Histopathology 10: Renal Disease Flashcards

1
Q

List the triad of symptoms that define Nephrotic syndrome?

A

breakdown in selectivity of glomerular filtration barrier leading to protein leak

-Proteinuria (>3g/24h)
-Hypoalbuminaemia
-Oedema
(hyperlipidaemia)

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2
Q

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

A

Minimal change disease

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3
Q

Electron microscopy shows loss of podocyte foot processes With no signs of immune deposits on immunofluorescence. This suggests ………. a cause of Nephrotic syndrome.

A

Minimal change disease

glomeruli look normal on light microscopy
effacement of foot processes on electron microscopy
responds to immunosuppression

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4
Q

Microscopy shows ‘Spikey” subepithelial deposits and diffuse glomerular membrane thickening. This is most likely ………… a cause of Nephrotic syndrome with a poor prognosis.

A

Membranous glomerular disease

membrane deposits on the outside of GBM
common cause of nephrotic syndrome in adults
primary disease is AI - antibody against phospholipase A2 type M receptor

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5
Q

Light microscopy shows focal and segmental glomerular consolidation and scarring. The Afro-Caribbean lady is most likely suffering with ……… a cause of Nephrotic syndrome.

A

Focal segmental glomerulosclerosis (FSGS)

some glomeruli are partly scarred
less likely to respond to immunosuppression

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6
Q

What causes Apple green birefringence with Congo red stain ?

A

Amyloidosis

deposition of EC proteinaceous material exhibiting B sheet structure

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7
Q

Does multiple myeloma cause AL or AA amyloidosis ?

A

AL

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8
Q

List 2 causes of AA amyloidosis?

A

Arthritis
TB

(Cause by chronic inflammation)

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9
Q

A mnemonic for the characteristic features of Nephritic syndrome is PHAROH. List the characteristic features?

A
Proteinuria (less than Nephrotic syndrome)
Haematuria 
Azootemia (high urea and creatinine) 
Red cell casts
Oliguria 
Hypertension
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10
Q

List 3 causers of Nephrotic syndrome ?

A

Minimal change disease
Membranous glomerular disease
Focal segmental glomerulosclerosis (FSGS)

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11
Q

Post streptococcal glomerulonephitis usually occurs 1-3 weeks post throat infection with ………………. or after Impetigo. The bloods show increased ……. and decreased …….. . Immunoflouresence shows …………………………. in the ……….. and this is a cause of nephritic syndrome.

A
group A alpha haemolytic streptococcus (S.pyogenes)
ASOT (antistreptolysin O titre) 
C3
Granular deposits of IgG and C3 
Basement membrane
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12
Q

Which cause of Nephritic syndrome characteristically occurs 1-2 days after a URTI with frank haematuria?

A

IgA nephropathy

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13
Q

Which cause of Nephritic syndrome is most aggressive and presents with particularly pronounced renal failure and oliguria?

A

Rapidly progressive Glomerulonephritis

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14
Q

What is the characteristic feature of all types of rapidly progressive glomerulonephritis?

A

Crescents in the glomeruli

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15
Q

A patient has pulmonary haemorrhage and haematuria. ImmunoFluorescence shows IgG deposition in the GBM. This is most likely ……………….

A

Goodpasture’s syndrome

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16
Q

Which HLA type is associated with Goodpasteures syndrome ?

A

HLA-DRB1

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17
Q

The main pathogenesis of Goodpasteures syndrome is ………

A

Anti-GMB antibodies

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18
Q

SLE causes Rapidly progressive glomerulonephritis. Immunoflourescence shows ……… deposition on the GMB.

A

Granular IgG immune complex

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19
Q

List 2 Pauci-immune causes of Rapidly progressive glomerulonephritis?

A

Granulomatosis with polyangitis (Wegener’s granulomatosis)

Microscopic polyangitis

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20
Q

Match these

Granulomatosis with polyangitis
Microscopic Polyangitis.

p-ANCA
c-ANCA
ANA
AMA

A

Granulomatosis with polyangitis c-ANCA

Microscopic Polyangitis. p-ANCA

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21
Q

Immunoflourescence shows scanty immune complex deposition. This suggests a ………… cause off rapidly progressive glomerulonephritis

A

Pauci-immune (granulomatosis with polyangitis, Microscopic polyangitis)

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22
Q

List the triad of characteristic features of Alport syndrome?

A

Nephritic syndrome
Sensorineural deafness
Eye disorders (cataracts, lens dislocation)

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23
Q

Benign familial haematuria is caused by ………… and shows asymptomatic haematuria which has ………….. inheritance.

A

Thinning of GBM

Autosomal dominant

24
Q

List 3 diseases that cause asymptomatic haematuria ?

A

Alport syndrome
IgA nephropathy
Benign familial haematuria (thin basement membrane syndrome)

25
Q

List 3 causes of ATN ?

A

Hypovolaemia
Drugs (NSAIDs)
Contrast for radiological imaging
heavy metals

26
Q

What is the most common cause of acute pyelonephritis ?

