(31) Renal System Disease 1 Flashcards

1
Q

What are the functions of the kidney?

A
  • eliminating metabolic waste products
  • regulating fluid and electrolyte balance
  • influencing acid-base balance
  • production of some hormones
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2
Q

Which hormones does the kidney produce and what is their function?

A

Renin = fluid balance

Erythropoietin = stimulates red cell production

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3
Q

How many people develop acute renal failure?

A

Around 26,000 per year in England

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4
Q

What is the prognosis for patients with acute renal failure?

A

Most will recover but around 10,000 will need dialysis. For these patients, mortality is around 50%

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5
Q

How many people develop chronic renal failure?

A

Around 5,500 per year in England

Around 41,000 patients with CRF in England at any one time

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6
Q

What proportion of those with chronic renal failure need transplant or dialysis?

A
  • 50% will have a transplant at some point
  • 40% haemodialysis
  • 10% peritoneal dialysis
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7
Q

How many transplants occur?

A
  • 1020 living donor renal transplants
  • 1667 deceased donor transplants
  • 7000 patients still on the transplant waiting list

(in 2010-2011)

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8
Q

In what 6 ways can patients with renal disease present?

A
  • acute renal failure
  • nephrotic syndrome
  • acute nephritis/nephritic syndrome
  • chronic renal failure
  • isolated haematuria
  • isolated proteinuria
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9
Q

Patients with renal disease may present with acute renal failure. What is this?

A
  • rapid rise in creatinine and urea
  • generally unwell

(most common presentation)

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10
Q

Patients with renal disease may present with nephrotic syndrome. What is nephrotic syndrome?

A
  • oedema + proteinuria + hypoalbuminaemia
  • proteinuria >3g per 24 hours (mostly albumin)

Combination of these 3 things define nephrotic syndrome

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11
Q

Patients with renal disease may present with acute nephritis/nephritic syndrome. What is this?

A

Oedema + proteinuria + haematuria + renal failure

proteinuria normally not as heavy as in nephrotic syndrome

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12
Q

What defines chronic renal failure?

A

Slowly declining renal function

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13
Q

Which 5 different specialties are involved in diagnosing renal disease?

A
  • renal physician
  • biochemist
  • pathologist
  • urologist
  • radiologist
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14
Q

What role does a renal physician play in diagnosis of renal disease?

A
  • clinical history

- examination

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15
Q

What role does a biochemist play in diagnosis of renal disease?

A

Blood tests:

  • urea
  • creatine

Urine analysis:

  • protein
  • electrolytes
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16
Q

What role does a pathologist play in diagnosis of renal disease?

A

Renal biopsy:

  • light microscopy (common)
  • immunofluorescence (special test)
  • electron microscopy (special test)
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17
Q

What role does a urologist play in diagnosis of renal disease?

A

Cytoscopy may be needed:

  • obstruction
  • some cases of haematuia
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18
Q

Why might a urologist be needed if haematuria is found?

A

Common symptom of bladder cancer

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19
Q

What role does a radiologist play in diagnosis of renal disease?

A
  • obstruction
  • kidney size
  • structural abnormalities
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20
Q

What is the glomerulus?

A

A network of capillaries located at the beginning of a nephron in the kidney. It serves as the first stage in the filtering process of the blood carried out by the nephron in its formation of urine - then passes along tubules

Coiled capillary

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21
Q

What is the glomerulus surrounded by?

A

Bowman’s capsule

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22
Q

What are the 2 main parts of the kidney?

A
  • cortex (outside part)

- medulla (inside part)

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23
Q

What does the renal cortex contain?

A

Renal tubules and glomeruli

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24
Q

What are the 3 layers of the wall of the glomerul?

A
  • podocyte foot processes
  • basement membrane
  • endothelial cells

(and then the capillary lumen)

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25
Q

What allows filtration at there glomerulus?

A

The podocyte foot processes, basement membrane and endothelial cells (endothelial cells have little pores) - damage to any of these can cause kidney disease

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26
Q

What are the different parts (in order) of the renal tubules in the nephron?

