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Urinary System > Glomerular Disease - Clinical > Flashcards

Glomerular Disease - Clinical Flashcards

1
Q

What is glomerulonephritis?

A

Inflammatory disorder of the kidney

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2
Q

What percentage of end-stage kidney disease is glomerulonephritis responsible for?

A

Up to 30%

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3
Q

What is the presentation of glomerulonephritis?

A 
Can present in many ways 
Mostly vague symptoms/signs 
- proteinuria
- renal failure
- hypertension
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4
Q

What is the function of mesangial cells?

A

Provide extracellular support for the capillaries

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5
Q

What is the permeability of the glomerular basement membrane?

A

Selectively permeable - in normal, healthy people, protein should not be present in the urine

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6
Q

What is present in the nuclei/cells in proliferative glomerulonephritis?

A

Overlap and congestion of the nuclei/cells

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7
Q

What are the classes of glomerulonephritis?

A

Proliferative

Non-proliferative

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8
Q

What are the features of non-proliferative glomerulonephritis?

A

Glomeruli look normal or have areas of scarring
Normal numbers of cells
Tubules and interstitium may be damaged

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9
Q

What are the features of proliferative glomerulonephritis?

A

Characterised by excessive numbers of cells in the glomeruli, including infiltrating leukocytes

Presents with nephrotic syndrome
Blood on dipstick, no or minimal proteinuria

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10
Q

What are the types of proliferative glomerular disease?

A

Diffuse proliferative
Focal proliferative
Focal necrotising (crescentic) nephritis
Membrano-proliferative nephritis

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11
Q

What are the features of post-infective glomerulonephritis?

A

Diffuse, congested nuclei
Immune deposits due to activation of the complement system
Follows 10-21 days after infection, typically of throat or skin

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12
Q

What type of bacteria is post-infective glomerulonephritis most commonly associated with?

A

Lancefield group A streptococci

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13
Q

Acute nephritis is typical of what?

A

Post-infective glomerulonephritis

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14
Q

What are the features of acute nephritis?

A

Fluid retention with oedema - periorbital oedema common
Normal serum albumin
Little proteinuria
Renal impairment

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15
Q

What is the treatment of post-infective glomerulonephritis?

A

Antibiotics for any active infection
Loop diuretics e.g. furosemide for oedema
Vasodilator drugs e.g. amlodipine for hypertension
Consider immunosuppression in severe/unresolving disease

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16
Q

What is the commonest cause of glomerulonephritis?

A

IgA nephropathy

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17
Q

What are the features of IgA nephropathy?

A

Congestion
Mesangial and endocapillary inflammation
Most inflammatory deposits are in the mesangium
No characteristic serology
Diagnosed by renal biopsy

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18
Q

In what patients does IgA nephropathy typically occur in?

A

Young - presents in late 30s-40s

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19
Q

What indicates that a patient with IgA nephropathy is likely to deteriorate?

A

Heavy proteinuria
High creatinine
Hypotension

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20
Q

What is the presenting feature of IgA nephropathy?

A

Macroscopic haematuria

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21
Q

What is IgA nephropathy provoked by?

A

Intercurrent infection

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22
Q

What is the typical onset of IgA nephropathy in adults?

A

Insidious onset

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23
Q

What is the prognosis of IgA nephropathy in adults?

A

Worse in adults - 25% develop renal failure

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24
Q

What is the treatment of IgA nephropathy?

A

Non-specific

ACEIs and other hypotensives

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25
Q

What are the features of focal necrotising glomerulonephritis?

A

Segmental necrosis

Ruptured capillary

26
Q

In what 3 main settings does focal necrotising glomerulonephritis occur?

A

In presence of anti-glomerular basement membrane antibodies (Goodpasture’s syndrome)

Associated with systemic vasculitis e.g. granulomatosis with polyangiitis, microscopic polyangiitis

As a complication of other types of glomerulonephritis, including post-streptococcal glomerulonephritis and mesangial IgA disease

27
Q

What is Goodpasture’s disease?

