医学专题一新生儿黄疸诊治.ppt

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1、2023/4/251新生儿黄疸新生儿黄疸(hungdn)(hungdn)诊治诊治 第一页，共三十四页。Paediatrics&Child Health1999;4(2):161-164ReferenceNo.FN98-02RevisioninprogressMay2007Paediatrics&Child Health2007;12(5):1B-12BReferenceNo.FN07-02Guidelinesfordetection,managementandpreventionofhyperbilirubinemiaintermandlatepretermnewborninfants参考文献

2、第二页，共三十四页。Hyperbilirubinemiaisverycommonandusuallybenigninthetermnewborninfantandthelatepreterminfantat35to36completedweeks.Criticalhyperbilirubinemiaisuncommonbuthasthepotentialforcausinglong-termneurologicalimpairment.Earlydischargeofthehealthynewborninfant,particularlythoseinwhombreastfeedingmayn

3、otbefullyestablished,maybeassociatedwithdelayeddiagnosisofsignificanthyperbilirubinemia.高胆红素血症很常见，多为良性。危险的高胆红素血症并不常见，但是(dnsh)有潜在的导致长期神经损害的可能。第三页，共三十四页。胆红素水平胆红素水平(shupng)与胆红素脑病发生与胆红素脑病发生Itisestimatedthat60%oftermnewbornsdevelopjaundiceand2%reachaTSBconcentrationgreaterthan340mol/L(19.8mg/dl).Acuteenc

4、ephalopathydoesnotoccurinfull-terminfantswhosepeakTSBconcentrationremainsbelow340mol/LandisveryrareunlessthepeakTSBconcentrationexceeds425mol/L(24.85mg/dl).Abovethislevel,theriskfortoxicityprogressivelyincreases.Morethanthree-quartersoftheinfantsintheUnitedStateskernicterusregistry(between1992and200

5、2)hadaTSBconcentrationof515mol/L(30.1mg/dl)orgreater,andtwo-thirdshadaconcentrationexceeding600mol/L(35mg/dl).Evenwithconcentrationsgreaterthan500mol/L(29.2mg/dl),therearestillsomeinfantswhowillescapeencephalopathy.第四页，共三十四页。Kernicterus（核黄疸核黄疸）:thepathologicalfindingofdeep-yellowstainingofneuronsand

6、neuronalnecrosisofthebasalganglia（基底节）andbrainstemnuclei（脑干神经元）.Acutebilirubinencephalopathy（急性（急性(jxng)胆红素脑病）胆红素脑病）:aclinicalsyndrome,inthepresenceofseverehyperbilirubinemia,oflethargy（昏睡）,hypotoniaand（肌张力减低）poorsuck,whichmayprogresstohypertonia(withopisthotonos（角弓反张）andretrocollis（颈后倾）)withahigh-p

7、itchedcryandfever,andeventuallytoseizures（发作）andcoma.Chronicbilirubinencephalopathy（慢性胆红素脑病）（慢性胆红素脑病）:theclinicalsequelaeofacuteencephalopathywithathetoidcerebralpalsy（手足徐动症样大脑麻痹）withorwithoutseizures,developmentaldelay,hearingdeficit,oculomotor（眼球运动异常）disturbances,dentaldysplasia（牙发育异常）andmentaldef

8、iciency.Severehyperbilirubinemia（严重的高胆红素血症）（严重的高胆红素血症）:atotalserumbilirubin(TSB)concentrationgreaterthan340mol/Latanytimeduringthefirst28daysoflife.Criticalhyperbilirubinemia（危险的高胆红素血症）（危险的高胆红素血症）:aTSBconcentrationgreaterthan425mol/Lduringthefirst28daysoflife.第五页，共三十四页。具有危险具有危险(wixin)因素人群中患者与非患者之比相当

9、于不具有危险因素人群中患者与非患者之比相当于不具有危险(wixin)因素人群中患者与非患者之比的倍数因素人群中患者与非患者之比的倍数第六页，共三十四页。脱水，高渗，呼吸(hx)窘迫，水肿，早产，酸中毒，低白蛋白血症，缺氧，抽搐可增加急性脑病的发生率与败血症的关系?Allofthereasonsforthevariablesusceptibilityofinfantsarenotknown;however,dehydration,hyperosmolarity,respiratorydistress,hydrops,prematurity,acidosis,hypoalbuminemia,hyp

