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Urinary system > Glomerular disease - glomerulonephritis > Flashcards

Glomerular disease - glomerulonephritis Flashcards

1
Q

What % of end stage renal disease is caused by glomerulonephritis?

A

30%

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2
Q

What is glomerulonephritis?

A

Group of disorders causing injury to the glomeruli

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3
Q

What are the 4 main clinical features of glomerulonephritis?

A
  • Haematuria
  • Proteinuria
  • Hypertension
  • Renal insufficiency
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4
Q

Describe the 2 types of haematuria

A
  • Macroscopic - tea- or cola coloured or frank blood

* Microscopic - >5 RBC per high power field

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5
Q

In which syndrome is haematuria most commonly seen in - nephritic or nephrotic syndrome?

A

Nephritic > nephrotic

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6
Q

Where can the blood causing haematuria come from?

A

kidney, ureter, bladder, prostate, urethra

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7
Q

Describe the haematuria seen in glomerulonephritis

A

In glomerulonephritis: persistent microscopic haematuria, microscopy shows dysmorphic RBC (Mickey-mouse-like)

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8
Q

In which syndrome is proteinuria most commonly seen in - nephritic or nephrotic syndrome?

A

Presents equally in Nephritis and nephrosis

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9
Q

Describe the proteinuria seen in glomerulonephritis

A

In glomerulonephritis: persistent, proteinuria of more than 1 gram /mmol creatinine

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10
Q

Define hypertension

A

> 140/80 mmHg

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11
Q

In which syndrome is hypertension most commonly seen in - nephritic or nephrotic syndrome?

A

Nephritic > nephrotic

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12
Q

Describe nephritic state

A

Caused by inflammation of the glomeruli; involves:

o Active urine sediment: haematuria, dysmorphic RBCs, cellular casts
o Hypertension
o Renal impairment

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13
Q

Describe nephrotic syndrome

A 
Collection of symptoms associated with kidney damage:
o	Oedema
o	Proteinuria >3.5 g/day
o	Hypoalbuminemia
o	Hyperlipidemia

Can be caused by primary (idiopathic) glomerular disease or secondary glomerular diseases

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14
Q

What are the differential diagnoses of nephrotic syndrome?

A
  • Congestive Heart Failure (JVP raised, normal albumin, minimal proteinuria)
  • Hepatic Disease (abnormal LFTs, no proteinuria)
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15
Q

What are the 2 main classifications of glomerulonephritis?

A
  • Proliferative glomerulonephritis - Excessive numbers of cells in glomeruli. These include infiltrating leucocytes
  • Non-proliferative glomerulonephritis - Glomeruli look normal or have areas of scarring. They have normal numbers of cells
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16
Q

Describe diffuse glomerulonephritis

A

• Diffuse: >50% of glomeruli affected

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17
Q

Describe focal glomerulonephritis

A

• Focal: <50% of glomeruli affected

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18
Q

Describe global glomerulonephritis

A

• Global: all the glomerulus affected

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19
Q

Describe segmental glomerulonephritis

A

• Segmental: part of the glomerulus affected

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20
Q

What types of glomerulonephritis are associated with nephrotic syndrome?

A
  • Minimal change disease

* Membranous nephropathy

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21
Q

What type of glomerulonephritis is associated with urinary sediment abnormalities?

A

IgA nephropathy

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22
Q

What type of glomerulonephritis is associated with proteinuria?

A

IgA nephropathy

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23
Q

What types of glomerulonephritis are associated with nephritic state?

A
  • Anti-neutrophil cytoplasmic antibody (ANCA) associated glomerulonephritis
  • Post-infection glomerlunephritis
24
Q

Give 4 examples of proliferative glomerulonephritis

A
  • Diffuse proliferative - post-infective nephritis
  • Focal proliferative - mesangial IgA disease
  • Focal necrotizing (crescentic) nephritis
  • Membrano-proliferative nephritis
25
Q

Describe the histopathology of Post-Infective Glomerulonephritis

A
  • Global and diffuse enlarged hypercellular glomeruli
  • Proliferation of endothelial and mesangial cells
  • Infiltration of leukocytes (neutrophils and monocytes)
  • Crescent formation in severe cases
  • Red cell casts
26
Q

Describe how Post-streptococcal glomerulonephritis presents and what causes it

A

Follows 10-21 days after strep infection; typically of throat or skin.
Most commonly with Lancefield group A Streptococci.
Genetic predisposition: HLA-DR, -DP, -DP.

Produces nephritic syndrome:
o	Haematuria
o	Oliguria
o	Uraemia
o	Hypertension
o	Mild proteinaemia
27
Q

How is Post infective glomerulonephritis treated?

A
  • Antibiotics for infection, debatable.
  • Loop diuretics such as frusemide for oedema
  • Vasodilator drugs (e.g. amlodipine) for hypertension
28
Q

What is the most common cause of glomerulonephritis worldwide?

A

IgA nephropathy

29
Q

Describe IgA nephropathy

A
  • Commonest cause of glomerulonephritis worldwide.
  • Initially described in 1960s by Berger and Hinglais.
  • Characterized by IgA deposition in the mesangium + mesangial proliferation and depositon of matrix
  • Most common in 2nd and 3rd decade of life with males more commonly affected.
  • Up to 40% of cases can progress to end stage kidney disease
30
Q

What age group and sex is more at risk of IgA nephropathy?

A

• Most common in 2nd and 3rd decade of life with males more commonly affected.

31
Q

What % of those with IgA nephropathy progress to end stage renal disease?

A

40%

32
Q

How do those with IgA nephropathy present?

