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Physiology · Blood and immunity: factor XIII
Sample itemA newborn has prolonged bleeding from the umbilical stump and later a delayed haemorrhage after minor trauma. Platelet count, PT, aPTT and thrombin time are all normal. Which defect is most likely?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
B. Failure to cross-link fibrin because of absent fibrin-stabilising factor
Factor XIII, activated by thrombin and calcium, cross-links fibrin strands into a stable clot. Its deficiency gives normal screening tests but weak clots that bleed late, classically with umbilical stump bleeding. Standard PT and aPTT end at fibrin formation, so they miss the defect.
Option by option
A. Absent vitamin K-dependent carboxylation of clotting factors
Vitamin K deficiency would prolong the PT and often the aPTT.
B. Failure to cross-link fibrin because of absent fibrin-stabilising factor (correct)
Correct. Unstabilised fibrin clots form but break down, and standard tests are normal.
C. Absent von Willebrand factor multimers
Von Willebrand deficiency usually prolongs the aPTT through low factor VIII and the bleeding time.
D. Deficient platelet glycoprotein Ib receptor
Glycoprotein Ib deficiency causes thrombocytopenia with giant platelets and a prolonged bleeding time.
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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