OfficialreprintfromUpToDate

www.uptodate.com2015UpToDate

Approachtothesepticappearinginfant
Authors
RichardJScarfone,MD,FAAP
ChristineCho,MD,MPH,MEd

SectionEditors
GeorgeAWoodward,MD
JanEDrutz,MD

DeputyEditor
JamesFWiley,II,MD,MPH

Alltopicsareupdatedasnewevidencebecomesavailableandourpeerreviewprocessiscomplete.
Literaturereviewcurrentthrough:Aug2015.|Thistopiclastupdated:Feb26,2014.
INTRODUCTIONTheevaluationofcriticallyillneonatesandyounginfantsischallengingbecausetheclinicalmanifestationsofillness(ie,lethargy,
poortone,poorfeeding,orirritability)areoftenatypicalornonspecific.Althoughmanyofthesesepticappearingpatientshaveoverwhelminginfections,
somemayhavecongenital(eg,congenitaladrenalhyperplasia)oracquired(eg,inflictedheadinjury)conditionsthatrequirepromptrecognitionand
specificmanagement.
Thecausesofoverwhelmingillnessamongyounginfantswhoaresepticappearingarereviewedhere.Theevaluationandinitialmanagementdecisions
arealsodiscussed.Analgorithmicapproachtoestablishingthediagnosisissuggested(algorithm1).Specificdiagnosesarereviewedseparately.The
evaluationoffeverininfantslessthanthreemonthsofageisdiscussedelsewhere.(See"Evaluationandmanagementoffeverintheneonateandyoung
infant(youngerthanthreemonthsofage)".)
CAUSESAlthoughinfectionisthemostlikelycauseofoverwhelmingillnessamongneonatesandyounginfants,anumberofotherclinicalconditions
havesimilarmanifestations(table1).
Infectiouscauses
Bacterialinfections
BacterialsepsisNeonatescandevelopsepsiswithorwithoutlocalizedinfectionssuchasurinarytractinfections,pneumonia,orcellulitis.(See
"Definitionandetiologyoffeverinneonatesandinfants(lessthanthreemonthsofage)",sectionon'Seriousbacterialinfection'.)
Possiblepathogensincludethefollowing:
Intheimmediatenewbornperiod,groupBstreptococcusandEscherichiacoliarethetwomostcommonpathogensassociatedwithsepsis
Listeriamonocytogenesisalesscommoncause.
Beyondthefirstweeksoflife,lateonsetdiseasewithanyofthesepathogensmayoccur,aswellasinfectionswithStreptococcus
pneumoniae,Neisseriameningitidis,and,toamuchlesserextent,Haemophilusinfluenzaetypeb.
InfectionwithcommunityacquiredmethicillinresistantStaphylococcusaureus(MRSA)mustbeconsideredforinfantswithskininfectionsor
withknownexposures.

(See"MethicillinresistantStaphylococcusaureusinfectionsinchildren:Epidemiologyandclinicalspectrum",sectionon'CAMRSAinfection'
and"MethicillinresistantStaphylococcusaureusinchildren:Treatmentofinvasiveinfections",sectionon'Treatmentapproach'.)
Inyounginfants,theoriginsofosteomyelitisandsepticarthritisaretypicallyhematogenous.(See"Hematogenousosteomyelitisinchildren:
Epidemiology,pathogenesis,andmicrobiology",sectionon'Pathogenesis'and"Bacterialarthritis:Epidemiology,pathogenesis,and
microbiologyininfantsandchildren".)
BacterialmeningitisBacterialmeningitisamongneonatesandinfantsiscausedbythesameorganismsthatcausesepsis.Theincidenceof
bacterialmeningitisinthisagegrouphasbeendecliningastheresultofuniversalscreeningandintrapartumantibioticprophylaxisforgroupB
StreptococcaldiseaseandtheintroductionofconjugatevaccinesagainstHaemophilusinfluenzatypebandpneumococcus.(See"Bacterial
meningitisintheneonate:Clinicalfeaturesanddiagnosis"and"Bacterialmeningitisinchildrenolderthanonemonth:Clinicalfeaturesand
diagnosis",sectionon'Epidemiology'.)
PyelonephritisUrinarytractinfectionsarethemostcommonseriousbacterialinfectionsinneonatesandyounginfants.Fewerthan10percentof
thesechildrenwillhavecoexistingbacteremiaorurosepsis.Escherichiacolicausesmorethan80percentoftheseinfections.Clinically,itisnot
possibletodistinguishlowerfromupperurinarytractinfectioninthisagegroup.Aconservativeandappropriateapproachistoassume
pyelonephritisexistsamongfebrileyoungchildrenwithpyuria.(See"Urinarytractinfectionsininfantsandchildrenolderthanonemonth:Clinical
featuresanddiagnosis",sectionon'Youngerchildren'.)
PertussisPertussisisaubiquitousandhighlycontagiousinfectionwithsignificantmorbidityandmortalityforyounginfants.Pertussisshouldbe
consideredamonginfantswithrespiratoryfailure,apneaand/orbradycardia,oranapparentlifethreateningevent(ALTE).Symptomsmaybenon
specific,includingfeedingdifficulties,tachypnea,andcough.Gagging,apnea,cyanosis,andbradycardiaoftendevelopduringparoxysmsofcough.
(See"Bordetellapertussisinfectionininfantsandchildren:Clinicalfeaturesanddiagnosis",sectionon'Infants'.)
InfantbotulismInfantsdevelopbotulismfromtheingestionofClostridiumbotulinumspores(airborneorfromfood),ratherthanpreformed
botulinumtoxin.Thetoxin,whichimpairsimpulsesattheneuromuscularjunctionbyblockingacetylcholinerelease,isthenproducedbyorganisms
thatcolonizetheinfant'sgastrointestinaltract.Symptomsinitiallyincludehypotonia,constipationandpoorfeedingandprogresstorespiratory
failure.Mostinfantsrequireintensivecareandmanyneedmechanicalventilation.Themedianageofpresentationisfourmonths.Thediseaseis
morecommonamongbreastfedinfants.(See"Neuromuscularjunctiondisordersinnewbornsandinfants",sectionon'Infantbotulism'.)
Viralinfections
OverwhelmingviralinfectionLifethreateningviralinfectionsamongneonatesaremostoftencausedbyHerpessimplexvirus(HSV)or
enterovirus.
HSVcancauselifethreateningdisseminatedorcentralnervoussysteminfectioninthenewborn.Asmanyasonethirdoftheseneonatesdo
nothaveskinvesiclesatpresentation,andmanyareafebrile,makingthediagnosismorechallenging[1].Initialsymptomscanoccuranytime
betweenbirthandfourweeks.ThepeakincidenceofCNSdiseaseisfrom10to17daysoflife.Thosewithdisseminatedinfectionmayhave
earlierclinicalmanifestations.Thediagnosisshouldbesuspectedandconsiderationshouldbegiventopresumptiveuseofacycloviramong
infantslessthanfourweeksoldwhohaveanyofthefollowingriskfactors:maternalHSV,vesicularrash,seizures,CSFpleocytosis,or
elevatedliverenzymes.(See"Neonatalherpessimplexvirusinfection:Clinicalfeaturesanddiagnosis",sectionon'Clinicalmanifestations'.)

 Enteroviralserotypes,suchasgroupBcoxsackievirusserotypes2to5andechovirus11,mayproducefulminantmyocarditisorhepatitis
amongneonates.Theinfectionismostoftenacquiredfromasymptomaticmotherintheperinatalperiod.Symptomstypicallydevelop
betweenthreeandsevendaysoflife.However,approximatelyonethirdofcaseshaveabiphasicillnesswithaperiodofonetosevendaysof
apparentwellbeinginterspersedbetweentheinitialsymptomsandtheappearanceofmoreseriousmanifestations.(See"Clinical
manifestationsanddiagnosisofenterovirusandparechovirusinfections",sectionon'Infectionsinneonates'.)
BronchiolitiswithapneaYounginfants,particularlythosewhoarelessthanonemonthofageorwhowerebornprematurely,maydevelop
apneawithbronchiolitis[2].Somemaypresentwithsevereapneabeforetheydeveloptypicalsignsofbronchiolitis,suchasrespiratorydistressor
wheezing.(See"Bronchiolitisininfantsandchildren:Clinicalfeaturesanddiagnosis",sectionon'Apnea'.)
InfluenzaTheinfluenzavirusishighlycontagiousresultinginseasonalepidemics.Influenzalikeillnessismarkedbyfeverandsignsoflower
respiratorytractdiseasesuchascoughing.Infantsmayalsopresentwithvomiting,poorfeeding,ormalaiseandalongwiththeelderly,theysuffer
thegreatestmorbidityandmortality.Thosewithsignificantcomorbiditiessuchasprematurityorpulmonaryorcardiacdiseasesareatgreatestrisk
foradverseoutcomes.(See"Seasonalinfluenzainchildren:Clinicalfeaturesanddiagnosis",sectionon'Clinicalfeatures'.)
MyocarditisEvidenceofviralmyocarditishasbeendescribedinassociationwithapparentlifethreateningevents(ALTE)andsuddeninfant
death.Myocarditisinchildrenisusuallycausedbyenteroviruses(coxsackieBgroup)oradenovirus.Infantsmaypresentwithafulminantillness
characterizedbysignsofdecreasedcardiacoutput,includinghypotension,poorpulses,anddecreasedperfusion.Malignantarrhythmiasare
common.(See"Clinicalmanifestationsanddiagnosisofmyocarditisinchildren".)
Congenitalconditions
Congenitalheartdisease(CHD)InfantswithpreviouslyundiagnosedCHDwhoareseriouslyillusuallyfallintooneofthreecategories:cyanotic
lesions,obstructivelesions,or(rarely)acoronaryarteryabnormality.Infantswithcyanoticorobstructiveheartdisease,whoaredependentonblood
flowthroughtheductusarteriosus(DA)forpulmonaryorsystemiccirculation,developseveresymptomsastheDAclosesoverseveraldaysto
severalweeksoflife[3].Dependingonthespecificcardiaclesionandthedelayinseekingcare,infantsmaypresentwithsomecombinationof
respiratorydistress,cyanosis,shock,orcongestiveheartfailure.(See"Diagnosisandinitialmanagementofcyanoticheartdiseaseinthenewborn"
and"Clinicalmanifestationsanddiagnosisofcoarctationoftheaorta",sectionon'Neonates'and"Congenitalandpediatriccoronaryartery
abnormalities",sectionon'Variationsofcoronaryarteryoriginfromthepulmonaryartery'.)
Commoncausesofcyanoticheartdiseaseincludetranspositionofthegreatvessels,tetralogyofFallot,truncusarteriosus,tricuspidatresia,
andtotalanomalouspulmonaryvenousreturn.
Obstructiveheartlesionsincludehypoplasticleftheart,coarctationoftheaorta,andotheraorticarchanomalies.
Forinfantswithananomalousoriginofoneormorecoronaryarteriesfromthepulmonaryartery,myocardialischemiamaydevelopas
pulmonaryvascularresistancenormalizespostnatally.
Congenitaladrenalhyperplasia(CAH)CAHisagroupofinheriteddisordersofimpairedcortisolsynthesis.Morethan95percentofcasesare
dueto21hydroxylasedeficiency,whichclassicallymanifestsininfancyasvirilizationandadrenalinsufficiency.Maleinfantsareusuallymore
difficulttorecognizeandmaypresentwithadrenalcrisis.Adrenalcrisistypicallydevelopswithinthefirstfewdaystoweeksoflife.Clinical
manifestationsincludevomiting,diarrhea,hypovolemia,hyponatremia,hyperkalemia,hypoglycemia,andhypotension.(See"Causesandclinical