10、oxiaandseizuresaresaidtoincreasetheriskofacuteencephalopathyinthepresenceofseverehyperbilirubinemia,althoughreliableevidencetoconfirmtheseassociationsislacking.Inaddition,someinfantswithseverehyperbilirubinemiaarefoundtohavesepsis,butbothsepsisandhyperbilirubinemiaarecommonintheneonatalperiod,andsep

11、sisappearstobeuncommoninthewell-appearinginfantwithseverehyperbilirubinemia.第七页，共三十四页。黄疸的发生（总体(zngt)发生情况）Early(days1-2)-uncommonHaemolyticjaundice(ABO,others)Normal(days3-10)-verycommonUncomplicatedComplicated-seebelowLate(days14+)Breastmilk-commonConjugatedjaundice-uncommonInheriteddeficiencyofgluc

12、uronyltransferaseenzymes-veryrare第八页，共三十四页。Investigations:Measurementofbilirubin经皮测并不准确（与是否光疗后，皮肤颜色及厚度经皮测并不准确（与是否光疗后，皮肤颜色及厚度(hud)都有关）都有关）ThereareseverallimitationstoTcBmeasurements:theybecomeunreliableafterinitiationofphototherapy,andtheymaybeunreliablewithchangesinskincolourandthickness.However,the

13、resultsaremoreaccurateatlowerlevelsofbilirubin,andtherefore,useofTcBasascreeningdeviceisreasonableClinicalevaluationKramersRuleRatherthanestimatingthelevelofjaundicebysimplyobservingthebabysskincolour,onecanutilisethecephalocaudalprogressionofjaundice.Kramerdrewattentiontotheobservationthatjaundices

14、tartsonthehead,andextendstowardsthefeetasthelevelrises.ThisisusefulindecidingwhetherornotababyneedstohavetheSBRmeasured.Kramerdividedtheinfantinto5zones,theSBRrangeassociatedwithprogressiontothezonesisasfollows:第九页，共三十四页。ClinicalmanagementofhyperbilirubinemiaininfantsTABLE1:Laboratoryinvestigationfo

15、rhyperbilirubinemiaintermnewborninfantsIndicated(ifbilirubinconcentrationsreachphototherapylevels)SerumtotalorunconjugatedbilirubinconcentrationSerumconjugatedbilirubinconcentrationBloodgroupwithdirectantibodytest(Coombstest)Hemoglobinandhematocritdeterminations第十页，共三十四页。Optional(可选择(xunz)的)Complete

16、bloodcountincludingmanualdifferentialwhitecellcountBloodsmearforredcellmorphologyReticulocytecountGlucose-6-phosphatedehydrogenasescreenSerumelectrolytesandalbuminorproteinconcentrations第十一页，共三十四页。TimedTSBmeasurements定时胆红素水平监测，适时干预UmbilicalcordbloodTSB（脐带血胆红素水平并无特异性）（脐带血胆红素水平并无特异性）ATSBconcentrationg

17、reaterthan30mol/LinumbilicalcordbloodisstatisticallycorrelatedwithapeakneonatalTSBconcentrationgreaterthan300mol/L,butthepositivepredictivevalue（阳性(yngxng)预测值）isonly4.8%fortheterminfant,risingto10.9%inthelatepreterminfant,andthespecificityisverypoorUniversalhemoglobinassessment（常规脐带血血红蛋白或红细胞比容（常规脐带血

18、血红蛋白或红细胞比容测定并不能预测严重高胆红素血症的发生）测定并不能预测严重高胆红素血症的发生）Althoughbilirubinisderivedfromthebreakdownofhemoglobin,routineumbilicalcordbloodhemoglobinorhematocritmeasurementdoesnotaidinthepredictionofseverehyperbilirubinemia第十二页，共三十四页。BloodgroupandCoombstesting（血型及（血型及Coombs试试验）验）ABO溶血是常见原因，大部分新生儿黄疸与ABO溶血有关（blo

19、odgroupAorBinfantsborntoamotherwithgroupOblood）ABO溶血患儿直抗阳性者比阴性者更需光疗TheneedforphototherapyisincreasedinABO-incompatibleinfantswhoaredirectantiglobulintest(DATdirectCoombstest)-positivecomparedwiththosewhoareDAT-negative对型血母亲(mqn)及有高危因素的黄疸患儿进行DAT检测TestingallbabieswhosemothersaregroupOdoesnotimproveout

20、comescomparedwithtestingonlythosewithclinicaljaundice.Therefore,itisreasonabletoperformaDATinclinicallyjaundicedinfantsofmotherswhoaregroupOandininfantswithanelevatedriskofneedingtherapyTheresultswilldeterminewhethertheyarelowriskorhighrisk,andmaythereforeaffectthethresholdatwhichtherapywouldbeindic

