Glomerular Diseases

Question 1

Which genetic mutation is associated with congenital nephrotic syndrome and affects the slit-pore membrane at birth?

A. TRPC6
B. NPHS1
C. APOL1
D. COL4A5

Answer 1

A

Congenital nephrotic syndrome from mutations in NPHS1 (nephrin) and NPHS2 (podocin) affects the slit-pore membrane at birth.”

Question 2

Which gene polymorphism is a major risk factor for nearly 70% of African Americans with nondiabetic end-stage renal disease (ESRD)?

A. NPHS2
B. APOL1
C. TGF-β
D. COL4A3

Answer 2

B

Polymorphisms in the gene encoding apolipoprotein L1, APOL1, are a major risk for nearly 70% of African Americans with nondiabetic endstage renal disease (ESRD), particularly FSGS

Question 3

What is the primary mechanism of injury in Alport’s syndrome?

A. Antibody-mediated attack on type IV collagen
B. Mutations in the α3, α4, or α5 chains of type IV collagen
C. Deposition of circulating immune complexes
D. Deficiency of α-galactosidase A

Answer 3

B

Alport’s syndrome, from mutations in the genes encoding for the α3, α4, or α5 chains of type IV collagen, produces split basement membranes with glomerulosclerosi

Question 4

Which condition is caused by mutations affecting complement pathway regulation and is associated with membranoproliferative glomerulonephritis (MPGN)?

A. Atypical hemolytic-uremic syndrome (aHUS)
B. Focal segmental glomerulosclerosis (FSGS)
C. Minimal change disease
D. Lupus nephritis

Answer 4

A

Mutations in control of the complement pathway increasingly associate with various forms of membranoproliferative glomerulonephritis (MPGN) and C3 glomerulopathies including dense deposit disease, or atypical hemolytic-uremic syndrome (aHUS).

Question 5

What is the primary antigen targeted in Goodpasture’s syndrome?

A. Subepithelial immune deposits
B. α3 NC1 domain of type IV collagen
C. Mesangial immune complexes
D. Complement proteins (C3 and C4)

Answer 5

B

Antiglomerular basement membrane disease producing Goodpasture’s syndrome primarily injures both the lung and kidney because of the narrow distribution of the α3 NC1 domain of type IV collagen that is the target antigen

Question 6

Which immune response mechanism is responsible for damage in poststreptococcal glomerulonephritis?

A. Direct attack by neutrophils
B. Deposition of circulating immune complexes
C. Antibody-mediated activation of the complement system
D. T-cell-mediated cytotoxicity

Answer 6

B

Poststreptococcal glomerulonephritis…typically associated with immune deposits along the GBM

Question 7

Which lysosomal storage disease is associated with focal segmental glomerulosclerosis (FSGS)?

A. Fabry’s disease
B. Minimal change disease
C. Dense deposit disease
D. Alport’s syndrome

Answer 7

A

Lysosomal storage diseases, such as α-galactosidase A deficiency causing Fabry’s disease…produce FSGS

Question 8

Gross hematuria is rare with the exception of ____

Answer 8

IgA Nephropathy
Sickle Cell Disease

Question 9

A mean of 8–10 mg/24 h of ____ appears in the urine in the absence of kidney disease.

Answer 9

albumin

Question 10

Proteinuria of 30–300 mg/24 h

Answer 10

Microalbuminuria

Question 11

Proteinuria of >300mg/24hr

Answer 11

Frank proteinuria

Question 12

_____ >1–2 g/24 h is also commonly associated with glomerular disease.

Answer 12

Sustained proteinuria

Question 13

This class of proteinuria is nonsustained, generally <1 g/24 h

Answer 13

Benign/Functional/Transient Proteinuria

Question 14

Proteinuria only seen with upright posture is called _____proteinuria and has a benign prognosis.

Answer 14

orthostatic

Question 15

Proteinuria in most adults with glomerular disease is _____containing albumin and a mixture of other serum protein

Answer 15

nonselective

Question 16

Some patients with inflammatory glomerular disease, such as_____, have pyuria characterized by the presence of considerable numbers of leukocytes.

Answer 16

acute poststreptococcal glomerulonephritis or MPGN

Question 17

If only large amounts of proteinuria are present without clinical manifestations, the condition is sometimes called _____

Answer 17

nephrotic-range proteinuria.

