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Renal Pathology Flashcards

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USMLE® Step 1 flashcards
1
Q

What are the different types of casts?

A
  1. CELLULAR casts - e.g. epithelial, RBC, WBC
  2. GRANULAR casts - breakdown of cellular casts
  3. HYALINE casts
  4. FATTY casts
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2
Q

Where are cellular casts formed?

A

Within the tubular lumen

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3
Q

What are casts primarily composed of?

A

Tamm-Horsfall protein - secreted from the renal tubular cells

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4
Q

What do hyaline casts indicate?

A

Usually nothing, tend to be of little diagnostic significance. May be seen in volume depleted states

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5
Q

From what are granular casts derived?

A

The breakdown of cellular casts, especially epithelial cell casts

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6
Q

What do epithelial cell casts indicate?

A
  1. Acute tubular necrosis
  2. Heavy metal poisoning
  3. Acute rejection of the transplant graft
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7
Q

What do RBC casts indicate?

A
  1. Glomerulonephritis
  2. IgA nephropathy
  3. Post streptococcal glomerulonephritis
  4. Goodpasture syndrome
  5. Malignant HTN
  6. Vasculitis
  7. Renal ischaemia
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8
Q

What do WBC casts indicate?

A
  1. Pyelonephritis
  2. Interstitial nephritis
  3. Lupus nephritis
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9
Q

What do granular casts indicate?

A
  1. Acute tubular necrosis
  2. Chronic renal failure
  3. Nephrotic syndrome
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10
Q

What do fatty casts indicate?

A

Nephrotic syndrome

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11
Q

What is the structure of fatty casts?

A

Maltese-cross configuration of cholesterol

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12
Q

What are the different types of glomerular disease?

A
  1. Nephrotic syndromes

2. Nephritic syndromes

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13
Q

What causes nephrotic syndrome?

A

Increased filtration barrier permeability

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14
Q

What are the nephrotic syndromes?

A
  1. MCD
  2. Focal segmental glomerulosclerosis
  3. Membranous glomerulopathy
  4. Membranoproliferative glomerulonephritis
  5. Diabetic nephropathy
  6. Renal amyloidosis
  7. Lupus nephritis
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15
Q

What causes nephritic syndrome?

A

Inflammatory damage to the glomeruli

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16
Q

What are the nephritic syndromes?

A
  1. Acute proliferative glomerulonephritis
  2. RPGN
  3. Anti-GBM disease
  4. Goodpasture syndrome
  5. IgA nephropathy (Berger disease)
  6. Alport syndrome (hereditary nephritis)
  7. Lupus nephritis
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17
Q

What are the characteristic symptoms of nephrotic syndrome?

A

Puffiness around the eyes
Pitting edema of the legs
Pleural effusion
Ascites

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18
Q

What are the hallmarks/signs of nephrotic syndrome?

A 
Proteinuria
Hypoalbuminaemia
Edema
Hyperlipidaemia
Urinary fatty casts
Hyper-coagulation
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19
Q

Why is there an increase in the permeability of the filtration barrier?

A

Mononuclear cells release cytokines.

These cytokines cause podocyte fusion + obliterate the -ve charge of the GBM

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20
Q

What is the definition of the proteinuria?

A

Urinary protein >3.5g/24hrs

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21
Q

What is the definition of hypoalbuminaemia?

A

Plasma albumin <3g/dL

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22
Q

What is the physiology of the edema in nephrotic syndrome?

A

Albumin loss = lower oncotic pressure
Lower oncotic pressure = loss of fluid from the circulation into the interstitial space
= activation of RAAS, increased sympathetic activity, release of vasopressin, decreased ANP release
All these changes^ = increased electrolyte and water retention

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23
Q

Why does hyperlipidaemia occur in nephrotic syndrome?

A

Increased production of lipoproteins by the liver in order to attempt to main the falling oncotic pressure in nephrotic syndrome

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24
Q

Why does hypercoaguability occur in nephrotic syndrome?

A

Loss of antithrombin III from the damaged glomeruli

= increased risk of renal vein thrombosis

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25
Q

What is the most common cause of nephrotic syndrome?

