyte and Granulocyte

14、精选pptPlatelet count Normal value for the platelet count are about 150250109/L and vary little with age.精选pptBlood 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.精选pptAnemiaDefinat

15、ion : 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 , hemoglobin concentration, or the RBC count. Anemia is not a diagnosis, but a sign of underlying disea

16、se.精选pptThe criteria of anemiaAgeHb concentration 28 days 145 g/L14 months 90 g/L46 months 100g/L6 months6 years 110g/L614 years 120g/L精选ppt Anemia1. Classification 1) degree : mild moderate severe Very severe 2) Morphology of RBC3)Causes: lost blood , hemolytic , deficiency of forming Hb and RBC精选p

17、pt 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 精选pptMorphologynanemia with microcytosis and hypochromianAnemia with macrocytosisnAnemia with normalcytosis Anemia精选pptMore anemia MCV MCH MCHCNormal 80-94 28-32 32-38Micro-hypochrom

18、ia 80 28 94 32 32-38microcytosis 80 28 32-38 mean corpuscular volume(MCV), means corpuscular hemoglobin (MCH), mean corpuscular hemoglobin concentration(MCHC) 精选pptCauses1.lost blood :acute chronic2. hemolysis Intrinsic membrane hereditary spherocytosis Glycolysis pyruvate kinase hemoglobin sickle c

19、ell,unstable Hb oxidation G6PD extrinsic : immune, infection, DIC精选pptCauses 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 in

20、vasion bone marrow 精选pptSymptoms 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 c

21、onstipation.精选pptSymptoms 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 disorders of attention. 精选ppt2.

22、DiagnosisHistory 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, WBC, platelet count, bone ma

23、rrow cell smear, HB ,special examination精选ppt3.Treatment Elimination etiology General Medicine Intravenous blood Transplantations : BM , stem cells Other精选ppt nutritional anemia with microcytosis and hypochromiaDefinition nutritional iron deficiency anemia (IDA) Hb、 most common 、 6-24ms、 special pre

24、vention 精选pptIron metabolism 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精选pptIron metabolismIron absorption: The primary regulator of iron homeostasis is intestinal iron a

25、bsorption. 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.精选pptIron metabolismIron storage: Most body iron is contained in HB,

26、 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 second storage form, he

27、mosiderin appears.精选pptIron 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 reserve

28、s by 8 weeks of age.精选pptIron 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

29、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.精选pptcauses1.inadequate iron stores: preterm infant, ake iron deficiency3.growth and de

30、velopment increased iron requirement4.iron absorb abnormal5.a amount of iron loss: hookworm infestation, repeated venesection, Meckels diverticulum, recurrent epistaxis(反复鼻出血).精选pptpathogenesis IRON Hb microcytosis and hypochromia RBC 精选pptThree stage of iron deficiencyDeficiency of iron progresses

31、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 satu

32、ration and/or elevated total iron binding capacity. 精选pptThree stage of iron deficiency iron deficiency anemia (IDA): iron deficiency following depletion of both marrow store and circulating iron. IDIDEIDA精选pptclinical manifestation1. general manifestation: mild iron deficiency is Asymptomatic , pal

33、lor 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 精选pptclinical manifestation 4. cardiovascular and respiratory manifestation: tachycard

34、ia, increased artery pressure, wheeze, increased pulse. Severe anemia may cause heart expansion and congestive cardiac failure. 5. nervous system manifestation: vertigo, irritability.精选pptclinical manifestation Main signs may be pallor of the skin and mucous membranes. Severe anemia may cause conges

35、tive cardiac failure. IDA in infancy and early childhood is associated with developmental delay and poor growth.精选pptlaboratory test1.blood smear2.bone marrow3.iron metabolism 精选pptInequality of size of erythrocytes,small cell,Central olistherozone obviously 精选ppthypercellular , erythroid hyperplasi

36、a , the development of cytoplasm falls behind nucleus. leukocytes and megakaryocytes are normal.精选pptBone 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精选pptlaboratory test The decrease of HB concentration is more t

37、han 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.精选pptBlood count in iron deficiencyHB75g/L120g/LRBC3.541012/L4.241012/LMCV64fl86flMCHC18.5pg3

