Renal Pathology Practice Questions

Q: Most specific test for diagnosing Fabry disease on kidney biopsy?

Electron microscopy. ***Electron microscopy*** - **Electron microscopy** is the most specific test as it can visualize the characteristic **lamellated zebra bodies** (lysosomal inclusions of globotriaosylceramide) in various cell types, including podocytes, which are pathognomonic for Fabry disease. - While other stains might show lipid accumulation, EM provides the definitive ultrastructural evidence by identifying the specific morphology of the accumulated glycosphingolipids. *Silver stain* - **Silver stains** are primarily used to highlight **reticular fibers**, basement membranes, or certain microorganisms, and are not specific for the lipid inclusions seen in Fabry disease. - They would not differentiate Fabry inclusions from other forms of cellular deposits or normal cellular components. *H&E stain* - **Hematoxylin and Eosin (H&E) stain** is a general histological stain that can show enlarged podocytes or vacuolization in Fabry disease, but these findings are **non-specific** and can be seen in other conditions. - H&E does not specifically highlight the characteristic lysosomal lipid inclusions. *PAS stain* - **Periodic Acid-Schiff (PAS) stain** detects **carbohydrates** and mucosubstances and may stain some of the accumulated glycosphingolipids with variable intensity. - However, PAS staining is not specific for the diagnosis of Fabry disease, as other conditions can also show PAS-positive material, and it does not reveal the characteristic lamellated structure of the inclusions.

Q: Characteristic feature of membranous nephropathy on electron microscopy?

Subepithelial deposits. ***Subepithelial deposits*** - The presence of **subepithelial immune complex deposits** is the hallmark feature differentiating **membranous nephropathy** from other glomerular diseases on electron microscopy [1]. - These deposits are located between the **glomerular basement membrane (GBM)** and the podocytes, triggering localized inflammation and thickening of the GBM [1]. *No deposits* - While some conditions like **minimal change disease** may show no visible deposits on electron microscopy, this finding is inconsistent with **membranous nephropathy**, which is defined by immune complex deposition. - This condition is characterized by diffuse effacement of **podocyte foot processes** but lacks organized immune deposits [1]. *Mesangial deposits* - **Mesangial deposits** are characteristic of conditions like **IgA nephropathy** or early stages of lupus nephritis, where immune complexes primarily accumulate within the mesangium, the central part of the glomerulus [1]. - This location is distinct from the **subepithelial space** seen in membranous nephropathy, where deposits are outside the capillaries, beneath the podocytes [1]. *Subendothelial deposits* - **Subendothelial deposits** are typically found in diseases such as **lupus nephritis (Class III or IV)** or **membranoproliferative glomerulonephritis (Type I)**, where immune complexes are situated between the endothelium and the glomerular basement membrane [1]. - This location is anatomically distinct from the **subepithelial deposits** characteristic of membranous nephropathy [1]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 911, 921.

Q: A patient with diabetes presents with kidney dysfunction and nodular glomerulosclerosis. What is the underlying process?

Mesangial expansion. ***Mesangial expansion*** - Nodular glomerulosclerosis, characteristic of **Kimmelstiel-Wilson lesions** in diabetic nephropathy, is primarily due to the **expansion of the mesangial matrix** and cells [1]. - This mesangial expansion leads to the formation of discrete, PAS-positive, and typically pericapillary nodules [1]. *Basement membrane thickening* - While **glomerular basement membrane (GBM) thickening** is a hallmark of diabetic nephropathy, it typically occurs early and diffusely [2]. - It is a precursor to more advanced changes but does not directly explain the formation of the distinct **nodular lesions** seen in advanced stages [2]. *Amyloid deposition* - **Amyloid deposition** can cause kidney dysfunction and enlargement, but it is a distinct pathological process characterized by the basis of accumulation of misfolded proteins. - It typically presents with a different microscopic appearance (e.g., Congo red positivity, apple-green birefringence) and is not the primary cause of nodular glomerulosclerosis in diabetes. *Hyaline arteriosclerosis* - **Hyaline arteriosclerosis** affects the afferent and efferent arterioles in the kidney, often seen in hypertension and diabetes, and contributes to renal ischemia and capillary damage [3]. - However, it represents damage to the **arterioles** and not directly the formation of the **nodular lesions within the glomeruli** [1]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Endocrine System, pp. 1121-1122. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Endocrine System, p. 1121. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 907-908.