A

MCD

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26
Q

How is MCD diagnosed?

A

Renal biopsy + microscopy (specifically electron microscopy)

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27
Q

When is a definitive diagnosis not required for MCD?

A

Children with a typical presentation of MCD may be given an empiric steroids without a renal biopsy

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28
Q

What is seen on an electron microscope in MCD?

A

Effacement (fusion) of the visceral epithelial foot processes

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29
Q

What is seen on a light microscope in MCD?

A

Lipoid nephrosis - lipoid appearance of cells in proximal tubules

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30
Q

How is MCD treated?

A

High dose oral glucocorticoids (prednisolone). Alkylating agents (e.g. cyclophosphamide or chlorambucil) in failure to respond to steroids

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31
Q

What is the prognosis of MCD in an adult vs. children?

A 
Adult = 50% response
Children = 90% response to treatment
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32
Q

What proportion of cases of nephrotic syndromes does focal segmental glomerulosclerosis account for?

A

1/3 in adults; 50% of cases in African Americans

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33
Q

What is the most common glomerular disease in HIV patients?

A

Focal segmental glomerulosclerosis (FSGS)

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34
Q

What are the differences in presentation between FSGS and MCD?

A

FSGS = non-selective proteinuria (whereas MCD = selective proteinuria of albumin)
Patients with FSGS also have HTN and mild haematuria on top of the usual symptoms of nephrotic syndrome

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35
Q

What is seen on light microscopy in FSGS?

A

FOCAL accumulation of hyaline material

SEGMENTAL sclerosis

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36
Q

What is the prognosis of FSGS?

A

Poor - 50% develop ESRD within 10 years

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37
Q

What is the leading cause of nephrotic syndrome in adults?

A

Membranous glomerulopathy (30-40% of cases)

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38
Q

What are the demographics of membranous glomerulopathy?

A

More often seen in men - 2:1 ratio

Peak incidence = 30-50y/o

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39
Q

Is the proteinuria in membranous glomerulopathy selective or non-selective?

A

Non-selective

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40
Q

What systematic diseases are glomerulopathy associated with?

A 
SLE
RA
Hep B
Hep C
Syphillis
Schistomiasis
Malaria
Leprosy
Drugs - e.g. gold and penicillamine
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41
Q

How do sub-epithelial deposits appear under the electron microscope?

A

‘Spike’ = extensions of GBM around the deposits + ‘Dome’ = deposits in the GBM

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42
Q

How is membranous glomerulopathy USUALLY treated? How otherwise might it be treated?

A

High rate of spontaneous remission, therefore no treatment needed. Otherwise, in severe disease, immunosuppressive therapy of cyclophosphamide + cyclosporine + glucocorticoids = used

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43
Q

What are the 2 types of membranoproliferative glomerulonephritis?

A

Type I - 2/3 of cases - type III hypersensitivity, deposition of immune complexes
type II - 1/3 of cases - associated with C3 nephritic factor. C£ present, but no IgG deposits

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44
Q

How does type I membranoproliferative glomerulonephritis appear under the electron microscope?

A

Subendothelial deposits of IgG + C3 + tram-track appearance from mesangium ingrowth into the GBM

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45
Q

How does type II membranoproliferative glomerulonephritis appear under the electron microscope?

A

Intramembranous deposits + increased glomeruli size + tram-track appearance

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46
Q

In whom with membranoproliferative glomerulonephritis may PE be useful?

A

In patients with circulating C3NeF

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47
Q

How does the prognosis differ between type I and II membranoproliferative glomerulonephritis?

A

Type I = 70-85% = no chronic decline in GFR

Type II = worse prognosis. Majority of patients progress to ESRD after 5-10 years

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48
Q

In which type of DM is diabetic nephropathy more common?

A

Type I

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49
Q

After how long of microalbuminaemia do other symptoms develop?

A

5-10 years

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50
Q

What is the leading cause of ESRD in the West?

A

Diabetic nephropathy

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51
Q

What is a complication seen in DM nephropathy?

