Glomerulonephropathies

Question 1

5 characteristics of nephrotic syndrome

Answer 1

1. proteinuria > 3.5 g/day
2. hypoalbuminemia
3. hypogammaglobulinemia
4. hypercoagulable state
5. hyperlipidemia and hypercholesterolemia

Question 2

Most common cause of nephrotic syndrome in children

Answer 2

Minimal change disease

Question 3

Cancer associated with minimal change

Answer 3

Hodgkin lymphoma

Question 4

Minimal change findings on H&E stain, EM, and IF

Answer 4

H&E: normal glomeruli

EM: effacement of foot processes due to production of cytokines

IF: negative

Question 5

Symptoms of minimal change disease

Answer 5

Selective loss of albumin, periorbital edema following an upper respiratory tract infection or vaccination

Has an excellent response to steroids

Question 6

Populations most likely to have focal segmental glomerulosclerosis

Answer 6

Hispanics and African Americans, also associated with HIV, heroin use and sickle cell disease

Question 7

Focal segmental findings on H&E stain, EM, and IF

Answer 7

H&E: focal (some glomeruli) and segmental (involving only part of the glomerulus) sclerosis

EM: effacement of foot processes

IF: negative

Question 8

Populations most likely to have membranous nephropathy

Answer 8

Caucasian adults, also associated with HBV and HCV, solid tumors (lung, breast, colon), SLE or drugs (NSAIDS and pencillamine, gold)

Question 9

Membranous nephropathy findings on H&E stain, EM, and IF

Answer 9

H&E: thick glomerular basement membrane

EM: subepithelial deposits with spike and dome appearance

IF: immune complex deposition giving a granular appearance

Question 10

Membranoproliferative, type I findings on H&E stain and IF

Answer 10

H&E: thick glomerular basement membrane often with tram track appearance due to basement membrane splitting by mesangial ingrowth

IF: immune complex deposition giving a granular appearance

Question 11

Diseases associated with membranoproliferative, type I

Answer 11

HBV and HCV

Question 12

Membranoproliferative, type II findings on IF

Answer 12

IF: intramembranous dense deposits

Question 13

Association with membranoproliferative, type II

Answer 13

C3 nephritic factor: autoantibody that stabilizes C3 convertase, leading to over activation of complement, inflammation and low levels of circulation C3

Question 14

Microscopic findings in diabetic glomerulonephropathy

Answer 14

Sclerosis of the mesangium with formation of Kimmelstiel-Wilson nodules

Question 15

Microscopic findings in systemic amyloidosis affecting the kidney

Answer 15

Amyloid deposits in the mesangium, characterized by apple-green birefringence under polarized light after staining with Congo Red

Question 16

4 characteristics of nephritic syndrome

Answer 16

1. limited proteinuria < 3.5 g/day
2. oliguria and azotemia
3. periorbital edema and hypertension
4. RBC casts and dysmorphic RBCs in urine

Question 17

Acute poststreptococcal glomerulonephritis findings on H&E stain, EM, and IF

Answer 17

H&E: glomeruli enlarged and hypercellular

EM: subepithelial immune complex humps

IF: granular appearance due to IgG, IgM, and C3 deposition along GBM and mesangium

Question 18

Symptoms of PSGN

Answer 18

Peripheral and periorbital edema, dark urine, and hypertension usually 2-3 weeks following infection

Question 19

RPGN findings on EM, and IF

Answer 19

EM: crescents in Bowman’s space composed of fibrin and macrophages

IF: varies on disease

Question 20

RPGN condition with linear IF pattern

Answer 20

Goodpasture syndrome: antibody against collagen in glomerular and alveolar basement membranes

Question 21

RPGN conditions with granular IF pattern

Answer 21

PSGN or diffuse proliferative glomerulonephritis

Question 22

RPGN conditions with negative IF pattern (pauci immune)

Answer 22

Wegener’s, microscopic polyangiitis, and Churg-Strauss

Question 23

Differentiate between microscopic polyangiits and Churg-Strauss

Answer 23

Both are p-ANCA+

Granulomatous inflammation, eosinophilia, and asthma are only associated with Churg-Strauss

Question 24

Berger’s disease findings on H&E stain, EM, and IF

Answer 24

H&E: mesangial proliferation

EM: mesangial IC deposits

IF: IgA-based IC deposits in mesangium

Question 25

Symptoms of Berger’s disease

Answer 25

Usually presents in childhood with episodic gross or microscopic hematuria with RBC casts usually following mucosal infections

Question 26

Mutation found in Alport syndrome and inheritance pattern

Answer 26

Inherited defect in type IV collagen, most commonly X-linked

Question 27

Symptoms of Alport syndrome

Answer 27

Isolated hematuria, sensory hearing loss, and ocular disturbances

Question 28

Diffuse proliferative glomerulonephritis findings on H&E stain, EM, and IF

Answer 28

H&E: “wire looping” of capillaries

EM: subendothelial and sometimes intramembranous IgG-based ICs often with C3 deposition

IF: granular appearance