Genetics: Beckwith-Wiedemann syndrome: practice question

A Pediatrics 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.

Try it

Pediatrics · Genetics: Beckwith-Wiedemann syndrome

Sample item

A neonate has macroglossia, omphalocele, hemihypertrophy and hypoglycaemia. To which tumour is the child predisposed, requiring ultrasound surveillance?

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

The answer and the reasoning

B. Wilms tumour and hepatoblastoma

Beckwith-Wiedemann syndrome is an overgrowth disorder involving imprinting at 11p15, with predisposition to Wilms tumour and hepatoblastoma. Abdominal ultrasound every 3 months until 8 years and alpha-fetoprotein screening are advised. Hypoglycaemia arises from hyperinsulinism.

Option by option

  • A. Neuroblastoma

    Neuroblastoma is not the main risk.

  • B. Wilms tumour and hepatoblastoma (correct)

    Correct. Wilms tumour and hepatoblastoma are the risks.

  • C. Osteosarcoma

    Osteosarcoma is linked to RB1 and Li-Fraumeni.

  • D. Retinoblastoma

    Retinoblastoma is linked to the RB1 gene.

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.

More Pediatrics questions

Questions about this Pediatrics question

What is the answer to this Pediatrics question?

The best answer is B, Wilms tumour and hepatoblastoma. Beckwith-Wiedemann syndrome is an overgrowth disorder involving imprinting at 11p15, with predisposition to Wilms tumour and hepatoblastoma.

Why is "Neuroblastoma" not the answer?

Neuroblastoma is not the main risk.

Why is "Osteosarcoma" not the answer?

Osteosarcoma is linked to RB1 and Li-Fraumeni.

Why is "Retinoblastoma" not the answer?

Retinoblastoma is linked to the RB1 gene.

What concept does this question test?

It tests Genetics: Beckwith-Wiedemann syndrome in Pediatrics. Abdominal ultrasound every 3 months until 8 years and alpha-fetoprotein screening are advised. Hypoglycaemia arises from hyperinsulinism.

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 Genetics: Beckwith-Wiedemann syndrome?

Memorise milestones and the vaccine schedule as tables and revise them often. Then use the flashcards and the other Pediatrics 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.