manifestationsofprimaryadrenalinsufficiencyinchildren",sectionon'Adrenalcrisis'.)
Inbornerrorsofmetabolism(IEM)Althoughindividualdefectsareuncommoninthegeneralpopulation,inbornerrorsofmetabolismaccountfor
asignificantportionofdiseaseamonginfants.
SeveralcategoriesofIEM(aminoaciddisorders,organicacidemias,ureacycledisorders,disordersofcarbohydratemetabolism,fattyacid
oxidationdefects,andmitochondrialdisorders)maypresentwithanacutemetaboliccrisisthatistriggeredbycircumstancessuchasintake
ofproteinorcertaincarbohydratesorinfection.Thedeteriorationtypicallyoccursafteraperiodofapparentwellbeing.Asanexample,
newbornswithureacycledisordersororganicacidemiasgenerallypresentwithanacute,severeillnesscharacterizedbylethargy,poor
feeding,vomiting,andshock,withhyperammonemiaandprofoundacidosis(algorithm2).
Infantswithgalactosemiamaypresentwithsepsis,usuallyfromaurinarytractinfectionwithEscherichiacoli.(See"Galactosemia:Clinical
featuresanddiagnosis",sectionon'Classicgalactosemia'.)
AlthoughIEMmaybeincludedinnewbornscreeningtests,infantscanpresentbeforetheresultsareavailable.(See"Inbornerrorsof
metabolism:Metabolicemergencies"and"Newbornscreening".)
Surgicalconditions
MalrotationwithvolvulusMalrotationdevelopsasaresultofanarrestofnormalrotationoftheembryonicgut.Abnormalmobilityofthesmall
bowel,astheresultofanarrowmesentericbase,allowsthemesenterytotwist.Volvulusoccurswhensmallboweltwistsaroundthesuperior
mesentericartery,causingvascularcompromisetolargeportionsofthemidgut(figure1).Thisleadstoischemiaandnecrosisofthebowelthatcan
quicklybecomeirreversible.Vomiting,whichisalmostalwaysbilious,occursin>90percentofnewbornswithvolvulusandisbyfarthemost
commonpresentingsymptomofmalrotationininfancy.Inonecaseseries,90percentofpatientswerelessthaneightweeksofageatdiagnosis
[4].(See"Intestinalmalrotation".)
IncarceratedherniaAninguinalherniadevelopswhenintraabdominalcontentsentertheinguinalcanalthroughapatentprocessusvaginalis.An
incarcerationresultswhentheherniacannotbereducedbackintotheintraabdominalcavity.Incarcerationcanrapidlyprogresstostrangulation,in
whichherniacontentsbecomeischemic.Inguinalherniaissixtimesmorecommoninboysandhasagreaterincidenceamongprematureinfants.
Incarcerationdevelopsmostcommonlyduringthefirstyearoflife.(See"Overviewofinguinalherniainchildren",sectionon'Incarceratedmass'.)
PyloricstenosisHypertrophyofboththecircularandlongitudinalmuscularlayersofthepylorusresultsinobstruction.Thisisacommon
conditionestimatedtooccurinabout1of300livebirths.Patientstypicallycometomedicalattentionatagethreetosixweekswithacomplaintof
progressivelyworseningprojectile,nonbiliousemesis.Eightypercentofpatientswithpyloricstenosisaremale[5].Aheightenedclinical
awarenessandtheliberaluseofultrasoundtoestablishthediagnosishasledtolessdelayindiagnosisandbetteroutcomes.(See"Infantile
hypertrophicpyloricstenosis".)
AppendicitisNeonatalappendicitishasbeenreportedinfrequently.Theappendixistypicallyperforatedatthetimeofdiagnosisamonginfants.
Symptomsarenonspecificandincludelethargy,irritability,andvomiting.Infantsoftenhavesignsofsepsis,suchashypotension.(See"Acute
appendicitisinchildren:Clinicalmanifestationsanddiagnosis",sectionon'Clinicalmanifestations'.)
Othercauses

 InflictedinjuryYounginfantswithsevereinflictedinjury(typically,headinjury)oftenpresentwithalteredmentalstatus,seizures,and/or
respiratorydistress.Thereisusuallynoclearhistoryoftrauma.Sincesignsofexternalinjury,suchasburnsorcontusionsareoftenminimalor
absent,onemustmaintainahighlevelofsuspicion.(See"Childabuse:Evaluationanddiagnosisofabusiveheadtraumaininfantsandchildren".)
Acquiredmetabolicconditions:
HyponatremiaHyponatremiausuallyoccursastheresultofwaterintoxication(intakeofexcessiveamountsoffreewater),syndromeof
inappropriateantidiuretichormonesecretion,orfromexcessiverenallosses(suchaswithcongenitaladrenalhyperplasia).Occasionally,
infantswithcysticfibrosismaypresentwithhyponatremicdehydration[6].Younginfantswithhyponatremiamaydeveloplethargyorseizures,
andtheseizuresmayberefractorytoanticonvulsantsuntiltheunderlyingmetabolicderangementiscorrected.(See"Fluidandelectrolyte
therapyinnewborns",sectionon'Hyponatremia'.)
HypernatremiaCausesofhypernatremia(150mEq/Lormore)includesodiumpoisoning,excessivelossoffreewater(ascanoccurwith
diabetesinsipidus),orlossofwaterinexcessofsodiumlosses.Severehypernatremicdehydrationhasbeenreportedinassociationwith
breastfeedingdifficulties[7,8].Lethargy,irritability,seizures,and/orcomamayoccurwithhypernatremia.(See"Fluidandelectrolytetherapy
innewborns",sectionon'Hypernatremia'.)
HypoglycemiaSeveralfactorsplaceinfantsatincreasedriskforhypoglycemia(plasmaglucosevalueof40mg/dL[2.22mmol/L]).These
includelowmusclemass,diminishedglycogenstoragecapacity,immaturityofgluconeogenesisandketogenesis,increasedglucosedemand,
anddecreasedoralintakeduringtimesofstress.Hypoglycemiacanbecausedbyvariousmetabolic,endocrinologic,toxic,andinfectious
etiologies.Timelyrecognitionandtreatmentiscrucialsinceprolongedand/orseverehypoglycemiacanprecipitateseizuresand/orpermanent
braindamage[9].(See"Pathogenesis,screening,anddiagnosisofneonatalhypoglycemia".)
SeizuresSeizuresoccurmorecommonlyininfancythanatothertimesduringchildhood,yettheyremaindifficulttorecognizebecause
generalizedtonicclonicactivitytypicallydoesnottooccur.Hypoxicischemicinjuryisthemostcommoncauseofneonatalseizures[10].Other
causesincludeinfections,metabolicdisturbances,trauma,structuralbraindisease,ordrugwithdrawal(table2).(See"Etiologyandprognosisof
neonatalseizures".)
ArrhythmiasArrhythmias,ofwhichsupraventriculartachycardiaisthemostcommon,maygounrecognized.Initialsignsarenonspecificandthe
infanttypicallytoleratestherapidheartrate.Eventually,congestiveheartfailuredevelops.(See"Supraventriculartachycardiainchildren:AV
reentranttachycardia(includingWPW)andAVnodalreentranttachycardia",sectionon'Heartfailure'.)
ToxicexposuresInfantsmaybecomeillfromexposuretoenvironmentaltoxins,therapeuticorintentionaloverdoseofmedications,or(rarely)
fromsubstancesinbreastmilk.Conditionsthatresultfromenvironmentalexposuresincludethefollowing:
MethemoglobinemiaMethemoglobinemiahasbeendescribedinyounginfantsinassociationwithseverediarrhealillnessandfollowing
exposuretooxidants(suchaswaterorfoodshighinnitritesandsometopicalanesthetics)[11,12].Infantsaresusceptibletoacute
methemoglobinemiabecauseoftherelativeimmaturityofthehemoglobinreductaseenzymesystemthatmaintainshemoglobinironina
reducedstate.Patientswithmethemoglobinemiatypicallyarecyanoticorashenanddonotimprovewithsupplementaloxygen.Oxygen
saturation,asmeasuredwithpulseoximetry,isnormalornearnormal.Inaddition,bloodsamplesaredarkred,chocolate,orbrownishtoblue
incoloranddonotchangewiththeadditionofoxygen(figure2).(See"Clinicalfeatures,diagnosis,andtreatmentofmethemoglobinemia".)

