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1、Anemia in childhood (小儿贫血) Disease of hematopoietic systeminfantile anemia1nutritional iron deficiency anemia(IDA)2nutritional megaloblastic anemiaPrimary/immunity thrombocytopenia Purpura(ITP)Leukemia haematogenesis of childrenhematopoiesis -produced blood extramedullary before birth and postnatal
2、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 stage3、spleen:12ws begin erythrocyte, granule ,lymphocyte4、Haematogenesis of ly
3、mphatic organ1.thoracic gland:8ws 2.lymphatic nodes:11wsEmbryo stage5、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 haemopoiesis increase,liver
4、、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 contributes to the development of physiolo
5、gic 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 shortened surviva
6、l 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 HbPhysiological haem
7、olysis 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. The RBC co
8、unt 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 umbilical
9、 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 birthReticulocyteReticulocyte Reticulocyte is 0.04-0.06 in the first 3 days. Reticul
10、ocyte 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 hou
11、rs, 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 about 3
12、0% 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
13、at 46 years. After 7 years white cell classification in infants is similar to that in adult.4-6 DaysGranulocyteLymphocyte4-6 yearsChange of proportion in Lymphocyte and GranulocytePlatelet count Normal value for the platelet count are about 150250109/L and vary little with age.Blood volume Blood vol
14、ume 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.AnemiaDefination : Anemia is defined as a reduction of the red blood cell volume or hemoglobin concentration below the range of values occurring in
15、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 anemiaAgeHb concentration 28 days 145 g/L14 months 90 g/L46 months 100g/L6 months6 years 110g/L614 years 120g/L Anem
16、ia 1. Classification 1 degree : mild moderate severe Very severe 2 Morphology of RBC 3Causes: lost blood , hemolytic , 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 Morphologynanemia with microcytosis a
17、nd hypochromianAnemia with macrocytosisnAnemia with normalcytosis AnemiaMore anemia MCV MCH MCHCNormal 80-94 28-32 32-38Micro-hypochromia 80 28 94 32 32-38microcytosis 80 28 32-38 mean corpuscular volume(MCV), means corpuscular hemoglobin (MCH), m e a n c o r p u s c u l a r h e m o g l o b i n conc
18、entration(MCHC) Causes1.lost blood :acute chronic2. hemolysis Intrinsic membrane hereditary spherocytosis Glycolysis pyruvate kinase hemoglobin sickle cell,unstable Hb oxidation G6PD extrinsic : immune, infection, DICCauses 3.deficiency of forming Hb and RBCdeficiency of hematopoiesis substance medu
19、llary hematopoiesis 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 muc
20、ous membranes, lethargy, 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 cau
21、se heart expansion 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,
22、course, symptoms, feeding, past medical history ,family history Morphology of RBC, reticulocyte count, WBC, platelet count, bone marrow cell smear, HB ,special examination3.Treatment Elimination etiology General Medicine Intravenous blood Transplantations : BM , stem cells Other nutritional anemia w
23、ith microcytosis and hypochromiaDefinition 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. Content of elemental iron (mg/kg)Adult females40Adult
24、 males50newborn75Iron metabolismIron 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 the basolateral membran
25、es of these cells to reach the circulation.Iron metabolismIron 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.The major iron stor
26、es are in the form of ferritin.As iron continues to accumulate in the cell, a second storage form, hemosiderin appears.Iron 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 ha
27、ve limited iron stores and because of their higher rate of growth, they outstrip their reserves by 8 weeks of age.Iron 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
28、 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.causes1.ina
29、dequate iron stores: preterm infant, ake 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 m i c r o c y t o s i s
30、 a n d hypochromia RBC Three stage of iron deficiencyDeficiency 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):
31、 loss of circulating 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. IDIDEIDAclinical manifesta
32、tion1. general 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 manifestati
33、on 4. cardiovascular 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 s
34、kin and mucous membranes. 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 smear 2.bone marrow 3.iron metabolism Inequality of size of erythrocytes,small cell,Central olistherozone ob
35、viously hypercellular , erythroid 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正常骨髓铁染正常骨髓铁染色色IDA iron stain铁缺乏骨铁缺乏骨髓铁染色髓铁染色laboratory test The decr
36、ease 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. .Blood count in iron deficiencyHB75g/L120g/LRBC3.541012/L4
37、.241012/LMCV64fl86flMCHC18.5pg32pgreticulocyte1.3%1.4%WBC7.54109/L7.64109/Lproportionnormalnormalplatelet254109/L257109/Llaboratory test Bone marrow reveals increased basophilic normoblast and polychromatic normoblast. Granulocyte system and megakaryocyte system are normal.Iron metabolisms Serum fer
38、ritin (SF) (血清铁蛋白) Free erythrocyte protoporphyrin(FEP) Serum iron, total iron binding capacity Iron in bone marrow Iron metabolismsIron study ID IDEIDASerum ferritin (SF)Iron store Red blood cell protoporphyrin (FEP) N Percent sideroblasts N Serum iron NN / diagnosis first consider - history + clin
39、ical manifestation + blood smear Decide 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 ar
40、e normal.) treatment 1. nursing feeding 2. get rid of etiology 3. iron medicine 4. interfusions bloodOral administration of simple ferrous salts ferrous sulfate(硫酸亚铁硫酸亚铁) ferrous gluconate葡萄糖酸亚铁葡萄糖酸亚铁ferrous fumaratepolysaccharide iron Dosage: 4-6mg/kg elemental iron per day Oral iron preparation Ad
41、ministration 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 pr
42、eparationParenteral iron preparation To 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 proper
43、ly 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.Blood Transfusion With a severe anemia, immediate red blood cell transfusion may advisable, especially in cardiac failure
44、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 RBCs should
45、 be considered. Iron 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: 6-8wsPrevention 4 points mother milk feeding specter food with iron preterm infa
46、nt Nutritional megaloblastic anemia 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 int
47、eractions 4.requirement increased Pathogenesis folic acid folic acid with 4 hydrate 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
48、macrocytic anemia produced by deficiency of vitamin B12, the symptoms and signs include those of anemia and neuropathy. nVitamin B12 deficiency neurology psychology symptom Patients develop a demyelinating lesion of neurons of the spinal column and cerebral cortex. This condition results in paresthe
49、sias 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, positive Babinskis sign may appear.Clinical manifesta
50、tion1. 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 : anorexia, nausea, diarrhea. Laboratory tests 1.blood smear 2.bo
51、ne marrow3.blood biochemistry tests4. othersvariation in BRC shape and size, macrocytosis , reticulocyte count is low , nucleated RBCs and megaloblastic morphology are often seen , thrombocytopenia Hypercellular , Megaloblastic changes, hypersegmentation Laboratory tests Blood routine examination: m
52、acrocytic 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 polychromatic normoblastic. Granulocytic system and megakaryocyte system : normal/less
53、 than normal.Laboratory 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 deficiency. others :LDH : serum lactic dehydrogenase(LDH) is increaseDiagnosis first cons
54、ider - 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 Treatment1.nursing feeding 2.get rid of etiology 3. medicine vit B12 ,folic acid Vitamin B12 preparation Vit B12 preparatio
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