Renal - Glomerulopathies Flashcards

1
Q

What is the most common cause of nephrotic syndrome in children?

A

Minimal change disease

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2
Q

List some of the features of nephritic syndrome?

A

Haematuria (macro or micro)
Proteinuria
Oliguria
Fluid retention

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3
Q

What is the criteria a patient must fulfil to have nephrotic syndrome?

A

Peripheral oedema
Proteinuria of >3g in 24 hours
Serum albumin of <25g per litre (hypoalbuminemia)
(Can also present with or have the complication of hypercholesterolemia)

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4
Q

List the different types of glomerulonephritis:

A
Minimal change disease
IgA nephropathy  (aka Berger's disease or mesangioproliferative)
Goodpasture syndrome
Membranous glomerulonephritis
Focal segmental glomerulosclerosis
Mesangiocapillary glomerulonephritis
Rapidly progressive glomerulonephritis 
Post streptococcal (aka diffuse proliferative)
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5
Q

Define interstitial nephritis:

A

Inflammation of the space between the tubules (interstitium) and the cells of the kidney.

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6
Q

What are the two different forms of interstitial nephritis:

A

Acute interstitial nephritis

Chronic Tubulointerstitial nephritis

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7
Q

Which types of glomerulonephritis tend to present with nephritic syndrome symptoms?

A

IgA
Rapidly progressive
Alport’s syndrome

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8
Q

A male patient presents with some of the following symptoms:
microscopic haematuria and progressive renal failure
bilateral sensorineural deafness
lenticonus, retinitis pigmentosa
What is the likely diagnosis?

A

Alport’s syndrome

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9
Q

How is Alport’s syndrome diagnosed?

A

Genetic testing

Renal biopsy:
electron microscopy: characteristic finding is of the longitudinal splitting of the lamina densa of the glomerular basement membrane, resulting in a ‘basket-weave’ appearance

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10
Q

What is the mode of inheritance in Alport’s syndrome?

A

Autosomal dominant

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11
Q

Alport’s syndrome is caused by a defect in collagen type ? which leads to defective basement menbranes.

A

4

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12
Q

List the glomerulonephritides which cause nephrotic syndrome:

A
Focal segmental
Membranous
Minimal change
Diabetic
Amyloidosis
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13
Q

List the glomerulonephritides which can cause a mixture of nephritic and nephrotic syndrome?

A

Post streptococcal
Diffuse proliferative
Membranoproliferative

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14
Q

What is the most common type of glomerulonephritis in adults?

A

Membranous

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15
Q

At what age does membranous glomerulonephritis peak?

A

20’s and 60’s

There is a bimodal peak

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16
Q

What is the main symptom associated with membranous GN?

A

Proteinuria

A lot tend to be asymptomatic

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17
Q

List some of the main causes of membranous GN?

A

Idiopathic (>70%)

Infection
NSAIDs
Malignancy

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18
Q

What is the main course of management for Membranous GN?

A

30% will resolve on their own

All should receive either an ACE inhibitor or an ARB

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19
Q

What are the two main principles of management in any glomerulonephritis?

A

Steroids (immunosuppression)

ACE inhibitors or ARBs to control BP

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20
Q

What can nephrotic syndrome predispose a patient to?

A
Thromboembolism
Hypertension
Hypercholesterolemia
Increased risk of infections
Hypocalcemia
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21
Q

Loss of what substance can lead to the development of thrombus in patients with nephrotic syndrome?

A

Antithrombin III

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22
Q

Which form of glomerulonephritis is the most common cause of nephrotic syndrome in adults?

A

Focal segmental glomerulosclerosis

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23
Q

What are the main causes of minimal change disease?

A

Mainly idiopathic

Others include:
Hodgkin's lymphoma
Thymoma
NSAIDs &amp; rifampicin
Infectious mononucleosis
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24
Q

Why are patients with nephrotic syndrome more predisposed to infections?

A

There is a decrease in serum IgG levels and complement as well as a decreased T cell funciton

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25
Q

What does minimal change disease look like under:
Light Microscopy
Electron microscopy

A

Light : minimal change (hence name)

Electron: Podocyte foot process effacement

26
Q

What are the main principles of treatment for nephrotic syndrome?

A

Reduce oedema
Reduce proteinuria
Reduce risk of complications e.g. anticoagulate
Treat underlying cause

27
Q

Injury to what cell type in the kidney leads to high loss of protein and why?

A

Podocytes
They wrap around the glomerular capillaries and maintain the filtration barrier. when damaged there is a high excretion of protein

28
Q

What changes are seen on histology in membranous glomerulonephritis?

A

Diffusely thickened glomerular basement membrane with IgG and complement deposits

29
Q

What antibodies are found in up to 80% of patients with idiopathic membranous glomerulonephriits?

A

Anti-phospholipase A2 antibodies

30
Q

Berger’s disease is another name for what?

