CSL Behring Monograph Berinert - page 20

19
However, the most common sites are the extremities, face,
and GI tract. Single-site episodes occur most frequently,
but migratory, simultaneous, or closely spaced attacks can
also occur. Moreover, abdominal involvement can arise
concurrently with cutaneous edema.
5,21
Bork et al collected data from 221 HAE patients with
131,110 episodes of angioedema. As shown in Table 1,
209 patients were symptomatic, and cutaneous swelling
accounted for about half of all attacks and occurred in
nearly all symptomatic patients. Likewise, abdominal
attacks occurred in the vast majority of patients. Although
dangerous laryngeal attacks accounted for less than 1%of
all HAE attacks, they occurred at least once in more than
half of the symptomatic patients.
31
This highlights the point
that laryngeal edema can occur at any time, or age.
Farkas et al noted that pediatric patients were more often
affected by cutaneous edema than abdominal or laryngeal
edema (Table 2). Cutaneous edema of the extremities or
genitals typically persisted for 2 to 3 days.
34
Furthermore, nearly 90% of the 1,168 patients in the
PREHAEAT registry experienced episodes of cutaneous
edema, 81% experienced abdominal attacks (Table 3).
23
Similar to the findings of Bork et al, 43% of patients
experienced at least 1 laryngeal attack.
23
a
Total n-size was 221; 12 patients were asymptomatic.
Number (%)
of Attacks
n=131,110
Number (%)
of Patients
a
n=209
Cutaneous
Abdominal
Laryngeal
Type of Attack
65,102 (50%)
62,503 (48%)
1,229 (1%)
201 (96%)
195 (93%)
108 (52%)
Table 1 – Number and Type of Hereditary
Angioedema Attacks in 209 Patients
31
Table 2 – Number and Type of Attacks
in 26 Pediatric Patients
34
Number (%) of Patients
n=26
Cutaneous
Abdominal
Laryngeal
Type of Attack
20 (77%)
14 (54%)
7 (27%)
Table 3 – Type of Angioedema Attacks
in 1,168 Patients
23
Number
(%)
of Patients
n=1,168
Number
(%)
of Males
n=523
Number
(%)
of Females
n=645
Cutaneous
Abdominal
Laryngeal
Genitourinary
1,037 (89%)
940 (81%)
498 (43%)
153 (13%)
Type of
Attack
NA
387 (74%)
183 (35%)
NA
NA
553 (86%)
315 (49%)
NA
NA=Data not available.
HAE
1...,10,11,12,13,14,15,16,17,18,19 21,22,23,24,25,26,27,28,29,30,...78
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