儿科学之小儿贫血.ppt

儿科学之小儿贫血儿科学之小儿贫血 Teaching purposeTo 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,pathogenosis,diagnosis,therapy and prevention of nutritional iron deficiency anemia and nutritional megaloblastic anemia.Hematopoesis in bone marrow Yellow bone marrow（黄骨髓）can come back to red bone marrow（红骨髓）when hematopoetic need increases.Yellow bone marrow is deficiency in children,especially in infant and toddler period.When hematopoietic demand increases,liver,spleen and lymph nodes come back to the Status to produce blood cells,hepatomegaly（肝肿大）and splenomegaly（脾肿大）appears,and maybe there are immature erythrocytes and granulocytes in circulating blood.Extramedullary hemopoiesis is the specific phenomena only appearing in infant and toddler.Extramedullary hemopoiesis（髓外造血）Features of blood in childrenErythrocyte and hemoglobin 7654321012h 10d 3m6m1yRBC(1012/L)YearsHemoglobin At birth1 yr2 yrLeucocyte countWBC109/L9h10d1y8y123%2040608005d5yDifferential count of WBC(白细胞分类计数)Lymphocyte淋巴细胞Granulocyte粒细胞MorphologicalclassificationofanemiaTypesMCV（fl）MCH(pg)MCHC(%)Normal809428323238macrocytic（大细胞性）（大细胞性）94323238normocytic（正细胞性）（正细胞性）809428323238microcytic（小细胞性）（小细胞性）80283238hypochromicmicrocytic（小细胞低色素性）（小细胞低色素性）802832Blood routine Hypochromicmicrocytic Macrocytic Normocytic（HbRBC）（HbRBC)（Hb=RBC）IDA（缺铁性贫血）（缺铁性贫血）megaloblastic acute bleedingThalassemia Vit B12 hemolytic anemiasideroblastic anemia folic acid aplastic anemiaChronic infection most of secondary anemia AgeHb concentration(g/L)28 d 14514 mon 9046 mon 1006 mon6 y 110614 y 120nThe criterion of anemia.Nutritional iron deficiency anemia(IDA)营养性缺铁性贫血营养性缺铁性贫血 Zhou,male,10 months old.Paleness in face and lips for 4 mons.His facial expression became more and more pale since 6 months of age.Without fever and bleeding.He was premature birth at 33 weeks of gestational age and fed with Mothers milk only.Case 1PE:Pallor.no jaundice and hemorrhagic spots in skin and sclera;Bilateral submandibular lymph nodes are 0.80.8cm.Breath sounds in lungs are distinct;there are no rhonchi and moist rale.HR 100/min.There is II/VI systolic murmur in precordial region.The liver and spleen enlarge to 4 cm and 3 cm below lower costa margin respectively.Case 1Blood routine testWBC4.0109/LLym2.4(60%)109/LGran1.6(40%)109/LRBC2.181012/LHb47g/LMCV70.4flMCH22.4pgMCHC176g/LRDW23.7%RC0.026Plt194109/Lquestions Do an anemia exist?Which kind of anemia does it belong?answerHypochromicmicrocytic anemia(小小细细胞低色素性胞低色素性贫贫血）血）questions What is the cause of anemia in the case above?answer The cause of anemia in the case above is deficiency of iron because he is premature and is fed with Mothers milk only.etiology The etiological factors of nutritional iron deficiency anemia:v Stored iron deficiency at