ACP- L23, L24 Medical renal diseases Flashcards

1
Q

The endothelial cells of the glomerulus are with fenestration. The Glomerular basement membrane (GBM) contains __________ with heparin sulfate producing ________charge. This serves as the main filtration barrier, blocking large MW molecules like albumin.

What is the possible pathology in GBM? (2)

A

Contains Type IV collagen;
Negative charge;

Pathology - Thickened in:

  1. Membranous nephropathy
  2. DM: increased type IV collagen synthesis
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2
Q

Visceral epithelial cells (VEC) produce GBM and they are podocytes (intracellular junctions) that serve as the final filtration barrier.
Possible pathology of VECs?

A

Minimal change disease: fusion of podocytes

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3
Q

Mesangial cells support glomerular capillaries.

Possible pathology?

A

IgA nephropathy:

IC deposition > release inflammatory mediators

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4
Q

Parietal epithelial cells are the lining of the Bowman capsule. Possible pathology?

A

Crescentic glomerulonephritis: proliferate and compress on glomerular tuft (blood vessels)

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5
Q

Which of the following about glomerular diseases are correct?
A. More than half is diffuse type
B. Less than half is focal type
C. Can be divided into segmental or global
D. Light microscope using H&E staining, PAS can be used for classifying the type of glomerular disease
E. Immunofluoresence can be used to identify protein deposition

A

All of the above

E:
IF - e.g. Anti-IgA Ab
Identify protein deposition, e.g.
1. Linear pattern in IF: anti-GBM disease, e.g. Goodpasture syndrome
2. Granular pattern in IF: Immune-complex type GN

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6
Q

______________ can be used to detect submicroscopic defects, such as podocytes fusion in nephrotic syndrome.
It can also be used to detect site of IC deposition, e.g. subendothelial, subepithelial and intramembranous

A

Electron microscope

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7
Q

In most nephritic syndrome, pathology is due to? (1)

explain (3)

A

Immune complex -
1. Type III HSR >

  1. IC circulate and deposit in glomeruli >
  2. activate C’ > C5a > neutrophil attracted > glomerular damage
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8
Q

What is the pathology of Goodpasture syndrome?(2)

A

Type II HSR - Antibodies against GMB antigens

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9
Q

Minimal change disease is due to _____ cell disorder, which produces cytokines.
This affects the GBM by affecting its ______, podocytes are damaged and fused.

A

T-cell disorder;
charge (lost negative charge)

(Visceral epithelial cell)

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10
Q

Clinical manifestation of glomerular disease is often chronic and will progress to?
There is no specific Tx, supportive Tx is given to slow disease progression.

A

End stage renal disease (ESRD)

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11
Q

Give 2 examples of primary glomerulonephritis

A
  1. Membranous nephropathy
  2. Minimal change nephropathy
  3. Cresenteric glomerulonephritis
  4. IgA nephropathy
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12
Q

Give 2 examples of secondary glomerulonephritis.

A
  1. Lupus nephritis
  2. Diabetic glomerulosclerosis
  3. Amyloidosis
  4. Post-streptococcal GN
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13
Q

Give 2 examples of heriditary glomerulonephritis.

A
  1. Alport’s sydrome (kidney disease, hearing loss, eye abnormalities)
  2. Thin membrane disease (hematuria)
  3. Fabry’s disease (deficiency of enzyme that builds up a type of fat in body)
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14
Q

What are difference in definition between nephritic syndrome and nephrotic syndrome?

A

Nephritic syndrome - Glomerular injury due to neutrophils

Nephrotic syndrome:
Glomerular injury due to cytokines but not neutrophils

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15
Q

Nephritic/ Nephrotic?

A. Hypertension is due to increase fluid retention in azotaemia (increased N-containing components in blood)

B. Hypertension is due to RAAS activation that reduced ECF

A

Nephritic - A

Nephrotic - B

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16
Q

In nephritic syndrome, patients experience periorbital puffiness +/- uputting edema: salt-retention in loose skin

Proteinuria >150 mg/daybut <3.5 g/day. (Ddx from nephrotic syndrome)

What are the other clinical manefestations?

