Histopathology 8 - Renal disease Flashcards

1
Q

Which part of the nephron is impermeable to water?

A

Distal convuluted tubule

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2
Q

What is a “horse shoe kidney”?

A

Congenital renal fusion

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3
Q

What is the inheritance pattern of adult polycystic kidney disease?

A

Dominant

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4
Q

What is the triad of symptoms/signs associated with adult PCKD?

A

Hypertension
Flank pain
Haematuria

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5
Q

What are the genetic associations of PCKD?

A

PDK1 PDK2

implicated protein: polycystin 1 and polycystin 2

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6
Q

What is the most strongly associated aneurysm with PCKD?

A

Berry aneurysm

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7
Q

In which patients are kidney cysts most likely to form?

A

End stage renal failure and on dialysis

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8
Q

How can the causes of acute renal failure be classified?

A

Pre-renal/renal/post-renal

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9
Q

What is the most common cause of acute renal failure?

A

Acute tubular injury

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10
Q

How does acute tubular injury affect glomerular filtration?

A

Blockage of tubules by casts –> leakage into interstitial space –> secondary haemodynamic changes that affect GFR

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11
Q

What is acute tubulo-interstitial nephritis, and what causes it?

A

Injury to tubules and interstitium that is usually immune but can also be caused by infection/drugs

these drugs can include NSAIDS and antibiotics

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12
Q

What is the most common cause of acute tubulo-interstitial nephritis?

A

Drugs (especially NSAIDs)

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13
Q

How does acute glomerulonephritis present?

A

Oligouria

Urine casts containing erthrocytes and leucocytes

Crescents present when it is bad enough to cause acute renal failure

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14
Q

What is acute crescentic glomerulonephritis?

A

Immune mediated inflammation of glomerulonephritis with crescents as main histopathological finding

Includes:
Anti-GBM disease
Pauci-immune disease

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15
Q

Which aetiologies might cause immune complex associated crescentic glomerulonephritis?

A

SLE
IgA nephropathy
Post-infectious glomerulonephritis

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16
Q

What is anti-GBM disease?

A

Rare and severe disease caused by Ig directed against the GBM

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17
Q

How can anti-GBM disease affect the lung?

A

Cross-reaction with alveolar basement membrane leading to pulmonary haemorrhage

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18
Q

What are the features of Pauci-immune crescentic glomerulonephritis?

A

Only scanty glomerular Ig deposits

Usually ANCA associated

Vasculitis everywhere

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19
Q

What on earth is thrombotic microangiopathy? (no seriously if someone works this out pls pm me)

A

Damage to endothelium in glomeruli, arterioles and arteries leading to thrombosis??

*HUS, TTP, DIC

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20
Q

What is nephrotic syndrome, and what are is its four diagnostic requirements?

A

Breakdown in selectivity of glomerular filtration barrier leading to protein leak

Proteinuria (>3.5g/day)

Hypoalbuminaemia

Oedema

Hyperlipidaemia

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21
Q

Recall a pre-renal cause of acute renal failure

A

Failure of perfusion of kidney

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22
Q

Recall 3 renal causes of acute renal failure

A

Acute tubular injury
Acute glomerulonephritis
Thrombotic microangiopathy

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23
Q

How can acute renal failure be caused post-renally?

A

Obstruction

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24
Q

How can trauma cause acute renal failure?

A

Release of myoglobin damages tubular epithelial cells, causing acute tubular injury (rhabodmyolysis)

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25
Q

Which antibodies are present in pauci-immune acute crescentic glomerulonephritis?

A

Anti-neutrophil cytoplasm Ig

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26
Q

How can immune complexes in the glomerulus be identified?

A

Immunohistochemistry
Electron microscopy

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27
Q

Which syndromes are associated with Pauci-immune Crescentic Glomerulonephritis?

A

Wegener’s
Churg Strauss
Microscopic polyangiopathy

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28
Q

Which kidney disease is associated with E coli diarrhoea?

A

Thrombotic microangiopathy

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29
Q

Recall 2 examples of non-immune complex related, primary nephrotic syndrome

A

Minimal change disease

Focal segmental glomerulosclerosis

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30
Q

Recall an example of a primary cause of nephrotic syndrome that is immune mediated?

A

Membranous glomerulonephritis

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31
Q

Recall 3 systemic diseases that can cause nephrotic syndrome

A

Diabetes mellitus
Amyloidosis
SLE

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32
Q

In minimal change glomerulonephritis, how do glomeruli appear under electron microscopy?

A

Effacement of foot processes

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33
Q

What is a common cause of the nephrotic syndrome in children?

