Histopathology 8 - Renal disease Flashcards

(94 cards)

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
Which antibodies are present in pauci-immune acute crescentic glomerulonephritis?
Anti-neutrophil cytoplasm Ig
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?
Thrombotic microangiopathy
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 minimal change glomerulonephritis, how do glomeruli appear under electron microscopy?
Effacement 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
35
Does focal segmental glomerulonephritis produce the nephrotic or nephritic syndrome?
Nephrotic
36
What is the broad pathophysiology of primary membranous glomerulonephritis? Description of membraneous glomerulonephritis?
Immune complex related Immune deposits are subepithelial, outside GBM Description: spikey subepithelial deposits
37
What age group does membranous glomerulonephritis usually affect?
Adults
38
What is the most common cause of membranous glomerulonephritis?
75% are immune Antipody against phospholipase A2 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?
X-linked dominant Affects alpha 5 subunit
42
What are the symptoms of Alport's syndrome?
Renal failure in middle age Deafness Ocular disease
43
What is the most common form of glomerulonephritis worldwide?
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
What socring system is used to score IgA renal disease?
Oxford classification
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
What would be the histological appearance of acute tubulo-interstitial nephritis?
Eosinophils and granulomas also white cell casts
54
What is the usual cause of non-diarrhoea-associated thrombotic microangiopathy?
ADAMTS13 deficiency (eg scleroderma and anti-phospholipid syndrome)
55
Describe each stage of diabetic nephropathy
Stage 1: Basement Membrane thickening Stage 2: Mesangial matrix expansion Stage 3: Nodular lesions (Kimmelstiel-Wilson) Stage 4: Advanced glomerulosclerosis
56
How is IgA nephropathy graded?
Oxford classification (MEST-C)
57
What two properties does the filtration barrier created by the podocytes depend on?
Charge dependent and size dependent
58
Role of the PCT
Actively resorbs sodium Carries out hydrogen exchange to allow carbonate resorption Co-transport of amino acids, PO4 and glucose Reabsorption of potassium
59
Role of the LoH
* 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
Role of DCT
* 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
Role of the collecting duct
Aldosterone acts on these cells → Na retention and K excretion Reabsorbs water (principle cells, ADH) Regulates pH (intercalated cells, proton excretion)
62
What is the renal corpuscle?
Consists of the glomerulus and bowman's capsule
63
Membranous glomerulonephritis histology Treatment of membranous glomerulonephritis?
* 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
What protein is deficient in adult polycystic kidney disease?
polycystin-1
65
Complications of adult PKD
- subarachnoid haemorrhage (berry aneurysms) - 10% mortality from renal failure
66
Which cancer do renal cysts increase the risk of?
Papillary renal cell cancer
67
Definition of AKI
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
Drugs that affect glomerular blood flow (and therefore predispose to pre-renal AKI)
69
ATN histology
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
What intrinsic renal failure can pre-renal AKI lead to?
Can eventually lead to ATN if damage is sufficient
71
Most common cause of AKI
Acute tubular necrosis \*\*intrinsic renal failure\*\*
72
73
Histology of HUS
Fibrinoid necrosis \*because you get fibrin strand deposition which leads to MAHA\*
74
Which form of glomeruloneprhitis is a medical emergency?
Crescentic
75
Causes of crescentic glomerulonephritis
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
What cells are seen on biopsy in goodpasture's disease?
Crescent cells
77
Histology of acute tubulointerstitial nephritis
heavy interstitial inflammatory infiltrate with tubular injury: Eosinophils Granulomas
78
How does rhabdomyololysis cause AKI? Tx
Myoglobin causes acute tubular necrosis Treatment: fluids to wash out the myoglobin
79
3 common causes of nephrotic syndrome
_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
Minimal change disease: light and electron microscopy
Light microscopy: no change Electron microscopy: effacement of podocytes
81
IgA nephropathy findings
main clinical feature: frank haematuria
82
PSGN histology findings
* Light microscope (LM): ↑cellularity of glomeruli * Fluorescence Microscope (FM): * granular deposits of IgG and C3 in GBM * Electron Microscope (EM): * Subendothelial humps
83
Membranoproliferative glomerulonephritis hsitology
* mesangial cell proliferation * and capillary thickening
84
What are two hereditary causes of microscopic haematuria?
Alport's syndrome Thin basement membrane disease - usually get microscopuc haematuria unlike frank haematuria in IgA nephropathy
85
Classification+ description of lupus nephritis
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
Histology of diabetes causing nephrotic sydnrome
Diffuse glomerular basmenet membrane thickening Mesangial matrix nodules- aka Kimmelstiel Wilson nodules
87
Histology of acute pyelonephritis
leucocytic casts
88
Histology of acute interstitial nephritis
Histology: inflammatory infiltrate with tubular injury, eosinophils & granulomas
89
HUS vs TTP
90
Commonest causes of chronic renal failure in the UK
Diabetes (19.5%) ● Glomerulonephritis (15.3%) ● Hypertension & Vascular disease (15%) ● Reflux nephropathy (chronic pyelonephritis) (9.5%) ● Polycystic kidney disease (9.4%)
91
3 types of renal cell carcinoma
Clear cell carcinoma – well differentiated ● Papillary carcinoma – commonest in dialysis-associated cystic disease ● Chromophobe renal carcinoma – pale, eosinophilic cells
92
What is alport syndrome? Inheritance pattern?
nephritic syndrome, cataracts and sensorineural deafness suggests what underyling diagnosis? x linked dominant
93
Classification of nephritic and nephrotic syndromes
94
Which protein is responsible for dark urine in rhabdomyololysis?
myoglobin