Nephrotic Syndrome Flashcards
What is the most common cause of nephrotic syndrome in children?
Minimal change disease
What is the hallmark of nephrotic syndrome?
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
What is the cause of minimal change disease? What can it be associated with?
Idiopathic
Associated with Hodgkin lymphoma
What layer from the glomerulal filtration barrier is affected?
Lose podocytes Foot processes
Explain how Hodgkins Causes symptoms?
Read Sternberg cells – >massive cytokine production into blood – >
– B symptoms = fever night sweats weight loss
– Cytokines hit glomerular filtration barrier – > knockout podocytes – >MCD
What do we see @:
Light microscopy
Electromicroscopy
Immunofluorescence?
- H+E stain=normal glomeruli – Poss see lipid @PCT
- Defacement of thought processes
- not driven by the position of immunocomplexes – > IF negative
@Minimal change disease what do you know about the proteinuria ?
Selective protein urea =
lose albumin
NOT lose Ig
Treatment for minimal change disease?
Corticosteroids – >decrease cytokine production
What is the most common cause of nephrotic syndrome in Hispanics + African-Americans?
Focal segment glomerulosclerosis FSGS
HIV patient presents with nephrotic syndrome. What has patient got?
Focal segmental glomerulosclerosis
Patient with sickle-cell disease develops nephrotic syndrome . What nephrotic syndrome does he have?
Focal segment glomerulosclerosis
A heroin user develops nephrotic syndrome. What nephrotic syndrome does he have?
Focal segmental glomerulosclerosis
What is the primary reason for FSGS?
What is the secondary reason for FSGS?
- idiopathic
2. HIV, sickle-cell, Heroin
If a patient with minimal change disease doesn’t respond to corticosteroids what happens?
Progresses to focus segmental glomerulosclerosis and then chronic renal failure
Where do we see +
LM
IF
EM
- segmental sclerosis + hyalinosis
- not driven by IC deposition = IF negative
- effacements of foot processes
What is the most common cause of nephrotic syndrome @ Caucasian adults ?
Membranous nephropathy
What are the primary and secondary causes of membranous nephropathy?
Primary = idiopathic
Secondary = SHADS Solid tumours Hep B + C A B's to phospholipase A2 Drugs = NSAIDs + penicillinamine SLE
Explain how immunocomplexes the position + membranous nephropathy Appears
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
What do we see @membranous nephropathy
IF
EM
LM
IF = positive = Granular due to IC deposition
EM =
spike + dome appearance
+
subepithelial deposits
LM = Diffuse capillary + glomerular BM thickening
What are the two types of membranoproliferative glomerulonephritis
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
Explain how diabetes mellitus leads to glomerulonephropathy
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
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?
Different
Different
Ace inhibitors = angiotensin II – >
decreased pressure on afferent arterial is – > decrease hyperfiltration injury
As systemic amyloidosis which organ is the most commonly involved?
What diseases cause amyloid depositions @ mesangium ?
Explain what stain + what colour + polarised light
Kidney
Mr T: multiple myeloma, rheumatoid arthritis, TB
@LM: Congo red stain shows apple green birefringence @polarised light