21
Fluid shifts, fluid loss from vomiting and diarrhea, plasma
extravasation, decreased oral intake, and vasodilation can
lead to hypovolemia and hypovolemic shock, including
circulatory collapse and/or loss of consciousness.
5,36
Bork et al retrospectively reviewed 33,671 abdominal
attacks in 153 patients, including a prospectively examined
subgroup of 23 patients with 169 attacks.
36
The majority
of attacks were severe, with a mean maximal pain rating
of 8.4 on a scale of 10. Symptoms of hypotension, such as
lightheadedness and dizziness, were the most commonly
reported experience of abdominal attacks and transpired
in 9 of every 10 episodes. Vomiting was also frequent,
occurring in nearly 75% of attacks. Circulatory collapse
and shock were atypical in the vast majority of attacks, but
did occur in about 4% of all abdominal attacks and about
40% of patients (Table 5). The majority of abdominal
attacks lasted 2 to 4 days.
36
Laryngeal Attacks
Upper airway obstruction from edema of the larynx,
glottis, or tongue can occur in HAE.
5,38,39
It is estimated
that only 10 years ago 25% to 50% of undiagnosed
HAE patients experiencing laryngeal attacks died from
asphyxiation.
5,38
Today, survival rates have improved because
of the availability of C1-INH therapy and other effective
treatments.
5,38,39
Undiagnosed patients are at a higher risk of death from
asphyxiation than patients who know their diagnosis of
HAE. Increased awareness of diagnosed patients and
repeated education and training of emergency treatment
personnel are a prerequisite for successful management of
laryngeal attacks.
40
Signs and symptoms that may herald the onset of a
laryngeal attack may include any of the following:
38,39
•
Dysphagia
•
Sensation of tightness or a lump in the throat
•
Voice changes, such as hoarseness or roughness
•
Dyspnea
•
Aphonia.
Fatal laryngeal edema has been known to arise as the first
symptom of HAE, and fatal upper airway attacks can occur
at any age. However, several factors have been identified
as increasing the risk of laryngeal attacks:
5,38
•
Oral surgery
•
General anesthesia with intubation
•
Head injury
•
Age 11 to 45 years
•
History of 1 or more episodes of laryngeal attacks
•
Facial edema.
Attacks of facial edema have been known to progress to
laryngeal edema, but facial attacks are far more common
than laryngeal attacks. About 14% to 29% of laryngeal
attacks begin with facial swelling.
31,38
Number (%)
of Attacks
n=33,671
Number (%)
of Patients
n=153
Lightheadedness or dizziness
Vomiting
Diarrhea
Circulatory collapse and shock
Loss of consciousness
Abdominal Attack
30,608 (90%)
24,696 (73%)
13,682 (41%)
1,468 (4%)
739 (2%)
149 (97%)
133 (87%)
98 (64%)
61 (40%)
41 (27%)
Table 5 – Symptoms Associated With
33,671 Abdominal Attacks in 153 Patients
36
HAE
