Renal Pathology Practice Questions

Q: A 51-year-old cancer patient undergoing chemotherapy presents with clinical features of a urinary tract infection, including fever with rigors and flank pain. Urine examination reveals variable amounts of protein, leukocytes, and a few RBCs. Urine culture shows E. coli. Bladder wash shows characteristic polygonal granular cells and Michaelis-Gutmann bodies. Which of the following conditions is associated with these features?

Malakoplakia. Explanation: 1. Why Malakoplakia is Correct: Malakoplakia is a rare chronic inflammatory condition, most commonly affecting the urinary bladder, often in immunocompromised patients (like those on chemotherapy). It results from the defective phagocytic killing of bacteria (usually E. coli) by macrophages. The hallmark histological finding is the presence of von Hansemann cells (large, polygonal granular macrophages) containing Michaelis-Gutmann bodies. These bodies are pathognomonic laminated calcified inclusions (iron and calcium deposits) formed due to the incomplete digestion of bacterial components. 2. Why Other Options are Incorrect: * Xanthogranulomatous pyelonephritis (XPN): While also a chronic inflammatory response to infection (often Proteus or E. coli), it is characterized by "foamy" lipid-laden macrophages and is typically associated with staghorn calculi and a "bear paw" appearance on CT. It lacks Michaelis-Gutmann bodies. * Nail-patella syndrome: A genetic disorder (LMX1B mutation) characterized by nail dysplasia, absent/hypoplastic patellae, and iliac horns. Renal involvement manifests as nephrotic syndrome with basement membrane thickening, not inflammatory bodies. * Xanthelasma: These are localized lipid deposits (xanthomas) typically found on the eyelids, associated with hyperlipidemia, and have no relation to urinary tract pathology. Clinical Pearls for NEET-PG: * Pathognomonic sign: Michaelis-Gutmann bodies (PAS positive, Von Kossa positive for calcium). * Common Organism: Escherichia coli (80% of cases). * Key Association: Immunosuppression or chronic debilitating diseases. * Staining: Michaelis-Gutmann bodies stain positive for Iron (Prussian blue) and Calcium (Von Kossa).

Q: Which of the following renal cystic diseases is autosomal recessive?

Childhood polycystic kidney disease. **Explanation:** The classification of renal cystic diseases is a high-yield topic for NEET-PG, primarily distinguished by their inheritance patterns and clinical presentation. **1. Why Option B is Correct:** **Childhood Polycystic Kidney Disease (ARPKD)** is inherited in an **autosomal recessive** manner [1]. It is caused by mutations in the **PKHD1 gene** on chromosome 6, which encodes **fibrocystin** [1]. Pathologically, it is characterized by bilateral, symmetrical enlargement of kidneys with small, elongated, cylindrical cysts arranged radially in the cortex and medulla, giving the kidney a "sponge-like" appearance. It is almost always associated with **congenital hepatic fibrosis** [1]. **2. Why Other Options are Incorrect:** * **Adult Polycystic Kidney Disease (ADPKD):** As the name suggests, it follows an **autosomal dominant** inheritance (mutations in PKD1 or PKD2 genes) [1]. It presents later in life with large, multicystic kidneys. * **Medullary Sponge Kidney:** This is typically a **sporadic** condition, not inherited. It involves cystic dilatations of the collecting ducts in the renal papillae. * **Adult-onset Medullary Cystic Disease (Nephronophthisis-Medullary Cystic Disease Complex):** The adult-onset form (MCKD) is inherited in an **autosomal dominant** pattern, whereas the juvenile form (Nephronophthisis) is autosomal recessive [1]. **Clinical Pearls for NEET-PG:** * **ARPKD Triad:** Bilateral renal cysts + Congenital hepatic fibrosis + Potter sequence (due to oligohydramnios). * **ADPKD Associations:** Berry aneurysms (Circle of Willis), hepatic cysts, and Mitral Valve Prolapse (MVP). * **Imaging:** In ARPKD, ultrasound shows a "salt and pepper" appearance due to tiny cysts. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 950-955.

Q: Mutation in the PKHD1 gene causing autosomal recessive polycystic kidney disease (ARPKD) maps to which chromosome?

