Atopic dermatitis and skin diseaseFatal laryngeal attacks and mortality in hereditary angioedema due to C1-INH deficiency
Section snippets
Methods
Our partly retrospective and partly prospective study analyzed death cases among patients with HAE-C1-INH. To determine mortality, a series of 182 unrelated families with HAE-C1-INH registered at our Angioedema Outpatients Service (AOS) of the University of Mainz was studied. These 182 families included 728 patients with HAE-C1-INH, identified from their family history and corresponding family pedigrees.
On diagnosis of HAE-C1-INH, the family pedigree was constructed on the basis of the
Estimation of mortality based on total number of deaths
A total of 182 families with HAE-C1-INH were analyzed (Table I). According to the pedigrees and the criteria mentioned earlier, 728 patients with HAE-C1-INH could be identified. At the time of evaluation (October 2011), 514 of these patients were alive and 214 patients had died. Seventy of these 214 patients (32.7%) had died of asphyxiation due to an LA (Fig 1). In 63 patients (36 men and 27 women) who died of asphyxiation (29.4%; 95% CI, 23%-36%), the death occurred before HAE-C1-INH had been
Discussion
The results of our study showed that in all patients whose death could be attributable to HAE-C1-INH, the death occurred because of asphyxiation. With some caution, it could be concluded that asphyxiation is the exclusive cause of death when specifically attributable to HAE-C1-INH.
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Disclosure of potential conflict of interest: K. Bork has received consultancy fees from CSL Behring, Shire, and Viropharma. J. Hardt has received research support from Stiftung Innovation. G. Witzke declares that he has no relevant conflicts of interest.