Q: Which renal pathology is characterized by crescent formation in the glomeruli?

Rapidly progressive glomerulonephritis. ***Rapidly progressive glomerulonephritis*** - This condition is histologically defined by the presence of **crescent formation** in a significant number of glomeruli [1]. - **Crescents** are formed by the proliferation of parietal epithelial cells, inflammatory cells, and fibrin within Bowman's space, indicating severe glomerular injury [1]. *Focal segmental glomerulosclerosis* - This pathology is characterized by **segmental sclerosis** and hyalinosis of some glomeruli, rather than widespread crescent formation. - It often leads to **nephrotic syndrome** and can progress to end-stage renal disease, but crescents are not its defining feature. *Membranous glomerulonephritis* - Characterized by diffuse thickening of the **glomerular basement membrane** due to immune complex deposition, often appearing as "spikes" and "domes" on electron microscopy. - It typically presents as **nephrotic syndrome** and does not primarily involve crescent formation. *Diffuse proliferative glomerulonephritis* - This condition involves diffuse proliferation of **endothelial and mesangial cells** within the glomeruli, often seen in post-streptococcal glomerulonephritis [1]. - While it can be severe, **crescent formation** is not its defining or most characteristic feature, usually being focal if present. **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. 528-529.

Q: A kidney biopsy from a patient with hematuria and proteinuria shows segmental sclerosis of the glomeruli. What is the most likely diagnosis?

Focal Segmental Glomerulosclerosis. ***Focal Segmental Glomerulosclerosis*** - Segmental sclerosis of the glomeruli is a defining characteristic of focal segmental glomerulosclerosis (FSGS) [1][2]. - It commonly presents with **hematuria** and **proteinuria**, making it the most likely diagnosis in this case [1][3]. *Minimal Change Disease* - Typically presents with **nephrotic syndrome** and does not show segmental sclerosis on biopsy [3][4]. - It is more common in children and is characterized by **foot process fusion** rather than scarring [3][4]. *IgA Nephropathy* - Often associated with **IgA deposition** in the mesangium and usually presents with **episodic hematuria** rather than segmental sclerosis. - There may be some proteinuria, but it is generally less prominent compared to FSGS. *Membranous Nephropathy* - Characterized by **thickening of the glomerular basement membrane** without segmental sclerosis [2][3]. - Presents often with **nephrotic syndrome** and is associated with **anti-PLA2R antibodies**, which are not indicated here [3]. **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. 530-531. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 531-532. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 927-928. [4] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 922-923.

Q: A 5-year-old child presents with a large abdominal mass. Histological examination reveals small, round blue cells. Which tumor is consistent with these findings?

Neuroblastoma. ***Wilms tumor*** - This tumor commonly presents as a **large abdominal mass** in children, especially around the age of 5 [1][2]. - Histological examination typically shows **small, round blue cells**, characteristic of this tumor type [1]. *Hepatoblastoma* - Usually presents in younger children with **liver involvement**, characterized by a **different histological pattern**, such as embryonal liver cells [2]. - It often shows **elevated alpha-fetoprotein (AFP)** levels, which are not mentioned in this case. *Rhabdomyosarcoma* - This tumor is associated with **soft tissue masses**, generally arises in older children and typically shows striated muscle differentiation. - While it can present with small, round blue cells histologically, it has a **different anatomical distribution** compared to Wilms tumor. *Neuroblastoma* - Commonly found in the **adrenal glands** or sympathetic chain, it can present as an abdominal mass but usually shows **neuroblastic differentiation** histologically, not just small blue cells [2]. - Often associated with **elevated catecholamines** and may spread to the bones or lymph nodes, rather than primarily presenting as a renal mass. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 488-490. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. (Basic Pathology) introduces the student to key general principles of pathology, both as a medical science and as a clinical activity with a vital role in patient care. Part 2 (Disease Mechanisms) provides fundamental knowledge about the cellular and molecular processes involved in diseases, providing the rationale for their treatment. Part 3 (Systematic Pathology) deals in detail with specific diseases, with emphasis on the clinically important aspects., pp. 211-212.

Q: What type of glomerulonephritis is associated with systemic lupus erythematosus and often involves "wire-loop" lesions?

