Pediatric hereditary angioedema due to C1-inhibitor deficiency 英文参考文献.docVIP

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Pediatric hereditary angioedema due to C1-inhibitor deficiency 英文参考文献.doc

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Pediatric hereditary angioedema due to C1-inhibitor deficiency 英文参考文献

FarkasAllergy,AsthmaClinicalImmunology2010,6:18 /content/6/1/18 ALLERGY, ASTHMA CLINICAL IMMUNOLOGY REVIEW OpenAccess Pediatrichereditaryangioedemadueto C1-inhibitordeficiency HenrietteFarkas Abstract Hereditaryangioedema(HAE)resultingfromthedeficiencyoftheC1inhibitor(C1-INH)isarare,life-threatening disorder.Itischaracterizedbyattacksofangioedemainvolvingtheskinand/orthemucosaoftheupperairways, aswellastheintestinalmucosa.Inapproximately50percentofcases,clinicalmanifestationsmayappearduring childhood.ThecomplexmanagementofHAEinpediatricpatientsisinmanyrespectsdifferentfromthemanage- mentofadults.Establishingthediagnosisearly,preferablybeforetheonsetofclinicalsymptoms,isessentialin caseswithapositivefamilyhistory.Complementstudiesusuallyaffordaccuratediagnosis,whereasmolecular geneticstestsmayprovehelpfulinuncertaincases.Appropriatetherapy,supportedbycounselling,suitablemodifi- cationoflifestyle,andavoidanceoftriggeringfactors(whichprimarilyincludemechanicaltrauma,mentalstress andairwayinfectionsinchildren)maysparethepatientunnecessarysurgeryandmaypreventmortality.Prompt controlofedematousattacks,short-termprophylaxisandintermittenttherapyarerecommendedastheprimary meansforthemanagementofpediatriccases.Medicinalproductscurrentlyusedforthetreatmentofchildren withhereditaryangioedemaincludeantifibrinolytics,attenuatedandrogens,andC1-INHreplacementtherapy.Cur- rentguidelinesfavourantifibrinolyticsforlong-termprophylaxisbecauseoftheirfavorablesafetyprofilebuteffi- cacymaybelacking.Attenuatedandrogensadministeredinthelowesteffectivedoseareanotheroption.C1-INH replacementtherapyisalsoaneffectiveandsafeagentforchildren.Regularmonitoringandfollow-upofpatients arenecessary. 1.Introduction (antifibrinolytics, attenuated androgens, and C1-INH ThedeficiencyoftheC1inhibitor(C1-INH)isinherited concentrate)hasnotchangedfordecades.Theprophy- as an autosomal dominant trait. It causes hereditary lacticuseofplasma-derivedC1-INH(pdC1-INH),how- angioedema (HAE-C1-INH), which i