Nephrotic syndrome Flashcards
What is nephrotic syndrome?
Inflammation of glomerulus (BOTH) can lead to increased permeability of glomerulus causing PROTEINURIA (3.5g+ / 24hr)
Aetiology of nephrotic syndrome?
1^ and 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)
Sx of Nephrotic syndrome?
Proteinuria (frothy apple juice)
Hypoalbuminemia
Hyperlipidemia + weight gain
Oedema (puffy eg. face)
Dx of nephrotic syndrome?
Urinalysis (proteinuria +/- haematuria)
24hr urine protein collection (>3.5g)
eGFR, U+E, LFT (low albumin), lipid profile (high lipid)
Renal biopsy
What would be seen in the renal biopsy for each 1^ diseases causing nephrotic syndrome?
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
Tx for all 3 1^ diseases and response to it?
steroids for 12 weeks with variable response
Minimal change = responds well
FSG + MN = less well
Aetiology of nephritic syndrome?
IgA nephropathy (Berger’s disease) MC
Post strep glomerulonephritis
SLE, Goordpastures, haemolytic uremic syndrome
All examples of T3 hypersensitivity (except good pastures T2)
Ig A nephropathy:
More in?
associated with?
in asian populations
HIV
Ig A nephropathy
Sx?
visible haematuria (ribena/coke)
1-2 days after tonsillitis - VIRAL INFECTION (or gastroenteritis infection)
Ig A nephropathy
Dx?
Tx?
Immunofluorescence microscopy shows: IgA complex deposition
Non curative; 30% progress to ESRF
1st line control = BP control (ACE-i)
Post strep glomerulonephritis
Sx?
visible haematuria (ribena/coke)
2 weeks after pharyngitis from Group A, beta haemolytic strep (s.pyogenes)
Post strep
Dx?
Light = hyper cellular glomeruli
E- = Subendothelial immune complex deposition
Immunofluorescemce shows starry sky appearance - Ig G,M + C3 deposits along GBM + Mesangium
Tx for post strep?
self limiting
usually sometimes may progress to RPGN (rapidly progressing glomerulonephritis)
SLE
What is 2^ to it?
Dx?
Tx?
Lupus nephritis 2^ to SLE (ANA deposition in endothelium)
Dx = ANA +VE anti dsDNA +ve
Tx = steroids, hydrochloroquine + immunosuppressants eg. cyclophosphamide
Goodpastures
what does it cause?
due to?
Tx?
Pulmonary and alveolar haemorrhage + glomerulonephritis
due to AutoAb (anti GBM)
Tx = steroids and plasma exchange
Haemolytic uremia
When does It occur?
what it Causes?
Tx?
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
What Is rapidly progressing glomerulonephritis?
Dx?
Causes?
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)
NephrOtic syndrome?
What appears in the urine and why?
Due to?
PrOteinuria (3.5g+ /24hr) +/- haematuria
Increased permeability of glomerulus walls due to podocyte injury and scarring
Sx of nephrOtic syndrome?
and why?
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)
NephrItic syndrome?
What gets into the urine and why?
Haematuria (+ little proteinuria) RBCs get through glomerulus
GBM breaks and inflammation + Bowmans crescent
Sx of nephrItic syndrome?
Oliguria (little urine, salt + water retention)
Hypertension
(inflammation = little function = can’t remove waste or fluid)
oedema (due to fluid overload)
What can present as both?
Diffuse proliferative glomerulonephritis
Membrane proliferative glomerulonephritis