 CarbonmonoxidepoisoningInfantsmaydevelopcarbonmonoxidepoisoningastheresultofoccultexposurefromsourcessuchas
improperlyventedhomeheatingsystemsorautomobileexhaustfumes[13,14].Thediagnosismaybedifficulttomakewithoutahistoryof
exposureorsymptomaticcontacts.Presentingsymptomsincludelethargyandirritability.(See"Carbonmonoxidepoisoning".)
Necrotizingenterocolitis(NEC)NECischaracterizedbybowelwallnecrosisthatmayleadtoperforation(image1).Itismostcommonin
prematureneonates,especiallythoseofverylowbirthweight.Itmayoccurinfullterminfants,usuallywithinthefirst10daysoflife.Terminfants
whodevelopNECtypicallyhaveanunderlyingcondition,suchascongenitalheartdiseaseorprotracteddiarrhea.Systemicsignsarenonspecific
andincludeapnea,respiratoryfailure,lethargy,poorfeeding,temperatureinstability,orhypotensionresultingfromsepticshockinthemostsevere
cases.(See"Clinicalfeaturesanddiagnosisofnecrotizingenterocolitisinnewborns".)
Acutebilirubinencephalopathy(ABE)Unconjugatedbilirubinisaneurotoxin,which,atveryhighlevels,cancauseencephalopathywith
permanentneurologicsequelae(kernicterus).Terminfantsmaydevelopbilirubinneurotoxicitywhentotalserumbilirubinconcentrationsexceed25
mg/dL(513mol/L).InfantswhoareatincreasedriskforABEincludethosewhoare<37weeksgestation,breastfed,havehemolyticdisease,
and/oraredischargedhomebefore48hours.(See"Clinicalmanifestationsofunconjugatedhyperbilirubinemiaintermandlatepreterminfants",
sectionon'Neurologicmanifestations'.)
Apparentlifethreateningevent(ALTE)ALTEisnotaspecificdiagnosisrather,itdescribesa"chiefcomplaint"thatbringsaninfanttomedical
attention.Itmaybedefinedasanepisodethatisfrighteningtotheobserverandischaracterizedbysomecombinationofapnea,colorchange,
changeinmuscletone,choking,orgagging.IllappearinginfantswithahistoryofALTEhaveamyriadofpotentialdiagnoses(table3).(See
"Apparentlifethreateningeventininfants".)
Kawasakidisease(KD)Kawasakidiseaseisrareamongyounginfants.However,inthisagegroup,thepresentationisfrequentlyincompleteor
atypical.Asaresult,patientsareatincreasedriskforcoronaryartery(CA)aneurysms,primarilybecauseofdelayintreatment.Thediagnosis
shouldbeconsideredinanychildunderagesixmonthswithsevenormoreconsecutivedaysofunexplainedfever.(See"Kawasakidisease:
Clinicalfeaturesanddiagnosis".)
EVALUATIONANDDECISION
HistorySymptomsreportedbycaretakersofsepticappearinginfantsaretypicallynonspecific:
Amongyounginfants,constitutionalsymptomssuchasincreasedsleepiness,irritability,orpoorfeedingarenonspecificcomplaintsthatoccurwith
avarietyofseriousconditions.
Absenceoffeverdoesnotexcludeinfection.Younginfantswithnormalorlowcoretemperaturesmayhaveseriousinfections.(See"Bacterial
meningitisintheneonate:Clinicalfeaturesanddiagnosis",sectionon'Temperatureinstability'.)
Coughcanbeassociatedwithpneumonia,bronchiolitis,orcongenitalheartdisease(CHD).
Infantswithinflictedheadinjuryoftenhavenonspecificsymptoms(suchasseizures,breathingdifficulty,apnea,orlifelessness).Thereistypically
noclearhistoryoraninconsistenthistoryoftrauma.(See"Childabuse:Evaluationanddiagnosisofabusiveheadtraumaininfantsandchildren",
sectionon'History'.)
Incontrast,thefollowingcomplaintsareoftenassociatedwithspecificconditions(table4):

 Biliousemesisisaserioussignofbowelobstruction.Inoneretrospectiveseries,97percentofinfantswithmalrotationandvolvulushadahistory
ofbiliousemesis[4].(See"Intestinalmalrotation".)
Neonateswhohavebeenwell,butdeveloplethargy,poorfeeding,vomiting,andshockmayhaveaninbornerrorofmetabolism(IEM).Theurineof
someinfantswithIEMmayhaveanunusualodor(table5).(See'Congenitalconditions'above.)
Herpessimplexvirusinfectionmustbeconsideredforaneonatewhosemotherhasgenitalvesicularlesions.
Abnormalrhythmicmovements(suchastwitching,blinkingorchewing)mayrepresentseizureactivity.
Infantswhodevelopprojectilevomitingmayhavepyloricstenosis.
Aninfantwhoisnotmovinganextremitymayhaveosteomyelitis,septicarthritis,orafracture.
Importantfeaturesoftheperinatalhistoryincludethefollowing:

Maternalinfections,fever,andGroupBstreptococcaltestingandresults
Modeofdelivery
Prematurity
Birthasphyxia
Needforneonatalintensivecare
Lengthofstayinthenewbornnursery

Finally,informationregardingfever,vomiting,typeandfrequencyoffeeding,stoolingpatterns,andillcontactsmayprovideusefulcluestotheetiologyof
theinfant'ssymptoms.
PhysicalexaminationInfantswhohaverespiratoryorcirculatorycompromisemustbequicklyidentifiedandtheirconditionsstabilized.(See"Initial
assessmentandstabilizationofchildrenwithrespiratoryorcirculatorycompromise",sectionon'Evaluation'.)
Bydefinition,infantswithconditionsthatmimicsepsisareillappearing.Thegeneralappearancetypicallyincludesnonspecificfeaturessuchas
irritability,lethargy,poortone,anddecreasedactivity.Acarefulphysicalexaminationmayidentifyacombinationorpatternofclinicalfeaturesthat
suggesttheetiologyofaninfant'ssymptoms(table6).
Featuresofvitalsignstoconsiderincludethefollowing:
Pulsesandbloodpressuremeasurementsshouldbeobtainedinbotharmsandbothlegs.Diminishedpulsesandbloodpressureinthelower
extremitiessuggestleftventricularoutflowobstruction,asoccurswithhypoplasticleftheartsyndrome,criticalaorticstenosis,orcoarctationofthe
aorta.(See"Clinicalmanifestationsanddiagnosisofcoarctationoftheaorta",sectionon'Bloodpressureandpulses'.)
Lackoffeverdoesnotexcludeaninfectiousillness.
Aninfantwithaheartrateover220beatsperminute(bpm)probablyhasatachyarrhythmia,mostcommonlysupraventriculartachycardia.Sinus
tachycardiararelyexceeds220bpm.(See"Supraventriculartachycardiainchildren:AVreentranttachycardia(includingWPW)andAVnodal
reentranttachycardia",sectionon'Clinicalfeatures'.)

Respiratorysymptoms(suchastachypnea,grunting,orretractions)maybenonspecific.However,ralesand/orwheezingsuggestapulmonarydisorder
orheartfailure.
Featuresofthecardiacexaminationmaysuggestacongenitaldefect.(See"Diagnosisandinitialmanagementofcyanoticheartdiseaseinthenewborn",
sectionon'Physicalexamination'and"Clinicalmanifestationsanddiagnosisofcoarctationoftheaorta",sectionon'Clinicalmanifestations'.)Findingsto
noteinclude:
Thepresenceofaheartmurmursuggestscardiacdisease,althoughtheabsenceofamurmurdoesnotexcludeit.
Agalloprhythm,thepresenceofrales,andhepatomegalylikelyindicateheartfailure.
Abdominaldistentionmayindicatebowelobstruction,butitisanonspecificfinding.Anormalabdominalexaminationdoesnotexcludeserious
conditions.Asanexample,inonecaseseriesdescribingchildrenwithmalrotation,60percentofthosewithvolvulushadanormalabdominal
examination[4].
Skinfindingsmayincludesignsofinfection,suchasvesicles,cellulitis,orabscessformation.Jaundicesuggestsacutebilirubinencephalopathy.
Acrocyanosismaybetheresultofpoorperfusion.Infantswithcentralcyanosisthatdoesnotrespondtosupplementaloxygen,however,mayhave
cyanoticheartdiseaseormethemoglobinemia.
Physicalfindingsthataresuggestiveofaspecificetiologyincludethefollowing:
Infantswithcongenitalobstructiveleftheartdiseaseandrespiratorydistressaremorelikelytohavecardiomegalyanddiminishedextremitypulses
thanareinfantswithsepsis[15].(See'Congenitalconditions'above.)
Aninfantwithapnea,bradycardia,afocalneurologicexamination,andretinalhemorrhageshasaninflictedheadinjuryuntilprovenotherwise
(picture1).(See"Childabuse:Evaluationanddiagnosisofabusiveheadtraumaininfantsandchildren",sectionon'Clinicalfeatures'.)
Volvulusistheprobablecauseofbiliousemesisandabdominaldistentionforaninfantwithmalrotation.[4].(See"Intestinalmalrotation".)
Incomparisontomyoclonus,theamplitudeofmovementsofaseizureistypicallynotalteredbygentlerestraint.Tachycardiaandhypertension
occurmorecommonlyduringaseizure.(See"Clinicalfeatures,evaluation,anddiagnosisofneonatalseizures",sectionon'Autonomicsigns'.)
Ancillarystudies
LaboratorystudiesInfantsundertwomonthsofagewhoaresepticappearingmaybeseriouslyill.Thefollowinglaboratorystudiesshould
generallybeperformed:
Culturesofbloodandurineshouldbeobtained.Infantswhoarestableenoughtoundergolumbarpuncture(LP)shouldusuallyhavecerebrospinal
fluid(CSF)sentforcultureaswell.LPmaybedeferredforthosewhoareafebrileandforwhomanalternativediagnosis(suchascongenitalheart
diseaseorvolvulus)isquicklyestablished.Specificcultures(suchasofstooloralocalizedinfection)shouldbeobtainedasindicatedfromthe
historyandphysicalexamination.
Urineshouldbecollectedbyurethralcatheterizationandsentforurinalysis(UA)andculture.AnenhancedUA(microscopicanalysisperformedon
anuncentrifugedspecimenthatincludesaGramstainandcellcountusingahemocytometer)improvestheaccuracyofUAfordetectingurinary

tractinfections.EnhancedUAshouldberequested,whenavailable.(See"Urinarytractinfectionsininfantsandchildrenolderthanonemonth:
Clinicalfeaturesanddiagnosis",sectionon'Rapidlyavailabletests'.)
CSFshouldbesentforcellcount,protein,glucose,gramstain,andculturewheneveraLPisperformed(table7andtable8).(See"Bacterial
meningitisinchildrenolderthanonemonth:Clinicalfeaturesanddiagnosis",sectionon'InterpretationofCSF'.)Additionaltesting(suchasfor
enterovirusorHSV)shouldbesentasindicatedfromthehistoryandphysicalexamination.
Herpessimplexvirus(HSV)culturesofskinvesicles,oropharynx,conjunctiva,urine,blood,stoolorrectum,andCSF,aswellaspolymerasechain
reaction(PCR)testingofCSFforHSVandliverfunctiontests,shouldbeperformedforinfants28daysofagewiththefollowingriskfactors:

Mucocutaneousvesicles
Seizure
CSFpleocytosiswithnegativeGramstain
MotherknowntohaveHSV