A

Minimal change disease

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34
Q

What treatment does minimal change glomerulonephritis usually respond to?

A

Immunosuppression

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35
Q

Does focal segmental glomerulonephritis produce the nephrotic or nephritic syndrome?

A

Nephrotic

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36
Q

What is the broad pathophysiology of primary membranous glomerulonephritis?

Description of membraneous glomerulonephritis?

A

Immune complex related

Immune deposits are subepithelial, outside GBM

Description: spikey subepithelial deposits

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37
Q

What age group does membranous glomerulonephritis usually affect?

A

Adults

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38
Q

What is the most common cause of membranous glomerulonephritis?

A

75% are immune

Antipody against phospholipase A2 receptor

39
Q

Does diabetic nephropathy cause nephrotic or nephritic syndrome?

A

Nephrotic

40
Q

What is amyloidosis?

A

Deposition of extracellular proteinaceous material exhibiting β-sheet structure

41
Q

What is the inheritance pattern of Alport’s syndrome?

A

X-linked dominant
Affects alpha 5 subunit

42
Q

What are the symptoms of Alport’s syndrome?

A

Renal failure in middle age
Deafness
Ocular disease

43
Q

What is the most common form of glomerulonephritis worldwide?

A

IgA nephropathy

44
Q

What eGFR is indicative of end-stage renal failure?

A

<15

45
Q

What is the most common cause of chronic renal failure?

A

Diabetes

46
Q

What are anti-GBM antibodies directed against?

A

The C terminal domain of type IV collagen

47
Q

What kidney disease is characterised by shrunken kidneys with granular cortices?

A

Hypertensive nephropathy

48
Q

What stain is used to detect amyloidosis?

A

Congo red (–> apple green birefringeance)

49
Q

What socring system is used to score IgA renal disease?

A

Oxford classification

50
Q

What might histopathology show in hypertensive nephropathy?

A

Nephrosclerosis

51
Q

What is the commonest cause of kidney failure requiring renal replacement therapy?

A

Diabetes

52
Q

How does SLE affect the kidney?

A

Anti-nuclear anti-dsDNA Ig directed against a broad range of intracellular and extracellular antigens

53
Q

What would be the histological appearance of acute tubulo-interstitial nephritis?

A

Eosinophils and granulomas

also white cell casts

54
Q

What is the usual cause of non-diarrhoea-associated thrombotic microangiopathy?

A

ADAMTS13 deficiency (eg scleroderma and anti-phospholipid syndrome)

55
Q

Describe each stage of diabetic nephropathy

A

Stage 1: Basement Membrane thickening
Stage 2: Mesangial matrix expansion
Stage 3: Nodular lesions (Kimmelstiel-Wilson)
Stage 4: Advanced glomerulosclerosis

56
Q

How is IgA nephropathy graded?

A

Oxford classification (MEST-C)

57
Q

What two properties does the filtration barrier created by the podocytes depend on?

A

Charge dependent and size dependent

58
Q

Role of the PCT

A

Actively resorbs sodium

Carries out hydrogen exchange to allow carbonate resorption

Co-transport of amino acids, PO4 and glucose

Reabsorption of potassium

59
Q

Role of the LoH

A
  • The descending and thin ascending limb is permeable to water
    • but NOT ions or urea
  • The ascending limb actively reabsorbs sodium and chloride
  • This creates a counter-current multiplier that is aligned with the vasa recta
    • vasa recta= straight arterioles, and the straight venules of the kidney

**basc point is to concentrate the urine

60
Q

Role of DCT

A
  • Impermeable to water
  • Regulates pH by active transport of proteins and bicarbonate
  • Regulates sodium and potassium by active transport (aldosterone regulated)
  • Regulates calcium (PTH, 1,25-dihydroxy vitamin D)
61
Q

Role of the collecting duct

A

Aldosterone acts on these cells → Na retention and K excretion

Reabsorbs water (principle cells, ADH)

Regulates pH (intercalated cells, proton excretion)

62
Q

What is the renal corpuscle?

A

Consists of the glomerulus and bowman’s capsule

63
Q

Membranous glomerulonephritis histology

Treatment of membranous glomerulonephritis?

A
  • IgG deposits with C3
  • In sub-epithelial area

**NB- this is immune complex mediated unlike minimal change disease and focal segmental glomerulosclerosis

Treatment: canot be treated with steroids unlike minimal change disease; treated with antihypertensives

64
Q

What protein is deficient in adult polycystic kidney disease?

A

polycystin-1

65
Q

Complications of adult PKD

A
  • subarachnoid haemorrhage (berry aneurysms)
  • 10% mortality from renal failure
66
Q

Which cancer do renal cysts increase the risk of?