A

Rare disease caused by autoimmunity to the glomerular basement membrane

28
Q

How does Goodpasture’s disease typically present?

A

As nephritis, with or without lung haemorrhage

29
Q

How is Goodpasture’s disease diagnosed?

A

By demonstrating anti-GBM antibodies in serum and kidney

30
Q

How is Goodpasture’s disease treated?

A

Plasma exchange and immunosuppression

Kidney can only be saved if detected and treated early

31
Q

What is the typical presentation of crescentic glomerulonephritis?

A

Rapidly progressive glomerulonephritis
May occur in isolation, or as a complication of other disease e.g. chronic nephritis
Medical emergency

32
Q

What is the management of crescentic glomerulonephritis?

A

Immunosuppression - prednisolone, cytotoxic drugs, plasma exchange

33
Q

What is the prognosis of crescentic glomerulonephritis?

A

Good, if treatment is started early enough

34
Q

CASE 1

7 year old boy presents to GP 
Generally unwell, dark urine due to haematuria 
Puffy face, no rash, no oedema 
BP 125/80 
Sore throat two weeks previously 
Hb 130 g/l
WCC 11.3 x 109
Urea 11.2 mmol/l
Creatinine 160umol/l
Urine microscopy – RBC +++, casts + 
Complement – low C3 
Spontaneous diuresis after 6 days 
Serum creatinine returned to normal

What is the most likely diagnosis?

A

Diffuse proliferative glomerulonephritis - post-streptococcal nephritis

35
Q

CASE 2

14 year old boy presents to GP with bright red urine
No other urinary symptoms
Concurrent upper respiratory tract infection
Physical examination normal
Urine dipstick shows high blood and trace of protein

What investigations would you do?

A 
FBC 
Creatinine, urea and electrolytes 
Urine microscopy 
Serology 
Renal ultrasound 
Renal biopsy
36
Q

CASE 2

14 year old boy presents to GP with bright red urine
No other urinary symptoms
Concurrent upper respiratory tract infection
Physical examination normal
Urine dipstick shows high blood and trace of protein
Urine microscopy shows dysmorphic erythrocytes and red cell casts

What is the most likely diagnosis?

A

Focal proliferative nephritis caused by mesangial IgA disease

37
Q

CASE 3

57 year old woman presents to GP
Three month history of increasing tiredness, anorexia and weight loss
Developed a cough with haemoptysis over last two weeks
Physical examination reveals rash on both ankles and lower legs and bilateral basal crepitations
Urine dipstick shows protein and blood

ANF and DNA binding negative
ANCA positive

What is the most likely diagnosis?

A

Focal necrotising glomerulonephritis secondary to systemic vasculitis

38
Q

What are the types of non-proliferative glomerulonephritis?

A

Minimal change disease
Focal glomerulonephritis
Membranous nephropathy
Causes nephrotic syndrome

39
Q

What is the presentation of nephrotic syndrome?

A

Clinical triad of pitting oedema, proteinuria (> 3.5g) and hypoalbuminaemia (< 30 g/l, normal > 45)

40
Q

What is nephrotic syndrome?

A

A clinical syndrome in which severe oedema is caused by hypoalbuminaemia due to loss of protein in the urine

41
Q

What are the additional features of nephrotic syndrome?

A

Hyperlipidaemia
Hypercoagulable state - increased DVT and venous thromboembolism risk
Increased risk of infection, especially pneumococcus

42
Q

What are the differential diagnoses of nephrotic syndrome?

A

Congestive heart failure - raised JVP, normal albumin, minimal proteinuria
Hepatic disease - abnormal LFTs, no proteinuria

Presence of proteinuria in nephrotic syndrome differentiates from these

43
Q

What are the principles of management of nephrotic syndrome?

A

Specific management
Treatment of oedema
Prophylaxis against complications - identify those at risk of DVT/VTE and give anticoagulation

44
Q

What is the specific management of nephrotic syndrome?

