Nephrotic Syndrome Flashcards
What is Nephrotic syndrome?
Occurs when the GBM becomes highly permeable to protein, allowing them to leak from the blood into the urine.
How does Nephorotic syndrome present?
- Frothy urine
- Generalised oedema
- Pallor
What is the classic triad seen of Nephrotic syndrome?
- Low serum albumin
- High urine protein content (>3+ protein on urine dipstick)
- Oedema
List 3 other features that occur in patients with nephrotic syndrome
- Deranged lipid profile, ↑cholesterol, TAGs and LDLs
- ↑BP
- Hyper-coagulability, ↑ risk of thrombis
What is the structure of the glomerulus?
- Fenestrated endothelial cells
- GMB
- Podocytes
Study light micrograph of a ‘normal’ glomerulus
Only 1 or 2 cells per capillary tuft
Capillary lumens are open
Thickness of the glomerular capillary wall (long arrow) is similar to tubular basement membranes (short arrow)
Mesangial cells and mesangial matrix are located in central regions of the tuft (arrows)
Pathophysiology of Proteinuria? (3)
- Podocyte foot process effacement, slit diaphragm disruption, depletion of podocytes
- Mutations
- Nephrin and podocin → maintain slit diaphragm
- Alpha-actinin-4 → integrity of podocyte cytoskeleton
- Autoantibodies to podocyte antigens or circulating factors that affect podocyte
Pathophysiology of oedema in Nephrotic syndrome?
What is the general management of Nephrotic syndrome?
- High dose steroids (i.e. prednisolone)
- Low salt diet
- Diuretics → oedema
- Albumin infusions in severe hypoalbuminaemia
- Antibiotic prophylaxis in severe cases
List 3 primary and 3 secondary cause of Nephrotic syndrome
Primary
- Minimal change disease
- Membranous GN
- Focal segmental glomerulosclerosis (FSGS)
Secondary
- Diabetic nephropathy
- Amyloidosis
- Lupus
What is the most common cause of Nephrotic syndrome in children?
Minimal change disease
Causes of Minimal change disease?
Which is the most common
Most commonly it occurs with no clear underlying condition or pathology
May be secondary to
- Intrinsic kidney disease
- HSP
- Diabetes
- HIV, hepatitis and malaria
What changes are seen in minimal change disease for the following:
- Light microscopy
- Immunofluorescence
- Electron microscopy
- LM – Normal
- IF – Normal (no immune deposits)
- EM – Fusion of podocytes and effacement of foot processes
Pathogenesis of minimal change disease?
T-cell and cytokine-mediated damage to the glomerular basement membrane → polyanion loss
Resultant reduction of electrostatic charge → ↑ glomerular permeability to serum albumin
Management and prognosis of minimal change disease
Management:
- Corticosteroids (i.e. prednisolone)
- Cyclophosphamide and ACEi for steroid-resistant cases
Prognosis: Good, most children make a full recovery (may relapse)