A

Papillary renal cell cancer

67
Q

Definition of AKI

A

Increase in SCr by ≥0.3mg/dL w/in 48h

Increase in SCr ≥ 1.5 x baseline w/in last week

Urine volume < 0.5 ml/kg/h for 6h

68
Q

Drugs that affect glomerular blood flow (and therefore predispose to pre-renal AKI)

A
69
Q

ATN histology

A

necrosis of short segments of tubules

Epithelial cell casts

(epithelial cells lining the tubules die and then block off the actual lumen of the tubule

70
Q

What intrinsic renal failure can pre-renal AKI lead to?

A

Can eventually lead to ATN if damage is sufficient

71
Q

Most common cause of AKI

A

Acute tubular necrosis

**intrinsic renal failure**

72
Q
A
73
Q

Histology of HUS

A

Fibrinoid necrosis

*because you get fibrin strand deposition which leads to MAHA*

74
Q

Which form of glomeruloneprhitis is a medical emergency?

A

Crescentic

75
Q

Causes of crescentic glomerulonephritis

A
  1. Immune complex associated
    • SLE
    • IgA nephropathy
    • post-infectious glomerulonephritis
  2. Anti-GBM disease
  3. Pauci-immune (pauci = not much)
    • ANCA mediated eg Wegener’s (c-ANCA) (WC)
76
Q

What cells are seen on biopsy in goodpasture’s disease?

A

Crescent cells

77
Q

Histology of acute tubulointerstitial nephritis

A

heavy interstitial inflammatory infiltrate with tubular injury:

Eosinophils

Granulomas

78
Q

How does rhabdomyololysis cause AKI? Tx

A

Myoglobin causes acute tubular necrosis

Treatment: fluids to wash out the myoglobin

79
Q

3 common causes of nephrotic syndrome

A

Primary glomerular causes (non-immune complex mediated)

  1. Miminal change disease
  2. focal segmental glomerulosclerosis - same changes on electron microscopy as minimal change disease (loss of podocyte foot processes) but on light microscopy you see FOCAL AND SEGMENTAL SCARRING

Primary (immune complex mediated)

Membranous glomeurlonephritis

Systemic diseases

  1. diabetic nephropathy
  2. Amyloidosis
  3. SLE
80
Q

Minimal change disease: light and electron microscopy

A

Light microscopy: no change

Electron microscopy: effacement of podocytes

81
Q

IgA nephropathy findings

A

main clinical feature: frank haematuria

82
Q

PSGN

histology findings

A
  • Light microscope (LM): ↑cellularity of glomeruli
  • Fluorescence Microscope (FM):
    • granular deposits of IgG and C3 in GBM
  • Electron Microscope (EM):
    • Subendothelial humps
83
Q

Membranoproliferative glomerulonephritis hsitology

A
  • mesangial cell proliferation
  • and capillary thickening
84
Q

What are two hereditary causes of microscopic haematuria?

A

Alport’s syndrome

Thin basement membrane disease - usually get microscopuc haematuria unlike frank haematuria in IgA nephropathy

85
Q

Classification+ description of lupus nephritis

A

Renal Histology: immune complex deposition in capillaries à ‘wire loop capillaries’, deposition of immune complexes & complement I the GBM in a lumpy-bumpy granular fashion

86
Q

Histology of diabetes causing nephrotic sydnrome

A

Diffuse glomerular basmenet membrane thickening

Mesangial matrix nodules- aka Kimmelstiel Wilson nodules

87
Q

Histology of acute pyelonephritis

A

leucocytic casts

88
Q

Histology of acute interstitial nephritis

A

Histology: inflammatory infiltrate with tubular injury, eosinophils & granulomas

89
Q

HUS vs TTP

A
90
Q

Commonest causes of chronic renal failure in the UK

A

Diabetes (19.5%) ● Glomerulonephritis (15.3%) ● Hypertension & Vascular disease (15%) ● Reflux nephropathy (chronic pyelonephritis) (9.5%) ● Polycystic kidney disease (9.4%)

91
Q

3 types of renal cell carcinoma

A

Clear cell carcinoma – well differentiated
● Papillary carcinoma – commonest in dialysis-associated cystic disease
● Chromophobe renal carcinoma – pale, eosinophilic cells

92
Q

What is alport syndrome?

Inheritance pattern?

A

nephritic syndrome, cataracts and sensorineural deafness suggests what underyling diagnosis?

x linked dominant

93
Q

Classification of nephritic and nephrotic syndromes

A
94
Q

Which protein is responsible for dark urine in rhabdomyololysis?

A

myoglobin