A

Make specific diagnosis e.g. by renal biopsy
Specific treatment when indicated
Consider steroid treatment with prednisolone if severe

45
Q

What is the treatment of oedema in nephrotic syndrome?

A

Fluid and sodium restriction
Normal/high protein diet
Loop diuretics e.g. furosemide
IV infusion of albumin furosemide for resistant oedema

46
Q

What prophylaxis can be given against potential complications of nephrotic syndrome?

A

Penicillin V for prophylaxis against infection
Heparin
Treat hyperlipidaemia in those with persistent nephrotic syndrome

47
Q

What are the features of minimal change nephrotic syndrome?

A

Most common form in children
Sudden onset of oedema
Complete loss of proteinuria with steroids
2/3rds of patients relapse despite most going into remission

48
Q

What is the treatment of minimal change nephrotic syndrome?

A

Prednisolone 60mg/m^2 per day, with rapid tapering of dose after a maximum of 8 weeks
Initial relapse treated in the same way
Subsequent relapses treated with 8 week course of cyclophosphamide or continuous cyclosporine
Treat any infection aggressively with antibiotics

49
Q

What are the complications of minimal change nephrotic syndrome?

A

Bacterial infection, especially with pneumococcus

Thrombosis

50
Q

What is the prognosis of minimal change nephrotic syndrome?

A

Resolves eventually, long term prognosis good

Patients do not develop renal failure

51
Q

What are the features of focal glomerulosclerosis (steroid resistant nephrotic syndrome)?

A

Severe nephrotic syndrome, especially in men in fourth decade
Very disabling symptoms
Incomplete (at best) response to steroids
Progresses to renal failure over 2-3 years
Can recur in renal transplants
Immunohistology negative
Sclerotic lesions

52
Q

Why is recover from focal glomerulosclerosis not possible?

A

Podocytes are killed by the disease process

53
Q

What is the aim of treatment of focal glomerulosclerosis?

A

To delay the time until end-stage kidney disease

54
Q

What is the treatment of focal glomerulosclerosis?

A

Initial trial of steroids - if complete response then prognosis is the same as for minimal change, if no response then poor prognosis
Continue steroids if clinically useful response
Try cyclophosphamide or cyclosporine if steroids fail
Steroid-sparing agents can be used, variable success
Non-specific drugs to reduce proteinuria e.g. ACEIs and NSAIDs
Non-specific treatment for nephrotic oedema and prophylaxis against nephrotic complications

55
Q

What is the commonest cause of nephrotic syndrome in adults?

A

Membranous nephropathy

56
Q

What is the cause of membranous nephropathy?

A

About half of cases are idiopathic
The other half are associated with another disease e.g. malignancy, systemic lupus, rheumatoid arthritis
Can also be caused by hypersensitivity to drugs

57
Q

Where are deposits located in membranous nephropathy?

A

Between the spikes

58
Q

What is the management of membranous nephropathy?

A

Control nephrotic symptoms
Immunosuppression for those with deteriorating renal function
Prednisolone and chorambucil, alternating monthly for six months
Cyclosporine probably ineffective
General measures in treatment should be done for 6 months before starting immunosuppression

59
Q

What is the prognosis of membranous nephropathy?

A

Resolves spontaneously in 25% over 5-10 years
Prognosis is good in treated patients whose proteinuria resolves
About 25% will be on dialysis at 10 years
Can recur in renal transplants

60
Q

CASE 4

22 year old woman 
Severe oedema which had developed suddenly over a week 
Breathless
Left sided chest pain 
Urine contained high level of protein 
Nephrotic syndrome 
Creatinine upper level of normal 
Serum albumin low - 15 g/l

What is the likely diagnosis?

A

Minimal change nephrotic syndrome

61
Q

CASE 5

66 year old man 
Steadily increasing oedema 
Recent change in bowel habit 
High level of proteinuria
Serum creatinine high at 135mmol/l

What is the likely diagnosis?

A

Membranous nephropathy

Need to determine if it is associated with malignancy

Urinary System (22 decks)

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