L76- Kidney diseases I Flashcards Preview

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Flashcards in L76- Kidney diseases I Deck (93):
1

Location of kidneys?

 Locate in retroperitoneum
 Vertebra: T12 - L3

2

Functions of Mesangium?

-Structural - hold capillary loops together
-produce ECM,
-analyze urine in distal
tubule,
-contract to reduce
perfusion

3

What is primary renal diseases?

kidney is the only/ predominant organ involved (no systemic manifestation)

4

What is secondary renal diseases?

kidney is injured in the
course of systemic diseases or infections

5

What is clinical-pathological diagnosis?

Combination of:
clinical hisotry and manifestations
+
Renal biopsy pathological diagnosis

6

3 methods to examine renal biopsy?

-Light microscopy
-Direct immunofluorescnce study
-Electron microscopy

7

Explain how nephrotic syndrome can lead to hyperlipidemia?

Kidney glomeruli leak albumin:
> heavy proteinuria , hypoalbuminemia = reduced oncotic pressure
> Generalized edema
> Liver synthesizes proteins (e.g. lipoproteins) to compensate oncotic pressure
> hyperlipidemia

8

How does nephrotic syndrome affect drug levels?

Decrease in serum-binding protein > some drugs less able to be transported

9

How does nephrotic syndrome affect clotting and cause Deep vein thrombosis?

Hypercoagulability
>> enhanced venous / arterial thromboembolism
>> DVT

10

How does nephrotic syndrome affect immunity?

Loss of Ig, complements, impaired neutrophil phagocytic function >> increase risk of infection

11

How does the kidney fail in nephrotic syndrome?

Progressively

12

Summarize all the complications of nephrotic syndrome?

Progressive renal failure

Increase risk of infection

Hypercoagulability

Decrease in serum-binding protein

Hyperlipidemia and lipiduria

13

Presentation of nephritic syndrome in urine?

Coke-colour Urine:
1) Mild to moderate proteinuria
2) Acute onset, gross visible hematuria or;
3) Microscopic hematuria with red cell casts

14

Presentation of nephritic syndrome in GFR and BP?

Hypertension

Decreased GFR

15

Which glomerular diseases are related to nephritic syndrome?

1) Acute poststreptococcal glomerulonephritis

2) Rapidly progressive glomerulonephritis

16

What is the presentation of acute kidney injury?

- rapidly decline in GFR in hours/ days

- Severe form: oliguria (reduced urine) or anuria (no urine)

17

What is the definition of chronic kidney disease?

presence of kidney structural / functional abnormalities for
at least 3 months

or

Persistent declined GFR (< 60mL/min/1.73m^2) for at least 3 months

18

What type of disease is acute kidney injury associated with?

Glomerular, interstitium, tububles, blood vessel diseases

19

GFR in end stage renal disease?

GFR < 5% of normal

Final and irreversible

20

Treatment for end stage renal disease?

Renal replacement therapy:
1) Haemodialysis
2) Peritoneal dialysis
3) Kidney transplant

21

Define Azotemia?

Biochemical abnormality referring to elevation of blood urea, nitrogen and creatinine levels
+
Decrease in GFR

22

Define Uremia?

Toxic condition resulting from kidney disease

Retention of waste products (normally excreted) in bloodstream

23

What are the 3 categories of glomerular diseases? *

Primary glomerulonephritis

Systemic disease with glomerular involvement

Hereditary disorders

24

Which glomerular diseases are related to nephrotic syndrome?

Just rmb the top 4

1) Focal segmental glomerulonephritis

2) Membranoproliferative glomerulonephritis

3) Membranous neuropathy

4) Minimal change disease

(Amyloidosis, Diabetes mellitus, SLE)

25

What are the primary glomerulonephritis/ glomerulopathy diseases?

Membranous nephropathy

Minimal change

Focal segmental glomerulonephritis

IgA neuropathy

Rapidly progressive (crescentic) Glomerulonephritis

Membranousproliferative glomerulonephritis

Acute post-infectious glomerulonephritis

Chronic glomerulonephritis

26

What are the systemic diseases with glomerular involvement?

SLE
Goodpasture syndrome
Diabetes
Amyloidosis
Bacterial endocarditis

(Microscopic polyangiitis, Wengener granulomatosis, Henoh- Scholein purpura)

27

Name 3 hereditary syndromes that causes kidney damage? *

Alport syndrome

Fabry disease

Thin basement membrane disease

28

What is the most common mechanism of glomerular injury pathogenesis?

Immune, Ag-Ab reactions

29

3 ways that immune mechanism causes glomerular injury? *

1) Ab react in-situ within glomerulus

2) Deposition of circulating Ag-Ab complexes in glomeruli

3) Ab directed against normal components of GBM

4) Cell-mediated or alternative complement immune injury

30

Apart from immune damage, what are the 2 other ways glomerular injury occurs?

