Renal Pathology Practice Questions

Q: An 8-month-old male infant presents with progressive renal and hepatic failure. Despite intensive medical therapy, the infant dies. At the time of autopsy, the external surfaces of his kidneys are found to be smooth, but the cut section reveals numerous cysts that are lined up in a row. What is the mode of inheritance of this renal abnormality?

Autosomal recessive. The clinical presentation and autopsy findings are classic for **Autosomal Recessive Polycystic Kidney Disease (ARPKD)**. **Why Option B is Correct:** ARPKD typically presents in the neonatal period or infancy. The hallmark gross finding is a **smooth external surface** (unlike the bosselated/nodular surface in the adult form) with a cut section showing **radially arranged, cylindrical/fusiform cysts** that extend from the medulla to the cortex. These cysts are formed by the dilation of collecting ducts [1]. Crucially, ARPKD is almost always associated with **congenital hepatic fibrosis**, leading to the combined renal and hepatic failure seen in this infant. It is caused by mutations in the **PKHD1 gene** on chromosome 6, which encodes **fibrocystin** [1]. **Why Other Options are Incorrect:** * **Option A (Autosomal Dominant):** ADPKD (Adult-onset) usually presents in the 3rd or 4th decade of life. Grossly, the kidneys are massive and have a **multicystic, irregular (bosselated) surface** with no specific radial orientation of cysts. * **Options C & D (X-linked):** While some rare renal syndromes (like Alport syndrome) can be X-linked, the major cystic diseases of the kidney are not inherited via sex chromosomes. **NEET-PG High-Yield Pearls:** * **ARPKD Triad:** Bilateral enlarged smooth kidneys + Congenital Hepatic Fibrosis + Potter sequence (due to oligohydramnios). * **Imaging:** On ultrasound, ARPKD kidneys appear "bright" or echogenic due to numerous small cyst interfaces. * **Genetics:** ADPKD involves **PKD1** (85%, Chromosome 16) or **PKD2** (15%, Chromosome 4). ARPKD involves **PKHD1** (Chromosome 6) [1]. * **Survival:** If the infant survives the neonatal period, the primary long-term complication is portal hypertension due to hepatic fibrosis. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 952-953.

Q: Hereditary nephritis is seen in:

Alport syndrome. **Explanation:** **Alport Syndrome** is the correct answer as it is a classic example of **hereditary nephritis** [1]. It is primarily an X-linked dominant disorder (85% of cases) caused by mutations in the genes encoding the **alpha-chains of Type IV collagen** (specifically COL4A3, COL4A4, and COL4A5) [1]. Type IV collagen is a structural component of the basement membranes in the kidney, ears, and eyes. Clinically, it presents with a triad of progressive hematuria (leading to ESRD), sensorineural hearing loss, and ocular abnormalities (e.g., anterior lenticonus). **Analysis of Incorrect Options:** * **Analgesic Nephropathy:** This is an **acquired** chronic interstitial nephritis caused by the long-term overuse of pain medications (e.g., phenacetin, NSAIDs). It is characterized by renal papillary necrosis. * **Balkan Nephropathy:** This is an **environmentally induced** chronic tubulointerstitial nephritis found in the Danube River basin [3]. It is linked to the ingestion of **aristolochic acid** from seeds of the *Aristolochia clematitis* plant [3]. * **Eosinophilic Nephritis:** This is typically a manifestation of **Acute Interstitial Nephritis (AIN)**, most commonly a hypersensitivity reaction to drugs (e.g., NSAIDs, antibiotics like Penicillin). It is an immunologic, not hereditary, condition. **High-Yield Clinical Pearls for NEET-PG:** * **Electron Microscopy (EM) Finding:** The pathognomonic feature of Alport Syndrome is the **"Basket-weave appearance"** (irregular thickening, thinning, and splitting of the Glomerular Basement Membrane) [1]. * **Thin Basement Membrane Disease (TBMD):** Also known as Benign Familial Hematuria; it is a differential for Alport but has a much better prognosis. * **Goodpasture Syndrome vs. Alport:** While both involve Type IV collagen, Goodpasture is an *autoimmune* attack against the alpha-3 chain [2], whereas Alport is a *genetic* defect [1].

Q: p-ANCA is sensitive and specific for which of the following conditions?

