Genetic and metabolic disorders: Alport 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.

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Pediatrics · Genetic and metabolic disorders: Alport syndrome

Sample item

A 9-year-old boy has recurrent episodes of haematuria, bilateral high-frequency sensorineural hearing loss, and his maternal uncle died of renal failure. Biopsy would show which feature on electron microscopy?

ASubepithelial humpsBEffacement of podocyte foot processes onlyCSubendothelial immune depositsDSplitting and lamellation of the glomerular basement membrane

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

The answer and the reasoning

D. Splitting and lamellation of the glomerular basement membrane

Alport syndrome is an X-linked disorder of type IV collagen (COL4A5) causing haematuria, deafness and eye changes, with a basket-weave splitting of the glomerular basement membrane. Males progress to renal failure. Subepithelial humps are seen in post-streptococcal glomerulonephritis.

Option by option

A. Subepithelial humps
Subepithelial humps occur in post-infectious glomerulonephritis.
B. Effacement of podocyte foot processes only
Effacement of foot processes alone is the finding in minimal change disease.
C. Subendothelial immune deposits
Subendothelial deposits occur in lupus nephritis and membranoproliferative disease.
D. Splitting and lamellation of the glomerular basement membrane (correct)
Correct. Lamellation of the basement membrane is typical of Alport 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.

Questions about this Pediatrics question

What is the answer to this Pediatrics question?

The best answer is D, Splitting and lamellation of the glomerular basement membrane. Alport syndrome is an X-linked disorder of type IV collagen (COL4A5) causing haematuria, deafness and eye changes, with a basket-weave splitting of the glomerular basement membrane.

Why is "Subepithelial humps" not the answer?

Subepithelial humps occur in post-infectious glomerulonephritis.

Why is "Effacement of podocyte foot processes only" not the answer?

Effacement of foot processes alone is the finding in minimal change disease.

Why is "Subendothelial immune deposits" not the answer?

Subendothelial deposits occur in lupus nephritis and membranoproliferative disease.

What concept does this question test?

It tests Genetic and metabolic disorders: Alport syndrome in Pediatrics. Males progress to renal failure. Subepithelial humps are seen in post-streptococcal glomerulonephritis.

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 Genetic and metabolic disorders: Alport 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.