Renal Pathology Practice Questions

Q: A mutation in the alpha 5 chain of collagen 4 leads to which diagnosis?

Alport's syndrome. **Explanation:** **Correct Option: A. Alport’s Syndrome** Alport’s syndrome is a hereditary nephritis caused by mutations in the genes encoding the **Type IV collagen** chains, which are essential components of the glomerular basement membrane (GBM), cochlea, and lens [1]. The most common form (85% of cases) is **X-linked dominant**, resulting from a mutation in the **COL4A5 gene**, which encodes the **alpha-5 (α5) chain** of Type IV collagen [1]. This leads to a defective GBM that appears thin initially but progresses to a characteristic "basket-weave" appearance (irregular thickening and splitting of the lamina densa) on electron microscopy. **Incorrect Options:** * **B. Thin Basement Membrane Disease (TBMD):** While also a Type IV collagen disorder, it most commonly involves mutations in the **COL4A3 or COL4A4** genes (alpha-3 or alpha-4 chains). It presents as isolated benign hematuria with diffuse thinning of the GBM. * **C. Nodular Glomerulosclerosis:** Also known as Kimmelstiel-Wilson lesions, this is pathognomonic for **Diabetic Nephropathy**. It involves the accumulation of mesangial matrix, not a primary genetic collagen mutation. * **D. Goodpasture Syndrome:** This is an autoimmune condition caused by **anti-GBM antibodies** directed against the non-collagenous (NC1) domain of the **alpha-3 chain** of Type IV collagen. It is not caused by a genetic mutation of the alpha-5 chain. **NEET-PG High-Yield Pearls:** * **Clinical Triad of Alport’s:** Sensorineural deafness, eye anomalies (Anterior Lenticonus), and progressive renal failure [1]. * **Electron Microscopy (EM):** "Basket-weave appearance" is the classic buzzword for Alport’s. * **Inheritance:** Most common is X-linked (COL4A5); Autosomal recessive/dominant forms involve COL4A3/A4 [1]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 928-930.

Q: Silver stain performed on renal biopsy shows characteristic findings. Immunostaining for Anti-PLA2R is positive. Based on the given findings, what is the likely diagnosis?

Membranous nephropathy. ***Membranous nephropathy*** - **Anti-PLA2R antibody** positivity is highly specific for **primary membranous nephropathy**, making it the diagnostic marker of choice. - Silver stain reveals the characteristic **spike-and-dome pattern** with thickened basement membrane projections between immune deposits. *Focal segmental glomerulosclerosis (FSGS)* - Shows **segmental sclerosis** and **hyalinosis** on silver stain, not the spike-and-dome pattern seen in membranous nephropathy. - **Anti-PLA2R** is typically negative in FSGS, as it lacks the characteristic immune complex deposits of membranous disease. *Membranoproliferative glomerulonephritis (MPGN)* - Silver stain demonstrates **double contour** or **tram-track** appearance of the basement membrane, distinct from spike-and-dome. - **Anti-PLA2R** is negative in MPGN, which involves different complement-mediated pathways and immune complex patterns. *Crescentic glomerulonephritis* - Characterized by **epithelial crescents** filling Bowman's space on silver stain, not basement membrane thickening. - **Anti-PLA2R** is not associated with crescentic GN, which typically involves **ANCA** or **anti-GBM antibodies** instead.

Question 1

A mutation in the alpha 5 chain of collagen 4 leads to which diagnosis?

Accepted Answer

Alport's syndrome

Answer

Thin basement membrane disease

Answer

Nodular glomerulosclerosis

Answer

Goodpasture syndrome

Question 2

Nephrotic syndrome is characterized by

Accepted Answer

Hematuria

Answer

Massive proteinuria

Answer

Edema

Answer

Hyperlipidemia

Question 3

In an adult, unilateral smooth contracted kidney with hypertension is seen in which condition?

Accepted Answer

Stenosis of renal artery

Answer

Chronic glomerulonephritis

Answer

Renal cell carcinoma

Answer

Pyelonephritis

Question 4

What is true about Minimal change disease?

Accepted Answer

Appears normal under light microscopy, but electron microscopy shows loss of foot processes.

Answer

Mesangial deposits are present.

Answer

Shows a 'tram track' appearance.

Answer

Presents with gross hematuria.

Question 5

All are features of hemolytic uremic syndrome, EXCEPT?

Accepted Answer

Neuropsychiatric disturbances

Answer

Hyperkalemia

Answer

Anemia

Answer

Renal microthrombi

Question 6

Which of the following is a characteristic feature of collapsing glomerulopathy?

Accepted Answer

Hypertrophy and necrosis of visceral epithelium

Answer

Tuft necrosis

Answer

Mesangiolysis

Answer

Parietal epithelial proliferation

Question 7

On electron microscopy, what characteristic splitting of the glomerular basement membrane (GBM) with subepithelial deposits is seen in most cases?

Accepted Answer

Membranous nephropathy

Answer

Rapidly progressive glomerulonephritis (RPGN)

Answer

Focal segmental glomerulosclerosis (FSGS)

Answer

Minimal change disease

Question 8

A 28-year-old man presents with a large amount of blood and protein in his urine. He has had sensorineural hearing loss since his teen years and anterior lenticonus. The physician suspects a genetic disorder that may lead to eventual kidney failure. If this is the case, the patient most likely has a mutation in which one of the following proteins?

Accepted Answer

Collagen

Answer

Spectrin

Answer

a1-Antitrypsin

Answer

Fibrillin

Question 9

Silver stain performed on renal biopsy shows characteristic findings. Immunostaining for Anti-PLA2R is positive. Based on the given findings, what is the likely diagnosis?

Accepted Answer

Membranous nephropathy

Answer

Focal segmental glomerulosclerosis (FSGS)

Answer

Membranoproliferative glomerulonephritis (MPGN)

Answer

Crescentic glomerulonephritis

Question 10

Dysmorphic RBCs are seen in which of the following conditions?

Accepted Answer

IgA glomerulopathy

Answer

Membranous glomerulopathy

Answer

Nil lesion glomerulopathy

Answer

None of the above

Renal Pathology — MCQs

Renal Pathology — MCQs

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