Nephrotic syndrome Flashcards

1
Q

What are the main abnormalities seen that define nephrotic syndrome? Why do they happen?

A
  1. Massive proteinuria (≥3.5g/day)
  2. Hypoalbuminaemia (serum albumin ≤30g/L) lost in urine due to gaps in podocytes
  3. Oedema - reduced intravascular oncotic pressure - fluid moves out of intravascular compartment into tissue
  4. Hyperlipidaemia - liver compensates loss of albumin and increases production w SE of also increasing production of lipids
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2
Q

How does urine appear?

A

frothy

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3
Q

What are the primary causes of nephrotic syndrome?

A
  1. minimal change disease
  2. Focal segmental glomerulosclerosis
  3. Membranous nephropathy
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4
Q

What are the secondary causes?

A
SLE
DM
Hep B/C
HIV
Malignancy - myeloma 
Amyloid 
Pre-eclampsia
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5
Q

How does nephrotic syndrome generally present?

A
  • Generalised pitting oedema (ankles if mobile, sacral pad/elbows if bed bound) and periorbitally due to low tissue resistance
  • Systemic sx if secondary cause
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6
Q

What are the DD of nephrotic syndrome, how would u differentiate?

A

CCF - raised JVP, palm oedema

Liver disease - (reduced albumin)

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7
Q

What is the management of nephrotic syndrome?

A
  1. Reduce oedema - 1L/day fluid + salt restriction, furosemide, aim for loss of 0.5-1kg loss/day to avoid intravascular volume depletion and secondary AKI
    Thiazide if resistant to loops
  2. Rx underlying cause
  3. Reduce proteinuria - ACEi/ARBs
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8
Q

Why shouldn’t a renal biopsy be performed in children?WHat are the exceptions?

A

Minimal change is most likely

Exceptions: <1yr, FHx, renal failure, haematuria, extra-renal disease

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9
Q

What are the potential complications of nephrotic syndrome and how do u manage them? Why do these complications occur?

A
  1. Thromboembolism:
    - Hypercoaguable state due to increase in clotting factors, reduced anti-thrombin and platelet abnormalities leading to increased risk of PE/DVT and renal vein thrombosis
    Rx: heparin + warfarin
  2. Infection:
    - Due to urine losses of Igs + immune mediators (increased risk of CNS, res and urinary)
  3. Hyperlipidaemia:
    - Due to raised cholesterol (>10), LDL + triglycerides and lower HDL levels
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10
Q

What are the causes of minimal change disease?

A

Mostly idiopathic

Assoc w drugs (NSAIDs, lithium) or paraneoplastic (haematological malignancy, usually Hodgkins)

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11
Q

Does minimal change disease lead to RF?

A

No

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12
Q

How is minimal change disease diagnosed?

A

Effacement of foot processes on podocytes on electron microscopy of biopsy

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13
Q

Why is it called minimal change disease?

A

Appears normal on light microscopy of renal biopsy

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14
Q

What is the treatment of minimal change diseasE?

A

Prednisolone 1mg/kg for 4-16 weeks

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15
Q

What is the treatment of relapses of minimal change disease?E

A

raise the dose of prednisone
OR
long term immunosuppression (cyclophosphamide, calcineurin inhibitors (tacrolimus))

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16
Q

What are the causes of focal segmental glomerulosclerosis (FSG)?

A
  1. Primary - idiopathic

2. Secondary - HIV, heroin, lymphoma, causes of reduced kidney/nephrons, kidney scarring due to GN

17
Q

What is the risk of FSG?

A

Progressing to CKD then kidney failure

18
Q

What indicates a worse prognosis of FSG?

A

Increased proteinuria

19
Q

What is FSG?

A

essentially scarring in small sections of glomeruli w limited no. of glomeruli affected at first

20
Q

What is the treatment FSG?

A
  1. BP control w ACEi/ARB
  2. CS in primary disease
  3. 2nd line - calcineurin inhibitors
21
Q

What is the treatment of FSG recurring in transplants?

A

plasma exchange and rituximab

22
Q

What are the causes of membranous nephropathy?

A
  1. Primary - idiopathic
  2. Secondary:
    i. malignancy - lung, breast, GI, prostate
    ii. infection - hep B/C, strep, malaria, schisto
    iii. Immune disease - SLE, RA, sarcoidosis, sjogrens
    iv. Drugs - gold, penicillamine, NSAIDs
23
Q

How is membranous nephropathy diagnosed?

A
  1. Anti-phospholipase A2 receptor ab found in most w idiopathic MN
  2. diffusely thickened GBM due to sub epithelial deposits
24
Q

What is the rx of membranous nephropathy ?

A

Immunosuppression if increased risk of progression (cyclophosphamide + CS)

25
Q

What are the two types of membranoproliferative GN?

A
  1. immune complex associated - increased of abnormal immune complexes which deposit in kidney and active complement
  2. C3 glomerulopathy - genetic or acquired defect in alternative complement pathway
26
Q

How is membranoproliferative GN diagnosed?

A

proliferative GN w electron dense depositis

Ig deposition distinguishes between types

27
Q

What is the treatment for membranoproliferative GN?

A

BP control ACEi/ARB

trial of immunosuppression if no underlying cause found and progressive decline in renal function