Metabolic alterations of GBM

Genetic defect of GBM synthesis and cellular protein

31

3 locations for Ag-Ab complexes to deposit to cause glomerular injury?

From circulation:
1) Subendothelial deposit

In-situ:
1) Within GBM
2) SubEPITHELIAL deposit (outside capillary)

32

2 things that lead to uremia?

Decrease GFR

Decrease glomerular blood flow

33

What causes primary membranous nephropathy? *

binding of autoantibodies

to M-type phospholipase A2 receptor (PLA2R) expressed on podocytes

34

What causes secondary membranous nephropathy?
*

-Infection (Hepatitic B or C)

-Malignancy

-Autoimmune disease (SLE, Rhuematoid arthritis)

-Sarcoidosis

- Drugs

35

Pathogenesis of membranous nephropathy?

 Chronic in situ deposit of Ig containing immune complex in subepithelial space along GBM

36

light microscopy appearance of membranous nephropathy? Give stains

• Holey, frothy, bubble, spiked appearance on GBM in Silver stain

• Diffuse thickening of capillary wall in H&E stain

37

Immunofluorescence appearance of membranous nephropathy?

Granular deposition in Subepithelial space

(in-situ deposition, not circulation)

38

Electron microscopy appearance of membranous nephropathy?

Electron-dense deposits
In subepithelial space

39

Main clinical manifestations of nephrotic syndrome?

Proteinuria (3.5 g or more in adults/day)

Hypoalbuminemia

Generalized oedema

Hyperlipidaemia and lipiduria

40

What is the marker for Primary membranous nephropathy?

PLA2R

41

What is the treatment of primary and secondary membranous nephropathy?

Primary = steroids

Secondary = Treat underlying cause

42

What are the outcomes of membranous nephropathy?

60% pateints have persistent proteinuria

10% progress to renal failure in 10 years

43

Age group affected by membranoproliferative glomerulonephritis ?

Children and young adults

44

Presentation of membranoproliferative glomerulonephritis?

Nephrotic syndromes (e.g. generalized edema, hyperlipiduria...etc)

Haematuria

45

What are the 4 types of membranoproliferative glomerulonephritis?

Idiopathic

Secondary

C3 glomerulonephritis

Dense Deposit disease

46

Light microscopy of membranoproliferative glomerulonephritis?

Hypercellularity: Cellular proliferation of mesangial cells + Leukocyte infiltration

Capillary wall thickening

Basement membrane splitting

47

Immunofluorescence appearance of membranoproliferative glomerulonephritis?

dense deposits at capillary walls subendothelium

48

Explain electron microscopy of membranoproliferative glomerulonephritis

Splitting of glomerular basement membrane

immune deposition at subendothelium causes formation of new GBM under original GBM

49

Treatment of membranoproliferative glomerulonephritis?

Treat underlying cause

Drugs targeting C3 glomerulonephritis

50

Outcome of membranoproliferative glomerulonephritis?

50% develop chronic renal failure within 10 years

51

Pathology of Minimal change disease?

injure podocytes and cause proteinuria with effacement of foot processes

Exact cause unknown

52

Age groups affected by minimal change disease?

Most common (90%) nephrotic syndrome in children

Peak incidence at 2-6 years old

53

Associated factors that can lead to minimal change disease?

Drugs (e.g. NSAID and Interferons)
Lymphoma
Infection and recent vaccination

54

Light microscopy of minimal change disease?

glomeruli that have a normal appearance

55

Immunofluorescence of minimal change disease?

No staining

56

Electron microscopy of minimal change disease?

Diffuse (more than 50%) effacement of foot processes of podocytes

>> loss of filtration slit

57

Treatment of minimal change disease>?

Steroid

(little steroid-dependence or resistance)

58

Outcome of minimal change disease?

Prognosis is excellent

59

Given the exact cause of minimal change is unknown, what are some possible etiologies?

 Circulating factor
 Abnormality in cell-mediated immunity
 Cytokines

60

Complication of minimal change disease? Explain

GBM lose protein filter function

Lose albumin > blood oncotic pressure decrease
> increase filtration into tissue and hypovolemic shock

> Pre-renal damage and acute tubular necrosis

> Acute renal failure

61

Pathology of Focal segmental glomerulosclerosis (FSGS)?

<50%, partial glomerulosclerosis:

Replacement of capillary loops of glomerulus by ACELLULAR, AMORPHOUS material*

*derived from plasma protein, collagen, basement membrane materials

62

Primary FSGS presentation and features are similar to which nephrotic disease?

