In a subanalysis (data not shown), the differences in attack frequency did not appear to be accountable to differences in prescribing of attenuated androgens. When attack frequency at the main three sites was compared for types I and II HAE, no differences were observed. The variation in attack frequency ranged

from 30% of patients who had had no attacks during the year to others with daily attacks. Information on the employment Selleckchem SCH772984 status of 213 patients shows that 76% were in employment (full- or part-time), were homemakers or students. Seven percent were unemployed and the remainder retired. The percentage in paid employment (full- and part-time) was 48% (Fig. 7). Information Selleckchem Tyrosine Kinase Inhibitor Library on days lost from work/school or where activities of daily living could not be performed was available on 116 patients, with an annual mean of 9 days per patient [standard deviation (s.d.) 24]; however, this is almost certainly an underestimate, as it was not

possible to analyse non-numerical data (e.g. yes: +++, plenty, very few, etc.) in 11 patients. The impact of HAE on quality of life was assessed by asking patients to rate the overall impact of their disease on their quality of life as either none, mild, moderate or severe. Information was available on 223 patients and the impact was noted to be moderate or severe in 37% of adult patients (Fig. 8a). While this approach

is Glycogen branching enzyme straightforward, detail and sensitivity is likely to be improved significantly using a validated disease-specific quality-of-life tool for HAE [22]. Swellings are generally rare before the age of 2 years and are relatively uncommon before adolescence, with a mean age of onset of swellings of 8–12 years [23]. The reported impact on quality of life in children available in 29 patients was rated as moderate in 14%, with no patients in the severe category. The annual frequency of swellings in children was peripheral, six; abdominal, six; and airway, 0·2 (Fig. 8b). Interpretation of attack frequency and impact upon quality of life in children is complicated by the fact that this information is reported by the parents. Furthermore, as there may be an increase in swellings during adolescence, consideration of childhood as less than 16 years of age may not capture important information. The questions on family history included one on deaths in the family related directly to an HAE attack. When multiple entries for the same family members (and two entries stating ‘uncertain’ or ‘possible’) were excluded there was a total of 55 deaths in 33 families, ranging from one to three deaths per family. This clearly underpins the lethal potential of this condition.