Heme metabolism: porphyria: practice question

A Biochemistry question in the style of the NEET PG paper. Answer it first, then read why the right option is right and why each of the others is wrong.

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Biochemistry · Heme metabolism: porphyria

Sample item

A patient has abdominal pain, neuropathy and darkening urine after barbiturate exposure, but no photosensitivity. Which enzyme is most likely deficient?

Original practice item, not a recalled exam question. Nothing here is recorded.

The answer and the reasoning

C. Porphobilinogen deaminase

Acute intermittent porphyria results from porphobilinogen deaminase deficiency. Hepatic CYP induction worsens attacks, and accumulated intermediates produce neurovisceral symptoms without cutaneous photosensitivity.

Option by option

  • A. Ferrochelatase

    Ferrochelatase deficiency contributes to erythropoietic protoporphyria.

  • B. Uroporphyrinogen decarboxylase

    Uroporphyrinogen decarboxylase deficiency causes porphyria cutanea tarda with photosensitivity.

  • C. Porphobilinogen deaminase (correct)

    Correct. PBG deaminase deficiency causes acute intermittent porphyria.

  • D. ALA dehydratase

    ALA dehydratase is inhibited by lead and is rarely deficient.

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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Questions about this Biochemistry question

What is the answer to this Biochemistry question?

The best answer is C, Porphobilinogen deaminase. Acute intermittent porphyria results from porphobilinogen deaminase deficiency.

Why is "Ferrochelatase" not the answer?

Ferrochelatase deficiency contributes to erythropoietic protoporphyria.

Why is "Uroporphyrinogen decarboxylase" not the answer?

Uroporphyrinogen decarboxylase deficiency causes porphyria cutanea tarda with photosensitivity.

Why is "ALA dehydratase" not the answer?

ALA dehydratase is inhibited by lead and is rarely deficient.

What concept does this question test?

It tests Heme metabolism: porphyria in Biochemistry. Hepatic CYP induction worsens attacks, and accumulated intermediates produce neurovisceral symptoms without cutaneous photosensitivity.

How hard is this question?

We tag it as an application question: it asks you to use a fact on a patient or a finding. The tag is our own editorial judgement, not an official difficulty rating, so read the reason for every option, not only the answer.

How should I study Heme metabolism: porphyria?

Build one table of enzyme deficiency, disease, inheritance and key finding, then revise it by spaced recall. Then use the flashcards and the other Biochemistry questions on this site to test what stuck.

Is this a real NEET PG question?

No. It is an original practice item (editorial status: sample, version 1). NextPrep does not publish recalled or leaked questions, and independent faculty review of this item has not been completed yet, so confirm important facts in a standard textbook.