Diffuse proliferative glomerulonephritis. ***Diffuse proliferative glomerulonephritis*** - This is the most common and severe form of **lupus nephritis**, often leading to significant renal damage [1]. - Presence of **"wire-loop" lesions** on light microscopy, representing subendothelial immune complex deposits, is a classic histopathologic feature [1]. *Focal segmental glomerulosclerosis* - Characterized by **scarring of some glomeruli** and only a portion of the glomerular tuft, not a global process [2]. - While it can be secondary to lupus nephritis, it does not typically present with "wire-loop" lesions and is not the primary form of active lupus nephritis with systemic involvement. *Membranous glomerulonephritis* - Identified by **subepithelial immune complex deposits** that can cause thickening of the glomerular basement membrane and a "spike and dome" appearance on microscopy [2]. - Though it can occur in lupus nephritis (Class V), it is typically associated with different pathological features than the "wire-loop" lesions of proliferative types [2]. *Minimal change disease* - Characterized by the **absence of significant changes** on light microscopy, with only effacement of podocyte foot processes on electron microscopy [2]. - This condition is the most common cause of **nephrotic syndrome in children** and is rarely associated with systemic lupus erythematosus [2]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of the Immune System, pp. 230-232. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 919-921.

Question 1

Most specific test for diagnosing Fabry disease on kidney biopsy?

Accepted Answer

Electron microscopy

Answer

Silver stain

Answer

H&E stain

Answer

PAS stain

Question 2

Characteristic feature of membranous nephropathy on electron microscopy?

Accepted Answer

Subepithelial deposits

Answer

No deposits

Answer

Mesangial deposits

Answer

Subendothelial deposits

Question 3

A patient with diabetes presents with kidney dysfunction and nodular glomerulosclerosis. What is the underlying process?

Accepted Answer

Mesangial expansion

Answer

Basement membrane thickening

Answer

Amyloid deposition

Answer

Hyaline arteriosclerosis

Question 4

Which renal pathology is characterized by crescent formation in the glomeruli?

Accepted Answer

Rapidly progressive glomerulonephritis

Answer

Focal segmental glomerulosclerosis

Answer

Membranous glomerulonephritis

Answer

Diffuse proliferative glomerulonephritis

Question 5

A young woman with systemic lupus erythematosus (SLE) presents with nephrotic syndrome. Which renal pathology is most commonly associated with SLE?

Accepted Answer

Diffuse proliferative glomerulonephritis

Answer

Membranous nephropathy

Answer

Focal segmental glomerulosclerosis

Answer

Minimal change disease

Question 6

A kidney biopsy from a patient with hematuria and proteinuria shows segmental sclerosis of the glomeruli. What is the most likely diagnosis?

Accepted Answer

Focal Segmental Glomerulosclerosis

Answer

Minimal Change Disease

Answer

Membranous Nephropathy

Answer

IgA Nephropathy

Question 7

A 5-year-old child presents with a large abdominal mass. Histological examination reveals small, round blue cells. Which tumor is consistent with these findings?

Accepted Answer

Neuroblastoma

Answer

Hepatoblastoma

Answer

Rhabdomyosarcoma

Answer

Wilms tumor

Question 8

A 40-year-old woman with a history of lupus presents with swelling and proteinuria. A kidney biopsy shows thickened glomerular capillary walls and subepithelial deposits. What is the most likely diagnosis?

Accepted Answer

Membranous nephropathy

Answer

Focal segmental glomerulosclerosis

Answer

Minimal change disease

Answer

IgA nephropathy

Question 9

A 55-year-old woman with a history of lupus presents with hematuria and nephrotic syndrome. Her renal biopsy shows 'wire-loop' glomerular lesions. What is the primary pathogenesis behind this finding?

Accepted Answer

Deposition of immune complexes in the subendothelial space

Answer

Deposition of IgA in the mesangium

Answer

Hyperfiltration of the glomeruli

Answer

Formation of glomerular crescents

Question 10

What type of glomerulonephritis is associated with systemic lupus erythematosus and often involves "wire-loop" lesions?

Accepted Answer

Diffuse proliferative glomerulonephritis

Answer

Focal segmental glomerulosclerosis

Answer

Membranous glomerulonephritis

Answer

Minimal change disease

Renal Pathology — MCQs

Renal Pathology — MCQs

On this page

Practice by Chapter

Want unlimited practice?