title: Short-term prophylaxis in hereditary angioedema due to deficiency of the C1-inhibitor--a long-term survey.
abstract: BACKGROUND
  Hereditary angioedema is a potentially life-threatening disorder, because edema occurring in the mucosa of the upper airways can lead to suffocation. The management of HAE consists of avoiding the triggering factors, prophylaxis, and the acute treatment of edematous episodes. Medical procedures can also provoke edematous attacks, and therefore, short-term prophylaxis (STP) is recommended before such interventions. Our aim was to evaluate the efficacy and safety of STP administered before medical procedures.
  
  
  METHODS
  We conducted a retrospective analysis before and a prospective survey after establishing the diagnosis in a group of 137 (60 males, 77 females; 20 pediatric and 117 adult) patients with HAE. Both were implemented using questionnaires, patient diaries and hospital charts focusing on medical interventions provoking edematous attack, and the medicinal products (C1-INH concentrate, tranexamic acid, and danazol) administered for STP.
  
  
  RESULTS
  Comparing surgical interventions performed without pre-event STP (in 39/89 patients before HAE was diagnosed), or after STP (in 3/55 cases after diagnosis), we found a significant (P < 0.0001, Fisher's exact test) reduction in the number of edematous episodes. Evaluating the efficacy of the drugs administered for STP revealed that C1-INH concentrate (Berinert(®) , CSL Behring, Marburg, Germany) was significantly (P = 0.0096, Fisher's exact test) superior to orally administered drugs in reducing the instances of postprocedural edema. None of the medicinal products caused adverse events potentially related to STP.
  
  
  CONCLUSIONS
  STP reduces the number of postprocedural edematous episodes. C1-INH concentrate is safe and effective for prophylaxis. When this agent is not available, danazol is a potential alternative for prophylaxis before elective medical interventions.
authors.affiliation: 3rd Department of Internal Medicine, Semmelweis University, Budapest, Hungary.
authors.forename: H
authors.initials: H
authors.lastname: Farkas
authors.forename: Z
authors.initials: Z
authors.lastname: Zotter
authors.forename: D
authors.initials: D
authors.lastname: Csuka
authors.forename: E
authors.initials: E
authors.lastname: Szabó
authors.forename: Z
authors.initials: Z
authors.lastname: Nébenfűhrer
authors.forename: G
authors.initials: G
authors.lastname: Temesszentandrási
authors.forename: L
authors.initials: L
authors.lastname: Jakab
authors.forename: L
authors.initials: L
authors.lastname: Varga
authors.forename: G
authors.initials: G
authors.lastname: Harmat
authors.forename: I
authors.initials: I
authors.lastname: Karádi
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D050718:Complement C1 Inhibitor Protein / Q000008:administration & dosage* / Q000009:adverse effects; D003613:Danazol / Q000008:administration & dosage* / Q000009:adverse effects; D005260:Female; D005500:Follow-Up Studies; D056829:Hereditary Angioedema Types I and II / Q000175:diagnosis / Q000517:prevention & control*; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D014148:Tranexamic Acid / Q000008:administration & dosage* / Q000009:adverse effects; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D050718:Complement C1 Inhibitor Protein; D014148:Tranexamic Acid; D003613:Danazol