2.1.1 Glomerular Disorders Nephritic and Nephrotic Syndromes II

Question 1

What systemic disease can lead to GBM thickening with nodular glomerulosclerosis (Kimmelstiel-Wilson nodules)?

Answer 1

Diabetes

Question 2

Give three examples of systemic diseases that can lead to nephrotic syndrome?

Answer 2

Diabetes, amyloidosis, lupus

Question 3

If you don’t know the answer, blame Miles or Joey.

Answer 3

Post-infectious glomerulonephritis (post-streptococcal GN)

Subepithelial electron dense “humps”

Question 4

Know it, bro?

Answer 4

Mutation in COL4A5

Alport Syndrome

Question 5

Name this dz: Most common in children, responsive to steriods, causes foot process effacement/fusion that can be seen on EM

Answer 5

Minimal change dz

Question 6

You can do it, doc

Answer 6

Diabetic nephropathy

Kimmelstiel-Wilson nodule (nodule glomerulosclerosis)

Question 7

What are the most common primary glomerular dz presenting with nephrotic syndrome in adults?

Answer 7

FSGS and membranous glomerulopathy

Question 8

What is the most common primary glomerular dz presenting with nephrotic syndrome in children?

Answer 8

Minimal change dz

Question 9

This could be your daughter. You better come up with the right answer

Answer 9

IF = negative; EM = foot process effacement

Minimal change dz

Steriods

Question 10

What systemic disease has this pattern of deposition?

Answer 10

SLE - leading to membranous nephropathy

Question 11

What are the three etiologic classifications of FSGS?

Answer 11

Primary (idiopathic), secondary, non-specific segmental glomerular scarring

Question 12

What are the four symptoms found in nephrotic syndrome?

Answer 12

Proteinuria (>3.5 gm/day), hypoalbuminemia, edema, hyperlipidemia

Question 13

“I done caught amnesia.” Which greatest rapper of all-time said this in a song?

Answer 13

Auto-Ab to COL4A3

Goodpasture syndrome

It was Kevin Gates if you were wondering

Question 14

What are the different causes of the two types of MPGN (Ig+ and Ig-)?

Answer 14

Question 15

What the answer, champ?

Answer 15

Henoch-Schonlein Purpura

Question 16

What morphological subtype of FSGS are we responsible for on STEP? It is related to HIV, drug, idiopathic; worse prognosis.

Answer 16

Collapsing glomerulopathy

Question 17

Name this dz: Proteinuria/nephrotic syndrome; diffuse, global thickening of GBM; subepithelial IgG and C3 by IF, EM; in situ immune complex formation for primary

Answer 17

Membranous glomerulopathy

Question 18

Need a little pick me up after those questions?

Answer 18

Here is a picture of Miles prior to getting into medical school. His life was definitely trending downwards…

Question 19

What is the most type of amyloidosis worldwide? What disease with a mutation in pyrin is of this type?

Answer 19

AA

Familial Mediterranean Fever

Question 20

Name this dz: More of a pattern of injury than it is one particular dz; lobular hypercelluarity; double contous or “tram tracks” IF = usually peripheral C3, and either Ig+ or Ig-; EM = subendothelial deposits (Ig+) or intramembranous dense deposits (Ig-)

Answer 20

Membranoproliferative glomerulonephritis

Question 21

Got an answer, pal?

Answer 21

ANCA (anti-GBM assay)

Wegner’s (GPA)

Question 22

Which systemic disease is characterized by peripheral neuropathy and can lead to abnormal protein with beta-pleated sheet structure forming fibril deposits in the glomeruli? It is often detected by congo red staining.

Answer 22

Amyloidosis

Question 23

What is the answer, bud?

Answer 23

Lupus nephritis

“Full house” (IgG, IgM, IgA, C3, C1q)

Question 24

Name this systemic disease that affects the glomeruli.

Answer 24

Diabetes

Question 25

Let me be your lighthouse

Answer 25

FSGS

Question 26

Name 5 diseases that primarily affect glomeruli and lead to nephrotic syndrome.

Answer 26

Membranous glomerulopathy

Minimal change disease

Focal segmental glomerulosclerosis (FSGS)

Membranoproliferative glomerulonephritis

Other proliferative glomerulonephritides

Question 27

Just keep swimming -DORY

Answer 27

Post-infectious GN

subepithelial electron dense “humps”

Question 28

What are some of the causes of secondary membranous GN?

Answer 28

Drug, infection, neoplasms, autoimmune

Question 29

What are some of the major histologic features of HIVAN?

Answer 29

Globally collapsed glomeruli

severe tubular microcytic dilation

Numerous tubular reticular inclusions (TRIs)

Question 30

You is smart

Answer 30

Question 31

Name this dz: Focal, segmental sclerosis, foot process effacement that can been seen on EM, unresponsive to sterioids.

Answer 31

Focal segmental glomerulosclerosis (FSGS)

Question 32

What is the most common type of amyloidosis in the US?

Answer 32

AL, which is Ig light chain, usually lambda

a/w myeloma