38、2pgreticulocyte1.3%1.4%WBC7.54109/L7.64109/Lproportionnormalnormalplatelet254109/L257109/L精选pptlaboratory test Bone marrow reveals increased basophilic normoblast and polychromatic normoblast. Granulocyte system and megakaryocyte system are normal.精选pptIron metabolisms Serum ferritin (SF) (血清铁蛋白) Fr

39、ee erythrocyte protoporphyrin(FEP) Serum iron, total iron binding capacity Iron in bone marrow 精选pptIron metabolismsIron study ID IDEIDASerum ferritin (SF)Iron store Red blood cell protoporphyrin (FEP) N Percent sideroblasts N Serum iron NN / 精选pptdiagnosis first consider - history + clinical manife

40、station + 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 cytoplasm falls behind one of nucleus. leukocytes and megakaryocytes are normal.)

41、精选ppt treatment 1. nursing feeding 2. get rid of etiology 3. iron medicine 4. interfusions blood精选pptOral 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精选ppt Admin

42、istration 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 when SF(Serum ferritin) and FEP(Free erythrocyte protoporphyrin) is normal. Oral iron prepa

43、ration精选pptParenteral iron preparation To be administered only for gastrointestinal malabsorption or severe intolerance prevents effective oral iron therapy. 精选pptParenteral iron preparation A parenteral iron preparation (iron dextran) is an effective form of iron and is usually safe when given in a

44、 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.精选pptBlood Transfusion With a severe anemia, immediate red blood cell transfusion may advisable, especially in card

45、iac 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. If there is evidence of frank congestive failure, a modified exchange transfusion using fresh-packed

46、 RBCs should be considered. 精选pptIron therapyNotice : 3 points1.Injection iron in danger 2.Reaction : 12-24h(irritability ,appetite )- 36-48h(erythroid hyperplasia )-48-72h(reticulocytosis)-5-7ds(peaking ) 2-3ws to reticulocytes3.Times: 6-8ws精选pptPrevention4 points mother milk feeding specter food w

47、ith iron preterm infant精选ppt Nutritional megaloblastic anemia Folic acid and vitamin B12 deficiency are primary causes of megaloblastic anemia. 精选ppt 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.精选pptCauses 1.les

48、s intake 2.absorb abnormal 3.drug interactions 4.requirement increased 精选pptPathogenesis folic acid folic acid with 4 hydrate vitamin B12 DNA Hb very large RBC Megaloblastic with Lot of Hb dihydrofolate reductase (THFA)精选pptVitaminB12 is importance in synthesis of nerve. deficiency of vitaminB12 can

49、 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. nVitamin B12 deficiency neurology psychology symptom精选ppt Patients develop a demyelinating lesion of neurons of the spinal column a

50、nd cerebral cortex. This condition results in paresthesias of the hands and feet, unsteadiness 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,

51、 positive Babinskis sign may appear.精选pptClinical manifestation1. General features: puffiness, poor nutrition, hair yellowed, mild edema, petechia (plt), mucocutaneous hemorrhage.2. feature of anemia: lethargy, extramedullary3. neurology psychology: irritability, vertigo.4. digestive symptoms : anor

52、exia, nausea, diarrhea. 精选pptLaboratory tests 1.blood smear 2.bone marrow3.blood biochemistry tests4. others精选pptvariation in BRC shape and size, macrocytosis , reticulocyte count is low , nucleated RBCs and megaloblastic morphology are often seen , thrombocytopenia 精选pptHypercellular , Megaloblasti

53、c changes, hypersegmentation 精选pptLaboratory tests Blood routine examination: macrocytic anemia, the decrease of red cell count is more than the decrease of HB. MCV94fl, MCH32pg. Rreticulocyte is decrease. WBC and platelets are also decreased. Bone marrow: increased basophilic normoblast and polychr

54、omatic normoblastic. Granulocytic system and megakaryocyte system : normal/less than normal.精选pptLaboratory tests Vitamine B12: normal serum vitamin 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 defic

55、iency. others :LDH : serum lactic dehydrogenase(LDH) is increase精选pptDiagnosis first consider - history + clinical manifestationMarked symptoms and signs of central nervous system.( it supports defiency of vitamin B12.) + .blood smear decide diagnosis-.bone marrow + metabolism(To distinguish the deficiency of folic acid with the deficiency of vitamin B12.) maybe see treatment with medicine 精选pptTreatment1.nursing feeding 2.get rid of etiology 3. medicine v

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