贫血、缺铁.ppt

,小儿贫血,2岁女性幼儿，Hb115g/L，WBC8.0109/L，N60%，L40%,，PLT120109/L。血象正常吗？,2.3天男性患儿，皮肤黄染2天就诊，BR：Hb115g/L，WBC14.0109/L，N60%，L40%,PLT120109/L。请问如何分析其黄疸原因？,Contents,DevelopmentalHaematopoiesis,MesoblasticHepaticMyeloid,EmbryonicHaemato-poiesis,Extramedullaryhematopoiesis,PostnatalHaematopoiesis,婴幼儿造血器官的特殊反应。因缺少黄髓，当造血需要增加时，肝恢复到胎儿期的造血状态出现肝肿大，同时出现脾和淋巴结肿大，外周血中出现有核红和/或幼稚中性粒细胞。贫血矫正后可恢复正常。,Extramedullaryhematopoiesis,Birth,23m,6m6y,6y,RBC:500700Hb:1522,RBCandHb,30011,45012,Hypoxiaoffetus,Physiologicalanemia,Physiologicalanemia,出生后由于EPO减少、及生理性溶血、循环血量增多，Hb、RBC逐渐下降，2-3个月时RBC为31012/L、Hb为100g/L左右。此过程自限，3个月以后逐渐恢复。,白细胞分类的变化,网织红血小板血容量,一、网织红生后3d内达46生理性贫血阶段1婴儿期同成人二、血小板初生：（150300）109/L3个月：（250300）109/L三、血容量新生儿约占体重10，儿童810成人68,Haem,Polypeptidechains,Hb,EmbryonicHbGower1,Gower2,PortlandFetalHb：HbFAdultHb：HbA，HbA2,Hb的构成,HbAHbA2HbF,30%1%70%,5%,6m6y110g/L6y14y120g/L,Definitionofanemia,Neonates145g/L14m90g/L46m100g/L,外周血单位体积红细胞数和血红蛋白低于正常。,轻度90g/L144120g/L中60g/L90g/L重30g/L60g/L极重30g/L60g/L,Neonates,Classificationofanemia,Classificationofanemia,Deficiencyofrawmaterial,Bloodloss,InadequateRBCproduction,Bonemarrowfailure,Infection,Chronicnephritis,Bonemarrowinfiltration,DefectiveRBCmembrane,enzymeorHb,Classificationofanemia,IncreasedRBCdestruction,Extracorpusculardefects,Normocyticanemia,Macrocyticanemia,Microcyticanemia,Classificationofanemia,MCV80-94MCH28-32MCHC32-38,Classificationofanemia,IDA,Microcytichypochromicanemia,-thalassemia,Leadpoisoning,Chronicinfection,Pulmonaryhemosiderosis,Chronicbloodloss,Sideroblasticanemia,一般表现造血器官反应非造血系统症状,CLINICALFEATURES,皮肤(面、耳轮、手掌等)，黏膜(口唇、睑结膜)苍白易疲倦、头晕、耳鸣、毛发干枯、营养低下体格发育迟缓,非造血系统表现循环、呼吸系统:呼吸、心率加快，重度时心脏扩大、心脏杂音、心衰;消化系统：食欲减退，偶有舌炎,舌乳头萎缩；神经系统：精神不振，注意力不集中免疫系统：免疫功能下降，易感染,小儿贫血的诊断,病史发病年龄：出生时产前、产时出血生后48小时内伴黄疸新生儿溶血病婴儿期营养性、遗传性儿童期失血、再障、其他,病程经过和伴随症状起病快、病程短：急性溶血或急性出血；起病缓慢：营养性贫血、慢性溶血、慢性失血；伴随症状：黄疸、血红蛋白尿，出血感染，神经症状，骨痛，肿块，肝脾肿大等；,喂养史：添加辅食，饮食质和量，食物搭配等过去史：感染史(结核、钩虫)慢性疾病史(肾病、风湿等)服药史（氯霉素、磺胺等）家族史：遗传性贫血,体格检查生长发育：发育障碍和特殊面容营养状况皮肤、黏膜指甲、毛发肝、脾淋巴结,Laboratoryfindings,HemogramRetBMCombstestRBCfragilityEnzymeanalysisHbanalysis,小儿贫血的治疗原则,去除病因一般治疗药物治疗铁剂IDA维生素B12、叶酸巨幼红细胞贫血皮质激素自溶、纯红再障联合免疫抑制再障,红细胞输注注意适应征、速度和量一般每次510ml/kg极重度或合并肺炎：57ml/kg,IronDeficiencyAnemia,Contents,Characteristics,Themostcommonkindofanemiainchildrenagedfrom6-24months,3%of12yr-oldchildrenhaveIDA2%ofadolescentgirlshaveIDAInadolescentboys,a50%decreaseinstoredironoccurs,IntheUnitedStates,InChina,10%ofchildrensufferedfromIDA,IDAisunusualbefore6months?,Sufficientstoresofironinthefirst4mooflifeinterminfants.2.Physiologicalanemiaduring2-3mo,3.Physiologicalhemolysis,铁的代谢,人体内铁含量新生儿75mg/kg成人男性50mg/kg女性35mg/kg铁的需要量早产儿：约2mg/（kgd）4月3岁：约1mg/（kgd）各年龄儿总摄入量:15mgd,血红蛋白约64%，肌红蛋白约3.2%;铁蛋白及含铁血黄素(肝、脾、骨髓等)约32%;微量(1%)存在于含铁酶和运转铁;,铁的分