Skeletal dysplasias: Camurati-Engelmann disease: practice question

A Orthopaedics 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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Orthopaedics · Skeletal dysplasias: Camurati-Engelmann disease

Sample item

A 10-year-old child has leg pain, a waddling gait, muscle weakness and symmetrical thickening of the diaphyses of the long bones on radiographs, with sparing of the epiphyses. Which treatment can reduce pain?

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

The answer and the reasoning

A. Corticosteroids

Camurati-Engelmann disease, or progressive diaphyseal dysplasia, is an autosomal dominant condition caused by a TGFB1 mutation. It causes symmetrical diaphyseal sclerosis and thickening, bone pain, muscle weakness and a waddling gait. Corticosteroids can relieve pain and improve function.

Option by option

  • A. Corticosteroids (correct)

    Correct. Corticosteroids reduce pain in progressive diaphyseal dysplasia.

  • B. Antibiotics

    There is no infection, so antibiotics are ineffective.

  • C. Chemotherapy

    The condition is benign, so chemotherapy is not used.

  • D. Calcitriol

    Calcitriol treats rickets and osteomalacia and not diaphyseal dysplasia.

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 Orthopaedics question

What is the answer to this Orthopaedics question?

The best answer is A, Corticosteroids. Camurati-Engelmann disease, or progressive diaphyseal dysplasia, is an autosomal dominant condition caused by a TGFB1 mutation.

Why is "Antibiotics" not the answer?

There is no infection, so antibiotics are ineffective.

Why is "Chemotherapy" not the answer?

The condition is benign, so chemotherapy is not used.

Why is "Calcitriol" not the answer?

Calcitriol treats rickets and osteomalacia and not diaphyseal dysplasia.

What concept does this question test?

It tests Skeletal dysplasias: Camurati-Engelmann disease in Orthopaedics. It causes symmetrical diaphyseal sclerosis and thickening, bone pain, muscle weakness and a waddling gait. Corticosteroids can relieve pain and improve function.

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 Skeletal dysplasias: Camurati-Engelmann disease?

Pair each fracture with its classic complication and nerve injured. Then use the flashcards and the other Orthopaedics 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.