21、ated第十三页，共三十四页。时间胆红素水平时间胆红素水平(shupng)曲线曲线第十四页，共三十四页。加强光疗(unlio)的指征曲线第十五页，共三十四页。Glucose-6-phosphatedehydrogenasedeficiency（葡萄糖（葡萄糖-6-磷酸脱氢酶缺乏症磷酸脱氢酶缺乏症）与严重胆红素血症相关Newbornswithglucose-6-phosphatedehydrogenase(G6PD)deficiencyhaveanincreasedincidenceofseverehyperbilirubinemiaG6PDdeficiencyincreasesthelikel

22、ihoodofrequiringexchangetransfusionininfantswithseverehyperbilirubinemia;therefore,atestforG6PDdeficiencyshouldbeconsideredinallinfantswithseverehyperbilirubinemia有家族种族高危因素的都应行此检查TestingforG6PDdeficiencyinbabieswhoseethnicgrouporfamilyhistorysuggestanincreasedriskofG6PDdeficiencyisadvised.有高危因素男女孩都因

23、检测AlthoughG6PDdeficiencyisanX-linkeddisease,femaleheterozygotescanhavemorethan50%oftheirredcellsdeficientintheenzymebecauseofrandominactivationoftheXchromosome.Femaleswithgreaterproportionsoftheirredcellsaffectedhaveanincreasedriskofsevereneonatalhyperbilirubinemia；therefore,testingofbothgirlsandboy

24、swhoareatriskisadvised有溶血病时，G-6-PD水平会被检测过高从而影响诊断Itshouldalsoberecognizedthatinthepresenceofhemolysis,G6PDlevelscanbeoverestimatedandthismayobscurethediagnosisFemalesinparticularcanhavemisleadingresultsonthecommonscreeningtests积极进行(jnxng)干预G6PD-deficientnewbornsmayrequireinterventionatalowerTSBconcen

25、trationbecausetheyaremorelikelytoprogresstoseverehyperbilirubinemia.Unfortunately,inmanycentres,itcurrentlytakesseveraldaysforaG6PDdeficiencyscreeningtestresulttobecomeavailable.Improvingtheturnaroundtimeforthistestwouldimprovecareofthenewborn.BecauseG6PDdeficiencyisadiseasewithlifelongimplications,

26、testinginfantsatriskisstillofvalue.第十六页，共三十四页。建议建议（每项均有建议的等级与研究的可靠性相关）：孕妇ABO,Rh血型检测及红细胞抗体筛查AllmothersshouldbetestedforABOandRh(D)bloodtypesandbescreenedforredcellantibodiesduringpregnancy脐血检测Ifthemotherwasnottested,cordbloodfromtheinfantshouldbesentforevaluationofthebloodgroupandaDAT(Coombstest)母型血的

27、早期黄疸患儿应做血型鉴定(jindng)及DAT检测BloodgroupevaluationandaDATshouldbeperformedininfantswithearlyjaundiceofmothersofbloodgroupO.G-6-PD筛查Selectedat-riskinfants(Mediterranean,MiddleEastern,AfricanorSoutheastAsianorigin)shouldbescreenedforG6PDdeficiency.严重的高胆红素血症时应做G-6-PD检测AtestforG6PDdeficiencyshouldbeconsider

28、edinallinfantswithseverehyperbilirubinemia第十七页，共三十四页。When?第十八页，共三十四页。怎样减少怎样减少(jinsho)严重高胆红素血症的发生？严重高胆红素血症的发生？HOW CAN THE RISK OF SEVERE HYPERBILIRUBINEMIA BE REDUCED?第十九页，共三十四页。Primarypreventionofseverehyperbilirubinemia一级预防一级预防Breastfeedingsupport对于母亲的宣教OtherineffectiveinterventionsRoutineuseofglyc

29、erinesuppositories（甘油(nyu)栓剂）routineglycerineenemas开赛露，L-asparticacid（天冬氨酸）,whey/caseinandclofibrate（氯贝丁脂）haveallbeenstudiedinsmallrandomizedcontrolledtrials(RCTs),buttheirusehasbeenfoundtohavenoeffectonclinicallyimportantoutcomes.第二十页，共三十四页。Preventionofseverehyperbilirubinemiaininfantswithhemolysis

