Pediatric emergency medicine trisk 1678 1678
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Adjunctive therapies including antihistamines and glucocorticoids may be
continued for 2 to 3 days per clinician discretion especially for patients with
persistent urticaria. Children should have close primary care follow-up. Referral
to an allergist for further evaluation and testing is recommended given 30% of
children may have subsequent reactions within 7 years.
HEREDITARY ANGIOEDEMA
CLINICAL PEARLS AND PITFALLS
Hereditary angioedema is a rare, life-threatening disorder triggered by
direct mechanical friction or trauma, or viral infections.
The diagnosis should be considered for patients with a positive family,
recurrent angioedema without urticaria, abdominal pain/vomiting, or
laryngeal edema.
Acute attacks may be life threatening and involve swelling of the
extremities, abdomen, genitourinary tract, face, oropharynx, or larynx.
C1-esterase inhibitor concentrate and plasma kallikrein inhibitor should
be used to manage acute attacks; epinephrine, antihistamines, and
steroids are not recommended to treat hereditary angioedema.
Current Evidence
Pathophysiology
Hereditary angioedema is a rare episodic disorder caused by a deficiency of C1inhibitor function. Affected individuals may have either low levels (type I) or
poor functionality (type II). C1-esterase inhibitor deficiency results in activation
of the kallikrein–kinin system, with resultant release of bradykinin causing
vasodilation and increased vascular permeability. The disease is autosomal
dominant and thus most patients have a family history; however, 20% to 25% of
cases are due to spontaneous mutations. Onset is most common in school-age
children and typically worsens during puberty. Presenting symptoms may include
subcutaneous angioedema (circumscribed areas of skin edema, most commonly
the extremities, without erythema or pruritus), abdominal attacks (severe pain,
distention, and vomiting), or laryngeal edema (life-threatening hoarseness,
stridor, and voice changes).
Triggers
