Renal 5

Question 1

What is seen in this photo?

“frog head”

Answer 1

Arrow is pointing to subepithelial side with a “hump”

• Immune complex (IgG complement/dead protein from strep)
• From head: Segmented nucleus, and lysosomes,(specific/non-specific neutrophils) jammed up against capillary loop

(to dx acute post strep GMN)

Question 2

What type of stain is this?

• Millions of immune complexes on subepithelial side
• Allows RBC into uroniferous space

Answer 2

• IF-C3 Granular Staining (Lumpy-Bumpy)
• Nephritic!

Question 3

Classic case of what?:

• young child w/ abrupt onset of malaise, fever, nausea, oliguria, hematuria, cola urine one to two weeks after recovering from sore throat
• Red cell casts in urine, mild proteinuria, periorbital edema, mild HTN

Answer 3

Acute Post-Strep GMN

(sxs of nephritic syndrome)

Question 4

What are the 4 sxs of pt w/ nephritic syndrome / Post strep GMN?

Answer 4

• Cola colored urine
• Periorbital edema in the morning
• Edema
• Mild HTN

Question 5

95% of children recover from Acute Post-Strep GMN / Nephritic Syndrome, and only 1 - 2 % will develop what?

Answer 5

Rapidly Progressive GMN

Question 6

Patients who have Rapidly Progressive GMN will develop what?

Answer 6

Crescent Formation

Question 7

Bowmans capsule compressing the tuft. Both kidneys will die within hours. Must get dialysis quickly or pt will die.

Answer 7

Crescent Formation of Acute Post Strep GMN / Rapidly Progressive GMN

Question 8

Answer 8

Crescent Formation

Question 9

Usually occurs after focal necrosis of the glomerular capillaries in diseases such as Goodpastures Syndrome

Answer 9

Rapidly Progressive (Cresentic) GMN

Question 10

Which condition does NOT lead to crescent formation?

Answer 10

Minimal Change Disease (Nils)

Question 11

How are crescents formed?

Answer 11

Inflammatory cells surround the compressed capillary loops in the form of a crescent moon

Question 12

Answer 12

Crescentic Glomerulonephritis

Question 13

• Autoimmune disease characterized by formation of antibodies to the body’s own basement membrane components (collagen type 4)
• Which 2 organs are affected?

Answer 13

• Goodpastures Syndrome
• Lungs & Kidneys

Question 14

• Lungs injured –> intraalveolar hemorrhage
• In glomeruli, antibodies cause rupture of BM, so macrophages exit through holes of BM
• Macrophages accumulate in urinary space (forming crescents that compress capillary loops)

Answer 14

Goodpastures Syndrome

Question 15

Answer 15

Goodpastures Syndrome w/ intra-alveolar hemorrhage

(brown macrophages in the lungs bc/ of iron)

Question 16

Answer 16

“ribbon pattern”

• Goodpastures Syndrome w/ LINEAR deposits in Glomerulus
• (no immune complex here! that would be called “lumpy bumpy”)

Question 17

W/ Goodpastures Syndrome, no blood is flowing through the compressed capillary loops, so what results?

Answer 17

Glomerular filtration ceases, causing anuria

Question 18

• What is the prognosis of Goodpastures Syndrome?
• Treatment?

Answer 18

• Most patients never recover.
• Survival depends on continuous dialysis or transplantation

Question 19

What 4 conditions lead to crescent formation?

Answer 19

• Wegeners
• PAN (Polyarteritis nodosa)
• Acute Post Strep GMN
• Goodpastures Syndrome

Question 20

• Characterized clinically by: Nephrotic Syndrome
• Pathologically by: fusion of visceral epithelial foot processes (podocytes)
• Most common cause of nephrotic syndrome in kids (protein spilled in urine)

Answer 20

Minimal Change Disease (Nils)

Question 21

• What is seen on Light Microscopy for Minimal Change Disease (Nils)?
• What is seen on IF?

