Gastroenterology: Dubin-Johnson syndrome: practice question

A General Medicine 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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General Medicine · Gastroenterology: Dubin-Johnson syndrome

Sample item

A young man has mild recurrent jaundice with conjugated hyperbilirubinaemia, normal liver enzymes and a grossly black liver on laparoscopy. What is the defect?

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

The answer and the reasoning

B. Impaired canalicular excretion through MRP2 transporter

Dubin-Johnson syndrome is a benign autosomal recessive disorder of canalicular bilirubin excretion due to ABCC2 (MRP2) mutation. The liver is black from melanin-like pigment and urinary coproporphyrin I predominates. No treatment is needed.

Option by option

  • A. Reduced hepatic uptake of bilirubin

    Reduced uptake occurs in Gilbert syndrome.

  • B. Impaired canalicular excretion through MRP2 transporter (correct)

    Correct. MRP2 defect explains conjugated hyperbilirubinaemia.

  • C. Absent UDP-glucuronosyltransferase

    UGT deficiency causes Crigler-Najjar or Gilbert syndrome with unconjugated hyperbilirubinaemia.

  • D. Autoimmune destruction of bile ducts

    Bile duct destruction is PBC.

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 General Medicine question

What is the answer to this General Medicine question?

The best answer is B, Impaired canalicular excretion through MRP2 transporter. Dubin-Johnson syndrome is a benign autosomal recessive disorder of canalicular bilirubin excretion due to ABCC2 (MRP2) mutation.

Why is "Reduced hepatic uptake of bilirubin" not the answer?

Reduced uptake occurs in Gilbert syndrome.

Why is "Absent UDP-glucuronosyltransferase" not the answer?

UGT deficiency causes Crigler-Najjar or Gilbert syndrome with unconjugated hyperbilirubinaemia.

Why is "Autoimmune destruction of bile ducts" not the answer?

Bile duct destruction is PBC.

What concept does this question test?

It tests Gastroenterology: Dubin-Johnson syndrome in General Medicine. The liver is black from melanin-like pigment and urinary coproporphyrin I predominates. No treatment is needed.

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 Gastroenterology: Dubin-Johnson syndrome?

For each disease know the classic presentation, the best test, and the first-line treatment. Then use the flashcards and the other General Medicine 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.