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Microbiology · Immunology: phagocyte disorders
Sample itemA 5-year-old boy has recurrent abscesses of the skin, lymph nodes and liver caused by Staphylococcus aureus and Aspergillus. The nitroblue tetrazolium test remains colourless. Which defect is present?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
A. Deficiency of NADPH oxidase, preventing the respiratory burst
Chronic granulomatous disease results from NADPH oxidase defects (most commonly X-linked gp91phox), so phagocytes cannot generate superoxide. Catalase-positive organisms such as Staphylococcus aureus, Aspergillus, Nocardia and Serratia are especially problematic because they destroy their own hydrogen peroxide. The NBT and dihydrorhodamine tests are abnormal.
Option by option
A. Deficiency of NADPH oxidase, preventing the respiratory burst (correct)
Correct. Failure of the oxidative burst leaves the NBT test unchanged.
B. Deficiency of the C5-C9 complement components
Terminal complement deficiency predisposes to Neisseria infections.
C. Deficiency of myeloperoxidase only
Myeloperoxidase deficiency usually causes few infections, other than Candida in diabetics.
D. Defective LFA-1 (CD18) adhesion
LAD-1 causes delayed umbilical cord separation and leucocytosis without pus.
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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