Thefollowingchemistrytestsshouldbesentforcriticallyillinfants:
Electrolytes,glucose,BUNandcreatinine.Infantswithhypernatremia,hyponatremia,orhypoglycemiafrequentlyhavenonspecificneurologic
symptoms,includingseizures.
Calcium,magnesium,andphosphatelevelsshouldbedeterminedforaninfantwhomayhavehadaseizure.
Totalanddirectbilirubinlevelsshouldbesentforinfantswhoappearjaundiced.
Bloodlevelsforammonia,lactate,pyruvate,aswellasbloodandurineketones,shouldbesentwhenaninbornerrorofmetabolismissuspected
(table9andtable10)
ImagingImagingstudiesshouldbeobtainedforspecificindications,includingthefollowing:
Achestradiographshouldbeobtainedforinfantswithsignsorsymptomsofpulmonaryorcardiacdisease[16,17].Infantswithcardiomegalyor
abnormalcardiacsilhouettesmayhavecongenitalheartdisease.
Plainfilmsoftheabdomenareindicatedforinfantswithabdominaldistentionorvomiting.Abnormalitiesthatmaybeseenwithnecrotizing
enterocolitis(NEC)includepneumatosisintestinalis(gaswithintheintestinalwall),portalvenousgas,orpneumoperitoneum(image1).With
malrotationandpyloricstenosis,theremaybeduodenalorgastricdistentionwithapaucityofairdistally.
Infantswithbiliousemesisshouldreceivecontrasteduppergastrointestinal(UGI)studieswithsmallbowelfollowthrough.Aduodenalbulbthat
overliesthespineand/oramediallydirectedcecumsuggestsmalrotation(image2andimage3).Acorkscrewappearanceinthesmallbowelcan
beseenwithvolvulus(image4).
Anabdominalultrasoundisthepreferredstudytodetectpyloricstenosis(image5).A"stringsign"maybeseenonUGIwithpyloricstenosis
(image6).

 Infantswithseizuresorfocalneurologicexaminationsshouldreceiveheadcomputedtomography(CT).Askeletalsurvey(plainfilmsofallbones)
toscreenforoldornewfracturesshouldbeperformedwheninflictedheadinjuryissuspected.(See"Childabuse:Evaluationanddiagnosisof
abusiveheadtraumaininfantsandchildren",sectionon'Imaging'.)
OtherstudiesAnelectrocardiogram(EKG)shouldbeperformedroutinelyforanyinfantbelievedtohaveCHD.EKGfindingsmaysuggesta
specificanatomiclesion(table11).Aninjurypatternmayidentifyinfantswhohavemyocardialischemiaastheresultofaberrantcoronaryarteries.An
emergentechocardiogrammaybeneededtoassesssomecriticallyillnewborns.
Thehyperoxiatestcanhelptodistinguishcardiacfrompulmonarydisease.Oxygensaturationismeasuredusingpulseoximetrybeforeandwhilethe
infantisbreathing100percentoxygen.Oxygensaturationshouldimprovebyatleast10percentforpulmonarycausesofcyanosis[18].Anabnormalor
equivocalresponsesuggestscardiacdiseaseandmustbeverifiedbymeasurementofanarterialbloodgas,takenfromtherightradialartery,whilethe
infantisbreathing100percentoxygen.(See"Diagnosisandinitialmanagementofcyanoticheartdiseaseinthenewborn",sectionon'Hyperoxiatest'.)
InitialmanagementdecisionsInfantswhoarebreathingspontaneouslyandeffectivelybuthaveevidenceofrespiratorydistressorcardiovascular
compromiserequireimmediateresuscitationwithsupplementaloxygenandintravenousfluids.Othermorecriticallyillinfantsmayneedtobesupported
withbagmaskormechanicalventilation.(See"Initialassessmentandstabilizationofchildrenwithrespiratoryorcirculatorycompromise"and
"Emergencyendotrachealintubationinchildren"and"Initialmanagementofshockinchildren".)
Somecriticallyillinfantsmayrequirespecificlifesavinginterventionsbeforedefinitivediagnoseshavebeenestablished.Inthissituation,theemergency
clinicianmustdeterminethelikelihoodthataninfantmayhavethediagnosis,whileconsideringthepotentialharmofthetreatment.Treatmentsthat
shouldbeconsideredincludethefollowing:
AntibioticsSymptomsofoverwhelminginfectionarenotoriouslynonspecificinyounginfants.Onceculturesofbloodandurine(andCSF,if
possible)havebeenobtained,mostillappearingyounginfantsshouldreceiveantibiotics(table12).(See"Evaluationandmanagementoffeverinthe
neonateandyounginfant(youngerthanthreemonthsofage)",sectionon'Evaluationandmanagement'.)
AcyclovirEarlytreatmentwithacyclovirisassociatedwithimprovedoutcomesamonginfantswithHSVinfections[1,19].However,thedefinitive
diagnosismaydependoncultureresultsorotherteststhatarenotimmediatelyavailable,suchaspolymerasechainreaction(PCR)testing.
SincetheincidenceofHSVamonginfantsislow,acyclovirshouldbeadministeredselectively.Infants28daysofagewhoareillappearingandhave
anyofthefollowingfeaturesshouldreceiveacyclovir:

Mucocutaneousvesicles
Neurologicsymptomssuchasseizures
CSFpleocytosiswithanegativeCSFGramstain
RedbloodcellsinCSFfromanatraumaticlumbarpuncture
MaternalhistoryofHSV

CulturesforHSV,aswellasaspecimenofCSFforHSVPCR,shouldbeobtainedbeforeacyclovirisgiven.(See"Evaluationandmanagementoffever
intheneonateandyounginfant(youngerthanthreemonthsofage)",sectionon'Evaluationandmanagement'.)
ProstaglandinE1(alprostadil)Forinfantswithcyanoticorobstructiveheartdiseasewhoaredependentonbloodflowthroughtheductus

arteriosus(DA)forpulmonaryorsystemiccirculation,severesymptomscandevelopwhentheDAclosesoverthefirstseveraldaystoweeksoflife.
StructuralclosureoftheDAisusuallycompletedby2to3weeksofage,makingthediagnosisofaductaldependentcardiacdefectunlikelyamong
infantsolderthan28days.(See"Clinicalmanifestationsanddiagnosisofpatentductusarteriosus",sectionon'Fetalandtransitionalductalcirculation'.)
Forhypoxic,hemodynamicallyunstableinfantswithductaldependentcongenitalheartdisease,treatmentwithprostaglandinE1(PGE1,alprostadil)to
reopentheductusarteriosus(DA)canbelifesaving[20,21].CirculationthroughtheDAtemporarilyrestorespulmonaryorsystemicbloodflowwhilethe
patientundergoesfurtherevaluationinpreparationfordefinitivetreatment.(See"Diagnosisandinitialmanagementofcyanoticheartdiseaseinthe
newborn",sectionon'ProstaglandinE1'.)
Infants28daysofagewhodonotrespondtoinitialresuscitativeeffortsandarelikelytohaveaductaldependentdefect,butforwhomdiagnosismay
bedelayed(suchasthosewhomustbetransferredtoanotherfacilityforechocardiography),shouldreceivePGE1,ideallyafterconsultationwitha
neonatologistorpediatriccardiologist[22].SideeffectsofPGE1infusionincludehypotension,tachycardia,andapnea.Equipmenttoprovideadvanced
airwaymanagementshouldbereadilyavailableandinfantsshouldhavereliableIVaccess.
PGE1shouldbestartedasanintravenousinfusionatadoseof0.05mcg/kgperminute.Inordertolimitsideeffects,thedosemaybetitrateddownto
thelowestdoseatwhichthepatient'sconditionremainsimproved.Apneaislesslikelywithdoses<0.01mcg/kgperminute[23].
Indicationsofaductaldependentcardiaclesionincludeoneofthefollowing:
Failedhyperoxiatest,suggestingcyanoticheartdisease.(See"Diagnosisandinitialmanagementofcyanoticheartdiseaseinthenewborn",
sectionon'Hyperoxiatest'.)
OR
Apulseorbloodpressuregradientbetweentheupperandlowerextremities,suggestinganobstructivecardiacdefect.(See"Clinicalmanifestations
anddiagnosisofcoarctationoftheaorta",sectionon'Bloodpressureandpulses'.)
HydrocortisoneAninfant(usuallymale)withpreviouslyundiagnosedcongenitaladrenalhyperplasiamaydevelopadrenalcrisisandpresentas
septicappearing.Treatmentwithhydrocortisonemaybelifesaving.
Specificmanifestationsofadrenalinsufficiencyincludehyponatremia,hyperkalemia,hypoglycemia,andhypotension.Patientswiththesefindingsmust
promptlyreceivestressdosesofhydrocortisone.(See"Treatmentofclassiccongenitaladrenalhyperplasiadueto21hydroxylasedeficiencyininfants
andchildren",sectionon'Managementinneonates'.)
ALGORITHMICAPPROACHAsystematicapproachtotheemergentevaluationofayounginfantwhoappearsseptic(includingafocusedhistory,
carefulphysicalexamination,andselectedancillarystudies)cangenerallyidentifythosewithaconditionotherthansepsisthatrequiresemergent
evaluationandtreatment(table1andtable4andtable6andalgorithm1).
SpecifichistoryorphysicalfindingsFeaturesofthehistoryorphysicalexaminationmaysuggestthediagnosisandguideevaluationandtreatment.
Cutaneousfindings
Focalareasoferythema,warmth,ortendernesssuggestsskininfectionwhichmaybelocalized(aswithmastitisoromphalitis).(See'Bacterial
infections'above.)

