Nephrotic syndrome

Question 1

What is nephrotic syndrome?

Answer 1

Inflammation of glomerulus (BOTH) can lead to increased permeability of glomerulus causing PROTEINURIA (3.5g+ / 24hr)

Question 2

Aetiology of nephrotic syndrome?
1^ and 2^?

Answer 2

1^
Minimal change disease - MC in children

Focal segmental glomerulosclerosis - MC In adults

Membranous nephropathy - also In adults, caucasian

2^
MC to diabetic (nephropathy)
also amyloidosis, lupus, viral infection (HIV), drugs (NSAIDs)

Question 3

Sx of Nephrotic syndrome?

Answer 3

Proteinuria (frothy apple juice)
Hypoalbuminemia
Hyperlipidemia + weight gain
Oedema (puffy eg. face)

Question 4

Dx of nephrotic syndrome?

Answer 4

Urinalysis (proteinuria +/- haematuria)
24hr urine protein collection (>3.5g)
eGFR, U+E, LFT (low albumin), lipid profile (high lipid)
Renal biopsy

Question 5

What would be seen in the renal biopsy for each 1^ diseases causing nephrotic syndrome?

Answer 5

Minimal change (young Px eg. 7y/o)
Light microscopy = no change
e- microscopy = Podocyte effacement + fusion

Focal segmental gomeruloSCLEROSIS (adult)
Segmental sclerosis; less than 50% glomeruli affected though

Membranous nephropathy
Light - thickened GBM
E- - subpodocyte immune complex deposition, spike + dome appearance

Question 6

Tx for all 3 1^ diseases and response to it?

Answer 6

steroids for 12 weeks with variable response
Minimal change = responds well
FSG + MN = less well

Question 7

Aetiology of nephritic syndrome?

Answer 7

IgA nephropathy (Berger’s disease) MC
Post strep glomerulonephritis
SLE, Goordpastures, haemolytic uremic syndrome

All examples of T3 hypersensitivity (except good pastures T2)

Question 8

Ig A nephropathy:
More in?
associated with?

Answer 8

in asian populations
HIV

Question 9

Ig A nephropathy
Sx?

Answer 9

visible haematuria (ribena/coke)
1-2 days after tonsillitis - VIRAL INFECTION (or gastroenteritis infection)

Question 10

Ig A nephropathy
Dx?
Tx?

Answer 10

Immunofluorescence microscopy shows: IgA complex deposition

Non curative; 30% progress to ESRF
1st line control = BP control (ACE-i)

Question 11

Post strep glomerulonephritis
Sx?

Answer 11

visible haematuria (ribena/coke)
2 weeks after pharyngitis from Group A, beta haemolytic strep (s.pyogenes)

Question 12

Post strep
Dx?

Answer 12

Light = hyper cellular glomeruli
E- = Subendothelial immune complex deposition

Immunofluorescemce shows starry sky appearance - Ig G,M + C3 deposits along GBM + Mesangium

Question 13

Tx for post strep?

Answer 13

self limiting
usually sometimes may progress to RPGN (rapidly progressing glomerulonephritis)

Question 14

SLE
What is 2^ to it?
Dx?
Tx?

Answer 14

Lupus nephritis 2^ to SLE (ANA deposition in endothelium)

Dx = ANA +VE anti dsDNA +ve

Tx = steroids, hydrochloroquine + immunosuppressants eg. cyclophosphamide

Question 15

Goodpastures
what does it cause?
due to?
Tx?

Answer 15

Pulmonary and alveolar haemorrhage + glomerulonephritis

due to AutoAb (anti GBM)

Tx = steroids and plasma exchange

Question 16

Haemolytic uremia
When does It occur?
what it Causes?
Tx?

Answer 16

5 days post infection vs SHIGA toxin (e.coli and shigella)
haemolytic anemia
aki (glomerulonephritis therefore uraemia)
Thrombocytopenia

Mostly self limiting but Sx medical emergency = fluids and Abx

Question 17

What Is rapidly progressing glomerulonephritis?
Dx?
Causes?

Answer 17

subtype of GN that progresses to ESRF very fast - weeks to months

Dx = inflammatory crescents in Bowmans spaces

causes = Wegener’s granulomatosis (GPA), MPA (pANCA +ve), goodpastures (C-ANCA +ve)

Question 18

NephrOtic syndrome?
What appears in the urine and why?
Due to?

Answer 18

PrOteinuria (3.5g+ /24hr) +/- haematuria
Increased permeability of glomerulus walls due to podocyte injury and scarring

Question 19

Sx of nephrOtic syndrome?
and why?

Answer 19

HypOalbuminemia (high protein in urine = low blood protein)

Hyperlipidemia (liver compensates by increasing albumin which increases lipids)

Oedema (low albumin - protein)

HypOgammaglobinemia (low Ig)
Hypercoagulable blood (due to loss of anti thrombin 3)
(low proteins such as anti thrombin 3, protein C+S and Ab)

Question 20

NephrItic syndrome?
What gets into the urine and why?

Answer 20

Haematuria (+ little proteinuria) RBCs get through glomerulus
GBM breaks and inflammation + Bowmans crescent

Question 21

Sx of nephrItic syndrome?

Answer 21

Oliguria (little urine, salt + water retention)

Hypertension
(inflammation = little function = can’t remove waste or fluid)

oedema (due to fluid overload)

Question 22

What can present as both?

Answer 22

Diffuse proliferative glomerulonephritis
Membrane proliferative glomerulonephritis