RevPaulPediatr.2016;34(4):395---396
REVISTA
PAULISTA
DE
PEDIATRIA
www.rpped.com.br
EDITORIAL
In
time:
Eosinophilic
esophagitis:
when
to
suspect
it
and
how
to
diagnose
it
in
children
and
adolescents
Em
tempo:
Esofagite
eosinofílica:
quando
suspeitar
e
como
diagnosticá-la
em
crianc
¸as
e
adolescentes
Mirna
Chehade
MountSinaiCenterforEosinophilicDisorders,JaffeFoodAllergyInstitute,IcahnSchoolofMedicineatMountSinai,NewYork, USA
Received23November2015
Prevalence
and
demographics
Eosinophilicesophagitis(EoE)isachronicimmune, antigen-mediated, disease of the esophagus characterized by symptomsrelatedtoesophagealdysfunctionandsignificant esophagealeosinophilicinfiltration.1EoEhasbeendescribed
inmanyplacesthroughouttheWorld,includingNorth Amer-ica,Europe,SouthAmerica,Australia,Asia,andtheMiddle East. There are no reported EoE cohorts in sub-Saharan Africaor India.2Multiplereportshaveoriginated inBrazil,
includingSãoPaulo,ofchildrenwithEoE.3---5Theprevalence
ofEoEhasbeensteadilyincreasing,2therefore,itis
impor-tant for pediatricians and pediatric specialists of various disciplinestobefamiliarwiththediseasepresentation,so thatdiagnosiscanbemadeinatimelymanner,andoptimal carecanbeprovided.
EoEis morecommoninboys,witha male:femaleratio of 3:1, and can present at any age in children, including ininfancy.6Familial clusteringhasbeen reportedin EoE,7
and wasfound to be due in a largerpart to shared fam-ily environment thanto genetics,the latter beingcaused bya complexratherthanMendelian inheritance.8A
num-berofearlylifeexposuressuchasantibioticuseininfancy, cesareandelivery,pretermbirth,andformula-onlyormixed (infantformulaandbreastmilk)feedingwerethoughttobe
E-mail:mirna.chehade@mssm.edu
potentiallyassociatedwiththedevelopmentofEoEinthe pediatricpopulation.9
50---70% of children with EoE have concomitant atopic diseases,includingasthma, allergicrhinoconjunctivitis,or atopicdermatitis.In addition,alarge numberof children withEoEhavecurrentorpasthistoryoffoodallergy.1
Fam-ilyhistoryofatopyispresentinalargenumberofchildren withEoE.10
Clinical
presentation
Children with EoE present with a variety of symptoms, dependingontheir ageand thedurationof theirdisease. Symptomsincludeabdominalpain,gastroesophagealreflux (GER) symptoms including nausea and emesis, solid food dysphagia,andesophagealfoodimpactions.10
Afewchallengescanfacetheclinicianinthisarea.The firstoneisthatchildrenwithEoEpresentattimeswith infre-quent or non-specific symptoms, therefore not perceived asalarming tothefamiliesor the clinician.While adoles-centsandolderchildrenmostlyreportdysphagiaandfood impactions,youngerchildrenandpatientswithshorter dura-tionofsymptomsaremorelikelytopresentwithabdominal pain,GER symptoms, and occasional emesis.11 Discerning
EoEfromacid-inducedGERdiseaseinthesepatientsby his-tory alone can bedifficult. Inquiring for other associated symptomssuchasearlysatiety,andassessingforthe pres-enceoffailuretothrivecanbeveryhelpful,asthesepoints tothepossibilityofEoE.Infact,failuretothrivecanoccur
http://dx.doi.org/10.1016/j.rppede.2016.07.001
396 ChehadeM
inup toa third of children withEoE,10 and is potentially
reversiblefollowingdiseaseremission.
Asecondchallengefacedbytheclinicianis that symp-toms can be subtle in nature, given that the disease is chronic and its symptoms evolve over time. Therefore, children with EoE learn to compensate through behav-ioral modifications in feeding patterns to prevent major symptoms such as emesis, dysphagia or esophageal food impactions. These behaviors include avoidance of large meals,avoidanceoffoodsthathavehardorlumpytextures suchasmeatsandbreads,prolongedchewing,cuttingfood intosmallerpieces,lubricatingfoodbiteswithcondiments, anddrinkingwithmostbitesoffood.1Thisemphasizesthe
importanceof obtainingadetailedhistoryfromboth chil-drenandadolescentswithsuspectedEoEandtheirfamilies topreventadelayindiagnosis.
Diagnosis
The diagnosis of EoE requires performing an upper endoscopywithmultiplebiopsiesoftheesophagealmucosa aswellasother partsof the gastrointestinaltract.Visual inspectionoftheesophagealmucosacanrevealoneormore findings,12includingfurrows,whiteplaquesandlossof
vas-cularpattern,allcommoninthepediatricpopulation.While thecauseoffurrows isunclear, whiteplaquesareformed byaggregatesofeosinophils closesttotheluminalsurface associatedwithsomesloughingofthesuperficialepithelial cells.13Inaddition,esophagealrings,strictures,narrowing,
or even shearing can be present in more severe cases. A combination of featuresis often present. Inup to20% of children withEoE, theesophagus may appearcompletely normal,highlightingtheimportanceofobtainingbiopsiesat alltimeswheneverEoEisclinicallysuspected.14
SinceEoEisapatchydisease,multipleesophageal biop-sies areneeded fromvarious locations of the esophageal mucosa, especially from lesional areas such as white plaques. Esophageal biopsies demonstrating at least 15 eosinophils perhigh power fieldinthe mostdensely infil-tratedareaupon microscopic examination ofhematoxylin andeosin-stainedsectionsareconsidereddiagnostic,inthe absenceofincreasedeosinophiliain theremainder ofthe gastrointestinaltract.1
Since acid-induced GER disease can also result in esophagealeosinophilicinfiltration,thoughmild, this pos-sibility needs to be ruled out. In addition, the entity of protonpump inhibitor-responsiveesophagealeosinophilia, currentlyconsideredaseparateentityuntilits pathogene-sisiselucidated,needstoberuledoutbeforeestablishing the diagnosis of EoE. Therefore,an empirictherapy with aproton pump inhibitor at adose of2mg/kg/day in chil-dren,uptoamaximumof20---40mgonceortwicedailyin adolescents,isrecommended.Esophagealbiopsies demon-stratingsignificantesophagealeosinophiliadespiteatleast 8---12weeks of this therapy are considered diagnostic for EoE.1
Conclusion
In conclusion, EoE is an increasingly prevalent disease in the pediatric population. Since symptoms can be subtle,
non-specific or infrequent, obtaining a thorough history focusingonalargenumberofsymptomsincludingfeeding historyandpatterns,recordingpersonalandfamilyhistory ofatopyandEoE,andassessinggrowthareimportant.These can cue thepediatrician tothe disease,and allow timely referralforfurtherwork-upandmanagement.
Funding
Thisstudydidnotreceivefunding.
Conflicts
of
interest
Theauthordeclaresnoconflictsofinterest.
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