On demand treatment and home therapy of hereditary angioedema in Germany - the Frankfurt experience 英文参考文献.docVIP

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On demand treatment and home therapy of hereditary angioedema in Germany - the Frankfurt experience 英文参考文献.doc

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On demand treatment and home therapy of hereditary angioedema in Germany - the Frankfurt experience 英文参考文献

Ayg?ren-Pürsünetal.Allergy,AsthmaClinicalImmunology2010,6:21 /content/6/1/21 ALLERGY, ASTHMA CLINICAL IMMUNOLOGY RESEARCH OpenAccess Ondemandtreatmentandhometherapyof hereditaryangioedemainGermany-the Frankfurtexperience EmelAyg?ren-Pürsün*,InmaculadaMartinez-Saguer,EvaRusicke,ThomasKlingebiel,WolfhartKreuz Abstract Background:Manifestationofacuteedemainhereditaryangioedema(HAE)ischaracterizedbyinterindividualand intraindividualvariabilityinsymptomexpressionovertime.Flexibletherapyoptionsareneeded. Methods:WedescribeandreportontheoutcomesofthehighlyindividualizedapproachtoHAEtherapy practicedatourHAEcenterinFrankfurt(Germany). Results:TheHAEcenterattheFrankfurtUniversityHospitalcurrentlytreats450adultswithHAEorAAEand107 pediatricHAEpatientswithhighlyindividualizedtherapeuticapproaches.73.9%oftheadultpatientstreatHAE attacksbyon-demandtherapywithpasteurizedpdC1-INHconcentrate,9.8%useadditionalprophylaxiswith attenuatedandrogens,1%ofthetotalpatientpopulationinFrankfurthasbeentreatedwithIcatibantuptonow. Inadditionadultandselectedpediatricpatientswithahighfrequencyofsevereattacksareinstructedtoapply individualreplacementtherapy(IRT)withpasteurizedpdC1-INHconcentrate.ImprovementonQualityofLifeitems wasshownforthesepatientscomparedtopreviouslong-termdanazolprophylaxis.HometreatmentofHAE patientswasdevelopedintheFrankfurtHAEcenterinlinewithexperiencesinhemophiliatherapyandhassofar beenimplementedoveraperiodof28years.Atpresent248(55%)oftheadultpatientsand26(24%)ofthe pediatricpatientsarepracticinghometreatmenteitherasondemandorIRTtreatment. Conclusions:Inconclusion,theindividualizedhometherapiesprovidedbyourHAEcenter,aimtolimitthe disruptiontonormaldailyactivitiesthatoccursformanyHAEpatients.Furthermore,weseektooptimizethe economicburdenofthediseasewhileofferingamaximumqualityoflifetoourpatients. Introduction time. The onset of the next attack, its location and its Ondemand treatment ofacute angioedema inHAE type I severity are unpredictable. Treatment options adapted