Nephropathies

Question 1

Amyloidosis is nephrotic or nephritic? what is it associated with?

Answer 1

Nephrotic. Associated with chronic conditions ex. multiple myeloma, TB, RA.

Question 1

Cause of DPGN

Answer 1

Due to SLE or MPGN. Most common cause of death in SLE. SLE and MPGN can present as nephrotic syndrome and nephritic syndrome concurrently.

Question 2

Minimal change disease is a nephrotic or nephritic syndrome?

Answer 2

nephrotic syndrome

Question 2

Answer 2

Diabetic glomerulonephropathy

• Nonenzymatic glycosylation ( EG) of GBM leads to increased permeability, thickening.
• NEG of efferent arterioles causes increased GFR leading to mesangial expansion.
• LM-mesangial expansion, GBM thickening, eosinophilic nodular glomerulosclerosis (Kimmelstiel-Wilson lesion).

Question 3

Bergers disease is a nephritic or nephrotic syndrome?

Answer 3

Nephritic syndrome

Question 4

This gets filtrated and is found in urine in minimal change disease

Answer 4

Selective loss of albumin, not globulins, caused by GBM polyanion loss.

Question 5

Most frequent patient population to see post-strept glomerulonephritis and presentation?

Answer 5

Most frequently seen in children. Peripheral and periorbital edema, dark urine, and hypertension. Resolves spontaneously.

Question 5

Is Acute poststreptococcal glomerulonephritis a nephrotic or nephritic syndrome?

Answer 5

Nephritic syndrome.

Question 5

Answer 5

Rapidly progressive (crescentic) glomerulonephritis (RPGN)

• LM and IF-crescent-moon shape.
• Crescents consist of fibrin and plasma proteins (e.g., C3b) with glomerular parietal cells, monocytes, and macrophages.

Question 6

What is MPGN Type I associated with?

Answer 6

Is associated with HBV, HCV

Question 7

Is Diabetic glomerulo-nephropathy a nephrotic or nephritic syndrome?

Answer 7

Nephrotic syndrome

Question 7

Nephritic syndrome presentation and associated with?

Answer 7

An inflammatory process. When it involves glomeruli, it leads to hematuria and RBC casts in urine. Associated with azotemia, oliguria, hypertension (due to salt retention), and proteinuria (

Question 8

Pathological process causing damage in glomerulo-nephropathy

Answer 8

*Nonenzymatic glycosylation (NEG) of GBM –>increased permeability, thickening *NEG of efferent arterioles –>increases GFR –>mesangial expansion

Question 8

Nephrotic syndrome presentation and associated with?

Answer 8

*Presents with massive proteinuria (>3.5g/day, frothy urine), hyperlipidemia, fatty casts, edema. *Associated with thromboembolism (hypercoagulable state due to AT III loss in urine) and increased risk of infection (loss of immunoglobulins)

Question 8

Berger’s disease (IgA nephropathy) is related to what clinical disorder?

Answer 8

Related to Henoch-Schonlein purpura

Question 8

What is the pathological process of Alport syndrome

Answer 8

Mutation in type IV collagen –> split basement membrane. X-linked.

Question 9

Berger’s disease (IgA nephropathy) often presents/flares with?

Answer 9

Often presents/flares with a URI or acute gastroenteritis.

Question 10

Whats the prognosis on RPGN

Answer 10

Poor prognosis. Rapidly deteriorating renal function (days to weeks)

Question 11

LM, EM, IM findings in Diffuse proliferative glomerulonephritis (DPGN)

Answer 11

**LM-“wire looping” of capillaries *EM-subendothelial and sometimes intramembranous IgG-based IC’s often with C3 deposition. *IF-granular

Question 12

Is RPGN a nephrotic or nephritic syndrome?

Answer 12

Nephritic syndrome

Question 14

Membrano proliferative glomerulonephritis (MPGN) type I findings

Answer 14

Subendothelial IC depositis with granular IF; “tram-track” appearance due to GBM splitting caused by mesangial ingrowth.

Question 15

Rapidly progressive (crescentic) glomerulonephritis (RPGN) LM and IF findings

Answer 15

LM and IF-crescent-moon shape. Crescents consist of fibrin and plasma proteins (ex. C3b) with glomerular parietal cells, monocytes, and macrophages.