 Infantswhoarejaundicedmayhaveacutebilirubinencephalopathy.(See"Clinicalmanifestationsofunconjugatedhyperbilirubinemiaintermand
latepreterminfants",sectionon'Neurologicmanifestations'.)
Centralcyanosisthatdoesnotimprovewhenthepatientisbreathing100percentoxygenoccurswithcyanoticcongenitalheartdiseaseand
methemoglobinemia.Bloodfrompatientswithmethemoglobinemiaisdarkred,chocolate,orbrownishtoblueincoloranddoesnotchangewiththe
additionofoxygen(figure2).Inaddition,patientswithmethemoglobinemiamayappearcyanoticorduskybuthavenormalornearnormaloxygen
saturationsasmeasuredbypulseoximetry.(See"Diagnosisandinitialmanagementofcyanoticheartdiseaseinthenewborn"and"Clinical
features,diagnosis,andtreatmentofmethemoglobinemia".)
RespiratorydistressPhysicalfindingssuchastachypnea,grunting,retractions,andapneaarenonspecificsignsofrespiratorydistressthatcan
occurinanumberofconditions,includinginflictedheadinjury,infantbotulism,bronchiolitis,orpertussis.(See"Childabuse:Evaluationanddiagnosisof
abusiveheadtraumaininfantsandchildren"and"Neuromuscularjunctiondisordersinnewbornsandinfants",sectionon'Infantbotulism'and"Bordetella
pertussisinfectionininfantsandchildren:Clinicalfeaturesanddiagnosis",sectionon'Infants'and'Viralinfections'above.)
Infantswithwheezesand/orralesmayhavepneumonia,bronchiolitis,orheartfailure.(See"Bronchiolitisininfantsandchildren:Clinicalfeaturesand
diagnosis",sectionon'Apnea'.)
AbnormalcardiacexaminationAnabnormalcardiacexaminationthatmayincludedecreasedpulsesorbloodpressureinthelowerextremities,
thepresenceofamurmurorgallop,aheartrateover220bpm,oraninjurycurrentonEKG,suggestsacardiacconditionsuchascongenitalheart
disease,myocarditis,pericarditis,supraventriculartachycardia,oraberrantcoronaryarteries.(See"Diagnosisandinitialmanagementofcyanoticheart
diseaseinthenewborn",sectionon'Physicalexamination'and"Clinicalmanifestationsanddiagnosisofcoarctationoftheaorta",sectionon'Clinical
manifestations'and"Clinicalmanifestationsanddiagnosisofmyocarditisinchildren"and"Supraventriculartachycardiainchildren:AVreentrant
tachycardia(includingWPW)andAVnodalreentranttachycardia",sectionon'Heartfailure'and"Congenitalandpediatriccoronaryarteryabnormalities",
sectionon'Variationsofcoronaryarteryoriginfromthepulmonaryartery'.)
MusculoskeletalfindingsInfantswhoarenotmovinganextremityorhaveswollenextremitiesorjointsmayhaveosteomyelitis,septicarthritis,
orafracture.Inflictedinjurymustbeconsideredforpatientswithfractures.(See"Hematogenousosteomyelitisinchildren:Clinicalfeaturesand
complications",sectionon'Birthtothreemonths'and"Orthopedicaspectsofchildabuse".)
BiliousvomitingInfantswithbiliousvomitingmustbeemergentlyevaluatedforcausesofbowelobstruction,particularlymalrotationwith
volvulus.(See"Intestinalmalrotation"and"Overviewofinguinalherniainchildren",sectionon'Incarceratedmass'.)
NospecificclinicalfeaturesYounginfantswhoappeartohavesepsisareseriouslyillandrequireancillarystudiestoidentifythecauseoftheir
symptoms.Thesestudiesmaybeparticularlyusefulforpatientswithoutspecificclinicalfeatures.However,infantswhoareseriouslyillmayhavevery
fewdistinguishingclinicalcharacteristicsandrelativelynormalancillarystudies.Examplesincludesomepatientswithsepsis,overwhelmingviral
illnesses,inflictedheadinjury,andinfantbotulism.
AbnormalcerebrospinalfluidInfantswithCSFpleocytosisusuallyhavemeningitisorencephalitis.HSVinfectionmustbeconsideredwhen
thereisCSFpleocytosiswithnoorganismsongramstainorthereareredbloodcellsfromanatraumaticlumbarpuncture(table8).(See"Bacterial
meningitisinchildrenolderthanonemonth:Clinicalfeaturesanddiagnosis",sectionon'InterpretationofCSF'and"Neonatalherpessimplexvirus
infection:Clinicalfeaturesanddiagnosis",sectionon'Clinicalmanifestations'and"Bacterialmeningitisintheneonate:Clinicalfeaturesanddiagnosis",
sectionon'Lumbarpuncture'.)

AbnormalchestradiographLunginfiltratesonachestradiographmayrepresentinfections(suchaspneumoniaorbronchiolitis)orheartfailure.
Infantswithcardiomegalyorabnormalcardiacsilhouettesmayhavecongenitalheartdiseaseormyocarditis.
PyuriaAnabnormalurinalysis,particularlywithpyuria,suggestspyelonephritisandpossibleurosepsisintheillappearinginfant.(See"Urinary
tractinfectionsininfantsandchildrenolderthanonemonth:Clinicalfeaturesanddiagnosis",sectionon'Microscopicexam'.)
AbnormalbloodchemistriesAbnormalitiesinbloodchemistriesmayhelptoidentifyaspecificcondition.Inaddition,manyofthese
abnormalitiesmustbeurgentlytreated.
Acidosisisanonspecificconsequenceofmanydisordersthatmaymimicsepsis,includingotherconditionsthatcauseshock,suchasCAH(table
13).Acidosismayalsooccurwithinbornerrorsofmetabolism,methemoglobinemia,carbonmonoxidepoisoning,dehydration,necrotizing
enterocolitis,andappendicitis.
Infantswithpyloricstenosismaydevelophypochloremicalkalosisfromlossofgastrichydrochloricacidastheresultofpersistentvomiting.(See
"Infantilehypertrophicpyloricstenosis",sectionon'Classicpresentation'.)
Hyponatremiamaydevelopastheresultofwaterintoxication(intakeofexcessiveamountsoffreewater),syndromeofinappropriateantidiuretic
hormonesecretion,orfromexcessivesodiumlosses(suchasrenallosseswithCAHorlossesfromtheskinwithcysticfibrosis).(See"Fluidand
electrolytetherapyinnewborns",sectionon'Hyponatremia'and'Othercauses'above.)
Hypernatremiatypicallyoccursastheresultofsodium(salt)poisoning,excessivelossoffreewater(ascanoccurwithdiabetesinsipidus),orloss
ofwaterinexcessofsodiumlosses.(See"Fluidandelectrolytetherapyinnewborns",sectionon'Hypernatremia'and'Othercauses'above.)
Infantswhoareseriouslyillarefrequentlyhypoglycemic.Severehypoglycemiaisalsoassociatedwithshock,congenitaladrenalhyperplasia,and
inbornerrorsofmetabolism.(See"Pathogenesis,screening,anddiagnosisofneonatalhypoglycemia".)
Hyperammonemiaisacharacteristicfindinginureacycledefects,organicacidemias,fattyacidoxidationdefects,andliverdysfunction(algorithm
2).
SUMMARYANDRECOMMENDATIONSYounginfantswhoareprofoundlyillareofteninitiallypresumedtohavesepsis.Althoughthatisoftenthe
case,somepatientsmayhaveotherconditionsthathavesimilar,nonspecificclinicalfeatures(table1).(See'Causes'above.)
Historicalfeatures,physicalfindings,andancillarystudiesmayidentifyaspecificdiagnosis(table4andtable6).(See'Evaluationanddecision'
above.)
Theinitialmanagementofsepticappearinginfantsconsistsofresuscitationwithsupplementaloxygenandintravenousfluids.Specificinterventions
includethefollowing(see'Initialmanagementdecisions'above):
Onceculturesofbloodandurine(andCSF,ifpossible)havebeenobtained,mostillappearingyounginfantsshouldreceiveantibiotics(table
12).(See"Evaluationandmanagementoffeverintheneonateandyounginfant(youngerthanthreemonthsofage)",sectionon'Neonates(0
to28days)'and"Evaluationandmanagementoffeverintheneonateandyounginfant(youngerthanthreemonthsofage)",sectionon'Ill
appearinginfants(29to90days)'.)
Infants28daysoldwhohavemucocutaneousvesicles,seizures,CSFpleocytosiswithanegativeGramstain,ormaternalherpessimplex

virus(HSV)infectionshouldreceiveacyclovir.HSVcultureandPCRforHSVtestingshouldideallybeobtainedpriortotreatment.(See
"Evaluationandmanagementoffeverintheneonateandyounginfant(youngerthanthreemonthsofage)",sectionon'Acyclovir'.)
Hypoxic,hypotensive,andacidoticinfants28daysold,whodonotrespondtoresuscitativeeffortsandarelikelytohaveductaldependent
congenitalheartdisease(assuggestedbyeitherafailedhyperoxiatestorapulseorbloodpressuregradientbetweentheupperandlower
extremities),shouldreceiveprostaglandinE1(alprostadil).Aneonatologistorpediatriccardiologistshouldbeconsulted.(See"Diagnosisand
initialmanagementofcyanoticheartdiseaseinthenewborn",sectionon'ProstaglandinE1'.)
Criticallyillinfantswithsignsofadrenalcrisis(hyponatremia,hyperkalemia,hypoglycemia,andhypotension)shouldreceivehydrocortisone
ideallyafterbloodhasbeendrawnforbaselineACTHandcortisolmeasurements.(See"Treatmentofadrenalinsufficiencyinchildren",
sectionon'Adrenalcrisis'.)
Analgorithmicapproachtotheemergentevaluationofthesepticappearinginfantcanbeuseful(algorithm1).(See'Algorithmicapproach'above.)
UseofUpToDateissubjecttotheSubscriptionandLicenseAgreement.
REFERENCES
1. KimberlinDW,LinCY,JacobsRF,etal.Naturalhistoryofneonatalherpessimplexvirusinfectionsintheacyclovirera.Pediatrics2001108:223.
2. WillwerthBM,HarperMB,GreenesDS.Identifyinghospitalizedinfantswhohavebronchiolitisandareathighriskforapnea.AnnEmergMed
200648:441.
3. LeeC,MasonLJ.Pediatriccardiacemergencies.AnesthesiolClinNorthAmerica200119:287.
4. BonadioWA,ClarksonT,NausJ.Theclinicalfeaturesofchildrenwithmalrotationoftheintestine.PediatrEmergCare19917:348.
5. HulkaF,CampbellTJ,CampbellJR,HarrisonMW.Evolutionintherecognitionofinfantilehypertrophicpyloricstenosis.Pediatrics1997100:E9.
6. BallesteroY,HernandezMI,RojoP,etal.Hyponatremicdehydrationasapresentationofcysticfibrosis.PediatrEmergCare200622:725.
7. OddieS,RichmondS,CoulthardM.Hypernatraemicdehydrationandbreastfeeding:apopulationstudy.ArchDisChild200185:318.
8. ShroffR,HignettR,PierceC,etal.Lifethreateninghypernatraemicdehydrationinbreastfedbabies.ArchDisChild200691:1025.
9. SperlingMA,MenonRK.Differentialdiagnosisandmanagementofneonatalhypoglycemia.PediatrClinNorthAm200451:703.
10. Gold,CR,Pierog,J.Arationalapproachtopediatricseizures.PediatricEmergencyMedicineReports20005:121.
11. PollackES,PollackCVJr.Incidenceofsubclinicalmethemoglobinemiaininfantswithdiarrhea.AnnEmergMed199424:652.
12. MuroneAJ,StuckiP,RobackMG,GehriM.Severemethemoglobinemiaduetofoodintoxicationininfants.PediatrEmergCare200521:536.
13. PiattJP,KaplanAM,BondGR,BergRA.Occultcarbonmonoxidepoisoninginaninfant.PediatrEmergCare19906:21.
14. O'SullivanBP.Carbonmonoxidepoisoninginaninfantexposedtoakeroseneheater.JPediatr1983103:249.
15. PickertCB,MossMM,FiserDH.Differentiationofsystemicinfectionandcongenitalobstructiveleftheartdiseaseintheveryyounginfant.Pediatr
EmergCare199814:263.
16. BramsonRT,MeyerTL,SilbigerML,etal.Thefutilityofthechestradiographinthefebrileinfantwithoutrespiratorysymptoms.Pediatrics1993
92:524.

