先天性甲状腺功能减低症15411.ppt

,PEDIATRICS,先天性甲状腺功能减低症,CongenitalHypothyroidism,introduction,name-previouslycalledcretinism,nowitisdiscardedConcept-Hypothyroidismresultsfromdeficientproductionofthyroidhormoneordefectinitsreceptor.Thecharacteristicsislowmetabolismlevelandretardationingrowthandmentaldevelopment.,etiology,1.errorsinembryologicdevelopmentofthyroid2.congenitaldefectofthyroidfunctiondefectivesynthesisofthyroxine2)poorresponsivenessofTSH3.congenitaldefectofhypothalamusandpituitarygland,clinicalmanifestation,growthanddevelopmentretardationshortstaturewithshortlimbs,bighead,enlargedanteriofontanelle,delayeddentitionandmotordevelopment2.mentalretardationtorpor,poorresponsive,awkwardactionandloweredIQ,clinicalmanifestation,3.characteristicfaciesThepatienthasdepressedbridgeofbroadnose.Eyesappearfarapart.Thechildhasthicklip,large,thickandprotrudingtongueandptyalism.Thehairsparseandtheskiniscoarse.,clinicalmanifestation,4.loweredphysiologicfunctionpresentaslowbodytemperature,lesssweat,coollimbs,weakandslowpulse,lowheartsound,distensiveabdomen,umbilicalherniaandconstipation.5.otersLowerrenalfiltration,hyponatremiaandmoderateanemia.,clinicalmanifestation,6.clinicalmanifestationinneonatalperiod1)Heavierbirthweightoftenmorethan4000g.2)Feedingdifficulties.3)Prolongedphysiologicjaundice.4)Distentiveabdomenandconstipation.,先天性甲低男孩，13岁,diagnosis,Inneonatalperiod,todiagnosisisdifficulty,soneonatalscreeningisimportant.Beforetheappearanceofcharacteristicclinicalsymptoms,mentaldisturbancehasappeared.soearlierperioddiagnosisisimportant.Todiagnosisdependsonlaboratoryexamination.Itisnoteasytodiagnosisdependsonclinicalfindings.,diagnosis,Estimationofthyroidfunction.skeletalX-rayexamination99mTcscanning,treatment,Exceptneonataltransienthypothyroidism,itisnecessarytotakethyroidhormoneallthelifetokeepthephysiologicalfunction.NoNSsequelaoccursasthethetherapyisbegunafterbirthorintwomonths.theearlierthetime,thebettertheeffects.Toadjustdoseasneeded,治疗前,治疗后,Question1:Girl,4months,wasborninwinter,full-term，normaldelivery,puremilkfeeding.Irritabilityforhalfmonths,nightsleepisnotgood.Whichsignsshouldweespeciallypayattentionto?A.CraniotabesB.costalrosaryC.wristknobbyorankleknooby,Question2:Thepatient,10months,irritability,sweatforaweek,nofever,milkfeeding.whichsignscanwefindinthephysicalexamination?A.Dehydration,dryskinB.enlargedanteriorfontanel,delayeddentitionC.normal,Question2:Thepatient,10months,irritability,sweatforaweek,nofever,milkfeeding.themechanismofthediseasemaybe:A.vitaminDdeficiencyB.excessiveexcretionofcalciumfromUrinaryC.Parathyroidhypersecretion,Question2:If1weekslaterthepatienthadsuddenlyconvulsion,physicalexamination:highmuscletension,eyesontheturn,normaltemperature,whichofthefollowingmethodsshouldbechoosedfirstly?A.antibioticsB.glucoseC.calcium+chloralhydrateD.vitaminD,Question3:Boy,1year,abdominaldistension,constipation,lowreaction,lesscry,moresleepfor11months.physicalexamination:T36.2,coolextremities,coarseskin,sparsehair,weakandslowpulse,lowheartsound,umbilical