Andersons pediatric cardiology 1393
Bạn đang xem bản rút gọn của tài liệu. Xem và tải ngay bản đầy đủ của tài liệu tại đây (160.98 KB, 3 trang )
FIG.53.3 EvaluationofsuspectedincompleteKawasakidisease.(1)In
theabsenceofagoldstandardfordiagnosis,thisalgorithmcannotbe
evidencebasedbutitrepresentstheinformedopinionoftheexpert
committee.Consultationwithanexpertshouldbesoughtanytime
assistanceisneeded.(2)ClinicalfindingsofKawasakidiseasearelistedin
Table53.3.Characteristicssuggestingthatanotherdiagnosisshouldbe
consideredincludeexudativeconjunctivitis,exudativepharyngitis,
ulcerativeintraorallesions,bullousorvesicularrash,generalized
adenopathy,orsplenomegaly.(3)Infantsupto6monthsofagearethe
mostlikelytodevelopprolongedfeverwithoutotherclinicalcriteriafor
Kawasakidisease;theseinfantsareatparticularlyhighriskfordeveloping
coronaryarteryabnormalities.(4)Echocardiographyisconsideredpositive
forpurposesofthisalgorithmifanyofthreeconditionsaremet:z-scoreof
leftanteriordescendingcoronaryarteryorrightcoronaryartery≥2.5;
coronaryarteryaneurysmisobserved;or≥3othersuggestivefeatures
exist,includingdecreasedleftventricularfunction,mitralregurgitation,
pericardialeffusion,orz-scoresinleftanteriordescendingcoronaryartery
orrightcoronaryarteryof2to2.5.(5)Iftheechocardiogramispositive,
treatmentshouldbegivenwithin10daysoffeveronsetorafterthetenth
dayoffeverinthepresenceofclinicalandlaboratorysigns(C-reactive
protein[CRP],erythrocytesedimentationrate[ESR])ofongoing
inflammation.(6)Typicalpeelingbeginsunderthenailbedsoffingersand
toes.ALT,Alaninetransaminase;WBC,whitebloodcells.(FromMcCrindle
BW,RowleyAH,NewburgerJW,etal.Diagnosis,treatmentandlong-term
managementofKawasakidisease:ascientificstatementforhealth
professionalsfromtheAmericanHeartAssociation.Circulation.
2017;135[17]:e927–e999.)
Laboratoryfeaturesarereflectiveofanacuteinflammatoryresponse.
Althoughnonspecific,theyprovidesupportfordiagnosisinpatientswitha
nonclassicbutsuggestivepresentation.Virtuallyallpatientsatpresentationhave
elevationoftheerythrocytesedimentationrateorC-reactiveprotein,whichcan,
however,bediscrepant.58,59Theaveragelevelofhemoglobinatthetimeof
presentationistwostandarddeviationsbelowthemeanforage,withtheanemia
beingnormocyticandnormochromic.Thenumberofwhitebloodcellsis
generallyincreased,atamedianof15,000/mm58withaleftwardshift.The
plateletcountisusuallynormalinthefirstweekoftheillness,peakinginthe
thirdweekoftheillnesstovaluessometimeshigherthan1,000,000/mm.58
Thrombocytopeniaisrarebutmayoccurduringtheacutephaseinasubsetof
patientswithmoreseveredisease.Plasmaγ-glutamyltranspeptidase,
transaminases,andbilirubinarefrequentlyelevated.60,61Synthesisofalbuminis
decreasedintheacutephase,andhypoalbuminemiaiscommon.Microscopic
evaluationoftheurinemayrevealanelevatedcountofwhitebloodcellswithno
identifiedinfectiousagent,so-calledsterilepyuria.62Cerebrospinalfluid
containsanincreasednumberofwhitebloodcells,predominantlymononuclear
cells,withnormallevelsofglucoseandprotein.63
Nobiomarkershavebeendemonstratedsuperiortoclinicalcriteriaand
inflammatorymarkers.N-terminalmoietyofB-typenatriureticpeptide(NTproBNP),likelyindicativeofmyocardialinvolvement,hasshowngood
specificityinidentifyingpatientswithKawasakidiseaseversusotherfebrile
illnessesbutdoesnothavesufficientsensitivityanddiscriminativeability.64,65
Moreover,cutoffvaluesforapositiveresulthavenotbeenclearlydefined.
