L58 + 59

Question 1

If you saw a hypercellular glomerulus, what would be happening in what you see?

Answer 1

Prolif of intracap glomerular cells

Crescents = prolif of extracap glomerular cells

Question 2

What happens to get sclerosis in the kidney?

Answer 2

GBM collapse + mesangial expansion
Damage to segmental structure
Get scar due to collagen deposition

Question 3

What happens to get hyalinosis in the kidney?

Answer 3

Accum protein in sclerotic areas in BV walls

Question 4

What disease process thickens the GBM itself?

Answer 4

Diabetes (vs immune complex deposition thickening)

Question 5

What is global vs segmental glomerulus damage?

Answer 5

Global - entire thing involved

Segmental - part of tuft involved w/ other part patent

Question 6

Use the name: what is focal segmental glomerulo-sclerosis?

Answer 6

Focal - dotted throughout kidney

Segmental - only part glomerulus affected

Question 7

Which cells are mediators of injury to the glomerulus?

Answer 7

Impt ones: neutrophils + mesangial cells

Question 8

What are the 3 soluble mediators of injury at the glomerulus?

Answer 8

Complement
Fibrinogen
TGF beta

Question 9

Clinical presentation nephritic syndromes

Answer 9

Hematuria - RBC casts
Mild proteinuria 
Periorbital edema 
Azotemia
Oliguria
HTN + edema

Question 10

Pathogenesis of post-infectious glomerular nephritis?

Answer 10

Strep pyogenes infection: impetigo > pharyngitis

Immune complex pathogenesis

Question 11

Post-infectious GN LM, IF, EM

Answer 11

LM: hypercellular (nephrotic)
GN: granular
EM: sub-epi humps of Ig

Question 12

What other labs will indicate post-strep GN

Answer 12

+ ASO

↓C3

Question 13

What is rapidly progressive GN? What are the categories of disease?

Answer 13

Crescent 
Renal failure progressing in wks/mos
Diseases may RESULT in RPGN
1. Immune complex mediated = granular IF
2. Anti-GBM = linear IF
3. Pauci-immune = nothing on IF

Question 14

Treat post-strep GN; what is the complication if not treated?

Answer 14

Will resolve on own if treat 1ary infection in kids

Can progress to RPGN

Question 15

What disease has anti-GBM antibodies?

Answer 15

Goodpature’s: T2 HST rxn

Linear IF

Question 16

What diseases have granular IF and can progress to RPGN?

Answer 16

Immune complex disorders

1. Post-strep GN
2. SLE (IgG)

Question 17

What disease are RPGN pauci-immune?

Answer 17

1. Wegener’s: c-ANCA
2. Microscopic polyarteritis (small vessel vasculitis) p-ANCA
3. Polyarteritis nodosa
4. Churg Strauss

Question 18

Which diseases that can result in RPGN are reno-pulmonary diseases?

Answer 18

Goodpastures
Wegener’s
Microscopic polyangitis

Question 19

What is ANCA?

Answer 19

Anti-neurtophilic cytoplasmic autoantibodies
c-ANCA = cytoplasmic staining
p-ANCA = perinuclear staining

Question 20

What is the ANCA in Goodpastures?

Answer 20

Confounder - doesn’t matter!

Question 21

Treat Goodpasture’s

Answer 21

Plasmapheresis + steroids + cytotox drug

Question 22

Treat RPGN

Answer 22

Steroids + cytotoxic drugs

Question 23

What are the characteristics of nephrotic syndrome?

Answer 23

Proteinuria > 3.5 g/d
Hypoalbumin
Hyperlipidemia
Edema

Question 24

What is the most common cause of nephrotic syndrome in kids?

Answer 24

1ary

Minimal change disease

Question 25

What is membranous glomerulopathy?

Answer 25

1ary nephropathy- long onset Ig @ sub-epi Diffuse thickening of glomerular capillary walls Idiopathic

Question 26

Membranous glomerulopathy LM, EM, IF

Answer 26

LM: diffuse thickening EM: sub-epi spike and dome

Question 27

What is minimal change disease? LM, IF, EM

Answer 27

Kids: response to steroids Lose negative change in GBM ONLY see on EM: effacement of podocytes

Question 28

Presentation of minimal change disease

Answer 28

Selective proteinuria: lose albumin but not globulins

Question 29

What might minimal change disease progress to?

Answer 29

Focal seg glomerulo-sclerosis

Question 30

Clinico-pathological definition of focal-seg GS

Answer 30

Proteinuria (nephrotic) non-selective FSGS - Focal makes biopies hard Effacement of foot processes

Question 31

Usually FSGS is primary and idiopathic. What are 3 secondary causes?

Answer 31

``` Focal scarring from another GN Reflux nephropathy Disproportion body:renal mass HIV Drug induced: heroin ```

Question 32

Treat FSGS

Answer 32

Not gonna - poor steroid response Likely progress renal failure High recurrence in transplant pts

Question 33

What is IgA nephropathy?

Answer 33

Nephrotic - recurrent hematuria | IgA deposits in mesangium -> alternative complement

Question 34

What is a systemic IgA disease that can cause IgA nephropathy, vasculitis, and skin purpura?

Answer 34

Henoch-Schoenlein purpura

Question 35

Presentation Alport's

Answer 35

Males Nephritis - hematuria w/ RBC casts Deafness

Question 36

Pathogenesis Alport's

Answer 36

T4 collagen mutation - don't see any of this collagen on skin biopsy X GBM synthesis

Question 37

What is thin membrane disease?

Answer 37

Asymptom hematuria | EM: diffuse thinning of GBM

Question 38

Is membrano-proliferative glomerulo-nephritis nephrotic or nephritic? Types

Answer 38

Mixed - kids/young adults 1. Immune complex mediated - Hep C/B, SLE 2. Complement - mutations or Abs vs complement regulating proteins 3. W/ thrombotic microangiopathy

Question 39

Does SLE cause nephritis or nephrotic?

Answer 39

Nephritis | IF: look for all Ig, complement components