A
  • Bowman’s capsule
  • proximal convoluted tubule (PCT)
  • descending limb of Henle
  • loop of Henle
  • ascending limb of Henle
  • distal convoluted tubule (DCT)
  • collecting duct (leading to the renal pelvis)
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27
Q

What are the different parts (in order) of the blood flow through the nephron?

A
  • branches of the renal artery
  • afferent arteriole
  • glomerulus (capillary bed)
  • efferent arteriole
  • vasa recta
  • branches of renal vein
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28
Q

In which part of the kidney is the loop of Henle?

A

The renal medulla

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29
Q

What is the renal tubule doing?

A

Concentrating the urine, excreting and reabsorbing substances

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30
Q

What else would a disease of the glomerulus affect?

A

It will cut off the blood supply to the rest of the nephron (tubules)

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31
Q

What rare the immunological mechanisms of glomerular damage?

A
  • circulating immune complexes deposit in glomerulus
  • circulating antigens deposit in glomerulus
  • antibodies to basement membrane or other components of glomerulus
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32
Q

What do all of the immunological mechanisms of glomerular damage lead to?

A

Damage to basement membrane

  • complement activation
  • neutrophil activation
  • reactive oxygen species
  • clotting factors

Glomerular damage

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33
Q

What are the non-immunological mechanisms of glomerular damage?

A
  • injury to endothelium of vessels
  • altered basement membrane due to hyperglycaemia in diabetes
  • abnormal basement membrane or podocytes due to inherited disease
  • deposition of abnormal proteins in the kidney e.g. amyloid
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34
Q

What are the 2 categories of glomerular damage mechanisms?

A
  • immunological

- non-immunological

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35
Q

What are the 2 categories of tubular damage mechanisms?

A
  • ischaemic

- toxic

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36
Q

What are the ischaemic mechanisms of tubular damage?

A
  • hypotension
  • damage to vessels within kidney
  • glomerular damage
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37
Q

What do the ischaemic mechanisms of tubular damage lead to?

A

Reduced blood supply to tubules and therefore tubular damage

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38
Q

What are the toxic mechanisms of tubular damage?

A
  • direct toxins
  • hypersensitivity reactions eg. to drugs
  • deposition of crystals in tubules eg. in gout
  • deposition of abnormal proteins in tubules e.g. amyloid
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39
Q

The degree of damage to renal tubules correlates with what?

A

Renal function

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40
Q

What are the potential mechanisms of vascular damage?

A
  • hypertension
  • diabetes
  • atheroma eg. renal artery stenosis
  • thrombotic microangiopathy
  • vasculitis
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41
Q

What is thrombotic microangiopathy?

A
  • thrombi in capillaries and small arterioles
  • due to endothelium damage
  • endothelium damage due to bacterial toxins, drugs, abnormalities in complement or clotting systems etc
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42
Q

Give an example of a thrombotic microangiopathy

A

Haemolytic uraemic syndrome

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43
Q

What is vasculitis?

A
  • acute or chronic inflammation of blood vessel walls
  • obliteration of lumen by inflammation
  • various different types
  • adults and children affected
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44
Q

Give an example of a type of vasculitis

A

Wegener’s granulomatosis

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45
Q

Damage to the vessels can also lead to damage to what?

A
  • damage to the glomerulus
  • damage to the tubule

(due to reduced blood flow)

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46
Q

Damage to the glomerulus can also lead to damage to what?

A

Damage to the tubule

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47
Q

Why are glomerular diseases so confusing?