birth.v Inadequate diet.v Rapidly growth.v Malabsorption.v Excessive loss of iron.questionsNextexaminations?Smear of Blood normalThe patient Bone marrow BonemarrowexaminationThe bone marrow is hypercellular,with erythroid hyperplasia（过 度 增 生）.The normoblasts(幼 红 细 胞)may have scanty,fragmented cytoplasm with poor hemoglobinization.Leukocytes and megakaryocytes are normal.Hemosidenin cannot be demonstrated in marrow specimens by Prussian blue staining.questionsHow do iron deficiency causeanemia?questions Pathogenesis of nutritional iron deficiency anemia.铁缺乏铁缺乏 贮存铁贮存铁 血清铁血清铁 血红蛋白铁血红蛋白铁 酶铁酶铁 红细胞内合成红细胞内合成 含铁酶含铁酶 细胞分裂不受影响细胞分裂不受影响 铁依赖酶铁依赖酶 ，RBC正常或正常或 某些细胞功能紊乱，如某些细胞功能紊乱，如 生物氧化、细胞呼吸生物氧化、细胞呼吸小细胞低色素性贫血小细胞低色素性贫血 神经递质分解与合成等神经递质分解与合成等 血液系统症状血液系统症状 非血液系统症状非血液系统症状 一般贫血表现一般贫血表现 行为发育异常行为发育异常 髓外造血表现髓外造血表现 皮肤粘膜损害皮肤粘膜损害 心血管系统症状心血管系统症状 细胞免疫功能细胞免疫功能缺铁性贫血发病机理缺铁性贫血发病机理Iron studyIron studyIDIDEIDA*serum ferritin(SF)Iron storeFEP(红细胞游离原卟啉）NsideroblastsNSerum ironNN orTherapy good nursingremoval of etiological factors treatment with iron preparation Oral administration of simple ferrous salts ferrous sulfateferrous gluconateferrors fumaratepolysaccharide iron Dosage:4-6mg/kg elemental iron per day Oral iron preparation Administration the iron prior 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 and FEP is normal.Oral iron preparationParenteral iron To be administered only for gastrointestinal malabsorption or severe intolerance prevents effective oral iron therapy.ReticulocytosisTransfusionPreventionNutritional Megaloblastic Anemia（营营养性巨幼养性巨幼细细胞性胞性贫贫血）血）Folic acid and vitamin B12 deficiency are primary causes of megaloblastic anemia.Case 2.Zhou,male,8 month old.pallor for 2 mo.progressive drowsiness and go asleep for 20 hr pre day.He can not smile and to raise his head,which can be done before.He was fed with Mothers milk only.Case 2.PE:emotional expression Sluggishness（呆滞）,Pallor.no jaundice,rashes and hemorrhagic spots in skin;pallor in his lips.Breath sounds are distinct and there are no rale in lungs.Heart rate is 110 times per minute.The liver and spleen enlarge to 3 centimeters and 4 centimeters below lower costa margin respectively.Blood routineBeforeAfternormallevelsWBC3.18.6(4.010.0)109/LLYM83.066.0(5070)%GRAN17.032(3050)%RBC1.982.93(3.55.5)1012/LHb6979(110160)g/LMCV103.388.0(8292)flMCH34.827.0(2734)pgMCHC337307(320360)g/LRDW19.420.9(11.514.5)%RC0.0180.090(0.0050.015)PLT80644(100300)109/Lquestions:1.Which kind of anemia it belongs?Answer The clinic data shows a macrocytic anemia.Smear of bloodpatientnormalSmear of patientbloodBM(骨髓骨髓)BonemarrowexaminationThe bone marrow is hypercellular because of erythroid hyperplasia.Megaloblastic changes are prominent,although some normal RBC precursors may also be found.Large,abnormal neutrophilic forms(giant metamyelocytes)with cytoplasmic vacuolation are seen,as well as hypersegmentation of the nuclei of megakaryocytes.questions:2.IsitcausedbydeficiencyoffolicacidorvitaminB12?Answer In this case,macrocyticanemiaiscausedbydeficiencyofvitaminB12.Why?questions:3.what is the differences in clinical manifestation between deficiency of folic acid and deficiency of vitamin B12?