Idiopathic crescentic glomerulonephritis. **Explanation:** **1. Why Option B is Correct:** Idiopathic Crescentic Glomerulonephritis (Type III Pauci-immune RPGN) is characterized by the presence of crescents in the glomeruli without significant immune complex or anti-GBM antibody deposits [1]. Approximately **80% of patients** with this condition are positive for **p-ANCA** (anti-myeloperoxidase) [1]. It is considered a renal-limited form of microscopic polyangiitis. **2. Analysis of Incorrect Options:** * **Option A (PSGN):** This is a Type II (Immune-complex mediated) hypersensitivity reaction. Diagnosis is based on low C3 levels and elevated ASO/anti-DNAse B titers, not ANCA. * **Option C (Diffuse glomerulosclerosis):** This is typically the end-stage of diabetic nephropathy or chronic glomerulonephritis. It is characterized by Kimmelstiel-Wilson nodules and basement membrane thickening, unrelated to ANCA. * **Option D (Wegener's Granulomatosis):** Now known as Granulomatosis with Polyangiitis (GPA), this condition is classically associated with **c-ANCA** (anti-proteinase 3), not p-ANCA. **3. High-Yield Clinical Pearls for NEET-PG:** * **c-ANCA (PR3-ANCA):** Associated with Wegener’s Granulomatosis (GPA). Shows cytoplasmic staining [1]. * **p-ANCA (MPO-ANCA):** Associated with Microscopic Polyangiitis (MPA), Churg-Strauss Syndrome (EGPA), and Idiopathic Crescentic Glomerulonephritis [1]. Shows perinuclear staining. * **Pauci-immune status:** Defined by a Negative Immunofluorescence (IF) for IgG and C3 [1]. * **Crescents:** Formed by the proliferation of parietal epithelial cells and infiltration of monocytes/macrophages into Bowman's space. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 917-918.

Question 1

Which of the following conditions is not related to immunity?

Accepted Answer

Diabetic nephropathy

Answer

Membranoproliferative glomerulonephritis (MPGN)

Answer

Post-streptococcal glomerulonephritis (PSGN)

Answer

IgA nephropathy

Question 2

Pauci-immune glomerulonephritis is seen in which of the following conditions?

Accepted Answer

Microscopic polyangiitis

Answer

Post-transplant glomerulopathy

Answer

Henoch-Schonlein nephritis

Answer

Lupus nephritis

Question 3

An 8-month-old male infant presents with progressive renal and hepatic failure. Despite intensive medical therapy, the infant dies. At the time of autopsy, the external surfaces of his kidneys are found to be smooth, but the cut section reveals numerous cysts that are lined up in a row. What is the mode of inheritance of this renal abnormality?

Accepted Answer

Autosomal recessive

Answer

Autosomal dominant

Answer

X-linked dominant

Answer

X-linked recessive

Question 4

A 40-year-old hypertensive male was admitted to the hospital with sudden onset of headache and altered sensorium. On examination, his blood pressure was observed to be 220/110 mm Hg. The patient died four hours later. What is the likely pathological finding in his kidneys?

Accepted Answer

Small kidney with petechial hemorrhages

Answer

Small kidney with granular surface

Answer

Large kidney with waxy appearance

Answer

Large kidney with granular surface

Question 5

Which of the following is MOST relevant in renal vascular hypertension?

Accepted Answer

Aldosterone

Answer

Renin

Answer

Hypokalemia

Answer

Angiotensin II

Question 6

What is the commonest histological finding in hypertensive nephrosclerosis?

Accepted Answer

Hyaline arteriosclerosis

Answer

Proliferative endarteritis

Answer

Necrotizing arteriolitis

Answer

Cystic medial necrosis

Question 7

Which of the following is NOT a steroid-responsive glomerulonephritis?

Accepted Answer

Membranoproliferative glomerulonephritis

Answer

Post-streptococcal glomerulonephritis

Answer

Minimal change disease

Answer

Focal segmental glomerulosclerosis

Question 8

Hereditary nephritis is seen in:

Accepted Answer

Alport syndrome

Answer

Analgesic nephropathy

Answer

Balkan nephropathy

Answer

Eosinophilic nephritis

Question 9

Minimal change glomerulopathy may be seen in association with all of the following except?

Accepted Answer

Hepatitis B

Answer

HIV

Answer

Drug-induced interstitial nephritis

Answer

Hodgkin's disease

Question 10

p-ANCA is sensitive and specific for which of the following conditions?

Accepted Answer

Idiopathic crescentic glomerulonephritis

Answer

Post-streptococcal glomerulonephritis

Answer

Diffuse glomerulosclerosis

Answer

Wegener's granulomatosis

Renal Pathology — MCQs

Renal Pathology — MCQs

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