Cardiac Manifestations from Non-FIP1L1-PDGFR-Associated Hypereosinophilic Syndrome in a 13-Year-Old African American Boy英文文献资料.docVIP

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Cardiac Manifestations from Non-FIP1L1-PDGFR-Associated Hypereosinophilic Syndrome in a 13-Year-Old African American Boy英文文献资料.doc

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Cardiac Manifestations from Non-FIP1L1-PDGFR-Associated Hypereosinophilic Syndrome in a 13-Year-Old African American Boy英文文献资料

HindawiPublishingCorporation JournalofAllergy Volume2009,ArticleID804910,5pages doi:10.1155/2009/804910 CaseReport CardiacManifestationsfromNon-FIP1L1-PDGFRα- AssociatedHypereosinophilicSyndromeina13-Year-Old AfricanAmericanBoy CindyM.Salm, NicoleE.St.Clair, JamesV.Lustig, andMargaretM.Samyn 1 2 3 4 1 2 3 DepartmentofMedicine,AuroraHealthCare,Milwaukee,WI53213,USA DepartmentofPediatrics,Children’sHospitalofWisconsin,Milwaukee,WI,USA DepartmentofPediatrics,Allergy,Asthma,andClinicalImmunology,Children’sHospitalofWisconsin, Milwaukee,WI,USA 4 DepartmentofPediatrics,Cardiology,HermaHeartCenter,Children’sHospitalofWisconsin, Milwaukee,WI,USA CorrespondenceshouldbeaddressedtoCindyM.Salm,cindy.salm@ Received12March2009;Revised2September2009;Accepted8October2009 RecommendedbyGarryWalsh Hypereosinophilicsyndrome(HES)isararedisordertypicallyseeninmales,aged20–50,withapredispositionforCaucasians. It is marked by overproduction of eosinophils (1,500/μL) and multi-organ system damage due to eosinophilic in?ltration andmediatorrelease.TherearemultiplevariantsofHES.CardiaccomplicationsaremorecommoninmyeloproliferativeHES associatedwiththeFIP1L1-PDGFRαmutation.Sequelaerangefromacutenecrosisandthrombusformationto?brosisofthe endomyocardium.Wedescribeayoungboywhopresentedwithchestpainanddyspnea.AdiagnosisofHESwasmadeafter allotheretiologiesofeosinophiliawereexcluded.AlthoughhewasfoundtobenegativefortheFIP1L1-PDGFRαmutation,his cardiac complications included pericardial e?usion and restrictive cardiomyopathy, without myocardial necrosis. Multi-organ involvementresultedinpericarditis,pleuritis,nephritis,anddermatitis.Inthisarticle,wereviewhiscaseanddiscusstheknown subtypesofHES,theclassiccardiaccomplications,andavailabletreatmentstrategies. Copyright?2009CindyM.Salmetal.ThisisanopenaccessarticledistributedundertheCreativeCommonsAttributionLicense, whichpermitsunrestricteduse,distribution,andreproductioninanymedium,provided