Answer 21

• Light: Glomeruli show NO CHANGES, hence name for Nils
• IF: the glomeruli do not contain deposits of immunoglobulins

Question 22

“Jigsaw Puzzle”

Answer 22

• Minimal Change Disease (Nils)
• Fusion of foot processes (podocytes)
• “Effacement”

Question 23

“stained glass”

Answer 23

Minimal Change Disease (Nils)

Question 24

Stained Glass

Answer 24

Minimal Change Disease

Question 25

In which condition is the proteinuria generally more selective than other types of Nephrotic Syndrome?

Answer 25

Minimal Change Disease

Question 26

Over 90% of children / slightly fewer adults who have Minimal Change Disease have complete remission of _______ within ___ weeks of initiation of ______ therapy.

Answer 26

* proteinuria * 8 weeks * corticosteroid

Question 27

What is the #1 cause of Nephrotic Syndrome in adults? (Spilling of proteins)

Answer 27

Membranous Glomerulonephritis

Question 28

Immune mediated glomerulopathy characterized by diffuse thickening of the glomerular basement membrane secondary to massive deposition of immune complexes

Answer 28

Membranous Glomerulonephritis

Question 29

* No evidence of ANY inflammatory cells in glomeruli * Urine is DEVOID of RBCs (like acute post strep GMN)

Answer 29

Membranous Glomerulonephritis

Question 30

Thick strings w/ pepper

Answer 30

Membranous Glomerulonephritis | (shows capillary loops w/ thick walls)

Question 31

Unlike Acute Post Strep GMN, Membranous GMN causes what?

Answer 31

Nephrotic Syndrome

Question 32

**Membranous GMN** * Etiology? * Light Microscopy: * EM: * IF:

Answer 32

* 85% idiopathic * **Light:** glomeruli w/ thickened BMs, but are normocellular * **EM:** thickening of BM attributed to deposition of dense immune complexes = lumpy bumpy * **IF:** granularity of deposits

Question 33

Looks like brain

Answer 33

Membranous Glomerulonephritis

Question 34

Avocado

Answer 34

Membranous GMN w/ deposits of immune complexes

Question 35

Sandy Ribbons

Answer 35

Membranous Glomerulonephritis Granular Deposits of IgG = Lumpy Bumpy

Question 36

* Typical signs/sxs of Nephrotic Syndrome * When followed 20 yrs, * 25% pts have \_\_\_\_\_ * 50% have _____ w/ stable renal function * 25% develop \_\_\_\_\_\_

Answer 36

* **Membranous GMN** * 25% have spontaneous remission * 50% have persistent proteinuria * 25% develop End Stage Renal Failure

Question 37

**Membranous GMN** * Treatment? * Prognosis?

Answer 37

* Corticosteroids for those w/ progressive renal failure * Prognosis better in children (higher rate of remission)

Question 38

Which forms of Glomerular Disease can become chronic?

Answer 38

ALL 17

Question 39

* Important cause of end-stage renal disease (presenting as chronic renal failure) * Most, if not all immune mediated glomerulopathies can progress to this end-stage disease, terminating in uremia (ammonia circulating which can go to brain and cause \_\_\_\_.)

Answer 39

* Chronic Glomerulonephritis * Can cause **coma.**

Question 40

* Grossly, kidneys are symmetrically shrunken * Surfaces are finely granular because of loss of \_\_\_\_.

Answer 40

* Chronic Glomerulonphritis * **Tubules**

Question 41

Answer 41

Chronic Glomerulonephritis

Question 42

* Histologically, the glomeruli have undergone hyalinization and appear as solid globules of homogenized matrix * Marked interstitial fibrosis, tubular atrophy, & thick walled arteries w/ narrowed lumina * Pts aware bc of/ which 4 things?

Answer 42

Chronic Glomerulonephritis * Proteinuria * Azotemia (nitrogen in blood) * HTN Edema

Question 43

"eye patch"

Answer 43

**Chronic Glomerulonephritis** (sclerosed, hyalinized glomeruli) * Dead glomerulus & live one (the pretty one with the white streaks is still alive)

Question 44

Jelly beans

Answer 44

Chronic Glomerulonephritis | (atrophic dilated tubules)