Question 18

heavy proteinuria (>3.0 g/24 h), h sion, hypercholesterolemia, hypoalbuminemia, edema/anasarca,

Answer 18

Nephrotic Syndrome

Question 19

Children with minimal change disease (MCD), the proteinuria is ____ and composed largely of albumin.

Answer 19

selective

Question 20

_____ producing 1–2 g/24 h of proteinuria, hematuria with red blood cell casts, pyuria, hypertension, fluid retention, and a rise in serum creatinine associated with a reduction in glomerular filtration

Answer 20

Acute nephritic syndrome

Question 21

Patients with a basement membrane syndrome either have genetically abnormal basement membranes (_____) or an autoimmune response to basement membrane collagen IV (____) associated with microscopic hematuria, mild to heavy proteinuria, and hypertension with variable elevations in serum creatinine.

Answer 21

Alport’s syndrome

Goodpasture’s syndrome

Question 22

What level of daily albumin excretion in urine is considered microalbuminuria, indicating early renal disease?

A. 10–30 mg/24 h
B. 30–300 mg/24 h
C. 300–500 mg/24 h
D. >1 g/24 h

Answer 22

B

Question 23

Which finding is most likely to be present in a patient with minimal change disease (MCD)?

A. Nonselective proteinuria
B. Hematuria with dysmorphic RBCs
C. Selective proteinuria with albumin predominance
D. Sustained proteinuria with red blood cell casts

Answer 23

C

Question 24

Which of the following findings distinguishes glomerular pyuria from pyuria due to a urinary tract infection (UTI)?

A. Positive leukocyte esterase
B. White blood cell casts in urine
C. Presence of dysmorphic RBCs
D. Positive urine culture

Answer 24

B

Question 25

Which level of proteinuria indicates frank proteinuria and significant glomerular disease?

A. 10–30 mg/24 h
B. 30–300 mg/24 h
C. 300–500 mg/24 h
D. >300 mg/24 h

Answer 25

D

Question 26

Which infectious agent is most commonly linked to glomerulonephritis in the Western Hemisphere?

A. Schistosomiasis
B. Malaria
C. HIV
D. Subacute bacterial endocarditis (SBE)

Answer 26

D

Question 27

_____ may be the most common causes of glomerulonephritis throughout the world, closely followed by HIV and chronic hepatitis B and C.

Answer 27

Malaria and schistosomiasis

Question 28

Light Microscopy Stains and Their Uses

• _____: Stains carbohydrate components of glomerular and tubular membranes.

Answer 28

Periodic Acid-Schiff (PAS)

Question 29

Light microscopy stains:

______ Evaluates cellularity and overall tissue architecture.

Answer 29

Hematoxylin & Eosin (H&E)

Question 30

Light microscopy stains:

______ : Identifies amyloid deposits.

Answer 30

Congo Red

Question 31

Light microscopy stains:

______: Enhances visualization of the glomerular basement membrane (GBM).

Answer 31

Jones-Methenamine Silver

Question 32

Light microscopy stains:

______: Detects collagen deposition and assesses glomerulosclerosis and interstitial fibrosis.

Answer 32

Masson’s trichrome

Question 33

When cells in the capillary tuft proliferate, it is called ____, and when cellular proliferation extends into Bowman’s space, it is called _______

Answer 33

endocapillary

extracapillary

Question 34

_____ are formed when epithelial podocytes attach to Bowman’s capsule in the setting of glomerular injury

Answer 34

Synechiae

Question 35

_____ , which in some cases may be the extension of synechiae, develop when fibrocellular/ fibrin collections fill all or part of Bowman’s space

Answer 35

Crescents

Question 36

Which of the following stains is best used to evaluate collagen deposition and assess the degree of glomerulosclerosis and interstitial fibrosis in a renal biopsy?

A. Hematoxylin and Eosin (H&E)
B. Masson’s Trichrome
C. Periodic Acid-Schiff (PAS)
D. Jones-Methenamine Silver

Answer 36

B

Question 37

Which of the following findings on renal biopsy suggests crescentic glomerulonephritis?

A. Thickened GBM with reduplication
B. Proliferative mesangial cells in the glomerulus
C. Fibrocellular collections filling Bowman’s space
D. Amyloid deposits highlighted by Congo red staining

Answer 37

C

Question 38

What is the histologic hallmark of sclerotic glomeruli in renal biopsy specimens?

A. Endocapillary hypercellularity
B. Epithelial proliferation extending into Bowman’s space
C. Acellular proteinaceous material with capillary obliteration
D. Tubular dropout with intact glomerular capillaries

Answer 38

C