A
  1. Oliguria,azotaemia: reduced GFR from inflamed glomeruli

2. Haematuria; inflammed glomeruli causes dysmorphic RBC

17
Q

In nephrotic syndrome, patients have pitting edema due to?

A

Hypoalbuminemia

18
Q

In nephrotic syndrome, proteinuria is > 3.5g/day?

A

> 3.5g/day

19
Q

Nephrotic syndrome patients is associated with DVT and Pulmonary embolism. Why?

A

Hypercoagulable state: loss of Anti-thrombin III (ATIII)

20
Q

Other than pitting edema, proteinuria and hypercoagulability, what else would a patient with nephrotic syndrome present? (2) (Why?)

What can be seen in microscopy?

A

Hyperlipidemia: reduced protein stimulates protein/ lipoprotein synthesis > Lipiduria

> fatty cases with Maltese crosses and oval fat bodies

21
Q

What is the most common Glomerulonephritis disease? It is preceded shortly by URTI (synphargyntic haematuria) (1)
Pathogenesis? (2)

A

IgA nephropathy (Berger disease)

  • Mutated IgA not degraded > Accumulation > recognised as foreign > AutoAb formed > IC form and deposit in mesangium > inflammation
22
Q

Post-streptococcal GN is the nephritic syndrome in children following Group A streptococci infection, which is a type _____ HSR. Pathology detected by? (3)

A

Type 3 - IC trapped in GBM

Light microscopy (LM): enlarged and hypercellular glomerulus

EM: subepithelial deposits (humps)

IF: granular

23
Q

Rapidly progressive GN/Crescentic GN causes rapid progression to AKI. What is the pathogenesis?

A

Glomerular inflammation > Proliferation of parietal epithelial cells in Bowman’s space (Crescent shape) > Renal failure

24
Q

MC cause of ESRF?

A

Diabetic nephropathy

25
Q

What are the 3 types of rapidly progressive GN that causes rapid progression to AKI?
Give examples for each type.

A
  1. Anti-glomerular basement membrane
    e. g. Goodpasture syndrome:
  2. IC disease: Lupus nephritis, SBE (subacute bacterial endocarditis)
  3. Pauci-immune: microscopic polyarteritis, Wegener’s granulomatosis
    - No Anti-GBM and no IC but ANCA (AutoAb against neutrophils) in blood.
    Therefore IF- but ANCA+.
26
Q

What is Goodpasture syndrome?
Pathogenesis (1)
Manifestations? (2)

A

Pathogenesis:
Anti-BM Ab against glomeruli and pulmonary alveolar capillary basement membrane

Manifestations

  • benign with haemoptysis
  • ends with AKI
27
Q

Prognosis of post-streptococcal GN VS rapidly progressive GN?

A

post-streptococcal GN: good

rapidly progressive GN: very poor

28
Q

What are the 2 main types of nephrotic syndrome?

They are MC nephrotic syndromes in what age groups?

A
  1. Minimal change disease
    - MC in children
  2. Membranous nephropathy
    - MC in adults
29
Q

Pathology of membranous nephropathy?

A

AutoAb targeting GBM > IC form and deposit subepithelially > inflammation

Overtime: GMB matrix deposits in between IC > thickened GBM seen in LM (spike and dome pattern subepithelially)

30
Q

Name the 2 secondary glomerulonephritis .

A
  1. Lupus nephritis

2. Diabetic nephropathy

31
Q

Pathophysiology of diabetic nephropathy? (8)

A
  1. Excess glucose in blood form glycation in basement membrane of efferent arteriole (> Hyaline arteriosclerosis)
  2. Obstruction to blood flow
  3. increase pressure of glomerulus
  4. increase GFR (hyperfiltration)
  5. Mesangial cells secrete more structural matrix > mesangium expansion and form nodules (Kimmelstiel-Wilson nodules - sclerosis - tiny balls of proteins)
  6. Thickening of GBM + Disruption of podocytes
  7. GBM more permeable > albuminuria
  8. reduced GFR > ESRF
32
Q

Hyaline arteriosclerosis + Kimmelstiel-Wilson nodules = what disease

A

Diabetic nephropathy

Nephrotic syndrome with microalbuminuria > macroalbuminuria