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Pathology · Haematology: lead poisoning
Sample itemA 30-year-old man who recycles old batteries has abdominal colic, constipation and anaemia. He has a bluish line on the gums, and the smear shows coarse basophilic stippling. Which pair of enzymes in haem synthesis is inhibited by his toxin?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
C. Delta-aminolevulinic acid dehydratase and ferrochelatase
Lead inhibits ALA dehydratase and ferrochelatase, so aminolevulinic acid and protoporphyrin accumulate and haem synthesis falls. It also inhibits pyrimidine 5-prime nucleotidase, which leaves ribosomal RNA in red cells and produces basophilic stippling. Diagnosis is by blood lead level, and chelators such as succimer or EDTA are used.
Option by option
A. ALA synthase and uroporphyrinogen decarboxylase
ALA synthase and uroporphyrinogen decarboxylase are not the main targets; the latter is deficient in porphyria cutanea tarda.
B. Heme oxygenase and biliverdin reductase
Haem oxygenase and biliverdin reductase are involved in haem breakdown, not synthesis.
C. Delta-aminolevulinic acid dehydratase and ferrochelatase (correct)
Correct. Blocking these two enzymes explains the anaemia and basophilic stippling in lead toxicity.
D. Porphobilinogen deaminase and uroporphyrinogen III synthase
Porphobilinogen deaminase is deficient in acute intermittent porphyria, and uroporphyrinogen III synthase in congenital erythropoietic porphyria.
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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