A nurse has just learned that her child has a life-threatening complement disorder known as hereditary angioneurotic edema (HAE). Due to deficiency in C1-INH, the nurse needs to be prepared for which possible life-threatening clinical manifestation?
A)
Bulging eyeballs
B)
Swelling of the airway
C)
Compressed carotid arteries
D)
Compression of brachial nerves
Ans:
B
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HAE is a rare, life-threatening complement disorder that results from deficiency of C1-inhibitor (HAE-C1-INH). It is an inherited autosomal dominant trait that causes mutation in the 11th chromosome. Deficiencies in C1-INH result in uncontrolled release of vasoactive substances that promote vascular permeability. The net result is the development of swelling in the subcutaneous tissues of the extremities, face/torso, or upper airway and GI tract. Laryngeal edema is a life-threatening manifestation that can lead to complete airway obstruction and death without interventions.
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