A

IgA nephropathy

31
Q

Which GN is the most common cause of primary glomerular nephropathy worldwide?

A

IgA nephropathy

32
Q

What changes will be seen in a patient with IgA nephropathy?

A

Proliferative changes within glomerular mesangial cell with mesangial deposition of IgA

33
Q

A male patient presents with macroscopic haematuria following an URTI only a few days ago, what is the most likely diagnosis?

A

IgA nephropathy (Bergers)

34
Q

What % of patients with IgA nephropathy will go on to develop end stage renal failure?

A

25%

35
Q

A renal biopsy taken from a young male shows the following:
acute, diffuse proliferative glomerulonephritis
endothelial proliferation with neutrophils
electron microscopy: subepithelial ‘humps’ caused by lumpy immune complex deposits
immunofluorescence: granular or ‘starry sky’ appearance. What is the likely diagnosis?

A

Post streptococcal nephropathy

36
Q

What is another name for post streptococcal glomerulonephritis?

A

Diffuse proliferative glomerulonephritis

37
Q

What are some of the common features of a post streptococcal GN?

A

Usually under 30 y/o
Presenting 1-3 weeks post URT infection (tonsillitis)
Nephritic syndrome symptoms

38
Q

What levels will be low in post streptococcal GN?

A

Complement

39
Q

What immune complexes are deposited in the glomeruli in a post streptococcal GN?

A

Complement (C3)
IgG
IgM

40
Q

What mode of inheritance is seen in Alport’s syndrome?

A

X linked (dominant / recessive)

41
Q

What is the difference between a glomerulonephritis and a glomerulopathy?

A

Glomerulonephritis - inflammation of the glomeruli can be seen
Glomerulopathy - no evidence of inflammation can be seen

42
Q

How should minimal change GN be managed?

A

High dose corticosteroid e.g. prednisalone for 4-6 weeks IF patient has significant proteinuria. Can be withheld for spontaneous remission
Up to 2/3rds will require further courses due to relapsing or non remittance

43
Q

How does focal segmental glomerulosclerosis present typically?

A

Massive proteinuria
Renal impairment
Hypertension
Haematuria

44
Q

Where is the kidney is typically affected first by focal segmental glomerulosclerosis?

A

The deep glomeruli of the corticomedullary junction

45
Q

WHat is seen on light microscopy for focal segmental glomerulosclerosis?

A

Segmental sclerosis is seen first. Then progresses to global sclerosis
Hyalinosis also seen

46
Q

What deposits will be seen on immunofluorescence in focal segmental glomerulosclerosis?

A

C3 and IgM

47
Q

What are the main causes of focal segmental glomerulosclerosis?

A
Idiopathic
Secondary to other renal pathology e.g. IgA nephropathy, minimal change disease
HIV
Heroin abuse
Sickle cell
Alport's
48
Q

How is FSGS treated?

A

<10% will go through spontaneous remission
Prednisolone (6 months)
+/- immunosuppressant e.g. ciclosporin

49
Q

Define azotaemia?

A

This is a collection of biochemical abnormalities including a raised blood urea, nitrogen and creatinine
often just referred to as uraemia

50
Q

In diabetic nephropathy the kidneys shrivel and get smaller. True or false?

A

False

They bilaterally enlarge and initially will have hyperfiltration

51
Q

What histological changes can be seen from early on in diabetic nephropathy?

A

Basement membrane thickening and mesangial expansion

52
Q

What is the most common cause of glomerular pathology and CKD in the UK?

A

Diabetic Nephropathy

53
Q

What causes the scarring in diabetic nephropathy?

A

The high concentration of glucose passing through the kidneys

54
Q

How are diabetics screened for diabetic nephropathy?

A

Albumin:creatinine ratio

Us and Es for kidney function

55
Q

What medication is first line for hypertension management in diabetic patients?

A

ACE inhibitors (or ARBs if not well tolerated)

56
Q

What form of glomerulonephritis is rarer, but has a significantly poor prognosis and is sometimes associated with hep C?

A

Membranoproliferative GN

57
Q

A patient presents with pulmonary haemorrhage (haemoptysis) and acute glomerulonephritis. What is the likely diagnosis?

A

Goodpasture’s disease a.k.a anti-glomerular basement membrane GN

58
Q

Anti-GBM / Goodpasture’s disease is a form of _______ glomerulonephritis?

A

Rapidly progressive

59
Q

How is Goodpasture’s disease managed?

A

Plasma exchange
Steroids
Cyclophosphamide

60
Q

Histology from a renal biopsy shows crescentic glomerulonephritis in an acutely unwell patient. What is the diagnosis?

A

Rapidly progressive GN

61
Q

What causes the crescentic shape in rapidly progressive GN?

A

An aggregate of macrophages and epithelial cells in the Bowman’s space