Renal Pathology Practice Questions

Q: Alport syndrome is associated with a defect in which type of collagen?

Collagen type IV. **Explanation:** **Alport Syndrome** is a hereditary nephritis caused by mutations in the genes encoding the **Type IV collagen** chains (specifically α3, α4, or α5) [1]. Type IV collagen is a crucial structural component of the **basement membranes**, particularly in the glomeruli, cochlea, and lens of the eye [1]. The defect leads to thinning and splitting of the Glomerular Basement Membrane (GBM), classically described as a **"basket-weave appearance"** on electron microscopy. **Analysis of Options:** * **Option B (Correct):** Type IV collagen is the "Basement Membrane Collagen." Mutations (most commonly X-linked) lead to the clinical triad of progressive hematuria (renal failure), sensorineural hearing loss, and ocular defects (e.g., anterior lenticonus) [1]. * **Option A:** **Type I collagen** is found in bone, skin, and tendons. Defects here lead to **Osteogenesis Imperfecta**. * **Option C:** **Type III collagen** (Reticulin) is found in blood vessels and fetal skin. Defects are associated with the vascular type of **Ehlers-Danlos Syndrome**. * **Option D:** **Type VII collagen** forms anchoring fibrils. Defects lead to **Dystrophic Epidermolysis Bullosa**. **High-Yield Clinical Pearls for NEET-PG:** 1. **Inheritance:** Most common is **X-linked Dominant** (COL4A5 mutation) [1]. 2. **Electron Microscopy (EM):** The gold standard for diagnosis; shows irregular thickening, thinning, and **lamellation** (splitting) of the lamina densa. 3. **Ocular Sign:** **Anterior lenticonus** is pathognomonic for Alport Syndrome. 4. **Goodpasture Syndrome Connection:** Patients with Alport syndrome who receive a kidney transplant may develop anti-GBM antibodies against the "new" Type IV collagen, leading to post-transplant glomerulonephritis. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 929-930.

Q: What is the commonest site of metastasis for Wilms' tumour?

Lungs. **Explanation:** Wilms’ tumour (Nephroblastoma) is the most common primary renal malignancy in children. The correct answer is **Lungs** because Wilms’ tumour typically spreads via the **hematogenous route**. Once the tumour cells enter the renal vein and inferior vena cava, the first major capillary bed they encounter is in the pulmonary circulation, making the lungs the most frequent site of distant spread [1]. **Analysis of Options:** * **B. Lungs (Correct):** Approximately 80% of patients with metastatic disease at diagnosis will have pulmonary involvement. These often appear as "cannonball" metastases on a chest X-ray. * **A. Bones:** Unlike Neuroblastoma (the main differential diagnosis), Wilms’ tumour rarely metastasizes to the bones. Bone pain or lesions should prompt a clinician to consider Clear Cell Sarcoma of the Kidney (CCSK) instead. * **C. Liver:** The liver is the second most common site of distant metastasis, but it occurs less frequently than pulmonary spread. * **D. Brain:** Brain metastasis is extremely rare in Wilms’ tumour and usually only occurs in the very late stages of aggressive or relapsed disease. **High-Yield Clinical Pearls for NEET-PG:** * **Triphasic Morphology:** On histology, Wilms’ tumour classically shows three components: Blastemal, Stromal, and Epithelial cells. * **WAGR Syndrome:** Associated with *WT1* gene deletion (Wilms’ tumour, Aniridia, Genitourinary anomalies, and intellectual disability/Retardation). * **Beckwith-Wiedemann Syndrome:** Associated with *WT2* gene (imprinting defect), characterized by macroglossia, organomegaly, and hemihypertrophy. * **Staging:** Unlike many other tumours, the presence of lung metastasis in Wilms' tumour classifies it as **Stage IV**. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 492-493.

Question 1

Alport syndrome is associated with a defect in which type of collagen?

Accepted Answer

Collagen type IV

Answer

Collagen type I

Answer

Collagen type III

Answer

Collagen type VII

Question 2

Which of the following statements is not true regarding IgA nephropathy (Berger's disease)?

Accepted Answer

Absence of associated proteinuria is pathognomonic.

Answer

The pathological changes are proliferative and usually confined to mesangial cells; usually focal and segmental.

Answer

Hematuria may be gross or microscopic.

Answer

On immunofluorescence, deposits contain both IgA and IgG.

Question 3

A 35-year-old man with AML and WBC of 100,000 cells/µL is treated with chemotherapy and develops oliguric renal failure. His urine is acidic and numerous crystals are noted in the urine. What is the most likely diagnosis?

Accepted Answer

Urate nephropathy

Answer

Nephrocalcinosis

Answer

Leukemic infiltration of the kidneys

Answer

Acute tubular necrosis secondary to a nephrotoxic effect of his chemotherapy

Question 4

Polycythemia is seen with which tumor?

Accepted Answer

Renal cell carcinoma

Answer

Endometrial carcinoma

Answer

Lung carcinoma

Answer

Fibrosarcoma

Question 5

What is the most common kidney disease associated with chronic heroin use?

Accepted Answer

Focal segmental glomerulosclerosis

Answer

IgA nephropathy

Answer

Lipoid nephrosis

Answer

Mesangioproliferative GN

Question 6

What is the commonest site of metastasis for Wilms' tumour?

Accepted Answer

Lungs

Answer

Bones

Answer

Liver

Answer

Brain

Question 7

Which of the following is seen in nephrotic syndrome?

Accepted Answer

Low serum calcium

Answer

Raised antithrombin III

Answer

Low lipids

Answer

Platelet activation

Question 8

Which chromosomal mutation is characteristic of low-grade transitional cell carcinoma of the bladder?

Accepted Answer

FGFR3

Answer

p53

Answer

Rb gene

Answer

HRAS

Question 9

Squamous cell carcinoma of the bladder is predisposed by which of the following factors?

Accepted Answer

Persistent urachus

Answer

Urolithiasis

Answer

Schistosomiasis

Answer

Smoking

Question 10

Papillary necrosis is most commonly seen in?

Accepted Answer

Analgesic nephropathy

Answer

Diabetes Mellitus

Answer

Sickle cell anemia

Answer

Acute pyelonephritis

Renal Pathology — MCQs

Renal Pathology — MCQs

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