17. CrainEF,BulasD,BijurPE,GoldmanHS.Isachestradiographnecessaryintheevaluationofeveryfebrileinfantlessthan8weeksofage?
Pediatrics199188:821.
18. BrousseauT,SharieffGQ.Newbornemergencies:thefirst30daysoflife.PediatrClinNorthAm200653:69.
19. KimberlinDW,LinCY,JacobsRF,etal.Safetyandefficacyofhighdoseintravenousacyclovirinthemanagementofneonatalherpessimplex
virusinfections.Pediatrics2001108:230.
20. FreedMD,HeymannMA,LewisAB,etal.ProstaglandinE1infantswithductusarteriosusdependentcongenitalheartdisease.Circulation1981
64:899.
21. Zahka,KG,Siwik,ES.Principlesofmedicalandsurgicalmanagement.In:NeonatalperinatalMedicine,9th,Martin,RJ,Fanaroff,AA,Walsh,MC
(Eds),MosbyElsevier,Philadelphia2011.Vol2,p.1290.
22. HallidieSmithKA.ProstaglandinE1insuspectedductusdependentcardiacmalformation.ArchDisChild198459:1020.
23. KramerHH,SommerM,RammosS,KrogmannO.EvaluationoflowdoseprostaglandinE1treatmentforductusdependentcongenitalheart
disease.EurJPediatr1995154:700.
Topic6467Version12.0

GRAPHICS
Approachtothesepticappearinginfant

ABC:airway,breathing,circulationIV:intravenouscatheterPE:physicalexaminationCSF:cerebrospinalfluidCXR:chest
radiographHSV:herpessimplexvirusSVT:supraventriculartachycardiaCAH:congenitaladrenalhyperplasia.
*Culturesofbloodandurine,CBC,enhancedUA,electrolytes,glucose.Forpatientsabletotoleratetheprocedure,performlumbar
punctureunlessanunderlyingcauseisrapidlyidentified(eg,congenitalheartdisease,abusiveheadinjury,malrotationwithvolvulus).
Chestradiographandotherstudies(eg,serumbilirubin,arterialbloodgas,EKG,ormetabolicstudies)mayalsobeindicateddepending
upontheclinicalfindings.
Patientswhocannottoleratelumberpuncture(LP)shouldhaveabloodcultureandreceiveantibiotics.AnLPshouldbeperformed
oncethepatient'sconditionisstabilizedunlessanetiologyotherthanseriousinfectionisidentified.
Graphic66424Version6.0

Causesofthesepticappearinginfant
Infections
Bacterialmeningitis
Sepsis
Urinarytract
Pneumonia
Cellulitis
Omphalitis
Mastitis
Septicarthritis
Osteomyelitis

Pertussis
Infantbotulism
Overwhelmingviralillness
Bronchiolitis
Myocarditis

Trauma
Inflictedheadinjury
Uintentionalinjury

Neurological
Seizures

Surgical/gastrointestinal
Pyloricstenosis
Malrotationwithvolvulus
Incarceratedhernia
Necrotizingenterocolitis

Appendicitis

Cardiac
Congenitalheartdisease
Cyanotic
Obstructive
Aberrantcoronaryartery

Supraventriculartachycardia

Endocrine
Congenitaladrenalhyperplasia

Metabolic
Hypoglycemia
Inbornerrorsofmetabolism

Hematologic
Acutebilirubinencephalopathy

Toxicexposures
Methemaglobinemia
Carbonmonoxidepoisoning

Apparentlifethreateningevent
Kawasakidisease
Graphic74535Version2.0

Diagnosticalgorithmforinitialevaluationof
hyperammonemia

ASA:argininosuccinicaciduriaCPS:carbamylphosphatesynthetaseOTC:
ormithinetranscarbamylase.
Graphic52126Version4.0

Midgutvolvulus

Volvulusoccursbecausethenarrowmesentericbase,whichdevelops
asaresultofmalrotation,allowsthesmallboweltotwistaroundthe
superiormesentericartery.Thisleadstovascularcompromiseoflarge
portionsofthemidgut.
Graphic78111Version2.0

Mostfrequentlyoccurringetiologiesofneonatalseizures
Neonatalandhypoxicischemicencephalopathy
Intracranialhemorrhage
Intraventricular
Intracerebral
Subdural
Subarachnoid

Centralnervoussysteminfection
Meningitis
Encephalitis
Intrauterine

Cerebralinfarction
Metabolic
Hypoglycemia
Hypocalcemia
Hypomagnesemia

Chromosomalanomalies
Congenitalabnormalitiesofthebrain
Neurodegenerativedisorders
Inbornerrorsofmetabolism
Benignneonatalconvulsions
Benignfamilialneonatalconvulsions
Drugwithdrawalorintoxication

Listedinrelativeorderoffrequency.Notlistedis"unknown"etiology,whichisencounteredinapproximately10percentof
cases(althoughsomeinthiscategorymaybebenignneonatalconvulsions).
Reproducedwithpermissionfrom:MizrahiEM,KellawayP.DiagnosisandManagementofNeonatalSeizures.LippincottRaven,Philadelphia
1998.Copyright1998LippincottWilliams&Wilkins.

http://www.lww.com
Graphic73867Version10.0

Methemoglobinemia

Samplesofbloodwithvaryingmethemoglobinlevelsdisplayedonwhiteabsorbentmaterial.
Reproducedfrom:ShihanaF,DissanayakeDM,BuckleyNA,DawsonAH.Asimplequantitativebedside
testtodeterminemethemoglobin.AnnEmergMed201055:184.Illustrationusedwiththe
permissionofElsevierInc.Allrightsreserved.
Graphic58540Version5.0

Radiographofnecrotizingenterocolitisinpremature
infants

Plainabdominalradiographsinprematureinfantswithnecrotizingenterocolitis.
Leftpanel:Thereismarkedabdominaldistentiondueinparttodilatedbowel
loops,andbubblesofgasinthebowelwallduetoextensivepneumatosis
intestinalis(arrow).Anorogastrictubeisinplace.Rightpanel:Thereismarked
abdominaldistention,pneumatosisintestinalis,andasuspicionofportalvenous
(arrow)and/orfreeintraperitonealair.
Graphic78676Version4.0

Differentialdiagnosisofanapparentlifethreateningevent(ALTE)
Normal(misinterpretedasabnormalbehavior)
Transientchoke,gagorcoughduringfeeding
IrregularbreathingofREMsleepininfants
Periodicbreathing
Respiratorypauses(5to15sec),andlongerpausesaftersigh

Acuteconditions
Infections
Respiratoryinfections(eg,pertussis,respiratorysyncytialvirus,bronchiolitis)
Sepsis,meningitis,encephalitis
Gastrointestinal
Intussusception
Volvulus
Drugeffect
Unintentionalorintentionalingestion(eg,coldmedicationsorethanol)
Postanesthesia
Metabolicdecompensation
Primaryinbornerrorofmetabolism
Otherendocrine,electrolyte,ormetabolicdisorder
Toxicexposure
Carbonmonoxide
Accidentalorintentionalingestionofatoxin
Childabuse
Intentionalsuffocation
Abusiveheadinjury
Intentionalpoisoningorintoxication

Factitiousillness

Chronicconditions
Gastrointestinal
Gastroesophagealreflux
Swallowingincoordination
Cardiovascular
Arrhythmia
Cardiomyopathy
Respiratory
Aspiration,withstimulationoflaryngealchemoreceptors,causingapnea
Breathholdingspellsorvariant
Abnormalitiesofrespiratorycontrol
Immaturityorprematurity
Centralhypoventilationsyndrome
Upperairwayobstruction
Vocalcorddysfunction
Laryngotracheomalacia
Vascularring
Neurologic
Seizure
Vasovagalsyncope
Otherneurologicconditionsaffectingrespiratorycontrol
ApneaassociatedwithChiariorotherhindbrainmalformation
CNShemorrhage

Nodefinablecause
REM:rapideyemovementCNS:centralnervoussystem.

