Patients’ Characteristics
The study cohort comprised 66 HAE patients (39 Femles, 27 Males). As expected from an autosomal inherited disorder, high prevalence was found among parents and siblings (74%, 60%, respectively). Half of the adults live with a partner and approximately half are parents. Mean age of onset of HAE was 9.8 years and mean age of diagnosis was 14.5 years (4.7 years diagnosis gap) (Table II).
In this study we received 223 reports on prodromes and /or attacks from the of the 66 patient cohort (attacks and its locations are presented in Figure 1). All the patients experienced attacks during the study period and 94% had their prodromes in the abdominal cluster. A considerable number (81.8%) had prodromes and attacks involving the extremities. The distribution of both events is depicted in Figure 2. More than 5th of the patients (22.7%) reported previous hospital admissions, most of them for facial involvement (45.2%), mouth-tongue-upper-airway involvement (12.2%), abdominal attacks (7.6%), and genital attacks (4.5%) (Presented in Table I). The vast majority (62/66, 92%) claimed that they experienced a prodromes before at least one of their attacks, and two-thirds (64%) affirmed that they can predict an oncoming attack by having a prodrome.