Nephrotic syndrome Flashcards

1
Q

Nephrotic syndrome : definition

A

Glomeruli are damaged therefore become more permeable and allow for plasma proteins from the blood to enter the urine resulting in proteinuria.
Triad of;
* Proteinuria >3g/24 hours
* Hypoalbuminaemia < 30g/L
* Oedema

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2
Q

Nephrotic syndrome : Causative diseases

A
  1. Minimal change disease
  2. Diabetic nephropathy
  3. Amyloidosis
  4. Membraneous Glomerulonephritis
  5. Membranoproliferative Glomerularnephritis
  6. Focal segmental glomerularnephritis
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3
Q

Nephrotic syndrome : Minimal change disease - Pathophysiology

A
  1. T cells in the blood release cytokines
  2. Damage the foot processes of the podocytes
  3. This causes effacement (flattening out of the podocytes)
  4. These foot processes are normally negatively charged thereby would normally repel large proteins like albumin
  5. Effacement means there is less of a charge barrier resulting in proteinuria.
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4
Q

Nephrotic syndrome : Minimal change disease - Clinical features

A

1.** Proteinuria >3.5g per day**
2. Hypoalbuinaemia : secondary to loss of albumin protein from the blood due to proteinuria
3. Oedema : reduced oncotic pressure from protein loss in the blood leads to reduced osmotic pressure, driving water out of the blood vessels and into the tissues.
4. Hyperlipidaemia and lipiduria - loss of protein from the blood inhibit synthesis of lipids leading to increased lipid levels in the blood and more lipid entering the urine.

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5
Q

Nephrotic syndrome : Minimal change disease - Incidence

A
  • Most common nephrotic syndrome seen in children
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6
Q

Nephrotic syndrome : Minimal change disease - Investigation

A

Light microscopy and H&E stain - doesn’t allow for foot process effacement to be seen, thus seems as though there is no change to the glomerulus, hence the name ‘minimal change;

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7
Q

Nephrotic syndrome : Minimal change disease - Mx

A

Corticosteroid therapy - 90% of children respond very well

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8
Q

Nephrotic syndrome : Focal segmental glomerulosclerosis (NORD) - Definition

A
  • continuation of minimal change disease also characterised by podocyte injury.
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9
Q

Nephrotic syndrome : Focal segmental glomerulosclerosis (NORD) - Pathophysiology

A
  1. Kidney disease which affects the kidney’s glomeruli and causes scarring, mainly only segments of some glomeruli are affected.
  2. Damage to foot processes of podocytes allows for plasma proteins and lipids to enter the urine.
  3. Over time - proteins and lipids get trapped and build up in the glomeruli resulting in hyalinosis - over time this results in scarring
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10
Q

Nephrotic syndrome : Focal segmental glomerulosclerosis (NORD) - Causes

A
  1. Primary FSGS : idiopathic
  2. Secondary FSGS : sickle cell disease, HIV, Heroin abuse
    Has high recurrence rate in renal transplants
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11
Q

Nephrotic syndrome : Focal segmental glomerulosclerosis (NORD) - Investigations

A
  • Light microscopy: Focal and segmental sclerosis and hyalinosis
  • Electron microscopy - Effacement of foot processes of podocytes
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12
Q

Nephrotic syndrome : Membranous Glomerulonephritis - Definition

A

Glomerular basement membrane which lines the glomeruli in the kidneys becomes inflamed and damages - secondary to binding of immune complexes to the GMB

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13
Q

Nephrotic syndrome : Membranous Glomerulonephritis - Incidence

A
  • Common type of glomerulonephritis in adults
  • Third common cause of end-stage renal failure
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14
Q

Nephrotic syndrome : Membranous Glomerulonephritis - Pathophysiology

A
  • Damage and inflammation is caused by binding of immune complexes (antibody-antigen complex) to the Glomerular basement membrane
  • Subendothelial deposits - Immune complexes become sandwiched between epithelial cells of podocyts and GBM
  • Auto antibodies may directly target the GBM : antibodies may form against two antigens expressed on the surface of podocytes - M- type phospholipase A2 receptor and neutral endopeptidase
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15
Q

Nephrotic syndrome : Membranous Glomerulonephritis - Causes

A
  1. Idiopathic : anti-phospholipase A2 antibodies
  2. Infections : Malaria, Syphillis, Hepatitis B
  3. Malignancy : prostate, lung, lymphoma and leukaemia
  4. Drugs : NSAIDs, Gold, pencillamine
  5. Autoimmune disease : SLE, Thyroiditis, Rheumatoid arthritis
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16
Q

Nephrotic syndrome : Membranous Glomerulonephritis - Incidence

A

Patients with end stage renal disease

17
Q

Nephrotic syndrome : Membranous Glomerulonephritis - Investigation

A

Light microscopy
1. Thickening of the GBM - GBM matrix is deposited between the immune complex,

Electron microscopy
1. Pattern of GBM Matrix on the sub epithelial deposits creates a characteristic ‘spike and dome pattern
* Effacement of the podocytes is also present.

18
Q

Nephrotic syndrome : Membranous Glomerulonephritis - Management

A
  1. Ace inhibitor or ARB : reduce proteinuria and improve prognosis
  2. Immunosupression : normally in severe progressive disease, combination of corticosteroids and cyclophosphamide.
19
Q

Nephrotic syndrome : Membranous Glomerulonephritis - Prognosis

A

Prognosis - rule of thirds
One third - Spontaneous remission
One third - Remain proteinic
One third - Develop End stage renal failure

20
Q

Nephrotic syndrome : Membranoproliferative Glomerulonephritis - definition

A
  1. Chronic infection - Antigens released -> Bind antibodies in the blood -> Immune complexes deposit in the GBM
  2. immune complex deposits which end up in the walls of the glomerulus
  3. Trigger immune reactions - causing inflammation and result in structural changes to the glomerulus.
  4. Cause mesangial proliferation
  • This leads to nephrotic syndrome - proetienuria, hypoalbumnaemia, oedema, hyperlipidaemia and lipiduria.
21
Q

Nephrotic syndrome : Membranoproliferative Glomerulonephritis - T1 MPGN - causes

A

Causes :
Chronic infection - Hepatitis C, Hepatitis B
Antigens released -> Bind antibodies in the blood -> Immune complexes deposit in the GBM

22
Q

Nephrotic syndrome : Membranoproliferative Glomerulonephritis - Investigations

A
  1. Electron microscopy : Subenthothelial and mesangium immune deposits resulting in ‘Tram-track appearance’