Nephrotic & Nephritic Syndromes Flashcards

1
Q

Which part of the nephron is affected in nephrotic and nephritic syndromes?

A

Glomerulus

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2
Q

Define nephrotic syndrome

A

Syndrome characterised by proteinuria >3g/24h, hypoalbuminaemia, and oedema. There will likely be hyperlipidaemia.

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3
Q

State the three primary causes of nephrotic syndrome

A

Minimal change disease, membranous glomerular disease, focal segmental glomerulosclerosis (GSGS)

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4
Q

Define nephritic syndrome

A

Syndrome characterised by PHAROH: Proteinuria (less than nephrotic syndrome), Haematuria, Azotaemia (high urea and creatinine), Red cell casts in urine, Oliguria, and Hypertension

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5
Q

State at least three causes of nephritic syndrome

A

Acute post-streptococcal glomerulonephritis, Berger disease (IgA nephropathy), rapidly progressive (crescentic) glomerulonephritis, hereditary nephritis (Alport’s syndrome), thin basement membrane disease(benign familial haematuria)

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6
Q

Describe the epidemiology of minimal change disease

A

75% of cases in children, second peak in elderly

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7
Q

Describe the epidemiology of focal segmental glomerulosclerosis (FSGS)

A

Most common in adults of Afro-Caribbean descent

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8
Q

Describe the signs of minimal change disease under light microscopy

A

No changes

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9
Q

Describe the signs of minimal change disease under electron microscopy

A

Loss of podocyte foot processes

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10
Q

Describe the signs of membranous glomerular disease under light microscopy

A

Diffuse glomerular basement membrane thickening

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11
Q

Describe the signs of membranous glomerular disease under electron microscopy

A

Loss of podocyte foot processes, subepithelial deposits giving a spikey appearance

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12
Q

Describe the signs of focal segmental glomerulosclerosis under light microscopy

A

Focal and segmental glomerular consolidation and scarring, hyalinosis

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13
Q

Describe the signs of focal segmental glomerulosclerosis under electron microscopy

A

Loss of podocyte foot processes

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14
Q

Describe the signs of minimal change disease under immunofluorescence

A

No immune deposits

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15
Q

Describe the signs of membranous glomerular disease under immunofluorescence

A

Ig and complement in granular deposits along entire glomerular basement membrane

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16
Q

Describe the signs of FSGS under immunofluorescence

A

Ig and complement in scarred areas

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17
Q

State the underlying causes of membranous glomerular disease

A

Idiopathic (primary), SLE, infection, drugs, malignancy

18
Q

State the underlying causes of FSGS

A

Idiopathic (primary), obesity, HIV nephropathy

19
Q

State two secondary causes of nephrotic syndrome

A

Diabetes mellitus, amyloidosis

20
Q

Describe the histological appearance of diabetic nephropathy

A

Diffuse glomerular basement membrane thickening, Kimmelstiel Wilson nodules (mesangial matrix nodules)

21
Q

Describe the histological appearance of renal amyloidosis

A

Apple green birefringence with Congo red stain

22
Q

Describe acute-post infectious glomerulonephritis

A

Occurs 1-3 weeks after streptococcal throat infection or impetigo. Immune complex deposition leads to glomerular damage, haematuria, proteinuria, oedema, and hypertension

23
Q

Describe the features of acute post-infectious glomerulonephritis on light microscopy

A

Increased cellularity of glomeruli

24
Q

Describe the features of acute post-infectious glomerulonephritis on electron microscopy

A

Subendothelial humps

25
Q

Describe the features of acute post-infectious glomerulonephritis on fluorescent microscopy

A

Granular deposits of IgG and C3 in the glomerular basement membrane

26
Q

Describe IgA nephropathy (Berger disease)

A

Deposition of IgA immune complexes in the glomeruli 1-2 days after an UTRI, causing frank haematuria

27
Q

Describe the features of IgA nephropathy on fluorescent microscopy

A

Granular deposition of IgA and complement in mesangium

28
Q

State the three types of rapidly progressive glomerulonephritis

A

Type 1: anti-glomerular basement membrane antibody
Type 2: immune complex
Type 3: pauci-immune/ ANCA-associated

29
Q

How is rapidly progressive glomerulonephritis characterised?

A

Presence of crescents in glomeruli

30
Q

State the causes of type 1 rapidly progressive glomerulonephritis

A

Goodpasture’s syndrome - anti-glomerular basement membrane antibody against COL4-A3 (collagen type IV)

31
Q

Describe the appearance of type 1 rapidly progressive glomerulonephritis on fluorescent microscopy

A

Linear deposition of IgG in the glomerular basement membrane

32
Q

Describe the appearance of type 2 rapidly progressive glomerulonephritis on fluorescent microscopy

A

Granular IgG immune complex deposition on the glomerular basement membrane and mesangium

33
Q

Describe the appearance of type 3 rapidly progressive glomerulonephritis on fluorescent microscopy

A

Lack of significant immune complex deposition

34
Q

State the additional organs involved in type 1 rapidly progressive glomerulonephritis

A

Lungs (pulmonary haemorrhage)

35
Q

State the additional organs involved in type 2 rapidly progressive glomerulonephritis

A

None (except in SLE)

36
Q

State the additional organs involved in type 3 rapidly progressive glomerulonephritis

A

Blood vessels (vasculitis, presenting as skin rashes or pulmonary haemorrhage)

37
Q

State the causes of type 3 rapidly progressive glomerulonephritis

A

Granulomatosis with polyangiitis (c-ANCA), microscopic polyangiitis (p-ANCA)

38
Q

Define Alport’s syndrome

A

Hereditary (X-linked) glomerular disease caused by a mutation in the alpha 5 chain of type IV collagen

39
Q

State the clinical features of Alport’s syndrome

A

Nephritic syndrome, sensorineural deafness, eye disorders (lens dislocation, cataracts)

40
Q

When does Alport’s syndrome present?

A

Age 5-20 with nephritic syndrome progressing to end-stage renal failure

41
Q

Define thin basement membrane disease

A

Benign familial haematuria - diffuse thinning of the glomerular basement membrane caused by an autosomal dominant mutation in the alpha4 chain of type IV collagen. 5% prevalence, very rarely causes nephritic syndrome - usually incidentally diagnosed with microscopic haematuria