Renal Pathology Practice Questions

Q: The gross appearance of the kidney shown below is most compatible with which of the following conditions?

Chronic pyelonephritis. ***Chronic pyelonephritis*** - Shows characteristic **irregular cortical scarring** with **calyceal blunting and clubbing** due to recurrent infections. - Results in a **contracted, asymmetrically scarred kidney** with loss of renal parenchyma and distorted collecting system. *Cystic renal dysplasia* - Presents with **multiple cysts** of varying sizes replacing normal renal architecture from birth. - Shows **immature tubular structures** and **primitive mesenchyme** rather than inflammatory scarring. *Acute pyelonephritis* - Kidney appears **swollen and edematous** with **microabscesses** on the cortical surface. - Shows **acute inflammatory changes** without the chronic scarring and contraction seen here. *Acute glomerulonephritis* - Presents with **enlarged, edematous kidneys** with a **smooth cortical surface**. - Shows **red-brown discoloration** due to glomerular inflammation, not cortical scarring or calyceal changes.

Q: Alport syndrome is inherited as:

X-linked. **Explanation:** Alport Syndrome is a hereditary nephritis caused by mutations in the genes encoding **Type IV collagen**, which is a critical structural component of the glomerular basement membrane (GBM), cochlea, and lens [1]. **1. Why X-linked is Correct:** The most common mode of inheritance (approximately **85% of cases**) is **X-linked Dominant**, caused by a mutation in the **COL4A5** gene. Because it is X-linked, males are typically more severely affected, often progressing to end-stage renal disease (ESRD), while females (carriers) may present only with isolated hematuria. **2. Analysis of Incorrect Options:** * **Autosomal Recessive (D) & Autosomal Dominant (C):** While these forms do exist (due to mutations in *COL4A3* or *COL4A4*), they are significantly less common (approx. 15%). In the context of competitive exams like NEET-PG, if a single best answer is required, X-linked is the standard choice as it represents the vast majority of cases. * **Co-dominant (B):** This pattern is not associated with the inheritance of Type IV collagen disorders. **3. Clinical Pearls for NEET-PG:** * **Classic Triad:** Hereditary nephritis (hematuria/ESRD), Sensorineural hearing loss (high frequency), and Ocular defects. * **Pathognomonic Ocular Sign:** **Anterior Lenticonus** (conical protrusion of the lens). * **Electron Microscopy (High Yield):** Shows characteristic **"Basket-weave appearance"** due to irregular thinning and thickening of the GBM. * **Key Association:** Patients may develop Anti-GBM disease (Goodpasture-like syndrome) after receiving a kidney transplant [1].

Q: Which of the following is NOT a characteristic feature of nephronophthisis?

Cortical tubular hypertrophy. **Explanation:** Nephronophthisis (NPHP) is an autosomal recessive tubulointerstitial cystic kidney disease and is the most common genetic cause of end-stage renal disease (ESRD) in children and adolescents. **Why Option B is correct:** In nephronophthisis, the kidneys are typically **shrunken and small** [1]. The hallmark pathological process is **cortical tubular atrophy**, not hypertrophy [1]. This atrophy, combined with widespread interstitial fibrosis, leads to the characteristic contraction of the renal parenchyma [1]. **Analysis of other options:** * **Option A (Interstitial fibrosis):** This is a cardinal feature of the disease [1]. Chronic tubulointerstitial nephritis leads to dense fibrosis, which eventually causes renal failure. * **Option C (Cysts in the medulla):** Small cysts (typically 1–15 mm) are characteristically found at the **corticomedullary junction** and in the medulla [1]. While essential for the name, they may be absent in early stages or visible only on biopsy/ultrasound [1]. * **Option D (20% non-familial):** While NPHP is primarily a genetic (autosomal recessive) disorder, approximately 20% of cases occur sporadically without a documented family history, often due to de novo mutations or small family sizes. **NEET-PG High-Yield Pearls:** 1. **Genetics:** Most common gene involved is **NPHP1** (encoding Nephrocystin-1). It is a **ciliopathy**. 2. **Clinical Presentation:** Polyuria and polydipsia (due to impaired urinary concentrating ability) are early signs. Unlike many other cystic diseases, hypertension is a late finding. 3. **Extra-renal associations:** Senior-Løken syndrome (NPHP + Retinitis pigmentosa) and Joubert syndrome (NPHP + Cerebellar ataxia). 4. **Imaging:** Small, shrunken kidneys with loss of corticomedullary differentiation [1]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 953-954.

Question 1

Which of the following is associated with Von Brunn's nests?

Accepted Answer

Normal urothelium

Answer

Transitional cell carcinoma

Answer

Squamous cell carcinoma

Answer

Adenocarcinoma

Question 2

Rapidly progressive glomerulonephritis (RPGN) occurs in which of the following conditions?

Accepted Answer

Diabetic nephropathy

Answer

Systemic lupus erythematosus (SLE)

Answer

Post-streptococcal glomerulonephritis

Answer

Goodpasture syndrome

Question 3

A 27-year-old woman presents with a red rash over her cheeks, and pain and swelling in both knees as well as several small joints in her hands. She notes that the rash is worse with sun exposure. Medical evaluation reveals oral ulceration, positive ANA, and 3+ proteinuria. What is the most likely mechanism for the renal damage in this condition?

Accepted Answer

deposition of circulating immune complexes

Answer

vasculitis

Answer

micro-emboli

Answer

anti-basement membrane antibodies

Question 4

A 68-year-old man presents for repair of an abdominal aortic aneurysm. Severe complicated atherosclerosis is noted at surgery, prompting concern for embolism of atheromatous material to the kidneys and other organs. If the patient were to develop a renal cortical infarct as a result of surgery, which of the following would be the most likely outcome?

Accepted Answer

Scar formation

Answer

Chronic inflammation

Answer

Granulomatous inflammation

Answer

Hemangioma formation

Question 5

The gross appearance of the kidney shown below is most compatible with which of the following conditions?

Accepted Answer

Chronic pyelonephritis

Answer

Cystic renal dysplasia

Answer

Acute pyelonephritis

Answer

Acute glomerulonephritis

Question 6

Goodpasture's syndrome is characterized by which of the following findings?

Accepted Answer

Renal failure with lung hemorrhage

Answer

Renal failure with brain hemorrhage

Answer

Renal hemorrhage

Answer

Renal failure

Question 7

A 30-year-old man presents with generalized edema and hypertension. Urine examination shows subnephrotic proteinuria (< 2gm) and microscopic hematuria. Serum complement levels are decreased, and he is positive for anti-hepatitis C antibodies. What is the most likely diagnosis?

Accepted Answer

Membranoproliferative glomerulonephritis

Answer

Post-streptococcal glomerulonephritis (PSGN)

Answer

Mixed cryoglobulinemia

Answer

Focal segmental glomerulosclerosis (FSGS)

Question 8

Alport syndrome is inherited as:

Accepted Answer

X-linked

Answer

Co-dominant

Answer

Autosomal dominant

Answer

Autosomal recessive

Question 9

Which of the following findings is NOT associated with Adult Polycystic Kidney Disease?

Accepted Answer

Tricuspid valve prolapse

Answer

Autosomal dominant inheritance

Answer

Mutations affecting cell-cell-matrix interaction

Answer

Intracranial berry aneurysm

Question 10

Which of the following is NOT a characteristic feature of nephronophthisis?

Accepted Answer

Cortical tubular hypertrophy

Answer

Interstitial fibrosis

Answer

Cysts in the medulla

Answer

20% of cases are non-familial

Renal Pathology — MCQs

Renal Pathology — MCQs

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