Nephrotic Syndrome Flashcards

1
Q

What is Nephrotic syndrome?

A

Occurs when the GBM becomes highly permeable to protein, allowing them to leak from the blood into the urine.

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2
Q

How does Nephorotic syndrome present?

A
  1. Frothy urine
  2. Generalised oedema
  3. Pallor
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3
Q

What is the classic triad seen of Nephrotic syndrome?

A
  1. Low serum albumin
  2. High urine protein content (>3+ protein on urine dipstick)
  3. Oedema
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4
Q

List 3 other features that occur in patients with nephrotic syndrome

A
  1. Deranged lipid profile, ↑cholesterol, TAGs and LDLs
  2. ↑BP
  3. Hyper-coagulability, ↑ risk of thrombis
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5
Q

What is the structure of the glomerulus?

A
  1. Fenestrated endothelial cells
  2. GMB
  3. Podocytes
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6
Q

Study light micrograph of a ‘normal’ glomerulus

A

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)

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7
Q

Pathophysiology of Proteinuria? (3)

A
  1. Podocyte foot process effacement, slit diaphragm disruption, depletion of podocytes
  2. Mutations
  • Nephrin and podocin → maintain slit diaphragm
  • Alpha-actinin-4 → integrity of podocyte cytoskeleton
  1. Autoantibodies to podocyte antigens or circulating factors that affect podocyte
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8
Q

Pathophysiology of oedema in Nephrotic syndrome?

A
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9
Q

What is the general management of Nephrotic syndrome?

A
  1. High dose steroids (i.e. prednisolone)
  2. Low salt diet
  3. Diuretics → oedema
  4. Albumin infusions in severe hypoalbuminaemia
  5. Antibiotic prophylaxis in severe cases
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10
Q

List 3 primary and 3 secondary cause of Nephrotic syndrome

A

Primary

  • Minimal change disease
  • Membranous GN
  • Focal segmental glomerulosclerosis (FSGS)

Secondary

  1. Diabetic nephropathy
  2. Amyloidosis
  3. Lupus
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11
Q

What is the most common cause of Nephrotic syndrome in children?

A

Minimal change disease

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12
Q

Causes of Minimal change disease?

Which is the most common

A

Most commonly it occurs with no clear underlying condition or pathology

May be secondary to

  • Intrinsic kidney disease
  • HSP
  • Diabetes
  • HIV, hepatitis and malaria
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13
Q

What changes are seen in minimal change disease for the following:

  1. Light microscopy
  2. Immunofluorescence
  3. Electron microscopy
A
  1. LM – Normal
  2. IF – Normal (no immune deposits)
  3. EM – Fusion of podocytes and effacement of foot processes
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14
Q

Pathogenesis of minimal change disease?

A

T-cell and cytokine-mediated damage to the glomerular basement membrane → polyanion loss

Resultant reduction of electrostatic charge → ↑ glomerular permeability to serum albumin

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15
Q

Management and prognosis of minimal change disease

A

Management:

  • Corticosteroids (i.e. prednisolone)
  • Cyclophosphamide and ACEi for steroid-resistant cases

Prognosis: Good, most children make a full recovery (may relapse)

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16
Q

What is commonest cause of nephrotic syndrome in adults (non diabetic)?

A

Focal Segmental Gomerulosclerosis (FSGS)

17
Q

Causes of FSGS?

A
  1. Idiopathic
  2. Secondary to other renal pathology e.g. IgA nephropathy, reflux nephropathy
  3. HIV
  4. Heroin
  5. Alport’s syndrome or Sickle-cell
18
Q

What changes are seen in FSGS for the following:

  1. Light microscopy
  2. Immunofluorescence
  3. Electron microscopy
A

LM: focal and segmental sclerosis and hyalinosis

IF: IgM and C3 deposition in sclerotic areas

EM: effacement of foot processes

19
Q

FSGS is noted for having a high recurrence rate in ___________

A

Renal transplants

20
Q

Management and prognosis of FSGS?

A

Management: steroids +/- immunosuppressants

Prognosis: rule of thirds

  • 1/3: spontaneous remission
  • 1/3: remain proteinuric
  • 1/3: develop ESRF
21
Q

What is the commonest cause of cause of nephrotic syndrome in adults?

A

Membranous glomerulonephritis

22
Q

Causes of Membranous GN?

A
  1. Idiopathic: due to anti-phospholipase A2 antibodies
  2. Infections: hep B, malaria, syphilis
  3. Malignancy: prostate, lung, lymphoma, leukaemia
  4. Drugs: gold, penicillamine, NSAIDs
  5. Autoimmune: SLE, thyroiditis, RA
23
Q

What changes are seen in Membranous GN for the following:

  1. Light microscopy
  2. Immunofluorescence
  3. Electron microscopy
A

LM: Thickened capillary BM, BM spikes on silver stain

IF: diffuse granular GBM staining

EM: thickened BM with subepithelial deposits → spikes on silver stain

24
Q

Pathogenesis of Membranous GN?

A

Circulating antibodies permeate GBM and form immune complexes

(Phospholipase A2 receptor in 75-80% of primary MN)

25
Q

List 4 complications of Nephrotic syndrome

A
  1. Hypovolaemia: fluid leaks into interstitial space → oedema and ↓BP
  2. Thrombosis: ↓ATIII + ↑clotting factors
  3. Infection: kidneys leak Ig’s, ↓ immune system + exacerbated by steroid treatments
  4. AKI or CKD
  5. Relapse