Systemic paediatrics: autosomal recessive polycystic kidney disease: 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.

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Pediatrics · Systemic paediatrics: autosomal recessive polycystic kidney disease

Sample item

A neonate has bilateral massive flank masses with oligohydramnios, Potter facies and respiratory distress from pulmonary hypoplasia. Years later the child develops portal hypertension. Which liver lesion is expected?

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

The answer and the reasoning

C. Congenital hepatic fibrosis

Autosomal recessive polycystic kidney disease (PKHD1) presents in the neonate or fetus with large echogenic kidneys, oligohydramnios and pulmonary hypoplasia. All patients have congenital hepatic fibrosis with portal hypertension, varices and splenomegaly later. Adult-type ADPKD has liver cysts and berry aneurysm risk.

Option by option

  • A. Hepatic adenoma

    Hepatic adenoma is linked to glycogen storage disease and hormonal exposure, not ARPKD.

  • B. Multiple liver cysts with intracranial aneurysms

    Liver cysts with intracranial aneurysms describes adult-type ADPKD.

  • C. Congenital hepatic fibrosis (correct)

    Correct. Congenital hepatic fibrosis accompanies ARPKD and causes portal hypertension.

  • D. Biliary atresia

    Biliary atresia presents with persistent conjugated jaundice and pale stools in early weeks.

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 Pediatrics question

What is the answer to this Pediatrics question?

The best answer is C, Congenital hepatic fibrosis. Autosomal recessive polycystic kidney disease (PKHD1) presents in the neonate or fetus with large echogenic kidneys, oligohydramnios and pulmonary hypoplasia.

Why is "Hepatic adenoma" not the answer?

Hepatic adenoma is linked to glycogen storage disease and hormonal exposure, not ARPKD.

Why is "Multiple liver cysts with intracranial aneurysms" not the answer?

Liver cysts with intracranial aneurysms describes adult-type ADPKD.

Why is "Biliary atresia" not the answer?

Biliary atresia presents with persistent conjugated jaundice and pale stools in early weeks.

What concept does this question test?

It tests Systemic paediatrics: autosomal recessive polycystic kidney disease in Pediatrics. All patients have congenital hepatic fibrosis with portal hypertension, varices and splenomegaly later. Adult-type ADPKD has liver cysts and berry aneurysm risk.

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 Systemic paediatrics: autosomal recessive polycystic kidney disease?

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.