Profile of blood cells and inflammatory mediators in periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome 英文参考文献.docVIP

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Profile of blood cells and inflammatory mediators in periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome 英文参考文献.doc

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Profile of blood cells and inflammatory mediators in periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome 英文参考文献

Brownetal.BMCPediatrics2010,10:65 /1471-2431/10/65 RESEARCH ARTICLE OpenAccess Profileofbloodcellsandinflammatorymediators inperiodicfever,aphthousstomatitis,pharyngitis andadenitis(PFAPA)syndrome KellyLBrown1*?,PerWekell2,3*?,VeronicaOsla1,MartinaSundqvist1,KarinS?vman2,4,AndersFasth2,4, AnnaKarlsson1,StefanBerg2,4 Abstract Background:Thisstudyaimedtoprofilelevelsofbloodcellsandserumcytokinesduringafebrileandfebrile phasesofperiodicfever,aphthousstomatitis,pharyngitisandadenitis(PFAPA)syndrometoadvance pathophysiologicalunderstandingofthispediatricdisease. Methods:Acohortofpatientswithamedianageof4.9yearsexperiencing‘typicalPFAPA’episodesparticipated inthisstudy.BloodcellsandserumcytokineswereanalyzedbyCBCanalysisandmultiplexELISA. Results:OscillationsintheconcentrationofbloodcellsduringtheafebrileandfebrilephasesoftypicalPFAPA syndromewereobserved;novelfindingsincludeincreasedmonocytesanddecreasedeosinophilsduringafebrile episodeandincreasedthrombocytesintheafebrileinterval.Relativelymodestlevelsofpro-inflammatorycytokines werepresentinsera.IFNg-inducedcytokineIP10/CXCL10wasincreasedaftertheonsetoffeverwhileTcell- associatedcytokinesIL7andIL17weresuppressedduringafebrileandfebrileperiods. Conclusions:Identificationofdysregulatedbloodcellsandserumcytokinesisaninitialsteptowardsthe identificationofbiomarkersofPFAPAdiseaseand/orplayersindiseasepathogenesis.Futureinvestigationsare requiredtoconclusivelydiscernwhichmediatorsareassociatedspecificallywithPFAPAsyndrome. Background intheEasternMediterraneanbasinwhereFMFismore Theperiodicfever,aphthousstomatitis,pharyngitisand common. Every pediatrician is likely to encounter at cervical adenitis (PFAPA) syndrome was originally least one case of PFAPA during his or her career [4]. described by Marshall et al in 1987 and the acronym, Autoinflammatory attacks in PFAPA syndrome occur PFAPA, was coined two years later together with the within a 2-8 week interval with remarkable clockwork diagnosticcriteria[1,2].PFAP