Histopathology - Renal disease Flashcards

1
Q

Which part of the nephron is impermeable to water?

A

Distal convuluted tubule
thick ascending loop

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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

2 mutations in PCKD?

A

PKD 1 on Chr 16 (polysystin 1)
PKD 2 on Ch 4 (polycystic 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

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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

red cell casts

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

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14
Q

What is rapidly progressive (crescentic) glomerulonephritis? what are the types

A

Immune mediated inflammation of glomerulonephritis with crescents as main histopathological finding

Includes:
type 1 - Anti-GBM disease
type 2- immune complex mediated
type 3 - ANCA associated

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15
Q

Which 5 aetiologies might cause immune complex associated crescentic glomerulonephritis?

A

SLE
IgA nephropathy
Post-infectious glomerulonephritis
alport
HSP

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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 3 features of Pauci-immune crescentic glomerulonephritis?

A

lack of/ scanty immune complex deposition

ANCA associated ( c- wegengers, p- MPA)

Vasculitis everywhere

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19
Q

outline the PHAROH of nephritic syndrome

A

proteinuria (less than nephrotic)
hamateuria
azootemia (high ureas & creatinine)
red cell casts in utrine
oliguria
hypertension

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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
vasculitis

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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
Which antibodies are present in pauci-immune acute crescentic glomerulonephritis?
Anti-neutrophil cytoplasm Ig aka ANCA
26
How can immune complexes in the glomerulus be identified?
Immunohistochemistry Electron microscopy
27
Which syndromes are associated with Pauci-immune Crescentic Glomerulonephritis?
Wegener's Churg Strauss Microscopic polyangiopathy
28
Which kidney disease is associated with E coli diarrhoea?
HUS
29
Recall 2 examples of non-immune complex related, primary nephrotic syndrome
Minimal change disease Focal segmental glomerulosclerosis
30
Recall an example of a primary cause of nephrotic syndrome that is immune mediated?
Membranous glomerulonephritis
31
Recall 3 systemic diseases that can cause nephrotic syndrome
Diabetes mellitus Amyloidosis SLE
32
in all the 3 primary nephrotic sydnromes, how do glomeruli appear under electron microscopy?
loss of foot processes
33
What is a common cause of the nephrotic syndrome in children?
Minimal change disease
34
What treatment does minimal change glomerulonephritis usually respond to?
Immunosuppression 1st line = steroids 2nd = cyclosporine
35
Does focal segmental glomerulonephritis produce the nephrotic or nephritic syndrome?
Nephrotic
36
What is the broad pathophysiology of membranous glomerular disease
Immune deposits are subepithelial, outside of GBM also thickening of BM
37
What age group does membranous glomerular disease sually affect?
Adults
38
what antibody is commonly seen in membranous glomerular disease?
anti- phospholipase A2 type M receptor
39
Does diabetic nephropathy cause nephrotic or nephritic syndrome?
Nephrotic
40
What is amyloidosis?
Deposition of extracellular proteinaceous material exhibiting β-sheet structure
41
What is the inheritance pattern of Alport's syndrome? what mutation
X-linked type IV collagen alpha 5 chain
42
What are the triad of symptoms of Alport's syndrome?
nephritic syndrome sensorineural deafness eye disease
43
What is the most common form of glomerulonephritis?
IgA nephropathy
44
What eGFR is indicative of end-stage renal failure?
\<15
45
What is the most common cause of chronic renal failure?
Diabetes
46
What are anti-GBM antibodies directed against?
The C terminal domain of type IV collagen
47
What kidney disease is characterised by shrunken kidneys with granular cortices?
Hypertensive nephropathy
48
What stain is used to detect amyloidosis?
Congo red (--\> apple green birefringeance)
49
in whom is FSGS most cmonnon
afro carribean adults
50
What might histopathology show in hypertensive nephropathy?
Nephrosclerosis
51
What is the commonest cause of kidney failure requiring renal replacement therapy?
Diabetes
52
How does SLE affect the kidney?
Anti-nuclear anti-dsDNA Ig directed against a broad range of intracellular and extracellular antigens
53
Does SLE cause the nephrotic or nephritic syndrome?
Nephrotic
54
What would be the histological appearance of acute tubulo-interstitial nephritis?
Eosinophils and granulomas
55
Describe each stage of diabetic nephropathy
Stage 1: Basement Membrane thickening Stage 2: Mesangial matrix expansion Stage 3: Kimmelstiel-Wilson nodules Stage 4: Advanced glomerulosclerosis (scarring)
56
How is IgA nephropathy graded?
Oxford classification (MEST-C)
57
muddy brown casts in urine =
pathognomonic for ATN
58
what bacteria is implicated in post strep GN
usually 1-3 WEEKS after thoroat infection or impetigo so group A haemolytic strep = strep pyogenes
59
2 bloods for post strep GN
C3 down ASOT titre up
60
what's seen on microscopy in post strep GN
granular despots of IgG and C3 in GBM
61
most common GN worldwide?
IgA nephropathy = BERGER disease
62
IgA nephropathy is more common in what race
east/south asiana
63
when does IgA nephropathy tend to present after an UTRI
1-2 DAYS (much earlier than post strep GN) PRESENTS W FRANK HAEMATURIA
64
blood results in IgA nephropathy
IgA up note than 1/3rd will progress to CKD
65
give a rare dominant inherited cause of nephritic syndrome. what mutation
thin membrane disease (benign familial haematuria) type IV collagen alphan 4 chain usually asymtomatic
66
give 3 conditions that typically cause asymptomatic haematuria
alport thin membrane disease IgA nephropathy
67
what is lupus nephritis. give a histo finding
GN caused by lupus immune complex deposition- in capillaries = wire loop capillaries in GBM = lumpy-bumpy granular fashion
68
give 3 priamry causes of nephrotic syndrome
minimal change disease membranous gloemrualr disease focal segmental sloemrulosclerosis
69
give 5 diseases that cause a nrphritic syndrome
post strep GN IgA nephropathy rapidly progressive/crescenteric alport's syndrome thin basement memebran disease
70
microscopy findings of membranous glomerular disease
loss of poocytes for all three nephrotics ALSO spikey subepithelial deposits
71
WHAT NEPHRTOIC SYDROM SHOWS DIFFUSE THICKENING OF GBM
MEMBRANOUS