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Pathology · Haematology: haemolytic uraemic syndrome
Sample itemA 4-year-old child had bloody diarrhoea 5 days ago and now has pallor and oliguria. Haemoglobin is 7 g/dL, platelets 40,000 per microlitre, creatinine is raised, schistocytes are present, and PT and aPTT are normal. Which mechanism underlies this illness?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
A. Shiga toxin-mediated injury to glomerular endothelium with microthrombi
Typical haemolytic uraemic syndrome follows infection with Shiga toxin-producing Escherichia coli such as O157:H7. The toxin binds Gb3 receptors on renal endothelium, causing thrombotic microangiopathy with haemolysis, thrombocytopenia and acute kidney injury. Coagulation tests are normal, and antibiotics are avoided because they may increase toxin release.
Option by option
A. Shiga toxin-mediated injury to glomerular endothelium with microthrombi (correct)
Shiga toxin damages endothelium to produce the triad after a diarrhoeal illness.
B. Severe deficiency of ADAMTS13 leaving ultra-large von Willebrand multimers
ADAMTS13 deficiency causes TTP, which classically has prominent neurological features and usually milder renal involvement.
C. Widespread tissue factor release consuming clotting factors
Consumption of clotting factors in DIC prolongs PT and aPTT, which are normal here.
D. Antibodies to platelet factor 4-heparin complexes
Anti-PF4-heparin antibodies cause heparin-induced thrombocytopenia and require heparin exposure.
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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