A

Arteriosclerosis of the real artery

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52
Q

What is seen under the light microscope in DM nephropathy?

A

Thickening of GBM
Expansion of the mesangium
Kimmelstiel-Wilson lesions

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53
Q

How should DM nephropathy be treated?

A

Good glucose control + ACE inhibitors at onset of microalbuminaemia

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54
Q

What are the characteristics of amyloidosis?

A

Fibrous, insoluble proteins in a beta-pleated sheet confirmation in the extracellular space of the organs. = a multi-system disorder

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55
Q

What are the different types of amyloidosis that affect the kidney?

A
  1. Amyloid L (AL)

2. Amyloid A (AA)

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56
Q

What happens if the chains are not beta-pleated? What is the disease called then?

A

Light-chain deposition disease

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57
Q

From where may a biopsy be taken for diagnosis of amyloidosis?

A

Renal, abdominal fat pad, rectally

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58
Q

Which stain is used to identify amyloidosis? What is seen upon the staining being +ve?

A

Congo red stain will show apple-green bifringence in amyloidosis

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59
Q

How is amyloidosis treated?

A

Melphalan + prednisone

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60
Q

What are the classes of lupus nephritis?

A 
I - no evidence of disease
II - mesangial involvement
III - focal proliferative nephritis
IV - diffuse proliferative nephritis
V - membranous nephritis
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61
Q

How is each class of lupus nephritis treated?

A 
I - general SLE treatment
II - corticosteroids
III - high doses of corticosteroids
IV - corticosteroids + immunosuppressants
V - attend to general symptoms
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62
Q

What are the hallmarks of nephritic syndrome?

A

Haematuria
Oliguria
Azotemia
HTN

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63
Q

What is azotemia?

A

Increased blood urea nitrogen (BUN) and creatine

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64
Q

How do the symptoms of nephritic syndrome come about?

A 
Haematuria = due to destruction of glomerular capillaries
Oliguria = obstruction of glomerular capillary lumen decreases the GFR leading to oliguria (<400ml/day) and azotemia
HTN = secondary to the increased fluid retention by the kidney due to decreased GFR
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65
Q

Which type of nephritic syndrome is most likely to affect children?

A

Acute proliferative glomerulonephritis (poststreptococcal)

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66
Q

After what does acute proliferative glomerulonephritis usually occur?

A

2-3 weeks after either pharyngeal or skin infections with GABHS

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67
Q

What are the diagnostic signs of acute proliferative glomerulonephritis?

A

Serum chemistry - ANCA and anti-GBM Ab’s are -ve, C3 = usually low, ASO tigers or other streptococcal Abs are elevated

Urinalysis - smoky brown urine, RBC casts

Pathology - renal biopsy

Light microscopy - enlarged glomeruli

Electron microscopy - subepithelial deposits - ‘humps’

Immunofluorescence - ‘bumpy appearance’ of IgG and C3 deposits

68
Q

What is RPGN also known as?

A

Crescentic RPGN

69
Q

What are the 3 types of RPGN?

A

Type I - Goodpastures
Type II - Post-streptococcal, SLE, IgA nephropathy, Henoch-Schonlein purpura
Type III - Wegener’s

70
Q

Which types of RPGN are ANCA -ve?

A

Type I - Goodpastures

Type II - Post-streptococcal, SLE, IgA nephropathy, Henoch-Schonlein purpur

71
Q

Which types of RPGN are ANCA +ve?

A

Type III - Wegener’s

72
Q

What feature characterises Goodpastures syndrome?

A

Ab’s against proteins in the GBM

73
Q

What is seen upon immunofluorescence in Goodpastures?

A

Linear, ribbon-like deposits of IgG

74
Q

Aside from nephritic symptoms, what other symptoms are seen in Goodpastures?

A

Pulmonary haemorrhage - presenting with haemoptysis

75
Q

What is the treatment of Goodpastures?

A

Emergency plasmapheresis performed daily until anti-GBM titers become negative. Prednisone + either cyclophosphamide or azathioprine are also started to prevent the formation of new GBM Abs

76
Q

What is the most common form of glomerulopathy worldwide?