A

E.coli

27
Q

Which 2 scans are used to investigate VUR in children ?

A

MCUG (micturition cystourogram)- identifies the reflux

DMSA - identifies renal scarring

28
Q

How does acute interstitial nephritis present?

A

Fever, skin rash, haematuria, anaemia, proteinuria caused by a hypersensitivity reaction after exposure to a drug such as NSAIDs or antibiotics

29
Q

List the triad for HUS ?

A

MAHA
Thrombocytopaenia (after renal thrombosis)
renal failure

30
Q

List pentad for TTP

A
MAHA
Thrombocytopaenia (after widespread thrombosis)
renal failure
Fever
Neurological signs
31
Q

List 2 causes of secondary Nephrotic syndrome ?

A
  • Amyloidosis

- Diabetes

32
Q

Match the type of RPGN with the immunofluorescence

Type

Type 1 (Goodpastures)
Type 2 (Immune complex e.g SLE)
Type 3 (Pauci-immune)

Immunoflourescence

Linear
Scanty
Granular

A
Type 1 (Goodpastures)- Linear
Type 2 (Immune complex e.g SLE)- Granular
Type 3 (Pauci-immune)- Scanty
33
Q

List 5 causes of nephritic syndrome ?

A
Post strep infection
IgA nephropathy
RPGN
Alport's syndrome
Benign familial haematuria
34
Q

What causes chronic interstitial nephritis ?

A

Long term analgesic consumption e.g NSAIDS or paracetamol

35
Q

List 3 pre-renal causes of renal failure ?

A
  • Hypovolaemia
  • Sepsis
  • Renal artery stenosis
36
Q

List 3 renal causes of renal failure

A
  • ATN
  • Acute glomerulonephritis
  • Thrombotic microangiopathy
37
Q

List 3 post-renal causes of renal failure

A
  • stones
  • tumours
  • prostatic hyperplasia
38
Q

What GFR defines end stage renal failure (stage 5)

A

GFR <15

39
Q

Which autosomal dominant disease is associated with renal failure and berry aneurysms ?

A

APCKD (adult polycystic kidney disease)

40
Q

List 5 causes of type 2 (immune complex) RPGN ?

A
SLE
Henoch–Schlönein purpura
post-streptococcal
Alport’s syndrome
IgA nephropathy
41
Q

list clinical features of renal disease

A
haematuria 
proteinuria
uraemia 
hypertension 
oliguria/anuria 
polyuria 
oedema 
colic
42
Q

list genitourinary malformations involving the kidney

A
agenesis 
renal fusion 
renal dysplasia 
pelvi-ureteric junction obstruction
ureteral duplication 
posterior urethral valves
43
Q

features of polycystic kidney disease

A

presents in adulthood with hypertension, flank pain, haematuria
PKD1, PKD2
berry aneurysm
increased risk of malignancy

44
Q

causes of renal failure

A

pre-renal: perfusion failure
renal: acute tubular injury, acute glomerulonephritis, thrombotic microangiopathy
post-renal: obstruction

45
Q

what is acute tubule-interstitial nephritis

A

immune injury to tubules and interstitium
also due to infection and drugs (NSAIDs/antibiotics/diuretics/allopurinol)
heavy interstitial inflammatory infiltrate with tubular injury
eosinophils and granulomas visible

46
Q

what is acute glomerulonephritis

A

acute inflammation of glomeruli

presents with oliguria with urine casts containing erythrocytes + leukocytes

47
Q

what is acute crescentic glomerulonephritis

A

immune complex
anti GBM disease
pauci-immune (anti-neutrophil cytoplasm antibodies)

48
Q

what is immune complex associated crescentic glomerulonephritis

A

aetiologies include SLE, IgA nephropathy and post-infectious glomerulonephritis

49
Q

what is anti-GBM disease

A

disease caused by antibodies directed against the glomerular basement membrane
c-terminal domain of type IV collagen
antibody can be detected with serology
linear deposition of IgG

50
Q

what is pauci-immune complex glomerulonephritis

A

only scanty Ig deposits
ANCA associated
vasculitis elsewhere

51
Q

what is thrombotic microangiopathy

A

damage to the endothelium in glomeruli, arterioles, arteries leading to thrombosis
red cells can be damaged by fibrin - MAHA, HUS
diarrhoea associated
non-diarrhoea associated

52
Q

features of diabetic nephropathy

A

high glucose levels cause direct injury

starts as microalbuminuria, progresses to proteinuria and nephrotic syndrome

53
Q

causes of thin basement membranes

A

hereditary defect in type 4 collagen synthesis
<250nm
haematuria

54
Q

what is alports syndrome

A

x linked dominant mutations affecting alpha 5 subunit
progressive, renal failure in middle age
deafness, ocular disease

55
Q

what is IgA nephropathy

A

commonest glomerulonephritis
IgA predominant mesangial immune complex deposition
30% develop end-stage kidney failure

56
Q

what is hypertensive nephropathy

A

narrowing of arteries and arterioles leading to scarring and ischaemia of glomeruli
shrunken kidneys and granular cortices