Renal Pathology Practice Questions

Q: What is the diagnosis for pulmonary hypoplasia associated with uropathy?

Potter syndrome. **Explanation:** **Potter Syndrome (Correct Answer):** Potter syndrome (or Potter sequence) refers to a constellation of physical findings caused by **oligohydramnios** (low amniotic fluid). In the context of uropathy—such as bilateral renal agenesis, posterior urethral valves, or polycystic kidney disease—the fetus fails to excrete urine into the amniotic sac. Amniotic fluid is essential for lung development; it stretches the airways and provides the necessary pressure for alveolar growth. Its absence leads to **pulmonary hypoplasia**, which is the most common cause of death in these neonates. Characteristic physical features include flattened "Potter facies" (low-set ears, flattened nose, recessed chin) and limb deformities due to uterine compression. **Incorrect Options:** * **Patau Syndrome (Trisomy 13):** This is a chromosomal anomaly characterized by midline defects such as holoprosencephaly, cleft lip/palate, polydactyly, and microphthalmia. While renal cysts can occur, it is not the primary cause of pulmonary hypoplasia via uropathy. * **Pehe Disease:** This is an obsolete term sometimes historically associated with infantile scurvy or specific metabolic bone conditions; it has no clinical relevance to renal-pulmonary pathology. **High-Yield Clinical Pearls for NEET-PG:** * **The "Sequence" Concept:** Potter syndrome is technically a *sequence*, where a single primary trigger (renal failure/leakage) leads to a cascade of secondary effects [1]. * **Mnemonic (POTTER):** **P**ulmonary hypoplasia, **O**ligohydramnios, **T**wisted face, **T**wisted skin, **E**xtremity defects, **R**enal failure. * **Most common cause:** Bilateral renal agenesis is the classic cause, but obstructive uropathy (like Posterior Urethral Valves) is a frequent trigger in males. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 460-462.

Q: What is the most common viral infection in the kidney?

Cytomegalovirus (CMV). **Explanation:** **Cytomegalovirus (CMV)** is the most common viral infection affecting the kidney, particularly in immunocompromised individuals and renal transplant recipients. In the kidney, CMV typically infects the **tubular epithelial cells**. Histologically, it is characterized by the presence of large, eosinophilic intranuclear inclusions surrounded by a clear halo, giving the classic **"Owl’s eye" appearance**. It can lead to tubulointerstitial nephritis and is a significant cause of graft dysfunction in transplant patients. **Analysis of Incorrect Options:** * **Epstein-Barr virus (EBV):** While EBV is associated with Post-Transplant Lymphoproliferative Disorder (PTLD) which can involve the kidney, it is not the most common primary viral infection of the renal parenchyma itself. * **Herpes simplex virus (HSV):** HSV rarely involves the kidney; it more commonly causes mucocutaneous lesions or encephalitis. Renal involvement is usually seen only in cases of severe disseminated viremia. * **Hepatitis B virus (HBV):** HBV is a major cause of **secondary glomerular diseases**, most notably **Membranous Nephropathy (MN)**, but it is not the most common direct viral infection of the kidney tissue. **High-Yield Pearls for NEET-PG:** * **BK Virus:** Another high-yield virus in renal pathology. It causes **BK Virus-associated Nephropathy (BKVAN)** in transplant patients, characterized by "decoy cells" in urine and intranuclear inclusions (Basophilic/Homogenous) that can mimic CMV [1]. * **HIV:** Associated with **HIV-Associated Nephropathy (HIVAN)**, which typically presents as a collapsing variant of Focal Segmental Glomerulosclerosis (FSGS). * **HCV:** Strongly associated with **Cryoglobulinemic Membranoproliferative Glomerulonephritis (MPGN)**. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 935-937.

Q: Which of the following is NOT a typical electron microscopic feature of Alport syndrome?