A

IgA nephropathy

77
Q

If IgA nephropathy is associated with external symptoms, what might the condition be?

A

Henoch-Schonlein purpura

78
Q

What are the external symptoms of Henoch-Schonlen Purpura?

A

Skin - puerperal lesions on the extensor surfaces
GI - abdominal pain/interstitial bleeding
Musculoskeletal - joint pain

79
Q

How does IgA nephropathy typically present?

A

Painless haematuria following infection

80
Q

What is seen on immunofluorescence in IgA nephropathy?

A

Granular IgA deposits

81
Q

How is Alport syndrome acquired?

A

X-linked recessive

82
Q

What is Alport syndrome caused by an error in?

A

Synthesis of the alpha-5 chain type IV collagen

83
Q

Aside from nephritic syndrome, what other symptoms do people with Alport’s have?

A

Nerve deafness
Lens dislocation
Cataracts

84
Q

What are the diagnostic findings in Alport’s?

A

Serum chemistry - ANCA and anti-GBM -ve, C3 = normal
Urinalysis - gross haematuria + mild proteinuria
Pathology - renal biopsy
Light microscopy - glomerular + mesangial proliferation. Foam cells - interstitial cells with accumulation of lipids
EM - splitting of the GBM

85
Q

A combination of what 3 symptoms indicates Wegener’s?

A
  1. Chronic sinusitis
  2. Haemoptysis
  3. Haematuria
86
Q

What are the 3 C’s of Wegener’s?

A

C-ANCA
Corticosteroids
Cyclophosphamide

87
Q

What are the different types of kidney stones?

A

Calcium oxalate/phosphate
Struvite
Uric acid
Cystine

88
Q

In what season do kidney stones most commonly form?

A

Summer - due to insufficient fluid intake

89
Q

How do calcium oxalate/phosphate stones appear on XR?

A

Radiopaque

90
Q

How do struvite stones appear on XR?

A

Radiopaque

91
Q

How do uric acid stones appear on XR?

A

Radiolucent

92
Q

How do cystine stones appear on XR?

A

Faintly opaque/ground glass

93
Q

Stones under what size are likely to pass ‘naturally’?

A

<9mm

94
Q

What is the characteristic pain of kidney stones?

A

Loin to groin

95
Q

What are the intervention options when stones are >9mm?

A

Extracorporeal shockwave lithotripsy (ESWL) or nephrolithotomy

96
Q

Which type of stone is most common?

A

Calcium oxalate and calcium phosphate

97
Q

What causes calcium stones to form?

A 
Hypercalcaemia
Cancer
Elevated PTH
Increased vitamin D
Idiopathic
98
Q

What is the composition of struvite stones?

A

Magnesium ammonium phosphate

99
Q

In which patients are struvite stones occuring?

A

Patients with persistently alkaline urine, e.g. from UTIs caused by urease +ve organisms - e.g. proteus vulgaris, klebsiella, pseudomonas and staphylococci

100
Q

When is it a stag horn kidney stone?

A

When the stone forms a cast of the renal pelvis and calyces system

101
Q

What conditions are associated with uric acid stones?

A 
Hyperuricaemia
Gout
Tumour lysis syndrome
Leukaemia
Myeloproliferative disease
102
Q

What are the most common UTI causing bacteria in women?

A

E coli

Staphylococcus saprophyticus

103
Q

What should be assessed for in children with recurrent UTIs?

A

Vesicoureteral reflux (VUR) + child abuse

104
Q

What are the antibiotic options for an uncomplicated UTI?

A

Trimethoprim
Sulfamethoxazole
Ciprofloxacin
Nitrofurantoin

105
Q

What may underly chronic pyelonephritis?

A

Obstructions - e.g. stones

VUR

106
Q

What systematic processes are associated with cortical necrosis (causing DCN)?

A

Diffuse or patchy infarction of the cortices of the kidney secondary to ischaemia

107
Q

What conditions are associated with diffuse cortical necrosis (DCN)?

