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.

Question 1

Which of the following is not a non-proliferative glomerulonephritis?

Accepted Answer

Mesangiocapillary glomerulonephritis

Answer

Membranous glomerulonephritis

Answer

Diabetic glomerulosclerosis

Answer

Amyloidosis

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

Mesangial deposits of Lambda chain are seen in which condition?

Accepted Answer

Amyloidosis

Answer

Focal segmental glomerulosclerosis

Answer

Membranoproliferative glomerulonephritis

Answer

Membranous nephropathy

Question 6

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

Accepted Answer

Aldosterone

Answer

Renin

Answer

Hypokalemia

Answer

Angiotensin II

Question 7

Which of the following is seen in nephrotic syndrome?

Accepted Answer

Low serum calcium

Answer

Raised AT–III

Answer

Low lipid

Answer

Platelet activation

Question 8

What is the commonest histological finding in hypertensive nephrosclerosis?

Accepted Answer

Hyaline arteriosclerosis

Answer

Proliferative endarteritis

Answer

Necrotizing arteriolitis

Answer

Cystic medial necrosis

Question 9

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 10

Which of the following proteins is most commonly associated with steroid-resistant nephrotic syndrome?

Accepted Answer

Podocin

Answer

Nephrin

Answer

Alpha-actinin-4

Answer

Transient Receptor Potential 6

Renal Pathology — MCQs

Renal Pathology — MCQs

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