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Physiology · Immunity: leukocyte adhesion deficiency
Sample itemA 3-month-old infant had delayed umbilical cord separation and now has recurrent skin infections without pus formation. Blood neutrophil count is very high. Which neutrophil step is defective?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
B. Firm adhesion to endothelium through beta-2 integrins (CD18)
In leukocyte adhesion deficiency type 1, absent CD18 (beta-2 integrin) prevents firm adhesion and transmigration, so neutrophils accumulate in blood but cannot reach tissues. Features are delayed cord separation, neutrophilia, and infections without pus. Other neutrophil defects present differently.
Option by option
A. Phagolysosome fusion through lysosomal trafficking regulator
A lysosomal trafficking defect is Chediak-Higashi syndrome, with giant granules and albinism.
B. Firm adhesion to endothelium through beta-2 integrins (CD18) (correct)
Correct. Without beta-2 integrins, neutrophils cannot adhere firmly and exit the vessel.
C. Generation of the oxidative burst by NADPH oxidase
A defective oxidative burst causes chronic granulomatous disease, where pus and granulomas do form.
D. Rolling along endothelium through sialyl Lewis X
A defect in rolling ligands is seen in type 2 leukocyte adhesion deficiency, which has a Bombay phenotype and developmental delay.
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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