布,铁的来源,红细胞释放的铁,食物,血红素铁：吸收率10%-25%；母乳含铁0.05mg/dl,吸收率49%;牛乳含铁0.05mg/dl,吸收率4%；非血红素铁:吸收率1.7%-7.9%,食物铁含量、吸收率比较食物铁含量（mg/100g）吸收率%菠菜2.91.3蛋黄6.53牛乳0.54黄豆8.27肉类3.425（1070）母乳0.54970,Metabolismofiron,Fe2+,Tf,Serumiron,apoferritin,Fe3+,Hb,MHb,enzyme,ironreleasedfromRBC,ferritin,Intestinalmucosa,Tf-R,ferritin,促进铁吸收:还原物质,如VitC、稀盐酸、果糖、氨基酸等,使Fe3+Fe2+;铁吸收下降:磷酸、草酸等与铁形成不溶性铁酸盐;抑制铁吸收:植物纤维、咖啡、蛋、牛奶、抗酸药等;,影响铁吸收的因素,血清铁（serumiron,SI）：与转铁蛋白结合的铁。未饱和铁结合力：未与铁结合的剩余2/3转铁蛋白仍有结合铁的能力，在体外加入一定量的铁即能使其饱和，所加入的铁量即未饱和铁结合力。血清总铁结合力（totalironbindingcapacity,TIBC）：血清铁+未饱和铁结合力转铁饱和度（transferinsaturation,TS）：SI/TIBC。,几个名词,Hemachrome,Polypeptidechain,Protoporphyrin,Iron,+,+,Pathogenesis,Hb,Hypochromicmicrocyticanemia,Pathogenesis,irondepletion（ID）irondeficienterythropoiesis（IDE）irondeficiencyanemia（IDA）,SFEF,SF,EFFEP,Pathogenesis,Hb,inadequateirondeposit,growthspurt,inadequatedietaryintake,bloodloss,malabsorption,ETIOLOGY,PepticulcerMenstruationHookworminfestationMeckel,sdiverticulumPolypHemangioma,临床表现,1.一般表现：皮肤黏膜苍白（唇、口腔黏膜、甲床明显）；疲乏，不爱活动；年长儿头晕、眼前发黑、耳鸣等。2.造血器官反应：髓外造血,3.非造血系统症状消化系统：食欲减退，异食癖；口腔炎、舌炎或舌乳头萎缩，严重者萎缩性胃炎或吸收不良综合征神经系统：烦躁不安或萎靡不掁，精神不集中，记忆力减退心血管系统：心率增快，严重者心脏扩大、心衰。免疫功能降低：易感染。上皮组织异常：如反甲。,LABORATORYFINDINGS,Bloodroute:HypochromicandmicrocyticanemiaRetNorBonemarrowsmear:Normal.,Hypochromicmicrocytearetypical.Largecentralpalloristhemarkerofhypochromia.,LABORATORYFINDINGS,FEPFEP提示红胞内缺铁铅中毒、慢性炎症和先天性原卟啉增多症，FEPSI,TIBC,TS（反映血浆中铁含量）IDA期：SI和TS，TIBC感染、肿瘤、慢性炎症时SI降低SF反映体内储存铁感染、肿瘤、肝脏、心脏疾病SF明显增高,DIAGNOSIS,1.Hypochromicmicrocyticanemia2.Thecausesofirondeficiency3.TheintensityofPrussianbluestainingofmarrowdecreases.4.FEP0.9mol/L(50g/dl）,5.SF16g/L6.Serumiron(SI)10.7mol/L7.TIBC62.7pmol/L,TS0.15or0.18.Regularresponsestoadequateiron,Diagnosis:The1st+atleast2among2nd-8th,DIAGNOSIS,DIFFERENTIALDIAGNOSIS,thalasse-mia,leadpoisoning,chronicinfection,pulmonaryhemosiderosis,extravascularhemolyticanemia,abnormalhemoglobinelectrophoresis,RetSF,chronicinfectionSF,PbSF,cough,hemo-sputum,anemia,abnormalCXR,hemosideringranule,Ret,Elementaliron:1-2mg/kg/timepo,BidorTidThedurationoftherapy:68weeksafterHbcontentsarenormal,IronAdmini-stration,TREATMENT,Retrises:the3th4thdaypeaking:the7th10thdayregainsnormal:23weeksHbincreases:12weeksregainsnormal:34weeks,TREATMENT,Responsestoirontherapy,注射铁剂副作用多，慎用适应征诊断肯定但口服铁剂无效。口服反应严重，改变剂型、剂量无改善者；胃肠手术后无法口服者。,常用剂型山梨醇构椽酸铁复合物；肌注。右旋糖酐铁复合物；肌注、静注。葡萄糖氧化铁：静注。,药品名称剂型规格含元素铁每日剂量硫酸亚铁0.3/片20%2030mg/kg2.5%合剂5mg/ml0.81.2ml/kg富马酸铁每片0.05或0.233%1520mg/kg干糖浆3.3%90180mg/kg葡萄糖酸亚铁0.3/片12%4050mg/kg糖浆0.3g/10ml1ml/kg琥珀酸亚铁0.1/片35%918mg/kg多糖铁化合物150mg/胶囊46%812mg/kg(力蜚能),常用铁剂剂量表,TREATMENT,Bloodtransfusion,1.Verysevereanemia,especiallycomplicatedwithcongestiveheartfailure2.Superimposedinfection,红细胞输注Hb小于30g/L者，等量换血Hb30g/L6