Question 16

LM findings of Amyloidosis

Answer 16

LM-congo red stain shows apple green bifringence under polarized light

Question 17

LM and EM findings in Minimal change disease (lipoid nephrosis)

Answer 17

LM-normal glomeruli EM-foot process effacement

Question 18

What are some disease processes that can result in RPGN

Answer 18

\*Good pastures syndrome-type II hypersensitivity; antibodies to GBM and aveolar basement membrane --\>linear IF. Present with Hematuria/hemoptysis. \*Granulomatosis with polyangiitis (c-ANCA) \*Microscopic polyangiitis (p-ANCA)

Question 20

Cause of Minimal change disease (lipoid nephrosis)

Answer 20

May be triggered by a recent infection or an immune stimulus.

Question 22

Most common patient found with minimal change disease and what is the Treatment?

Answer 22

Is most common in children and it responds to corticosteroid Tx.

Question 24

LM and EM findings in Focal segmental glomerulosclerosis

Answer 24

LM-segmental sclerosis and hyalinosis. EM-effacement of foot process similar to minimal change disease.

Question 25

LM findings in Diabetic glomerulo-nephropathy

Answer 25

LM-mesangial expansion, GBM thickening, eosiniphilic nodular glomerulosclerosis, (Kimmelstiel-Wilson lesion)

Question 27

Cause of Membranous nephropathy

Answer 27

Can be idiopathic or caused by drugs, infections, SLE, solid tumors.

Question 28

Focal segmental glomerulosclerosis associations

Answer 28

Associated with HIV infection, heroin abuse, massive obesity, interferon treatment, and chronic kidney disease due to congenital absence or surgical removal.

Question 29

Alport Syndrome presents with what?

Answer 29

Glomerulonephritis, deafness, and less commonly, eye problems

Question 31

MPGN is nephrotic or nephritic?

Answer 31

It is nephrotic, but it can also present as nephritic syndrome.

Question 32

Answer 32

* Minimal change disease (lipoid nephrosis) * LM-normal glomeruli. * EM-foot process effacement

Question 33

Berger's disease (IgA nephropathy) LM, EM, and IF findings

Answer 33

LM-mesangial proliferation EM-mesangial IC depositis IF-IgA-based IC depositis in mesangium

Question 34

Acute poststreptococcal glomerulonephritis LM, EM, and IM findings

Answer 34

\*LM-glomeruli enlarged and hypercellular, neutrophils, "lumpy-bumpy" appearance. \*EM-subepithelial immune complex (IC) humps. \*IF-granular appearance due to IgG, IgM, and C3 deposition along GBM and mesangium.

Question 35

Answer 35

* Membranous nephropathy * LM-diffuse capillary and GBM thickening. * EM-"spike and dome" appearance with subepithelial deposits. * IF -granular. SLE's nephrotic presentation.

Question 36

Focal segmental glomerulosclerosis is nephrotic or nephritic?

Answer 36

nephrotic

Question 37

What is MPGN type II associated with?

Answer 37

Is associated with C3 nephritic factor

Question 38

LM, EM, and IF findings in Membranous nephropathy

Answer 38

LM-diffuse capillary and GBM thickening EM-"spike and dome" appearance with subepithelial deposits IF-granular. SLE's nephrotic presentation

Question 39

2nd most common cause of primary nephrotic syndrome in adults?

Answer 39

Membranous nephropathy

Question 41

Most common cause of nephrotic syndrome in adults

Answer 41

Focal segmental glomerulosclerosis

Question 43

Membrano proliferative glomerulonephritis (MPGN) type II findings

Answer 43

Intramembranous IC depositis; "dense depositis."

Question 44

Answer 44

* Focal segmental glomerulosclerosis * LM-segmental sclerosis and hyalinosis. * EM-effacement of foot process similar to minimal change disease. * Most common cause of nephrotic syndrome in adults.

Question 45

Answer 45

Membranaproliferative glomerulonephritis * Type 1-subendothelial IC deposits with granular IF; "tram-track" appearance clue to GBM splitting caused by mesangial ingrowth (in picture) * Type I is associated with HBV, HCV. * Type II-intramembranous IC deposits; "dense deposits." * Can also present as nephritic syndrome. * Type II is associated with C3 nephritic factor.