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Microbiology · Immunology: complement deficiency
Sample itemA 20-year-old man has recurrent episodes of non-itchy, non-pitting swelling of the lips and face and severe abdominal colic. Antihistamines and adrenaline give no relief, and his father had laryngeal swelling. Which laboratory finding is expected?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
B. Low C4 with reduced C1 esterase inhibitor level or function
Hereditary angioedema results from C1 esterase inhibitor deficiency or dysfunction, causing uncontrolled bradykinin release and unchecked classical pathway activation. C4 is low even between attacks and is the screening test, confirmed by C1 inhibitor level and function. Attacks do not respond to antihistamines or adrenaline.
Option by option
A. Raised serum tryptase between attacks
Raised tryptase marks mast cell degranulation as in anaphylaxis, which would respond to adrenaline.
B. Low C4 with reduced C1 esterase inhibitor level or function (correct)
Correct. Low C4 with deficient C1 inhibitor confirms the diagnosis.
C. Low C3 with normal C4
Low C3 with normal C4 suggests alternative pathway consumption, not this disorder.
D. Markedly raised total serum IgE
Total IgE is raised in atopy and does not explain bradykinin-mediated swelling.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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