A
  • many names refer to microscopic appearances rather than actual disease
  • some names are both diseases (when idiopathic) or just appearances (when secondary to a known cause)
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48
Q

Give some examples of glomerular diseases where the name is both a disease and also just a description of appearance

A
  • membranous nephropathy (idiopathic disease vs. secondary to known cause)
  • FSGS
  • mesangiocapillary glomerulonephritis
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49
Q

Give some other examples of glomerular diseases (names of actual diseases)

A
  • minimal change disease
  • post-infective glomerulonephritis
  • anti-GBM disease
  • IgA nephropathy
  • Henoch-Schonlein purpura
  • lupus nephritis (in SLE)
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50
Q

Give some other examples of appearances of glomerular diseases (appearance on biopsy)

A
  • proliferative glomerulonephritis
  • crescentic glomerulonephritis
  • thrombotic microangiopathy
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51
Q

What is nephrotic syndrome always due to?

A

Damage to the glomerulus

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52
Q

What does nephrotic syndrome consist of?

A
  • oedema
  • proteinuria (>3g in 24h)
  • hypoalbuminaemia
  • +/- hypertension
  • +/- hyperlipidaemia
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53
Q

What are the complications associated with nephrotic syndrome?

A
  • infection

- thrombosis

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54
Q

What are the common causes of nephrotic syndrome in adults?

A
  • membranous nephropathy
  • focal segmental glomerulosclerosis (FSGS)
  • minimal change disease
  • other causes eg. diabetes, lupus nephritis, amyloid
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55
Q

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

A

Membranous nephropathy

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56
Q

What is membranous nephropathy?

A

Thickening of the glomerular basement membrane

  • primary glomerular disorder of unknown cause
  • idiopathic
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57
Q

Who is usually affected by membranous nephropathy?

A

Usually adults 30-60

M>F

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58
Q

How many with membranous nephropathy will progress to end stage renal failure?

A

20-30%

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59
Q

What is focal segmental glomerulosclerosis?

A

Scar tissue formation in some of the glomeruli

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60
Q

What are the causes of focal segmental glomerulosclerosis?

A

Various possible causes

  • most are idiopathic
  • genetic
  • heroin use
  • HIV
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61
Q

Which gender is more commonly affected by FSGS?

A

M>F

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62
Q

What is seen in minimal change disease?

A

Biopsy is normal on light microscopy

changes only seen on electron microscopy - glomerular damage

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63
Q

Which gender more commonly gets minimal change disease?

A

M=F

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64
Q

What are the common cause of nephrotic syndrome in children?

A
  • minimal change disease
  • focal segmental glomerulosclerosis
  • other causes are rare
65
Q

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

A

Minimal change disease

66
Q

What are the causes of minimal change disease?

A

Cause is unknown the disease may occur after or be related to:

  • allergic reactions
  • NSAID use
  • tumors
  • vaccinations
  • viral infection
67
Q

What signs make up acute nephritis?

A
  • oedema
  • haematuria
  • proteinuria
  • acute renal failure

(another presentation of kidney disease)

68
Q

What are the common causes of acute nephritis in adults?

A
  • post-infective glomerulonephritis
  • IgA nephropathy
  • vasculitis
  • lupus
  • other forms of primary glomerulonephritis
69
Q

Post-infective glomerulonephritis typically occurs after what?

A

A few weeks after Streptococcal throat infection

Most recover completely

70
Q

What is the most common primary glomerular disease worldwide?

A

IgA nephropathy

71
Q

What is the damage in IgA nephropathy caused by?

A

Abnormal deposits of IgA protein in the glomeruli

72
Q

What is the most common sign of IgA nephropathy?

A

Haematuria (also swelling of feet)

Typically teenagers and young adults with haematuria

73
Q

How many with IgA nephropathy will progress to renal failure?

A

20-50% will progress to renal failure over 20 years

74
Q

What are the signs of vasculitis?

A
  • fever
  • generally unwell
  • may have rash
  • myalgia
  • arthralgia
75
Q

Who typically gets lupus?

A

Young women

autoimmune disease with many manifestations

76
Q

What are the common causes of acute nephritis in children?

A
  • post-infective glomerulonephritis
  • IgA nephropathy
  • Henoch-Schonlein purpura
  • haemolytic-uraemic syndrome
77
Q

What is Henoch-Schonlein purpura?