Graphic51356Version6.0

Linkinghistoryanddiagnosisinthesepticappearinginfant
History

Likelydiagnosis

Motherwithgenitallesions

Herpessimplexvirus

Notusinganextremity

Osteomyelitis,septicarthritis,orlongbone
fracture

Mechanismofinjurynotconsistentwithdevelopmentalabilityofthechildand/or
severityofinjuries

Abusiveheadtraumaorotherinflictedinjury

Rhythmictwitching,briefjerks,tonicrigidity,repetitiveblinking,chewing,nystagmus,
bicyclingmovementsofextremities

Seizure

Nofeverprogressiveweakness,poorheadcontrol,floppiness,constipation,breastfed

Infantbotulism

Progressivelyworsening,projectile,nonbiliousemesis

Pyloricstenosis

Biliousemesis

Malroataionwithvolvulusorother
gastrointestinalobstruction

Sweatingwithfeeds

Congenitalheartdisease

Unusualodors

Inbornerrorsofmetabolism

Graphic76560Version2.0

Urinarycluestoinbornerrorsofmetabolism

Potentialdisorder

Urinecolor
Black(uponstanding/oxidation)

Homogentisicaciduria(alkaptonuria)

Blue

Tryptophanmalabsorption

Pink

Disorderswithhematuria,kidneystoneformation

Portwine(uponstanding/oxidation)

Porphyrias

Yelloworange

Disorderswithincreaseduricacid

Urineodor*
Acrid,sweatyfeet

GlutaricacidemiaII

Cabbage

Tyrosinemia

Fishy

Trimethlylaminuria,dimethylglycinuria

Maplesyrup,curry

Maplesyrupurinedisease

Mousy

Phenylketonuria

Sweatyfeet

Isovalericacidemia

Sweet

Betaketothiolasedeficiency

Swimmingpool

Hawkinsinuria

*Onlyinacutephasesordependingonfoodintake.
Adaptedfrom:WappnerRS,HainlineBE.Inbornerrorsofmetabolism.In:Oski'sPediatrics.PrinciplesandPractice,3rded,McMillanJA,
DeAngelisCD,FeiginRD,WarshawJB(Eds),Lippincott,Williams&Wilkins,Philadelphia,1999.p.1823andSaudubrayJM,ChappentierC.
Clinicalphenotypes:Diagnosis/algorithms.In:MetabolicandMolecularBasesofInheritedDisease,8thed,ScriverCR,BeaudetAL,SlyWS,
ValleD(Eds),McGrawHill,NewYork,2001.p.1327.
Graphic74441Version3.0

Linkingphysicalexaminationanddiagnosisinthesepticappearinginfant
Physicalfindings

Likelydiagnosis

Bulgingfontanelle,fever

Meningitis

Bulgingfontanelle,nofever

Hydrocephalusaswithinflictedhead
injury

Skinvesicles

Herpessimplexvirus

Temperature>39C,femaleoranuncircumcisedmale

UTI

Weakcry,hypotonia,hyporeflexic,diminishedorabsentgagreflex,ptosis,mydriasis,weaksuck,
opthalmoparesis

Infantbotulism

Pylorictumor("olive")intherightupperquadrant

Pyloricstenosis

Scrotumexamwithtenderswellingattheexternalring,aboveandlateraltothepubiswithout
anupperlimit

Incarceratedhernia

Apnea,bradycardia,temperatureinstability,bloodystools,abdominaldistention

Necrotizingenterocolitis

Murmur,gallop,hepatosplenomegaly,edema,rales,grunting,flaring,retracting

Congenitalheartdisease

Ambiguousgenitaliainfemales,virilizationinmales

Congenitaladrenalhyperplasia

Jaundice,opisthotonus,highpitchedcry

Acutebilirubinencephalopathy

UTI:urinarytractinfection.
Graphic63744Version2.0

Domeshapedretinalhemorrhage

Domeshapedretinalhemorrhagesmaybreakintothevitreous.
CourtesyofBrianForbes,MD,PhD.
Graphic69754Version1.0

Characteristicsofcerebrospinalfluidintermandpretermneonateswithoutbacterial
meningitis

Age

MeanWBC/mm 3
(rangeor90th
percentile)

ANC/mm 3or
percentPMNs
(range)

Meanprotein
(mg/dL)
(rangeorSD)

Meanglucose
(mg/dL)
(rangeorSD)

Termneonatesevaluatedinthenurserysetting
0to24hours

5(0to90)

3/mm 3(0to70)

63(32to240)

51(32to78)

0to10days

8.2(0to32)

61.3percent

90(20to170)

52(34to119)

0to32days
(n=24) [3]

11(1to38)

21percent(0to100)

NR

NR

(n=135)* [1]
(n=87) [2]

Termneonatesevaluatedintheemergencydepartmentsetting
0to7days

15.3(1to130)

4.4/mm 3(0to65)

80.8(30.8)

45.9(7.5)

0to7days

8.6(90 thpercentile:26)

NR

106.4(90 thpercentile:

NR

(n=17) [4]
(n=118) [5]

153)

1to28days
(n=297) [6]

6.1(0to18)

NR

75.4(15.8to131)

45.3(30to61)

0to30days

7.3(0to130)

0.8/mm 3(0to65)

64.2(24.2)

51.2(12.9)

8to14days

3.9(90 thpercentile:9)

NR

77.6(90 thpercentile:

NR

(n=108) [4]
(n=101) [5]

103)

8to14days

5.4(0to18)

0.1/mm 3(0to1)

69(22.6)

54.3(17)

15to22days
(n=107) [5]

4.9(90 thpercentile:9)

NR

71(90 thpercentile:
106)

NR

15to21days

7.7(0to62)

0.2/mm 3(0to2)

59.8(23.4)

46.8(8.8)

(n=33) [4]

(n=25) [4]

th

th

22to28days
(n=141) [5]

4.5(90 thpercentile:9)

NR

68.7(90 thpercentile:
85)

NR

22to30days
(n=33) [4]

4.8(0to18)

0.1/mm 3(0to1)

54.1(16.2)

54.1(16.2)

Pretermorlowbirthweightneonates
0to28days
(n=30 ) [2]

9(0to29)

57.2percent

115(65to150)

50(24to63)

0to32days
(n=22 ) [3]

7(0to28)

16percent(0to100)

NR

NR

Verylowbirthweightneonates [7]
<1000g

0to7days
(n=6)

3(1to8)

11percent(0to50)

162(115to222)

70(41to89)

8to28days

4(0to14)

8percent(0to66)

159(95to370)

68(33to217)

4(0to11)

2percent(0to36)

137(76to269)

49(29to90)

0to7days
(n=8)

4(1to10)

4percent(0to28)

136(85to176)

74(50to96)

8to28days
(n=14)

7(0to44)

10percent(0to60)

137(54to227)

59(39to109)

29to84days

8(0to23)

11percent(0to48)

122(45to187)

47(31to76)

(n=17)
29to84days
(n=15)
1000to1500g

(n=11)
WBC:whitebloodcellcountANC:absoluteneutrophilcountPMNs:polymorphonuclearleukocytesSD:standarddeviationNR:notreported
CSF:cerebrospinalfluid.
*CSFobtainedfromtermneonateswithoutanyobviouspathology.
CSFobtainedfromhospitalizedneonatesathighriskforinfection(eg,unexplainedjaundice,prolongedruptureofmembranes,maternal
fever,etc)infectionexcludedbysterilecultures(CSF,blood,urine)andlackofclinicalevidenceofbacterialorviralinfection.
CSFobtainedintheemergencydepartmentduringevaluationforpossibleinfectioninfectionwasexcludedbysterilecultures(CSF,blood,
urine,andnegativepolymerasechainreactionforenterovirus).
OnlytwoinfantshadCSFWBC>30/mm 3:one<7daysofagewith130WBC/mm 3,andone15to21daysofagewith62WBC/mm 3.

Includes29preterminfantsand1infantwhowas2190gat40weeks'gestation.
Includesallinfantswithbirthweight<2500g.
References:
1. NaidooBT.Thecerebrospinalfluidinthehealthynewborninfant.SAfrMedJ196842:933.
2. SarffLD,LynnH,PlattMD,etal.Cerebrospinalfluidevaluationinneonates:Comparisonofhighriskinfantswithandwithout
meningitis.JPediatr197688:473.
3. PappuL.CSFcytologyintheneonate.AmJDisChild1982136:297.
4. AhmedA.Cerebrospinalfluidvaluesinthetermneonate.PediatrInfectDisJ199615:298.
5. ChadwickSL,WilsonJW,LevinJE,MartinJM.Cerebrospinalfluidcharacteristicsofinfantswhopresenttotheemergencydepartment
withfever:establishingnormalvaluesbyweekofage.PediatrInfectDisJ201130:e63.
6. ByingtonCL,KendrickJ,ShengX.Normativecerebrospinalfluidprofilesinfebrileinfants.JPediatr2011158:130.
7. RodriguezAF,KaplanSL,MasonEO.Cerebrospinalfluidvaluesintheverylowbirthweightinfant.JPediatr1990116:971.
Graphic54464Version14.0

Typicalcerebrospinalfluidfindingsincentralnervoussysteminfections*
Glucose(mg/dL)

<10

10to40

Protein(mg/dL)
100to500

50to300

Totalwhitebloodcellcount
(cells/microL)
>1000

100to1000

5to100

More

Bacterial

Bacterial

Bacterial

Viralmeningitis

Bacterial

Bacterialor

Earlybacterial

common

meningitis

meningitis

meningitis

Nervoussystem

meningitis

viral
meningitis

meningitis

Lymedisease
(neuroborreliosis)

Less

TBmeningitis

Neurosyphilis

common

Fungal

Someviral

meningitis

infections
(suchas

TBmeningitis

Viral
meningitis

Neurosyphilis

Neurosyphilis

TBmeningitis

TBmeningitis

Somecasesof

Encephalitis

Encephalitis

mumpsand
LCMV

mumpsand
LCMV)
TB:tuberculosisLCMV:lymphocyticchoriomeningitisvirus.
*Itisimportanttonotethatthespectrumofcerebrospinalfluidvaluesinbacterialmeningitisissowidethattheabsenceofoneormoreof
thesefindingsisoflittlevalue.RefertotheUpToDatetopicreviewsonbacterialmeningitisforadditionaldetails.
<0.6mmol/L.
0.6to2.2mmol/L.
1to5g/L.
0.5to3g/L.
Graphic76324Version8.0

Distinguishingbiochemicalfindingsofinbornerrorsofmetabolism
Maple
syrup
urine
disease

Organic
acidemias

Urea
cycle
defects

Disordersof
carbohydrate
metabolism

Fatty
acid
oxidation
disorders

Mitochondrial
disorders

Peroxisomal
disorders

Lysoso
stora
disord

Metabolicacidosis

++

Respiratory

Hyperammonemia

++

Hypoglycemia

Ketones

A/H

A/H

A/L

A/H

Lacticacidosis

++

Findings

alkalosis

:usuallyabsent:sometimespresent+:usuallypresent++:alwayspresentA:appropriateH:inappropriatelyhighL:inappropriately
low.
*Withindiseasecategories,notalldiseaseshaveallfindingsfordisorderswithepisodicdecompensationclinicalandlaboratoryfindingsmaybe
presentonlyduringacutecrisisforprogressivedisorders,findingsmaynotbepresentearlyinthecourseofdisease.
Adaptedfrom:WeinerDL.MetabolicEmergencies.In:TextbookofPediatricEmergencyMedicine,5thed,FleisherGR,LudwigS,HenretigFM
(Eds),Lippincott,Williams&Wilkins,Philadelphia2006.p.1193.
Graphic76373Version5.0

Laboratoryevaluationforsuspectedinbornerrorsofmetabolism

Comments

Initialevaluation*
Bloodtests

CBCwith

differential
Blood

glucose
Electrolytes,
BUN,

creatinine,
uricacid
Arterial
bloodgas

Serum
ammonia

Shouldbeobtainedfromarteryorveinwithoutatourniquet.Thetubeshouldbeplacedonicefortransporttothe
laboratoryandanalyzedimmediately.Iftheplasmaammoniaconcentrationis>100micromol/L(1.7mcg/mL),the
measurementshouldberepeatedimmediately.

