Clinical biochemistry: Rotor syndrome: 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 · Clinical biochemistry: Rotor syndrome

Sample item

A 12-year-old has lifelong mild jaundice with mainly conjugated hyperbilirubinaemia and normal ALT and ALP. Liver biopsy shows normal histology without any pigment, and the gallbladder is visualised on oral cholecystography. What is the diagnosis?

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

The answer and the reasoning

A. Rotor syndrome

Rotor syndrome is a benign conjugated hyperbilirubinaemia due to defective hepatic uptake and storage of conjugated bilirubin by OATP1B1/1B3 deficiency. Unlike Dubin-Johnson syndrome, the liver is not pigmented and the gallbladder is visualised. No treatment is needed.

Option by option

  • A. Rotor syndrome (correct)

    Correct. Conjugated jaundice with an unpigmented liver and a visualised gallbladder is Rotor syndrome.

  • B. Crigler-Najjar syndrome type II

    Crigler-Najjar type II causes unconjugated, not conjugated, hyperbilirubinaemia.

  • C. Dubin-Johnson syndrome

    Dubin-Johnson syndrome has a darkly pigmented liver and a non-visualised gallbladder.

  • D. Primary sclerosing cholangitis

    Sclerosing cholangitis raises ALP and shows biliary strictures.

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 A, Rotor syndrome. Rotor syndrome is a benign conjugated hyperbilirubinaemia due to defective hepatic uptake and storage of conjugated bilirubin by OATP1B1/1B3 deficiency.

Why is "Crigler-Najjar syndrome type II" not the answer?

Crigler-Najjar type II causes unconjugated, not conjugated, hyperbilirubinaemia.

Why is "Dubin-Johnson syndrome" not the answer?

Dubin-Johnson syndrome has a darkly pigmented liver and a non-visualised gallbladder.

Why is "Primary sclerosing cholangitis" not the answer?

Sclerosing cholangitis raises ALP and shows biliary strictures.

What concept does this question test?

It tests Clinical biochemistry: Rotor syndrome in Biochemistry. Unlike Dubin-Johnson syndrome, the liver is not pigmented and the gallbladder is visualised. No treatment is needed.

How hard is this question?

We tag it as a multi-step question: it asks you to combine more than one idea. 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 Clinical biochemistry: Rotor syndrome?

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.