A
  • Microscopic haematuria.
  • Micoscopic haematuria + proteinuria
  • Nephrotic syndrome
  • IgA crescentic glomerulonephritis
33
Q

What can cause crescentic glomerulonephritis?

A

Anti-neutrophil cytoplasmic antibody associated conditions e.g. Wegeners/granulomatosis associated with polyangitis

Anti-glomerular basement membrane (anti GBM) disease

IgA vasculitis

Post infective glomerulonephritis

SLE

34
Q

What % of crescentic glomerulonephritis cases are caused by anti-GBM disease?

A

10-20%

35
Q

How is anti-GBM disease treated?

A 
Treatment is aggressive immunosuppression: 
o	Corticosteroids
o	Plasma exchange
o	Cytotoxics e.g. Cyclophosphamide
o	B-cell therapy e.g. Rituximab
o	Complement inhibitors
36
Q

What is the prognosis for those with anti-GBM disease?

A

Prognosis is good provided treatment is started early enough

37
Q

Describe the features of proliferative glomerulonephritis syndromes

A
  • Present with nephritic syndrome
  • Blood on dipstix – variable proteinuria
  • Can cause rapid decline in renal function leading to dialysis
  • Early diagnosis and appropriate treatment
38
Q

Give examples of non-proliferative glomerulonephritis

A
  • Minimal Change Disease
  • Focal and segmental glomerulonephritis
  • Membranous Nephropathy
39
Q

Hw is nephrotic syndrome managed?

A

General measures
• Treat oedema: salt and fluid restriction and loop diuretics.
• Hypertension: use Renin-Angiotensin-Aldosterone-blockade.
• Reduce risk of thrombosis: Heparin or Warfarin.
• Reduce risk of infection e.g. pneumococcal vaccine.
• Treat dyslipidemia e.g. statins.

Specific therapy towards cause of the non-proliferative glomerulonephritis

40
Q

What group of glomerulonephritis is associated with nephrotic syndrome?

A

Non-proliferative

41
Q

What is the most common form of glomerulonephritis in children?

A

Minimal change nephrotic syndrome

42
Q

Describe Minimal change nephrotic syndrome

A
  • Commonest form in children
  • Sudden onset of oedema - days
  • Significant proteinuria (++++) - treat with steroids
  • Two thirds of patients relapse
43
Q

How is minimal change nephrotic syndrome treated?

A

Prednisolone – 1mg/kg for up to 16 weeks. Once remission achieved, slow taper over 6 months.

Initial relapse treated with further steroid course.

Subsequent relapses treated with:
•	Cyclophosphamide
•	Cyclosporin
•	Tacrolimus
•	Mycophenolate mofetil
•	Rituximab
44
Q

What is the prognosis for those with minimal change nephrotic syndrome?

A
  • Despite relapsing behaviour, prognosis is favourable.
  • Risk of end stage kidney disease is low.
  • Steroids toxicity more prevalent following multiple exposure.
45
Q

How should those with minimal change nephrotic syndrome be managed initially?

A
  • Salt and fluid restrictions.
  • Daily weights.
  • Loop diuretic e.g. Furosemide.
  • Thromboprophylaxis e.g. low molecular weight heparin.
46
Q

How does focal and segmental glomerulonephritis present?

A

Nephrotic syndrome

47
Q

Describe the clinical features of focal and segmental glomerulonephritis

A

Is not a single disease, rather a syndrome with multiple causes that presents with nephrotic syndrome.

Pathology reveals focal and segmental sclerosis with distinctive patterns e.g. tip lesion, collapsing, cellular, perihilar, and not otherwise specified

Primary (idiopathic) or secondary.

Generally steroid resistant.

High chance of progression to end stage kidney disease.

  • Collapse of capillary loops
  • Increase in matrix and hyalinosis
  • Lipid droplets and foam cells in sclerotic areas
48
Q

How is Focal and segmental glomerulonephritis treated?

A
  • General measures, as previously described.
  • Trail of steroids, positive response, even partial remission, carries better prognosis.
  • Alternative options: cyclosporin, cyclophosphamide, and Rituximab
49
Q

What is the commonest cause of nephrotic syndrome?

A

Membraneous nephropathy

50
Q

What serological markers indicate membraneous glomerulonephritis?

A
  • Anti-phospholipase A2 receptor (PLA2R) antibody - positive in 70% of idiopathic cases
  • Thrombospondin type 1 domain containing 7A (THSD7A).
51
Q

What causes membraneous glomerulonephritis?

A

Majority of cases occur in isolation (idiopathic).

Secondary causes include:
•	Malignancies
•	SLE
•	Rheumatoid arthritis.
•	Drugs: NSAIDs, gold, penicillamine.
52
Q

Describe the histopathology of membraneous glomerulonephritis

A
  • Spikes surround deposits
  • Thick GBM in relation to tubular basement membrane
  • Mesangial expansion
53
Q

How is membraneous glomerulonephritis treated?

A
  • General measures for at least 6 months.
  • Immunosuppression if symptomatic nephrotic syndrome, rising proteinuria or deteriorating renal function.
  • Cyclophosphamide and steroids (alternate months) for 6 months.
  • Cyclosporine.
  • Rituximab.
  • Resolves spontaneously in a third.
54
Q

What is the prognosis for those with membranous glomerulonephritis?

A
  • Resolves spontaneously in a third.
  • Prognosis good in treated patients whose proteinuria resolves
  • About 25% are on dialysis after 10 years
  • Can recur in renal transplants
55
Q

Summarise the features of non-proliferative glomerulonephritis

A
  • Present with nephrotic syndrome.
  • Renal biopsy is key investigation.
  • Identify cause, if possible.
  • General measures are important in all cases.
  • Specific treatment as appropriate.

Urinary system (22 decks)

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