Minimal change

63

Differential diagnosis between minimal change and FSGS?

FSGS has no response to steroids (but minimal change has excellent response)

FSGS = more likely to progress to chronic renal failure and recurrence after transplant

64

Is Acute proliferative / post-infectious glomerulonephritis nephrotic or nephritic?

Nephritic syndrome

65

Pathology of Acute proliferative / post-infectious glomerulonephritis?

Immune complex glomerulonephritis

usually preceded by Group A, B-haemolytic streptococci infection of pharynx (1-4 weeks before)

66

Age group most affected by Acute proliferative / post-infectious glomerulonephritis?

Children

67

What other infections can precede Acute proliferative / post-infectious glomerulonephritis?

Staphylococcal
Pneumococcal
Virus (HBV/ HIV/ HCV)
Parasites (malaria/ toxoplasmosis)

68

Light microscopy of Acute proliferative / post-infectious glomerulonephritis?

Hypercellular glomeruli with neutrophils

69

Explain where the immune complex deposition occurs in Acute proliferative / post-infectious glomerulonephritis?

Immune-complexes formed by antibodies and circulating or planted antigens


>> depositing in the subepithelial space, forming lumps

(from circulation)

70

Immunofluorescence appearance of Acute proliferative / post-infectious glomerulonephritis?

Spotty immune deposits

"Starry sky" appearance due to deposits of IgG (and complement 3)

71

Electron microscopy appearance of Acute proliferative / post-infectious glomerulonephritis?

Electron dense deposits forming subepithelial humps at GBM

72

Outcome of appearance of Acute proliferative / post-infectious glomerulonephritis?

95% recover with conservative therapy

73

Explain Hypercellular glomeruli
in Acute proliferative / post-infectious glomerulonephritis?

Injury to glomerular tufts > leakage of plasma protein
stimulates Bowman’s capsule

>> parietal epithelium proliferates

>> enlarged, hypercellular glomeruli

74

What is rapidly progressive (crescentic) glomerulonephritis associated with?

Severe glomerular injury with multiple possible causes:

Type I,II, III

75

Name a type I rapidly progressive (crescentic) glomerulonephritis?

Goodpasture syndrome

= Anti-GBM antibody mediated glomeruplonephritis

76

Name 4 type II rapidly progressive (crescentic) glomerulonephritis?

Immune complex mediated:

Lupus nephritis
IgA nephropathy
Acute poststreptococcal glomerulonephritis

(Henoch- Schonlein purpura)

77

Name a type III rapidly progressive (crescentic) glomerulonephritis?

Pauci- immune mediated:

ANCA-associated (Wegener granulomatosis, microscopic polyangiitis)

78

Goodpasture syndrome pathology in kidney?

Autoantibody attack noncollagenous C terminal (NC1) domain of alpha 3 chain of collagen type IV at GBM

79

Goodpasture syndrome pathology in lungs?

Autoantibodies cross-react with pulmonary alveolar basement membranes>> cause pulmonary haemorrhage and death

80

What precedes goodpasture syndrome?

Flu-like illness
Hydrocarbon or solvent exposure

81

Light microscopy appearance of Goodpasture? *

Focal necrotizing lesions

Cellular crescent at Bowman's capsule

Diffuse parietal epithelium proliferation

Fibrin deposition

82

immunofluorescence appearance of Goodpasture?

" Linear ribbon deposition" of IgG along GBM

83

Electron microscopy appearance of Goodpasture?

No deposit by usual electron microscopy

>> antibodies too small to be detected

84

Treatment of goodpasture syndrome?

Plasmapheresis/ plasma exchange

High dose steroid and Cyclophosphamide

85

Outcome of good pastures syndrome?

Chronic renal replacement therapy or kidney transplant

86

What type of disease in IgA nephropathy?

Immune complex glomerulonephritis

87

What is the most common cause of Glomerulonephritis worldwide?

IgA nephropathy

88

What are the presentations of IgA nephropathy?

Microscopic/ macroscopic haematuria

Proteinuria (10% nephrotic range)

Chronic or acute kidney failure

89

Light microscopy appearance of IgA nephropthy? *PAS stain*

Mesangial hypercellularity with increased matrix

Capillary loop not much affected

90

immunofluorescence appearance of IgA nephropthy?

Dominant or co-dominant deposits of IgA at mesangial cells

91

Electron microscopy appearance of IgA nephropthy?

Mesangial electron dense deposits (immune complexes)

92

Treatment of IgA nephropathy?

Depends on clinical setting

Corticosteroid if significant proteinuria or rapidly progressive kidney injury

93

Outcome of IgA nephropathy?

Slow progression to chronic renal failure in 15-40% patients in 20 years

Recurrence after kidney transplant

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