CardiacFindings
Cardiovascularmanifestationsandcomplicationsarethemajorcauseof
morbidityandmortalityinKawasakidisease,bothintheacutephaseandon
long-termfollow-up.
Theacutephaseofthediseasemaybeassociatedwithinflammationofthe
myocardium,pericardium,orendocardiumincludingvalvesandcoronary
arteries.Cardiacauscultationtypicallyrevealsahyperdynamicprecordium,or
tachycardia.Almostallchildrenhaveaninnocentflowmurmurrelatedto
anemiaandfever.Murmursofvalvardysfunctioncanbeheardinapproximately
25%ofpatients,mostcommonlyapansystolicregurgitantmurmurofmitral
regurgitation.66Agalloprhythmcanalsosometimesbeheard,suggesting
decreasedcomplianceanddiastolicdysfunctionsecondarytomyocardial
inflammation.Thediseasemayoccasionallypresentwithlowcardiacoutput
syndromeorshock.67
Electrocardiographymayshowarrhythmia,aprolongedPRinterval,or
nonspecificST-andT-wavechanges.68IncreasedQTdispersion,abnormalities
ofventricularrepolarization,andelectrocardiographicsignssuggestiveofleft
ventriculardilationandmyocardialinvolvementhavealsobeendescribed.69,70
Basedonmyocardialbiopsiesduringtheacutestage,myocarditisisanearly
universalfeatureofKawasakidisease.71Evidenceofmyocardialinflammationis
presentin50%to75%ofpatientsonnuclearimaging.72,73Onlyasubsetof
patientspresentswithovertventriculardysfunction(25%to50%).72–74
Fortunately,myocardialfunctionusuallyimprovesrapidlyafteradministrationof
intravenousimmunoglobulin(IVIG),andlong-termabnormalitiesofsystolic
functionareuncommonintheabsenceofischemicheartdiseasesecondaryto
coronaryarteryaneurysms.75
Intheacutephase,mitralregurgitationisthemostcommonvalvedysfunction;
itmayresultfromvalvitisorfromtransientdysfunctionofthepapillary
muscles.66Approximatelyone-quarterofchildrenhavemitralregurgitationat
thetimeofpresentation.66Latemitralregurgitationisusuallytheresultof
ischemicdisease.Aorticregurgitationismuchlessfrequentandfoundinless
than1%ofpatients.Itmaybeassociatedwithaorticrootdilation,whichoccurs
in8%ofpatients.
Coronaryarteryabnormalitiesduringtheacuteillnessrangefromdilationto
aneurysm.Theprevalenceofcoronaryarteryaneurysmsmaybeashighas25%
whenuntreated,anddecreasesto4%withadministrationofIVIGinthefirst10
daysofillness.76,77
Themostfrequentcoronaryanomalyisdilation,whichisdefinedasa
coronaryarteryz-scorebetween2and2.5.Somepatientswillhavecoronary
arteryz-scoresalwayswithinthenormalrange(below2)butwithasignificant
reductioninluminaldimensionsuponfollow-up.78,79Itisstillunclearifthese
changes,whichoccurin30%to50%ofpatients,representresolutionof
inflammatorychangesorhemodynamicfactorsrelatedtofever.
Theidentificationofpatientswithmildcoronaryarterydilationintheacute
phasecanbeconfoundedbythefactthatnormalmeasurementsarebasedona
populationofhealthyafebrilechildren.Coronaryarteryenlargementhasbeen