A

Abruptio placentae
Eclampsia/pre-eclampsia
Septic shock
Haemolytic-uremic syndrome

108
Q

What conditions are associated with renal papillary necrosis?

A

DM
Acute pyelonephritis
Chronic analgesic use
Sickle cell disease

109
Q

In what condition may nephrocalcinosis be seen?

A

Renal papillary necrosis

110
Q

What are the characteristics of pre-renal, renal failure?

A

Urine osmolality - >500
Urine sodium - <10
Fractional excretion of sodium - <1%
Blood urea nitrogen/creatine ration - >20

111
Q

What are the characteristics of renal, renal failure?

A

Urine osmolality - <350
Urine sodium - >20
Fractional excretion of sodium - >2%
Blood urea nitrogen/creatine ration - <15

112
Q

What are the characteristics of post-renal, renal failure?

A

Urine osmolality - <350
Urine sodium - >40
Fractional excretion of sodium - >4%
Blood urea nitrogen/creatine ration - >15

113
Q

What are the 5 indications for dialysis?

A
  1. Severe uraemia
  2. Hyperkalaemia unresponsive to treatment
  3. Metabolic acidosis
  4. Refractory fluid overload
  5. Pericarditis
114
Q

What is the most common cause of acute renal failure (ARF)?

A

Acute tubular necrosis (ATN)

115
Q

What is normal GFR?

A

115-125ml/min

116
Q

What is the definition of polyuria?

A

Urine output >3L/24hrs

117
Q

What is the definition of oliguria?

A

Urine output <500ml/24hrs

118
Q

What is the definition of anuria?

A

Urine output <100ml/24hrs

119
Q

In ATN what is seen on microscopy?

A

Muddy brown casts

120
Q

What are the pre-renal causes of CKD?

A
  1. Renal artery stenosis

2. Embolism

121
Q

What are the renal causes of CKD?

A
  1. DM
  2. SLE
  3. HTN
  4. Amyloidosis
  5. Adult PCKD
  6. Renal cancer
  7. Chronic glomerulonephritis
122
Q

What are the post-renal causes of CKD?

A

Chronic urinary tract obstruction

123
Q

When is dialysis indicated in CKD?

A

GFR =20ml/min

124
Q

When is transplant indicated in CKD?

A

GFR <20ml/min

125
Q

What are the possible long term sequelae of renal failure?

A

Congestive heart failure (CHF)

Pulmonary edema

126
Q

What are the consequences of renal failure?

A
  1. Uremic syndrome
  2. Hyperkalemia
  3. Metabolic acidosis - when GFR<50%, there is impaired renal production of HCO3-, H+ cannot be excreted
  4. Sodium and water retention
  5. Anaemia
  6. Renal osteodystrophy
  7. HTN
  8. Fanconi syndrome
127
Q

What occurs during uremic syndrome?

A

Lethargy, seizures, myoclonus, asterixis, pericardial friction rub, hyperammonemia

128
Q

What are the consequences of hyperkalemia?

A

Peaked T waves on ECG, which can lead to ventricular fibrillation

129
Q

How does anaemia occur in renal failure?

A

There is failure of EPO production, causing decreased hematocrit

130
Q

What is autosomal dominant PCKD caused by?

A

Mutations in PKD1 or PKD2

131
Q

At what age does autosomal dominant PCKD present?

A

40s

132
Q

How should autosomal dominant PCKD be managed?

A

Anti-HTNs + diuretics + low-salt diet

133
Q

What condition is autosomal dominant PCKD associated with?

A

Saccular aneurism of the Circle of Willis = high incidence of subarachnoid haemorrhage

134
Q

What is autosomal recessive PCKD caused by?

A

PKHD1 mutation

135
Q

How do neonates with autosomal recessive PCKD present?

A

Enlarged kidneys

136
Q

How do we distinguish between autosomal recessive and autosomal dominant PCKD?

A

In autosomal recessive PCKD, parents will NOT have cysts

137
Q

What is the prognosis of autosomal recessive PCKD?