Chromosome 6. **Explanation:** The correct answer is **Chromosome 6**. Autosomal Recessive Polycystic Kidney Disease (ARPKD) is primarily caused by mutations in the **PKHD1** (Polycystic Kidney and Hepatic Disease 1) gene, which is located on the short arm of **Chromosome 6 (6p12)**. This gene encodes **fibrocystin** (also known as polyductin), a protein localized in the primary cilia of epithelial cells, which is essential for maintaining the structural integrity of the renal collecting ducts and bile ducts. **Analysis of Incorrect Options:** * **Option A (Chromosome 16):** This is the location of the **PKD1** gene [1]. Mutations here cause the most common form of Autosomal Dominant Polycystic Kidney Disease (ADPKD Type 1), accounting for ~85% of cases [1]. * **Option B (Chromosome 4):** This is the location of the **PKD2** gene [1]. Mutations here cause ADPKD Type 2, which generally has a slower progression to end-stage renal disease compared to PKD1 [1]. * **Option D (Chromosome 12):** This chromosome is not associated with the primary genes for polycystic kidney disease. **High-Yield Clinical Pearls for NEET-PG:** * **ARPKD Presentation:** Typically presents in the neonatal period with bilateral enlarged kidneys and pulmonary hypoplasia (due to oligohydramnios—part of the **Potter Sequence**). * **Liver Involvement:** ARPKD is invariably associated with **Congenital Hepatic Fibrosis**. On imaging, this may present as Caroli’s disease (dilatation of intrahepatic bile ducts). * **Imaging Sign:** On ultrasound, ARPKD kidneys appear large and echogenic with a loss of corticomedullary differentiation, often described as a **"salt and pepper"** appearance. * **Genetics Tip:** Remember **"6-PKHD"** (6 letters in PKHD) to link it to Chromosome 6.

Question 1

Which of the following statements about IgA nephropathy is false?

Accepted Answer

Complement levels are typically decreased

Answer

Characterized by mesangial proliferation

Answer

Also known as Berger's nephropathy

Answer

Associated with recurrent hematuria

Question 2

Glomerulonephritis associated with sensory neuronal deafness is seen in which condition?

Accepted Answer

Alport syndrome

Answer

Nail patella syndrome

Answer

Down syndrome

Answer

Fabry's disease

Question 3

Sickle cell disease is associated with which type of renal cell carcinoma?

Accepted Answer

Medullary

Answer

Papillary

Answer

Chromophobe

Answer

Collecting duct

Question 4

Kidney biopsy from a child with hemolytic uremic syndrome characteristically most likely presents features of which of the following?

Accepted Answer

Thrombotic microangiopathy

Answer

Proliferative glomerulonephritis

Answer

Focal segmental glomerulosclerosis

Answer

Minimal change disease

Question 5

Which of the following is NOT associated with urinary bladder carcinoma?

Accepted Answer

Human papilloma virus infection

Answer

Smoking

Answer

Schistosomiasis

Answer

Cyclophosphamide

Question 6

Which of the following statements is true about Heymann rat glomerulonephritis?

Accepted Answer

Electron dense deposits are found in the mesangium

Answer

Heymann antigen is called megalin

Answer

Electron dense deposits are found in the subendothelial space

Answer

Electron dense deposits are found on the subepithelial aspect of the basement membrane

Question 7

A 51-year-old cancer patient undergoing chemotherapy presents with clinical features of a urinary tract infection, including fever with rigors and flank pain. Urine examination reveals variable amounts of protein, leukocytes, and a few RBCs. Urine culture shows E. coli. Bladder wash shows characteristic polygonal granular cells and Michaelis-Gutmann bodies. Which of the following conditions is associated with these features?

Accepted Answer

Malakoplakia

Answer

Xanthogranulomatous pyelonephritis

Answer

Nail-patella syndrome

Answer

Xanthelasma

Question 8

Which of the following renal cystic diseases is autosomal recessive?

Accepted Answer

Childhood polycystic kidney disease

Answer

Adult polycystic kidney disease

Answer

Medullary sponge kidney

Answer

Adult onset medullary cystic disease

Question 9

Mutation in the PKHD1 gene causing autosomal recessive polycystic kidney disease (ARPKD) maps to which chromosome?

Accepted Answer

Chromosome 6

Answer

Chromosome 16

Answer

Chromosome 4

Answer

Chromosome 12

Question 10

Cholemic nephrosis is seen in:

Accepted Answer

Obstructive jaundice

Answer

Malaria

Answer

Addison's disease

Answer

Hemochromatosis

Renal Pathology — MCQs

Renal Pathology — MCQs

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