Renal - Glomerulopathies Flashcards

(61 cards)

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
What does minimal change disease look like under: Light Microscopy Electron microscopy
Light : minimal change (hence name) Electron: Podocyte foot process effacement
26
What are the main principles of treatment for nephrotic syndrome?
Reduce oedema Reduce proteinuria Reduce risk of complications e.g. anticoagulate Treat underlying cause
27
Injury to what cell type in the kidney leads to high loss of protein and why?
Podocytes They wrap around the glomerular capillaries and maintain the filtration barrier. when damaged there is a high excretion of protein
28
What changes are seen on histology in membranous glomerulonephritis?
Diffusely thickened glomerular basement membrane with IgG and complement deposits
29
What antibodies are found in up to 80% of patients with idiopathic membranous glomerulonephriits?
Anti-phospholipase A2 antibodies
30
Berger's disease is another name for what?
IgA nephropathy
31
Which GN is the most common cause of primary glomerular nephropathy worldwide?
IgA nephropathy
32
What changes will be seen in a patient with IgA nephropathy?
Proliferative changes within glomerular mesangial cell with mesangial deposition of IgA
33
A male patient presents with macroscopic haematuria following an URTI only a few days ago, what is the most likely diagnosis?
IgA nephropathy (Bergers)
34
What % of patients with IgA nephropathy will go on to develop end stage renal failure?
25%
35
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?
Post streptococcal nephropathy
36
What is another name for post streptococcal glomerulonephritis?
Diffuse proliferative glomerulonephritis
37
What are some of the common features of a post streptococcal GN?
Usually under 30 y/o Presenting 1-3 weeks post URT infection (tonsillitis) Nephritic syndrome symptoms
38
What levels will be low in post streptococcal GN?
Complement
39
What immune complexes are deposited in the glomeruli in a post streptococcal GN?
Complement (C3) IgG IgM
40
What mode of inheritance is seen in Alport's syndrome?
X linked (dominant / recessive)
41
What is the difference between a glomerulonephritis and a glomerulopathy?
Glomerulonephritis - inflammation of the glomeruli can be seen Glomerulopathy - no evidence of inflammation can be seen
42
How should minimal change GN be managed?
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
How does focal segmental glomerulosclerosis present typically?
Massive proteinuria Renal impairment Hypertension Haematuria
44
Where is the kidney is typically affected first by focal segmental glomerulosclerosis?
The deep glomeruli of the corticomedullary junction
45
WHat is seen on light microscopy for focal segmental glomerulosclerosis?
Segmental sclerosis is seen first. Then progresses to global sclerosis Hyalinosis also seen
46
What deposits will be seen on immunofluorescence in focal segmental glomerulosclerosis?
C3 and IgM
47
What are the main causes of focal segmental glomerulosclerosis?
``` Idiopathic Secondary to other renal pathology e.g. IgA nephropathy, minimal change disease HIV Heroin abuse Sickle cell Alport's ```
48
How is FSGS treated?
<10% will go through spontaneous remission Prednisolone (6 months) +/- immunosuppressant e.g. ciclosporin
49
Define azotaemia?
This is a collection of biochemical abnormalities including a raised blood urea, nitrogen and creatinine often just referred to as uraemia
50
In diabetic nephropathy the kidneys shrivel and get smaller. True or false?
False | They bilaterally enlarge and initially will have hyperfiltration
51
What histological changes can be seen from early on in diabetic nephropathy?
Basement membrane thickening and mesangial expansion
52
What is the most common cause of glomerular pathology and CKD in the UK?
Diabetic Nephropathy
53
What causes the scarring in diabetic nephropathy?
The high concentration of glucose passing through the kidneys
54
How are diabetics screened for diabetic nephropathy?
Albumin:creatinine ratio | Us and Es for kidney function
55
What medication is first line for hypertension management in diabetic patients?
ACE inhibitors (or ARBs if not well tolerated)
56
What form of glomerulonephritis is rarer, but has a significantly poor prognosis and is sometimes associated with hep C?
Membranoproliferative GN
57
A patient presents with pulmonary haemorrhage (haemoptysis) and acute glomerulonephritis. What is the likely diagnosis?
Goodpasture's disease a.k.a anti-glomerular basement membrane GN
58
Anti-GBM / Goodpasture's disease is a form of _______ glomerulonephritis?
Rapidly progressive
59
How is Goodpasture's disease managed?
Plasma exchange Steroids Cyclophosphamide
60
Histology from a renal biopsy shows crescentic glomerulonephritis in an acutely unwell patient. What is the diagnosis?
Rapidly progressive GN
61
What causes the crescentic shape in rapidly progressive GN?
An aggregate of macrophages and epithelial cells in the Bowman's space