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The inclusion criteria comprised the presence of the known types (I or II) of HAE-C1-INH confirmed by complement studies. The prospective study started once the diagnosis of HAE-C1-INH had been established. Upon diagnosis, the patients were warned of Regorafenib supplier the potential attack-triggering effect of the medical procedures mentioned above. Then, STP was recommended as a preventive measure, to be implemented after consultation with the follow-up center. The patients were asked to record in their diaries the following: the type of the intervention, the method of STP, as well as the dose and effect of the drug administered, the postoperative course, and the occurrence of edematous episodes potentially related to the intervention. We considered edema formation related to a medical intervention, if it occurred within 48?h after the procedure. At the annual follow-up visits, selleck compound the subjects underwent a laboratory screen (virus serology, such as hepatitis B and C, HIV, parvovirus B19) and the measurement of anti-C1-INH antibody titers. Our study was approved by the Institutional Review Board of Semmelweis University, and all patients gave informed consent. STP was administered using the following medicinal products before surgical or diagnostic procedures contemplated in the head and neck region (including dental procedures), and other types of major surgery: Danazol (Danoval; KRKA, Novo Mesto, Slovenia): 2.5�C10?mg/kg/day orally (maximum daily dose: 600?mg), initiated 5?days before and continued for additional 2?days after surgery. Tranexamic acid (TXA, Exacyl, Sanofi-Synthelabo; Produtos Farmaceuticos, SA, Porto Salvo, Portugal): 20�C40?mg/kg/day orally (maximum daily dose: 3?g in two to three divided doses), started 5?days before and continued for additional 2?days after the intervention. Human plasma-derived C1-INH concentrate (Berinert?; CSL Behring, Marburg, Germany): 500?IU i.v. 1?h before the procedure. During the intervention, an additional ALK vial of this medicinal product was kept at hand for use if an emergency occurs. The calculations were performed with the Prism 5 package (GraphPad Software, San Diego CA, USA). Fisher's exact test was used to compare the occurrence of edematous episodes with or without STP, whereas chi-square test was used to compare the efficacy of the three types of medicinal products used for STP. In all statistical analyses, P?