Renal Pathology Practice Questions

Q: Adult polycystic kidney disease is characterized by which mode of inheritance?

Autosomal dominant. **Explanation:** **Autosomal Dominant Polycystic Kidney Disease (ADPKD)** is the most common hereditary renal disorder. As the name implies, it follows an **Autosomal Dominant** inheritance pattern (Option A). It typically manifests in adulthood (3rd to 4th decade) because, while the genetic defect is present at birth, the cysts require time to enlarge and replace functional parenchyma [1]. * **Pathogenesis:** It is primarily caused by mutations in the **PKD1 gene** (85% of cases, Chromosome 16, encoding Polycystin-1) or the **PKD2 gene** (15% of cases, Chromosome 4, encoding Polycystin-2) [1]. PKD1 mutations usually lead to an earlier onset of end-stage renal disease (ESRD). **Why other options are incorrect:** * **Autosomal Recessive (Option B):** This characterizes **ARPKD**, the "infantile" form. It is caused by mutations in the **PKHD1 gene** (Chromosome 6) and presents with bilateral renal enlargement and congenital hepatic fibrosis in neonates or children. * **X-linked (Option C):** While Alport Syndrome can be X-linked, polycystic kidney disease is not. * **Mitochondrial (Option D):** This inheritance involves maternal transmission affecting high-energy organs; it is not associated with ADPKD. **High-Yield Clinical Pearls for NEET-PG:** 1. **Extra-renal manifestations:** The most common is **Liver cysts**. The most life-threatening is **Berry Aneurysms** (Circle of Willis), which can lead to Subarachnoid Hemorrhage. 2. **Other associations:** Mitral Valve Prolapse (MVP), diverticulosis, and pancreatic cysts. 3. **Gross Appearance:** Kidneys are massively enlarged (up to 4kg) with a "multicystic" appearance and no intervening normal parenchyma. 4. **Screening:** Family members are screened using **Ultrasonography**. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 950-951.

Q: Which of the following statements regarding Wilms tumor is true?

It is associated with deletion of chromosome 11p13.. **Explanation:** **Wilms Tumor (Nephroblastoma)** is the most common primary renal tumor of childhood. **1. Why Option B is correct:** Wilms tumor is strongly linked to genetic alterations on **chromosome 11**. Specifically, the **WT1 gene** is located at **11p13** [1]. Deletions or mutations in this region are associated with sporadic Wilms tumor and syndromic forms like **WAGR syndrome** (Wilms tumor, Aniridia, Genitourinary anomalies, and mental Retardation) [1]. Another locus, **WT2**, is located at **11p15.5** (associated with Beckwith-Wiedemann syndrome). **2. Why the other options are incorrect:** * **Option A:** Wilms tumor is a pediatric neoplasm, typically occurring between ages **2 and 5 years**. It is extremely rare in adults. * **Option C:** The **MIC 2 gene** (CD99) is a diagnostic marker for **Ewing Sarcoma** and PNET, not Wilms tumor. * **Option D:** The most common presentation is a **large, palpable, asymptomatic abdominal mass** (often discovered by a parent while bathing the child) [1]. While hematuria can occur, it is present in only about 25% of cases. **High-Yield Clinical Pearls for NEET-PG:** * **Triphasic Histology:** Characterized by three components: **Blastema** (sheets of small blue cells), **Stroma** (fibrocytic or myxoid), and **Epithelium** (abortive tubules/glomeruli) [1]. * **Prognosis:** The most important prognostic factor is **histology** (presence of anaplasia indicates poor prognosis). * **Associated Syndromes:** 1. **WAGR:** 11p13 deletion (WT1) [1]. 2. **Denys-Drash Syndrome:** WT1 mutation (presents with gonadal dysgenesis and early-onset nephropathy). 3. **Beckwith-Wiedemann Syndrome:** 11p15.5 (WT2), characterized by macroglossia, organomegaly, and hemihypertrophy. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 487-490.

Question 1

What is the pathological feature observed on renal biopsy in Wegener's granulomatosis?

Accepted Answer

Focal necrotising glomerulonephritis

Answer

Nodular glomerulosclerosis

Answer

Granulomas in the vascular wall

Answer

Granulomas in the parenchyma of the kidney

Question 2

What is the characteristic feature seen in Wegener's granulomatosis?

Accepted Answer

Focal necrotizing glomerulonephritis

Answer

Granuloma in the vessel wall

Answer

Nodular glomerulosclerosis

Answer

Interstitial granuloma

Question 3

Which of the following is NOT a cause of granular contracted kidney?

Accepted Answer

Diabetes mellitus

Answer

Chronic pyelonephritis

Answer

Benign nephrosclerosis

Answer

Chronic glomerulonephritis

Question 4

What is the pathological feature observed on renal biopsy in Wegener's granulomatosis?

Accepted Answer

Focal necrotizing glomerulonephritis

Answer

Nodular glomerulosclerosis

Answer

Granulomas in the vascular wall

Answer

Granuloma of parenchyma of kidney

Question 5

What is the commonest cause of renal papillary necrosis?

Accepted Answer

Diabetes mellitus

Answer

Analgesic nephropathy

Answer

Pyelonephritis

Answer

Sickle cell anemia

Question 6

What is the most common cause of glomerulonephritis?

Accepted Answer

Berger disease

Answer

Acute glomerulonephritis

Answer

Chronic glomerulonephritis

Answer

Focal segmental glomerulosclerosis

Question 7

Adult polycystic kidney disease is characterized by which mode of inheritance?

Accepted Answer

Autosomal dominant

Answer

Autosomal recessive

Answer

X-linked

Answer

Mitochondrial

Question 8

Following circulatory shock, which of the following occurs?

Accepted Answer

Acute tubular necrosis

Answer

Acute papillary necrosis

Answer

Both of the above

Answer

None of the above

Question 9

Michael Guttmann bodies are seen in which of the following conditions?

Accepted Answer

Malakoplakia

Answer

Nail Patella syndrome

Answer

Leukoplakia

Answer

Pyelonephritis

Question 10

Which of the following statements regarding Wilms tumor is true?

Accepted Answer

It is associated with deletion of chromosome 11p13.

Answer

It is common in adults.

Answer

It is associated with the MIC 2 gene.

Answer

Its commonest presentation is hematuria.

Renal Pathology — MCQs

Renal Pathology — MCQs

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