Nephrotic Syndrome

Question 0

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

Answer 0

Minimal change disease

Question 1

What is the hallmark of nephrotic syndrome?

Answer 1

Proteinuria > 3.5 g/day

HyperAlbuminemia – >oedema

Hypogammaglobulinaemia -> increased p(infection)

Preferentially decrease AT3 – >hypercoagulable state

Decreased protein –> thin blood – > liver throws some fat to thicken it up – >
hyperlipidaemia + hypocholesterolaemia

Question 2

What is the cause of minimal change disease? What can it be associated with?

Answer 2

Idiopathic

Associated with Hodgkin lymphoma

Question 3

What layer from the glomerulal filtration barrier is affected?

Answer 3

Lose podocytes Foot processes

Question 4

Explain how Hodgkins Causes symptoms?

Answer 4

Read Sternberg cells – >massive cytokine production into blood – >
– B symptoms = fever night sweats weight loss
– Cytokines hit glomerular filtration barrier – > knockout podocytes – >MCD

Question 5

What do we see @:
Light microscopy
Electromicroscopy
Immunofluorescence?

Answer 5

1. H+E stain=normal glomeruli – Poss see lipid @PCT
2. Defacement of thought processes
3. not driven by the position of immunocomplexes – > IF negative

Question 6

@Minimal change disease what do you know about the proteinuria ?

Answer 6

Selective protein urea =
lose albumin
NOT lose Ig

Question 7

Treatment for minimal change disease?

Answer 7

Corticosteroids – >decrease cytokine production

Question 8

What is the most common cause of nephrotic syndrome in Hispanics + African-Americans?

Answer 8

Focal segment glomerulosclerosis FSGS

Question 9

HIV patient presents with nephrotic syndrome. What has patient got?

Answer 9

Focal segmental glomerulosclerosis

Question 10

Patient with sickle-cell disease develops nephrotic syndrome . What nephrotic syndrome does he have?

Answer 10

Focal segment glomerulosclerosis

Question 11

A heroin user develops nephrotic syndrome. What nephrotic syndrome does he have?

Answer 11

Focal segmental glomerulosclerosis

Question 12

What is the primary reason for FSGS?

What is the secondary reason for FSGS?

Answer 12

1. idiopathic

2. HIV, sickle-cell, Heroin

Question 13

If a patient with minimal change disease doesn’t respond to corticosteroids what happens?

Answer 13

Progresses to focus segmental glomerulosclerosis and then chronic renal failure

Question 14

Where do we see +
LM
IF
EM

Answer 14

1. segmental sclerosis + hyalinosis
2. not driven by IC deposition = IF negative
3. effacements of foot processes

Question 15

What is the most common cause of nephrotic syndrome @ Caucasian adults ?

Answer 15

Membranous nephropathy

Question 16

What are the primary and secondary causes of membranous nephropathy?

Answer 16

Primary = idiopathic

Secondary = SHADS
Solid tumours
Hep B + C
A B's to phospholipase A2
Drugs = NSAIDs + penicillinamine
SLE

Question 17

Explain how immunocomplexes the position + membranous nephropathy Appears

Answer 17

IC deposits under epithelial cell – >
podocyte NOT like being kicked off BM by deposits – >
podocytes responds by laying down more BM – >

Make DOME over deposit
and get SPIKES between DOMES

Question 18

What do we see @membranous nephropathy
IF
EM
LM

Answer 18

IF = positive = Granular due to IC deposition

EM =
spike + dome appearance
+
subepithelial deposits

LM = Diffuse capillary + glomerular BM thickening

Question 19

What are the two types of membranoproliferative glomerulonephritis

Answer 19

Type 1 = subendothelial = hepatitis B plus C
More often associated with tram tracks

Type 2 = inside basement membrane = intramembranous
Auto-Ab in blood = C3 nephritic factor – >block + stabilise C3 convertase (Can’t be broken down)– > Over activate compliment – >inflammation
–>
decreased serum C3 + inflam damage @glomerulus

Question 20

Explain how diabetes mellitus leads to glomerulonephropathy

Answer 20

Increased serum glucose – >
1st change = non-enzymatic glycosylation of vascular BM (stick sugar + BM without enzyme) –>

increased permeability of BM –>
proteins leak into blood vessel wall = hyaline arteriolosclerosis – >
THICK blood vessel wall due to Protein like material – >increased lumen calibre –> increased GFP – >
increased GFR = hyperfiltration – >

Initially = microalbuminuria 
Later = sclerosis of mesangium = Kimmelstiel Wilson nodules

Question 21

Which artery is preferentially affected by hyaline arteriolosclerosis afferent or efferent?

Which vessel does angiotensin II affect?
What is the effect of ace inhibitors On nephrotic syndrome?

Answer 21

Different

Different

Ace inhibitors = angiotensin II – >
decreased pressure on afferent arterial is – > decrease hyperfiltration injury

Question 22

As systemic amyloidosis which organ is the most commonly involved?
What diseases cause amyloid depositions @ mesangium ?
Explain what stain + what colour + polarised light

Answer 22

Kidney

Mr T: multiple myeloma, rheumatoid arthritis, TB

@LM: Congo red stain shows apple green birefringence @polarised light