A
  • specific type of IgA nephropathy

- M>F

78
Q

What is the typical presentation of Henoch-Schonlein purpura?

A

Typically young boys/teenagers with

  • arthralgia
  • abdo pain
  • rash
  • haematuria
  • acute renal failure

Most recover completely

79
Q

What is haemolytic-uraemic syndrome characterised by?

A
  • acute nephritis
  • haemolysis
  • thrombocytopaenia
80
Q

Who typically gets haemolytic-uraemic syndrome?

A

Children with E. coli 0157 enteritis

81
Q

What is the diagnosis of acute renal failure based on?

A
  • anuria/oliguria
  • raised creatinine
  • raised urea
82
Q

What is oliguria?

A

Production of abnormally small amounts of urine

83
Q

What is the usual prognosis in acute renal failure?

A

Many patients will recover and have good renal function if they had previously healthy kidneys

Short term dialysis may be needed in some patients

84
Q

What are the 3 categories of causes of acute renal failure?

A
  • pre-renal (most common)
  • renal
  • post-renal
85
Q

What do pre-renal causes mean?

A

Reduced blood flow to kidney (MOST COMMON)

86
Q

What do renal causes mean?

A

Damage to kidney

87
Q

What do post-renal causes mean?

A

Obstructed urinary tract

88
Q

Give examples of pre-renal causes of acute renal failure

A
  • severe dehydration

- hypotension eg. bleeding, septic shock, left ventricular failure

89
Q

Give some examples of post-renal causes of acute renal failure

A
  • tumours of urinary tract
  • tumours in renal pelvis
  • bladder stones
  • prostatic enlargement
90
Q

In which causes of acute renal failure is a renal biopsy useful?

A

Useful in renal causes.

Not helpful in pre- or post- renal causes

91
Q

What will all biopsies show in acute renal failure?

A

ATN - acute tubular necrosis/injury/damage/acute kidney injury (ATI/ATD/AKI)

92
Q

What are the causes of acute renal failure in adults?

A
  • vasculitis

- acute interstitial nephritis/tubulointerstitial nephritis

93
Q

What is acute interstitial nephritis/tubulointerstitial nephritis?

A

Tubular damage with inflammation, most commonly caused by drug reactions - most recover

94
Q

What are the causes of acute renal failure in children?

A
  • Henoch-Schonlein purpura
  • haemolytic uraemic syndrome
  • acute interstitial nephritis
95
Q

What are the complications associated with acute renal failure?

A
  • cardiac failure (fluid overload)
  • arrhythmias (electrolyte imbalance)
  • GI bleeding
  • jaundice (hepatic venous congestion)
  • infection, especially lung and urinary tract
96
Q

What is the treatment for acute renal failure?

A
  • short term dialysis may be needed

- treat the underlying cause

97
Q

What is chronic renal failure defined by?

A

Permanently reduced GFR - reduced number of nephrons

98
Q

What is stage 1 chronic renal failure?

A

kidney damage with normal or increased GFR (>90mL/min/1.73m2)

99
Q

What is stage 2 chronic renal failure?

A

mild reduction in GFR (60-89mL/min/1.73m2)

100
Q

What is stage 3 chronic renal failure?

A

moderate reduction in GFR (30-59mL/min/1.73m2)

101
Q

What is stage 4 chronic renal failure?

A

severe reduction in GFR (15-29mL/min/1.73m2)

102
Q

What is stage 5 chronic renal failure?

A

kidney failure (GFR

103
Q

At what stage of chronic renal failure is treatment required and more problems start occurring?

A

Stages 4 and 5

104
Q

What are the common causes of chronic renal failure in adults?

A
  • diabetes
  • glomerulonephritis
  • reflux nephropathy
105
Q

What is the commonest cause of chronic renal failure in this country?

A

Diabetes

106
Q

What is reflux nephropathy?

A

Chronic reflux of urine up the ureter leading to repeated infections and scarring of the kidney

107
Q

What are the common causes of chronic renal failure in children?

