Paediatric surgery: Beckwith-Wiedemann syndrome: practice question

A General Surgery 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 Surgery · Paediatric surgery: Beckwith-Wiedemann syndrome

Sample item

A newborn has an omphalocele, macroglossia and a large birth weight. Which complication needs early monitoring in the first days of life?

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

The answer and the reasoning

D. Hypoglycaemia from pancreatic islet hyperplasia

Beckwith-Wiedemann syndrome results from dysregulation of imprinted genes on chromosome 11p15 and features macrosomia, macroglossia and abdominal wall defects. Neonatal hyperinsulinism causes hypoglycaemia, so glucose must be monitored. Affected children also have an increased risk of embryonal tumours such as Wilms tumour and hepatoblastoma, requiring ultrasound surveillance.

Option by option

  • A. Hypercalcaemia

    Hypercalcaemia is not characteristic of this syndrome.

  • B. Hyperthyroidism

    Thyroid overactivity is not part of the syndrome.

  • C. Hypernatraemia

    Sodium disturbance is not a feature of the syndrome.

  • D. Hypoglycaemia from pancreatic islet hyperplasia (correct)

    Correct. Islet cell hyperplasia produces hyperinsulinaemic hypoglycaemia.

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 Surgery question

What is the answer to this General Surgery question?

The best answer is D, Hypoglycaemia from pancreatic islet hyperplasia. Beckwith-Wiedemann syndrome results from dysregulation of imprinted genes on chromosome 11p15 and features macrosomia, macroglossia and abdominal wall defects.

Why is "Hypercalcaemia" not the answer?

Hypercalcaemia is not characteristic of this syndrome.

Why is "Hyperthyroidism" not the answer?

Thyroid overactivity is not part of the syndrome.

Why is "Hypernatraemia" not the answer?

Sodium disturbance is not a feature of the syndrome.

What concept does this question test?

It tests Paediatric surgery: Beckwith-Wiedemann syndrome in General Surgery. Neonatal hyperinsulinism causes hypoglycaemia, so glucose must be monitored. Affected children also have an increased risk of embryonal tumours such as Wilms tumour and hepatoblastoma, requiring ultrasound surveillance.

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 Paediatric surgery: Beckwith-Wiedemann syndrome?

Learn classic signs and eponyms alongside what they diagnose. Then use the flashcards and the other General Surgery 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.