叶酸叶酸 四氢叶酸四氢叶酸 核苷酸尤其胸苷合成核苷酸尤其胸苷合成 VitB12 DNA合成合成 RNA合成正常合成正常 神经髓鞘中脂神经髓鞘中脂 细胞分裂细胞分裂 蛋白质蛋白质,如如Hb 蛋白合成不足蛋白合成不足 细胞增殖细胞增殖 合成正常合成正常 神经纤维功能神经纤维功能 细胞巨幼变细胞巨幼变 不完整不完整 细胞数量下降细胞数量下降 表情呆滞表情呆滞,反应差反应差 RBC WBC PLT 其它组织其它组织 发育落后发育落后,常倒退常倒退 细胞细胞 肢体震颤肢体震颤 可有病理反射可有病理反射 精神神经症状精神神经症状 巨幼细胞性贫血巨幼细胞性贫血营养性巨幼细胞性贫血发病机理营养性巨幼细胞性贫血发病机理 Answer In the macroblastic anemia produced by deficiency of vitamin B12,the symptoms and signs include those of anemia and neuropathy.Patients develop a demyelinating lesion of neurons of the spinal column and cerebral cortex.This condition results in paresthesias(感觉异常)of the hands and feet,unsteadiness of gait,and eventually memory loss and personality（智力）changes.There is retard of intellective and physical development.Trembling（震颤）of Extremities or head,hypertension of muscle,tendon reflex reinforcement,positive Babinskis sign may appear.DiagnosisPreliminary assessment of megaloblastic anemiaDiagnosisFinal diagnosis:To distinguish the deficiency of folic acid with the deficiency of vitamin B12.Marked symptoms and signs of central nervous system.(it supports defiency of vitamin B12.)Measurement of serum folate level and cobalamin level.Final diagnosisDiagnosisTherapyTo remove the etiological factors and ameliorate diet.Special treatment:Vitamin B12 preparation to treat vitamin B12 deficiency.Not to use folic acid preparation in patients with vitamin B12 deficiency only.Folic acid preparation to treat folic acid deficiency.TreatmentTherapy循征医学思考题n什么是髓外造血？什么是髓外造血？n小儿红细胞和血红蛋白有何变化规律？什么是生理性小儿红细胞和血红蛋白有何变化规律？什么是生理性贫血？各年龄组贫血的诊断标准是什么？贫血？各年龄组贫血的诊断标准是什么？n小儿白细胞计数有何变化规律？白细胞计数两次交叉小儿白细胞计数有何变化规律？白细胞计数两次交叉的时间是什么？的时间是什么？n按形态学分类贫血分那几类？按形态学分类贫血分那几类？n儿科小细胞低色素性和大细胞性贫血常见于那些疾病儿科小细胞低色素性和大细胞性贫血常见于那些疾病？n那些病因可引起机体缺铁？最常见的病因是什么？那些病因可引起机体缺铁？最常见的病因是什么？n人体缺铁如何引起贫血及非造血系统系统症状？人体缺铁如何引起贫血及非造血系统系统症状？n检查机体缺铁的较灵敏的化验指标有哪些？这些指标检查机体缺铁的较灵敏的化验指标有哪些？这些指标反映是铁缺乏的哪一阶段？反映是铁缺乏的哪一阶段？n补铁首选那种剂型？注射铁剂是否比口服铁剂好？补铁首选那种剂型？注射铁剂是否比口服铁剂好？n补充铁剂的疗程应多长？是否血常规恢复正常后即可补充铁剂的疗程应多长？是否血常规恢复正常后即可停药？停药？n判断铁剂治疗有效的简便，灵敏指标是什么？判断铁剂治疗有效的简便，灵敏指标是什么？n缺铁性贫血病人什么情况下考虑输血？是成份输血还缺铁性贫血病人什么情况下考虑输血？是成份输血还是输全血？是否贫血越严重输血应越快，越多，为什是输全血？是否贫血越严重输血应越快，越多，为什么？么？n那些原因可致维生素那些原因可致维生素B12和叶酸缺乏？和叶酸缺乏？n维生素维生素B12和叶酸缺乏如何引起贫血？两者临床表现和叶酸缺乏如何引起贫血？两者临床表现有何不同？有何不同？n巨幼细胞性贫血的骨髓象有何特点？巨幼细胞性贫血的骨髓象有何特点？n缺铁性贫血与巨幼细胞性贫血的血常规，骨髓象有何缺铁性贫血与巨幼细胞性贫血的血常规，骨髓象有何不同？不同？n营养性巨幼细胞性贫血如何治疗？叶酸或维生素营养性巨幼细胞性贫血如何治疗？叶酸或维生素B12的疗程应多长？的疗程应多长？n早孕母亲为什么要服用叶酸，服多长时间，为什么？早孕母亲为什么要服用叶酸，服多长时间，为什么？结束结束