A
  • developmental abnormalities/malformations
  • reflux nephropathy
  • glomerulonephritis
108
Q

What does renal biopsy show in chronic renal failure?

A

Kidney shows severe scarring with loss of glomeruli and tubules

109
Q

Why is a renal biopsy often unhelpful in established chronic renal failure?

A

Similar changes are seen in end-stage renal disease due to any cause - so hard to identify the cause

Most biopsies will look the same at this stage

110
Q

What are the effects on the body of chronic renal failure?

A
  • reduced excretion of water and electrolytes cause oedema and hypertension
  • reduced excretion of toxic metabolites
  • reduced production of erythropoietin leading to anaemia
  • renal bone disease
111
Q

What is the likely diagnosis for an elderly patient with acute renal failure?

A
  • acute interstitial nephritis due to drug reactions

- myeloma

112
Q

What is the likely diagnosis for a young male with haematuria and rash?

A

Henoch-Schonlein purpura

113
Q

What is the likely diagnosis for a teenager/young adult with haematuria?

A
  • post-infective glomerulonephritis

- IgA nephropathy

114
Q

What is the likely diagnosis for an adult with acute renal failure, fever and myalgia?

A

Vasculitis

115
Q

What is the likely diagnosis for a young woman with haematuria and facial rash?

A

Lupus

butterfly rash

116
Q

What is the likely diagnosis for an adult with nephrotic syndrome?

A

Membranous nephropathy (most cases idiopathic)

117
Q

What is the likely diagnosis for a child with nephrotic syndrome?

A

Minimal change disease

118
Q

What tests may be need din isolated haematuria?

A

Cytoscopy and other urological investigating as bleeding may be from urinary tract tumours

119
Q

What is cytoscopy?

A

Camera into the bladder

120
Q

What are the likely renal causes of haematuria with normal renal function?

A
  • IgA nephropathy
  • thin basement membrane disease
  • Alport type hereditary nephropathy
121
Q

What is thin basement membrane disease?

A
  • inherited condition causing abnormally thin basement membranes in the glomeruli
  • if no other renal disease is present, kidney function will remain normal
122
Q

What is Alport type hereditary nephropathy?

A
  • inherited abnormalities of collagen type IV causing abnormal basement membrane in the glomerulus
  • sometimes have eye and ear problems
123
Q

What are the signs of Alport syndrome?

A
  • renal failure
  • +/- deafness
  • +/- ocular problems
124
Q

What is the genetic pattern in Alport syndrome?

A

May be autosomal or X-linked

125
Q

What is isolated proteinuria defined by?

A

Proteinuria less than the nephrotic range, without haematuria, renal failure or oedema

126
Q

Isolated proteinuria might be benign, due to what?

A
  • postural

- related to pyrexia or exercise

127
Q

Isolated proteinuria might be due to renal disease. What are the common causes in adults?

A
  • FSGS
  • diabetes
  • lupus
128
Q

Isolated proteinuria might be due to renal disease. What are the common causes in children?

A
  • Henoch-Schonlein purpura

- FSGS

129
Q

What causes renal artery stenosis?

A

Atheroma (most common)

or arterial dysplasia

130
Q

What does renal artery stenosis lead to?

A

Ischaemia of the affected kidney - kidney left scarred and shrunken - reduced renal function due to loss of renal tissue

Also hypertension

131
Q

What causes hypertension in renal artery stenosis?

A

Stenosis leads to activation of RAAS leading to hypertension

132
Q

What is pyelonephritis?

A

Infection of the kidney

133
Q

How can infection of the kidney (pyelonephritis) occur?

A
  • via haematogenous spread

- via ascending route from bladder

134
Q

What causes acute pyelonephritis?

A
  • more common in women (ascending infection)
  • instrumentation of urinary tract
  • diabetics
  • structural abnormalities or urinary tract
135
Q

What are the complications associated with acute pyelonephritis?

A
  • abscess formation
136
Q

What are the causes of chronic pyelonephritis?