30、Phenobarbitone（苯巴比妥）Tin-mesoporphyrinSnMP（锡中卟啉）抑制胆红素合成及活性，临床使用未见明显改变Prophylacticphototherapy（预防光疗(unlio)）是否加强光疗见表第二十一页，共三十四页。PreventionofseverehyperbilirubinemiaininfantswithmildormoderatehyperbilirubinemiaPhototherapyInterruptingbreastfeeding（停止母乳喂养）RCT未见明显区别Intravenousimmunoglobulin（静丙）与免疫因素相关的溶血S

31、upplementalfluids（补液）Agar（琼脂）可减少肠壁对未结合胆红素的吸收，没有(miyu)可靠的证据支持第二十二页，共三十四页。建议(jiny)：支持母乳喂养支持母乳喂养Aprogramforbreastfeedingsupportshouldbeinstitutedineveryfacilitywherebabiesaredelivered并不推荐给母乳喂养的婴儿常规补充水或糖水并不推荐给母乳喂养的婴儿常规补充水或糖水Routinesupplementationofbreastfedinfantswithwaterordextrosewaterisnotrecommended

32、静丙的使用静丙的使用InfantswithapositiveDATwhohavepredictedseverediseasebasedonantenatalinvestigationoranelevatedriskofprogressingtoexchangetransfusionbasedonthepostnatalprogressionofTSBconcentrationshouldreceiveIVIGatadoseof1g/kg胆红素水平及高危因素提示可能有发生严重高胆红素血症趋势的时候，即使出院也应追踪监测胆红素水平及高危因素提示可能有发生严重高胆红素血症趋势的时候，即使出院也应追踪

33、监测胆红素水胆红素水ATSBconcentrationconsistentwithincreasedriskshouldleadtoenhancedsurveillancefordevelopmentofseverehyperbilirubinemia,withfollow-upwithin24hto48h,eitherinhospitalorinthecommunity,andrepeatestimationofTSBorTcBconcentrationinmostcircumstances加强光疗加强光疗(unlio)Intensivephototherapyshouldbegivenac

34、cordingtotheguidelinesshowninFigure2常规光疗常规光疗ConventionalphototherapyisanoptionatTSBconcentrations35mol/Lto50mol/LlowerthantheguidelinesinFigure2光疗中也应继续母乳喂养光疗中也应继续母乳喂养Breastfeedingshouldbecontinuedduringphototherapy迅速进展可能需要换血时，应控制补液量迅速进展可能需要换血时，应控制补液量Supplementalfluidsshouldbeadministered,orallyorbyi

35、ntravenousinfusion,ininfantsreceivingphototherapywhoareatanelevatedriskofprogressingtoexchangetransfusion.第二十三页，共三十四页。HOWSHOULDSEVEREHYPERBILIRUBINEMIABETREATED?Phototherapy初始(chsh)治疗，监测胆红素水平，有指征时开始做换血准备补液，静丙Aninfantwhopresentswithseverehyperbilirubinemia,orwhoprogressestoseverehyperbilirubinemiades

36、piteinitialtreatment,shouldreceiveimmediateintensivephototherapy.Thebilirubinconcentrationshouldbecheckedwithin2hto6hofinitiationoftreatmenttoconfirmresponse.Considerationoffurthertherapyshouldcommenceandpreparationsforexchangetransfusionmaybeindicated.Supplementalfluidsareindicated,andIVIGshouldbeg

37、ivenifnotalreadycommencedfortheinfantwithisoimmunization.第二十四页，共三十四页。Phototherapy脱水脱水(tushu)高胆红素血症光疗高胆红素血症光疗Itisimportanttorecognizetherelationshipbetweendehydrationandhyperbilirubinemia.Dehydrationmaybeassociatedwithincreasedserumbilirubinconcentrationsandmaybeexacerbatedbyphototherapy.Alljaundiced

38、infantsshouldbeadequatelyhydratedbeforeandduringphototherapy.Breastfeedingisnotcontraindicatedinthepresenceofhyperbilirubinemiaandshouldbecontinued.Morefrequentbreastfeedingsmaybebeneficial.第二十五页，共三十四页。Exchangetransfusion光疗失败Ifphototherapyfailstocontroltherisingbilirubinconcentrations,exchangetransf

39、usionisindicatedtolowerTSBconcentrations.没有高危因素的健康足月儿Forhealthytermnewbornswithoutriskfactors,exchangetransfusionshouldbeconsideredwhentheTSBconcentrationisbetween375mol/L(21.9mg/dL)and425mol/L(24.8mg/dL)(despiteadequateintensivephototherapy).在换血前采血完善相关检查Becausebloodcollectedafteranexchangetransfusi

