angioedema

October 6, 2014  |  By  | 


triggerfactorsmightinfluencethisprocessatitsdifferent stages.Theroleoftriggerfactorsinthepathophysiologyof edemahasnotyetbeenclarified. Althoughmanyofthesefactorsareubiquitousin everydaylife,HAE-C1-INHpatientsdonotexperience anattackeveryday.Theobjectiveofourstudywasto appraisetheincidenceofvarioustriggerfactors,along withtheireffectonedematousattacks. Methods StageIofthestudy Duringtheinitialphaseofthestudyfrom2004to2010, weperformeddiagnosticevaluation(familyhistory,symp- toms,genetictesting,andcomplementstudies)of140 HAE-C1-INHpatientsattheHungarianHAECenter. Oursurveyreviewedtheentriesmadebythesubjectsin theirstandardizedPatientDiariesover7years.Attheini- tialvisit,patientsreceivedindividualizedinformationon thenatureofthediseaseanditspossiblemanifestations. Thelocations,severity,triggerfactors,andtreatmentof HAEattackswerealsodiscussed.Everyyear,eachpatient receivedastandardizedPatientDiary.Thelatterwas intendedforrecordingthedateofonset,severity,location, andpotentialtriggerfactorsoftheattacksaccordingto standardcriteria,alongwiththetreatmentreceived.The PatientDiarieswerereviewedattheannualfollow-up visits(includingevaluationoftherelevanceandregularity theentries).Attheendofeachyear,thepatientsreturned theirdiariesbymailorinperson.Overall,wecannottell howaccuratetheserecordsareasfarasthepropertiesof theeventsareconcerned.Thepatientsaffirmedthatthey hadmadeeffortstorecordeveryattackinthediary. Nevertheless,ascertainingtheoccurrenceofanattackwas feasibleonlyinthecaseofthosethatrequiredtreatment withC1-INHconcentrate.Thismedicinalproductwasad- ministeredbymedicalprofessionalswhodocumented theircasesandfurthermore,theusageofthisproduct ismonitoredbytheNationalHealthInsuranceFundsAd- ministration.Wefoundan88-per-centagreementbe- tweentheentriesofthepatientdiariesandofthemedical records,regardingtreatmentwithC1-INHconcentrate. Analyzingthesedata,weappraisedthecharacteristics (e.g.location,triggerfactors)oftheattacks,aswellas theirseasonalandmonthlydistribution. StageIIofthestudy Inthesecond,prospectivestage,westudied27patients. Thesesubjectsrecordedtheoccurrenceofpossibletrig- gerfactorseverydayforsevenmonths – whetherornot theyhadexperiencedanattack.Inparticular,thesepa- tientsmadeanoteeverydayoftheoccurrenceofevents empiricallyassociatedwithanincreasedriskofedema formation.Thesubjectshadreceivedalistofpossible triggerfactorsalongwiththepatientdiary,whichthey keptfromJulyuntilDecember2011.Thus,patientdata from7monthswereanalyzed.Inthesecondphaseof thestudy,patientswerecontactedmonthlybytelephone tosupporttheircompliancewiththeinstructionson keepingthePatientDiary. Statisticalmethods StatisticalanalyseswereperformedwiththeGraphPad Prism4.0program(GraphPadSoftwareInc,SanDiego, CA,www.graphpad.com).WeusedtheChi-squaretestto analyzedifferencesbetweenthedistributionoftriggerfac- torsandofedemalocations.Toexploreannualdistribu- tion,wesummarizedthenumberofattacksoccurring duringthefourtemperate-climateseasons.Furthermore, wesummarizedthemonthlynumbersoftheattacksre- cordedeachyear,from2004to2010.Aschangesinpa- tientcompliancemayintroducedifferences,wecalculated weightedaverages.Fromthese,wedeterminedthemean annualnumberofattacksandthen,identifiedthemonths withahigherweightedaverage.Next,weidentifiedthe monthswithahigherthanaveragenumberofattacksdur- ingtheseven-yearlongphaseIofthestudy.Allhypoth- esesweretestedagainsttwo-directionalalternativesand p<0.05wasconsideredsignificant. Results StageIofthestudy Ofthe140HAE-C1-INHpatients,sixremained symptom-freeuntiltheendofthestudy.Ofthe134sub- jectswithsymptoms,92(56femalesand36males,mean age38.35[range:12to48]years)maderecordssuitable foranalysistoidentifytriggerfactors. Duringtheseven-yearlongobservationperiod,these 92patientsrecordedinformationon3176episodes. Sixty-fivepercent(2187/3176)oftheseweresubcutane- ous,and35%(1151/3176)weresubmucosal(937ab- dominaland214upperairway)attacks.Seventy-oneper cent(2256/3176)oftheattacksoccurredinfemalepa- tients.In162instances,edemaformationaffectedmultiple locations – thatis,involvedsubcutaneousandsubmucosal tissuesconcomitantly.Eightofthe92patientscouldnot detectanypotentialtriggerintheirlives,whereas84sus- pectedatleastonesuchfactor. Thetriggerfactorsidentifiedbythepatients Thesubjectsidentifiedseveraltriggerfactors(Table1), mostcommonlyphysicalexertion(66subjects),followed bymentalstress,andmechanicaltrauma(55 – 55patients). Triggerfactorsandtotalattacknumber Thesubjectscouldidentifyatriggerfactorin30%(953/ 3176)ofallrecordedattacks,andin23.64%ofsubcuta- neous,38.13%ofabdominal,and28.50%ofupperairway episodes.Thefollowingtriggerfactorswereidentified: Zotter etal.OrphanetJournalofRareDiseases 2014, 9 :44Page2of6 http://www.ojrd.com/content/9/1/44

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