A
  • obstruction or urinary tract
  • reflux of urine up ureter
    leads to scarring of kidney and can lead to renal failure
137
Q

What are the complications associated with chronic pyelonephritis?

A
  • scarring of kidney

- chronic renal failure

138
Q

How can vasculitis affect the kidneys?

A
  • affect glomerular vessels leading to clotting with obliteration of capillary lumens and destruction of glomerulus
  • inflammation of larger arterioles within kidney leading to hypoxia of tubules
139
Q

Vasculitis also often affects other vessels around the body leading to what symptoms?

A
  • rash
  • muscle pain
  • joint pain

Can also have fever and weight loss due to the inflammation

140
Q

On histology, what does bright pink in the glomerulus indicate?

A

Bright pink = fibrin - indicated thrombosis within glomerulus

May not be able to see capillary lumens

141
Q

On histology, what do dark dots around an artery indicate?

A

Inflammatory cells surrounding artery which has fibrin in wall - indicating damage to wall

142
Q

How does hypertension affect the kidneys?

A
  • damages renal vessels leading to thickening of vessel wall and reduction in size of lumen
  • produces chronic hypoxia which leads to loss of renal tubules and reduced renal function
  • reduced blood flow activates RAAS which exacerbates hypertension
143
Q

How is hypertension shown on renal biopsy?

A

Can see thickening of the walls of small arteries ad arterioles and a reduction in the size of the lumen

144
Q

What is the commonest cause of end-stage renal failure in Europe and the USA?

A

Diabetes

145
Q

What about diabetes causes diabetic nephropathy?

A

Hyperglycaemia

146
Q

How does diabetes cause damage to the kidneys?

A
  • hyperglycaemia damages the basement membrane
  • basemement membrane becomes thicker and the glomerulus produces excess extacellular matrix with forms nodules
  • diabetes also damages small vessels leading to ischaemia and damage to renal tubules
147
Q

How is diabetic nephropathy seen on renal biopsy/histology?

A

Can see nodules within the glomerulus

148
Q

How does myeloma cause damage to the kidneys?

A
  • tumour of plasma cells producing immunoglobins
  • excess immunoglobins deposit in tubules
  • tubules damaged
  • inflammation and fibrosis of kidney
  • loss of tubules = loss of renal function
149
Q

Is renal impairment due to myeloma reversible?

A

No, usually irreversible

150
Q

How does a kidney affected by myeloma look on histology?

A
  • immunoglobin deposits in artery walls (smooth, pink, thickened walls)
  • tubules destroyed by immunoglobin deposits (bright pink)
  • fibrosis (scarring)
  • residual tubules
151
Q

What is the function of mesangial cells?

A

Involved in maintaining structure of glomerulus

remove trapped residues and aggregated protein from the basement membrane thus keeping the filter free of debris

152
Q

What is the main sign of membranous nephropathy on histology?

A

Thickened glomerular capillary walls

153
Q

What is the main sign of FSGS on histology?

A

Most of the glomerulus is normal, one area appears solid with loss of capillary lumens

154
Q

What can post-streptococcal glomerulonephritis and IgA nephropathy both show on histology?

A

Glomeruli which have an increased number of cells and loss of capillary lumens

155
Q

What is seen on histology in acute tubulointerstitial nephritis?

A

Many lymphocytes and other inflammatory cells (dark dots) present between renal tubules

156
Q

What is a crescent (seen on kidney biopsy)?

A

Cells proliferating within Bowman’s capsule as a response to severe glomerular injury

Crescent surrounds the glomerulus

157
Q

What conditions may crescents be seen in?

A
Any condition where there is severe glomerular injury
- anti-GBM disease
- severe vasculitis
- severe-infective glomerulonephritis
etc
158
Q

What is seen on histology in acute tubular necrosis/injury?

A

Loss of nuclei from tubular cells (cell death)

159
Q

What is seen on histology in chronic kidney damage?

A

Tubular atrophy, shrunken tubules surrounded by interstitial fibrosis