Thickening of the renal tubules. **Explanation:** Alport syndrome is a hereditary type IV collagen disorder (most commonly X-linked) caused by mutations in the **COL4A3, COL4A4, or COL4A5** genes [1]. These genes encode the alpha chains of type IV collagen, which is a structural hallmark of the **Glomerular Basement Membrane (GBM)**, cochlea, and lens. **Why Option D is correct:** The primary pathology in Alport syndrome is localized to the **basement membranes of the glomeruli**, not the renal tubules. While chronic disease may lead to secondary tubulointerstitial fibrosis, "thickening of the renal tubules" is not a diagnostic or typical electron microscopic (EM) feature of the condition [1]. **Why other options are incorrect:** * **Options A, B, and C:** These represent the classic EM triad of Alport syndrome. The GBM initially appears thin but progresses to show **irregular thickening** and thinning. The hallmark feature is the longitudinal splitting and **multilamellation of the lamina densa**, which creates a characteristic **"basket-weave" appearance**. These findings are pathognomonic for the disease. **High-Yield Clinical Pearls for NEET-PG:** * **Clinical Triad:** Hereditary nephritis (hematuria/ESRD), Sensorineural deafness, and Ocular defects (e.g., Anterior lenticonus) [1]. * **Genetics:** 80% are X-linked dominant (COL4A5) [1]. * **Light Microscopy:** Early stages show no specific changes; later stages show focal segmental glomerulosclerosis (FSGS) and interstitial foam cells (lipid-laden macrophages) [1]. * **Immunofluorescence:** Characteristically shows a "negative" or "absent" staining for Type IV collagen alpha chains [1]. * **Differential:** Thin Basement Membrane Disease (Benign Familial Hematuria) shows only diffuse thinning of the GBM without the basket-weave pattern. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 929-930.

Question 1

What is the diagnosis for pulmonary hypoplasia associated with uropathy?

Accepted Answer

Potter syndrome

Answer

Patau syndrome

Answer

Pehe disease

Answer

All of the above

Question 2

Distal and collecting duct cysts are seen in which condition?

Accepted Answer

Autosomal Recessive Polycystic Kidney Disease (ARPKD)

Answer

Autosomal Dominant Polycystic Kidney Disease (ADPKD)

Answer

Tuberous Sclerosis

Answer

Nephronophthisis

Question 3

Which of the following is NOT true about the condition shown?

Accepted Answer

Low Renin due to pressure atrophy of Juxtaglomerular apparatus

Answer

Cysts increase in size with age

Answer

Spider leg deformity

Answer

Anemia

Question 4

What is the characteristic pathological finding in the kidney in malignant hypertension?

Accepted Answer

Flea-bitten kidney

Answer

Irregular granular contracted kidney

Answer

Large white kidney

Answer

No change in the kidney

Question 5

What is the most common viral infection in the kidney?

Accepted Answer

Cytomegalovirus (CMV)

Answer

Epstein-Barr virus (EBV)

Answer

Herpes simplex virus (HSV)

Answer

Hepatitis B virus (HBV)

Question 6

The characteristic feature of benign nephrosclerosis is

Accepted Answer

Leather grain appearance

Answer

Flea bitten kidney

Answer

Onion skin appearance

Answer

Hyperplastic arteriosclerosis

Question 7

Papillary necrosis is seen in all the following conditions except:

Accepted Answer

Chronic alcoholism

Answer

Sickle cell anemia

Answer

Analgesic nephropathy

Answer

Diabetes mellitus

Question 8

Diabetic glomerulosclerosis is characterized by all of the following EXCEPT:

Accepted Answer

Fibrin clot

Answer

Diffuse glomerulosclerosis

Answer

Fibrin cap

Answer

Kimmelstiel-Wilson lesions

Question 9

Which of the following is NOT a typical electron microscopic feature of Alport syndrome?

Accepted Answer

Thickening of the renal tubules

Answer

Irregular thickening of the glomerular basement membrane (GBM)

Answer

Multilamellation of the lamina densa

Answer

Basket weave pattern of the lamina densa

Question 10

Which glomerular lesion is seen in cases of reflux nephropathy?

Accepted Answer

Focal segmental glomerulosclerosis (FSGS)

Answer

Rapidly progressive glomerulonephritis (RPGN)

Answer

Membranoproliferative glomerulonephritis (MPGN)

Answer

Membranous glomerulonephritis (MGN)

Renal Pathology — MCQs

Renal Pathology — MCQs

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