Nephrotic Syndrome Flashcards
What is nephrotic syndrome?
Nephrotic syndrome is defined as the presence of proteinuria, hypoalbuminaemia, and peripheral oedema.
V. Rare
To have nephrotic syndrome the patient must have what symptoms
- Hypogammaglobulinemia
- proteinuria
- hypoalbunameia
- Hyperlipidemia
- Hypercoagubility
Proteinuria would present as
frothy urine
Cause of nephrotic syndrome
Due to direct sclerosis of podocytes on the glomerulus
Primary aetiology of Nephrotic syndrome
- Minimal change disease
- Focal segmental glomerulosclerosis
- Membranous nephropathy
- membrano proliferative glomerulonephritis
Secondary aetiology of NS
- Diabetes
- Amyloidosis
- Infections (Hep B+ C)
- Drugs, NSAIDs
Pathophysiology of NS
- glomeruli are damaged and become more permeable
- plasma proteins pass through
- leads to proteinuria, hypoalbuminemia
Why is there peripheral + preorbital edema in NS?
there’s less oncotic pressure in the blood vessels so the fluid leaks out due to hypoalbuminemia
Why would a nephrotic patient present with a hypercoaguble state?
patient is also losing antithrombin III proteins which is the body’s anticoagulant.
This means that individuals with nephrotic syndrome are prone to thrombotic and thromboembolic complications
What is else is lost in urine in NS?
Immunoglobulins - higher risk of infection
Lipids - urine looks frothy - compensation liver increases lipoprotein synthesis resulting in hyperlipidaemia
Key presentations of NS
proteinuria, hypoalbuminaemia, oedema
S + S of NS
HT
Proteinura
Frothy urine
Recurrent infections
What is minimal change disease
Most common form of nephrotic syndrome characterised by heavy proteinuria, odema, hypoalbuminaemia and hyperlipidaemia affecting children
Investigations for NS
Urinalysis
ACR
24hr urine protein
U&Es
LFTS
Lipid profile
Complications of Nephrotic syndrome
CVD - hypercholesterolemia + hypertriglyceridemia, CKD
Haem - thrombosis
Infection
Pathophysiology of minimal change disease
- caused by t cells in the blood releasing cytokines - glomerular- permeability factor
- directly damages foot cells of podocytes making them flatten out (effacement)
- due to the damage , negatively charged ions such as albumin slip into the nephron
- albumin goes through but larger proteins like Ig dont - selective proteinuria
investigations for Minimal change
- On light microscopy, the glomeruli look completely normal.
- In some cases, there can be lipids in the proximal tubular cells.
- Immunofluorescence is negative.
- The only changes are seen on electron microscopy, where there’s effacement of podocyte foot processes.
How would you diagnose nephrotic syndrome
EM- effacement of podocyte foot processes
Treatment of minimal change disease
corticosteroids
What is focal segment glomerulosclerosis
disease in which scar tissue develops on the glomeruli
Aetiology of focal segment glomerulosclerosis
- idiopathic
- linked to IV Drug users, HIV
- adults, African + Hispanic
Pathophysiology of focal segment glomerulosclerosis
- not clear
- effacement of podocyte foot processes
- hyalinosis caused by deposition of lipids and proteins in the glomerulus
Investigations for FSG
- On light microscopy, there’s sclerosis and hyalinosis among the glomeruli.
- FSG is segmental - only a part of the glomeruli is affected - and focal - only some of the glomeruli are affected.
- Immunofluorescence is often negative, but can sometimes be positive for deposits of C3, C1 or IgM.
Management of focal segment glomerulosclerosis
-steroids
- ACE-I /ARB
What is membranous nephropathy
immunologically mediated disease of the glomerular basement membrane
What antibody would most likely be present in patients with membranous nephropathy
anti- phospholipase A2 receptor antibody
Management of membranous nephropathy
ACE-i / ARB
where is the deposition of immune complexes in membranous nephropathy
between podocytes and the glomerular basement
Pathophysiology of membranous nephropathy
Damage caused by immune complexes called subepithelial deposits (SD)
SD activate complement system - damage podocytes + mesangial cells
Key causes of nephrotic syndroem
-minimal change disease
- membranous gn
- focal segment glomerulosclerosis
- amyloidosis
- diabetic nephropathy