A

50% of neonates die
Of the 50% that survive, 1/3 will develop ESRD within 10 years
Other will develop liver cysts, and then congenital hepatic cirrhosis

138
Q

What causes Liddle syndrome?

A

Autosomal dominant gain of function mutation in the collecting duct ENaC channels

139
Q

How does Liddle syndrome present?

A

Children = tend to be asymptomatic

Adults = weakness, fatigue + palpitations
Hypokalemia
Metabolic alkalosis
HTN (severe)

140
Q

How is Liddle syndrome treated?

A

ENaC antagonists

141
Q

What causes Bartter syndrome?

A

Autosomal recessive mutations in any of the transporters of the thick ascending limb of Henle

142
Q

How does Bartter syndrome present?

A

Presentation in early life with growth + mental retardation

Hypochloremic metabolic alkalosis
Hypokalemia
HYPERcalciuria
Hypomagnesia (in many, but not all)
No HTN
143
Q

How is Bartter syndrome treated?

A

K+ supplements + spironolactone (to increase serum K+)

144
Q

What causes Gitelman syndrome?

A

Autosomal recessive defect in thiazide-sensitive Na+-Cl- cotransporter in the distal tubule

145
Q

How does Gitelman syndrome present?

A

Cramps + severe fatigue

Hypochloremic metabolic alkalosis
Hypokalemia
HYPOcalciuria
Hypomagnesia (in many, but not all)
No HTN
146
Q

How is Gitelman syndrome treated?

A

K+ supplements + spironolactone (to increase serum K+)

147
Q

What is the most common primary renal tumour?

A

Renal cell carcinoma

148
Q

What are the different types of renal cell carcinoma?

A
  1. Clear-cell carcinomas
  2. Papillary renal cell carcinomas
  3. Chromophobe renal carcinomas
149
Q

What are the RFs for renal cell carcinoma?

A
  1. Smoking
  2. Cadmium
  3. Petroleum
  4. Gasoline
  5. Asbestos
  6. Lead
150
Q

What is the classic RCC triad?

A
  1. Painless hematuria
  2. Palpable flank mass
  3. Flank pain
151
Q

From what does RCC arise?

A

Tubular epithelium

152
Q

What proportion of RCCs are clear cell carcinomas?

A

80%

153
Q

What proportion of RCCs are papillary renal cell carcinomas?

A

15%

154
Q

What proportion of RCCs are chromophore renal carcinomas?

A

<5%

155
Q

What genetics defects are associated with RCCs?

A

Clear cell carcinomas - VHL gene (tutor suppressor gene on chromosome 3)

Papillary renal cell carcinomas - MET gene (photo-oncogene on chromosome 7)

Chromophobe renal carcinomas - loss of entire chromosome (frequently 1, 2, 6, 10, 13, 17 and 21)

156
Q

What are the paraneoplastic syndromes associated with RCCs?

A

Hypercalcemia

Polycythemia (excess EPO production)

157
Q

How may RCCs spread?

A

Hemataogenous spread via renal vein and IVC to the bones or lungs

158
Q

How are RCCs treated?

A

Radical nephrectomy with removal of LNs

159
Q

How are RCCs with mets treated?

A

+ interleukin-2

160
Q

Where do transition cell carcinomas tend to occur?

A

Bladder

161
Q

In whom are bladder tumours associated?

A
  1. Smokers
  2. Cyclophosphamide
  3. Phenacetin
  4. Beta-naphthylamine
162
Q

How may bladder tumours be treated?

A

Transurethral resection and/or BCG injection

163
Q

What causes Wilms tumours?

A

Two-hit mechanism - mutation of one copy of WT1 on chromosome 11 in the gremlin, followed by an acquired mutation in the second copy of WT1

164
Q

What syndromes are associated with Wilms tumours?

A

Beckwith-Wiedemann
Denys-Drash syndrome
WAGR complex

165
Q

How is a Wilms tumour treated?

A

Nephrectomy with chemotherapy

Vincristine, actinomycin D and doxorubicin if lung mets are found

USMLE (14 decks)

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