40、onisofnovalueforinvestigatingmanyoftherarercausesofseverehyperbilirubinemia,theseinvestigationsshouldbeconsideredbeforeperformingtheexchangetransfusion.Appropriateamountsofbloodshouldbetakenandstoredfortestssuchasthoseforredcellfragility,enzymedeficiency(G6PDorpyruvatekinase丙酮酸激酶deficiency)andmetabo

41、licdisorders,aswellasforhemoglobinelectrophoresisandchromosomeanalysis.如果胆红素水平刚达到换血指征，在换血前应再次检测(jinc)胆红素水平。严格掌握换血指征。Preparationofbloodforexchangetransfusionmaytakeseveralhours,duringwhichtimeintensivephototherapy,supplementalfluidsandIVIG(incaseofisoimmunization)shouldbeused.IfaninfantwhoseTSBconcen

42、trationisalreadyabovetheexchangetransfusionlinepresentsformedicalcare,thenrepeatmeasurementoftheTSBconcentrationjustbeforeperformanceoftheexchangeisreasonable,aslongastherapyisnottherebydelayed.Inthisway,someexchangetransfusions,withtheirattendantrisks,maybeavoided.Exchangetransfusionisaprocedurewit

43、hsubstantialmorbiditythatshouldonlybeperformedincentreswiththeappropriateexpertiseundersupervisionofanexperiencedneonatologist.当有急性胆红素脑病的临床表现时应马上换血Aninfantwithclinicalsignsofacutebilirubinencephalopathyshouldhaveanimmediateexchangetransfusion.第二十六页，共三十四页。建议(jiny)：InfantswithaTSBconcentrationabovethe

44、thresholdsshownonFigure3shouldhaveimmediateintensivephototherapy,andshouldbereferredforfurtherinvestigationandpreparationforexchangetransfusion.Aninfantwithclinicalsignsofacutebilirubinencephalopathyshouldhaveanimmediateexchangetransfusion.第二十七页，共三十四页。换血(hunxu)指征第二十八页，共三十四页。Follow-up随访随访(sufn)：Routi

45、nenewbornsurveillance,whetherinhospitalorafterdischarge,shouldincludeassessmentofbreastfeedingandjaundiceevery24hto48huntilfeedingisestablished(usuallyonthethirdorfourthdayoflife).Alljaundicedinfants,especiallyhigh-riskinfantsandthosewhoareexclusivelybreastfed,shouldcontinuetobecloselymonitoreduntil

46、feedingandweightgainareestablishedandtheTSBconcentrationstartstofall.CommunityservicesshouldincludebreastfeedingsupportandaccesstoTSBorTcBtesting.Infantswithisoimmunizationareatriskforsevereanemiaafterseveralweeks;itissuggestedthatarepeathemoglobinmeasurementbeperformedattwoweeksifitwaslowatdischarg

47、eandatfourweeksifitwasnormal.Infantsrequiringexchangetransfusionorthosewhoexhibitneurologicalabnormalitiesshouldbereferredtoregionalmultidisciplinaryfollow-upprograms.Neurosensoryhearinglossisofparticularimportanceininfantswithseverehyperbilirubinemia,andtheirhearingscreenshouldincludebrainstemaudit

48、oryevokedpotentials.第二十九页，共三十四页。FurtherinvestigationsTheoccurrenceofseverehyperbilirubinemiamandatesaninvestigationofthecauseofhyperbilirubinemia.Investigationsshouldincludeaclinicallypertinenthistoryofthebabyandthemother,familyhistory,descriptionofthelabouranddelivery,andtheinfantclinicalcourse.Aph

49、ysicalexaminationshouldbesupplementedbylaboratoryinvestigations(conjugatedandunconjugatedbilirubinlevels;directCoombstest;hemoglobinandhematocritlevels;andcompletebloodcellcount,includingdifferentialcount,bloodsmearandredcellmorphology).Investigationsforsepsisshouldbeperformedifwarrantedbytheclinica

50、lsituation.第三十页，共三十四页。建议(jiny)：每个黄疸的患儿均应随访Adequatefollow-upshouldbeensuredforallinfantswhoarejaundiced.加强光疗的患儿应完善(wnshn)相关检查，以了解黄疸病因Infantsrequiringintensivephototherapyshouldbeinvestigatedfordeterminationofthecauseofjaundice.第三十一页，共三十四页。CONCLUSION严重的高胆红素血症的有发生急性胆红素脑病及慢性后遗症的可能严重的高胆红素血症的有发生急性胆红素脑病及慢性