Cell injury and adaptation: Hurler syndrome: practice question

A Pathology 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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Pathology · Cell injury and adaptation: Hurler syndrome

Sample item

A 2-year-old has coarse facies, corneal clouding, hepatosplenomegaly, a thoracolumbar gibbus and developmental delay. Which pair of glycosaminoglycans accumulates in the lysosomes?

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

The answer and the reasoning

C. Dermatan sulfate and heparan sulfate

Hurler syndrome (MPS I) is caused by alpha-L-iduronidase deficiency, so dermatan sulfate and heparan sulfate cannot be degraded. Their lysosomal accumulation produces coarse features, corneal clouding, organomegaly, skeletal dysostosis and cognitive decline. Keratan sulfate with chondroitin sulfate is the pattern of Morquio syndrome.

Option by option

  • A. Keratan sulfate and chondroitin sulfate

    This pairing marks Morquio syndrome, which spares intellect and affects the skeleton.

  • B. Chondroitin sulfate and hyaluronic acid

    Hyaluronic acid is not a lysosomal storage product here, and this pairing does not fit the enzyme defect.

  • C. Dermatan sulfate and heparan sulfate (correct)

    Correct. Both are degraded by a pathway that begins with alpha-L-iduronidase.

  • D. Heparan sulfate and keratan sulfate

    No single mucopolysaccharidosis stores this pairing; keratan sulfate is stored in Morquio syndrome.

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 Pathology question

What is the answer to this Pathology question?

The best answer is C, Dermatan sulfate and heparan sulfate. Hurler syndrome (MPS I) is caused by alpha-L-iduronidase deficiency, so dermatan sulfate and heparan sulfate cannot be degraded.

Why is "Keratan sulfate and chondroitin sulfate" not the answer?

This pairing marks Morquio syndrome, which spares intellect and affects the skeleton.

Why is "Chondroitin sulfate and hyaluronic acid" not the answer?

Hyaluronic acid is not a lysosomal storage product here, and this pairing does not fit the enzyme defect.

Why is "Heparan sulfate and keratan sulfate" not the answer?

No single mucopolysaccharidosis stores this pairing; keratan sulfate is stored in Morquio syndrome.

What concept does this question test?

It tests Cell injury and adaptation: Hurler syndrome in Pathology. Their lysosomal accumulation produces coarse features, corneal clouding, organomegaly, skeletal dysostosis and cognitive decline. Keratan sulfate with chondroitin sulfate is the pattern of Morquio syndrome.

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 Cell injury and adaptation: Hurler syndrome?

Learn each disease as a bundle: cause, key histology, markers, and the clinical hallmark. Then use the flashcards and the other Pathology 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.