AST,ALT,

Ifthepatienthassignsorsymptomsofmyopathy.

bilirubin,PT
LDH,
aldolase,

creatine,
kinase
Urinetests

Color,odor

Urinalysis

Reducing
substances

Myoglobin

Ifthepatienthassignsorsymptomsofmyopathy.

Specializedtests

Bloodtests

Quantitative

Plasmaaminoacidanalysismustbeperformedquantitativelyratherthanqualitatively.

plasma
aminoacids
Lactateand
pyruvate

Lactateandpyruvateshouldbemeasuredinarterialbloodandtransportedonice.

Acylcarnitine
profile

Analysisofacylcarnitineconjugatesisperformedbytandemmassspectrometryandcanbemeasuredinaplasma
sampleorafilterpaperbloodspot.Serumispreferredbecauseofinherentproblemsinquantitatingcompounds
fromafilterpaperbloodspot

Urinetests

Qualitative
urine

Minimumof2to5mLinsterilecontainerwithoutpreservative.

organicacids
CBC:completebloodcountBUN:bloodureanitrogenAST:aspartateaminotransferaseALT:alanineaminotransferasePT:prothrombin
timeLDH:lactatedehydrogenase.
*Ifpossible,bloodandurinesamplesshouldbeobtainedforboththeinitialandspecializedtestsatthetimeofpresentation.Samplesfor
specializedtestsshouldbeprocessedandstoredappropriatelyforfurthertestingifindicated.
Graphic67745Version6.0

Uppergastrointestinalcontraststudies
demonstratingtheduodenalbulb

Intheimageontheleft,theduodenalbulbistoleftofthespine.Inthe
imageontheright,withmalrotation,theduodenalbulbisoverlying
spine.
ReproducedwithpermissionfromCarloBuonomo,MD,Children'sHospitalBoston.
CopyrightCarloBuonomo,MD.
Graphic77126Version2.0

Contrastenemademonstratingamediallydirected
cecum

Thiscontrastenemaillustratesthehigh,mediallydirectedcecum(see
arrow)thatcanbeseeninmalrotation.
ReproducedwithpermissionfromCarloBuonomo,MD,Children'sHospital
Boston.CopyrightCarloBuonomo,MD.
Graphic72551Version2.0

Volvuluscorkscrew

Thisuppergastrointestinalcontraststudydemonstratesthecorkscrew
appearanceofthetwistedsmallbowelinvolvulus.
ReproducedwithpermissionfromCarloBuonomo,MD,Children'sHospital
Boston.CopyrightCarloBuonomo,MD.
Graphic75419Version2.0

Pyloricstenosisonradiographandultrasound

Thisfourweekoldmaleinfantpresentedwithprojectilenonbiliousemesisandfailuretothrive.ImageAisasupineradiograph
andimageBalateraldecubitusradiographoftheabdomen,revealingadilatedstomachwithasingleairfluidlevel(arrowhead)
andperistalticwaves(arrows),whichconstitutethe"caterpillarsign"ofpyloricstenosis.ImageCisasonographicimageofthe
distalstomachandpylorusandshowsadilatedstomach(asterisk)withperistalticwaves(arrow)andpyloricwallthickening
(arrowheads).ImageDisasonographicimageoftheepigastriumandshowswallthickening(arrows)andlengthening.Thepyloric
musclethickness(PMT)measures5mm,andpyloricmusclelength(PML)measures18mm,consistentwithpyloricstenosis.

CourtesyofJosephFarnam,MD.
Graphic96412Version1.0

Pyloricstenosis

UpperGIofpyloricstenosis.Notethecharacteristic"applecore"or
"string"signcausedbythenarrowedlumenofthepylorus(arrow).
CourtesyofMaryLBrandt,MD.
Graphic69939Version3.0

Typicalphysicalexamination,chestradiography,andelectrocardiographyfindingsinsome
formsofcyanoticheartdisease
Physicalexam

Chestradiography

Diagnosis
S2

Murmur

Heartsize

Electrocardiogram

Percent
RAA

PBF

QRSaxis

Hypertrophy

Incidence
(per
100,000
live
births)*

TGA

single

none

90to150

nml

21

TOF

single

sys

boot

20

90to150

nml

20to26

HLHS

single

sys

vc

90to150

LVforces

16

PAIVS

single

sys

30to90

LVH,RAE

PS

single

sys

30to90

RVH,RAE

TAPVC

split

sys

,nl

,vc

90to150

RAE

Tricuspid

single

sys

30to90

LVH,RAE

single

sys/dias

30

90to150

nml

split

sys

90to150

RAE

atresia
Truncus
arteriosus
Ebstein's

NOTE:Thistablerepresentsthecommonpresentationofeachlesion.Variationsdooccur.Forexample,tricuspidatresiausually
isassociatedwithasmallventricularseptaldefectandpulmonarystenosissomepatientswiththisdiagnosiscanhavealarge
ventricularseptaldefect,nopulmonarystenosis,andincreasedpulmonarybloodflow.
dias:diastolicHLHS:hypoplasticleftheartsyndromeLV:leftventricularLVH:leftventricularhypertrophynl:normalnml:normalfor
neonate(rightventricularpredominance)PAIVS:pulmonaryatresiaintactventricularseptumPBF:pulmonarybloodflowPS:pulmonary
stenosisRAA:rightaorticarchRAE:rightatrialenlargementsys:systolicTAPVC:totalanomalouspulmonaryvenousconnectionTGAIVS:
dtranspositionofthegreatarteries,intactventricularseptumTOF:tetralogyofFallotvc:venouscongestion.
*IncidencefromreportofNewEnglandRegionalInfantCardiacProgram.Pediatrics198065:375.
PatientswithTAPVCassociatedwithpulmonaryvenousobservationhavenormalheartsizeandvenouscongestiononthechestradiography
whilethosewithoutobstructionusuallyhavecardiomegalyandincreasedpulmonarybloodflow.
Graphic79941Version9.0

EmpiricaltreatmentofsuspectedSBIinfebrileinfantslessthan90daysofage*
Age

Mostlikelyorganism

Empirictreatment

Neonate

Common:GroupBStreptococcus,E.coli

(28

Lesscommon:Listeriamonocytogenes,Enterococcus,S.aureus,other

days)

Gramnegativeorganisms,Herpessimplexvirus

footnotes)

Infant
(29to90

Common:S.pneumoniae,H.influenzae,N.meningiditis

Wellappearing,noCSFpleocytosis:

Lesscommon:GroupBStreptococcus,E.coli,S.aureus,Enterococcus,
Listeriamonocytogenes,Pseudomonassp.,otherGramnegative

CeftriaxoneORcefotaxime

days)

organisms

Ampicillin&cefotaximeORampicillin&

aminoglycoside &acyclovir, asindicated(see

CSFpleocytosisorillappearing:
Vancomycin&ampicillin&ceftriaxoneOR
cefotaxime

*Broadspectrumcoverageisprudentuntilanorganismisidentified.
ThechoiceofregimenshouldbebasedonlocalsusceptibilitypatternsofE.coliandlikelihoodofL.monocytogenesinfection.
Acyclovirisindicatedininfants28dayswithillappearance,mucocutaneousvesicles,seizures,orCSFpleocytosis.
ThisregimendoesnotincludeanaminoglycosideandmaynotoptimallycoverinfectionwithL.monocytogenesorresistantGramnegative

organisms,especiallywhenmeningitisispresent.Antibiotictherapyshouldbeadjustedaccordinglyifinfectionwiththesepathogensis
identified.
Graphic55679Version3.0

Pediatriccausesofmetabolicacidosis
Elevatedaniongap
Lacticacidosis
Hypoperfusion
Cardiacfailure
Hypovolemia
Septicshock

Mitochondrialdisorders
Inbornerrorofmetabolism
Mitochondrial,Encephalomyopathy,LacticAcidosisandStrokelikeEpisodes(MELAS)
MyoclonusEpilepsyandRaggedRedFibers(MERRF)
KearnsSayresyndrome
Acquired(antiretroviraltherapyinHIVpatients)

Inbornerrorsofmetabolism(carbohydratemetabolism)
Fructose1,6diphosphatasedeficiency
Glycogenstoragedisease1(glucose6phosphatedeficiency)
Pyruvatedehydrogenaseorcarboxylasedeficiency

Ketoacidosis
Diabeticketoacidosis
Inbornerrorsofmetabolism
Organicacidemia
Maplesyrupurinedisease(branchedchainaminoacids)
Propionicacidemia
Methylmalonicacidemia
Ketothiolasedeficiency
Multiplecarboxylasedeficiency(impairedbiotinutilization)

Fattyacidoxidationdefects

Ingestions
Cyanidepoisoning
Ethanolintoxication
Ethyleneglycol
Ironpoisoning
Metforminpoisoning
Methanol
Nonsteroidalantiinflammatorydrugpoisoning
Salicylates
Renalfailure
Massiverhabdomyolysis
Tumorlysissyndrome

Normalaniongap(hyperchloremicmetabolicacidosis)
LossofHCO3
Gastrointestinalloss
Diarrhea
Chroniclaxativeabuse
Entericfistulae
Ureterosigmoidostomy

Renalloss
Proximal(Type2)renaltubularacidosis

DiminishedH+secretion
Distal(Type1)renaltubularacidosis
Earlyrenalfailure
Graphic51117Version5.0

Disclosures
Disclosures:RichardJScarfone,MD,FAAPNothingtodisclose.ChristineCho,MD,MPH,MEdNothingtodisclose.GeorgeA
Woodward,MDNothingtodisclose.JanEDrutz,MDNothingtodisclose.JamesFWiley,II,MD,MPHNothingtodisclose.
Contributordisclosuresarereviewedforconflictsofinterestbytheeditorialgroup.Whenfound,theseareaddressedbyvettingthrougha
multilevelreviewprocess,andthroughrequirementsforreferencestobeprovidedtosupportthecontent.Appropriatelyreferencedcontentis
requiredofallauthorsandmustconformtoUpToDatestandardsofevidence.
Conflictofinterestpolicy