Renal - Glomerular disease Flashcards

(59 cards)

1
Q

Nephritic or nephrotic syndrome is caused by

A

damage to the glomeruli leading to disruption of glomerular filtration barrier

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2
Q

Inflammatory renal syndrome that presents as:
- hematuria with acanthocytes
- azotemia (increased BUN and creatinine)
- RBC casts
- hypertension
- proteinuria < 3.5 g/day

A

nephritic syndrome (nephritis)

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3
Q

Non-inflammatory glomerulopathy that presents with:
- generalized edema
- hypoalbuminemia
- hyperlipidemia, fatty casts
- heavy proteinuria > 3.5 g/day

A

nephrotic syndrome (nephrosis)

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4
Q

Inflammatory response within the glomeruli leading to GBM disruption

A

nephritic syndrome

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5
Q

Damage to podocytes causing structural damage of the glomerular filtration barrier

A

nephrotic syndrome

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6
Q

What are some of the important complications of nephrotic syndromes?

A
  • hypercoagulability (renal vein thrombosis is common)
  • hypogammaglobulinemia with increased infection risk (especially pneumococcal infection)
  • iron deficiency anemia
  • vitamin D deficiency
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7
Q

Why are patients hyper coagulable and susceptible to DVT in nephrotic syndromes?

A

Decreased antithrombin III, protein C, protein S, increased fibrinogen synthesis and loss of fluid to the extravascular space = hypercoagulability

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8
Q

Irregular cell surface as a result of osmotic stress inside the tubules (“mickey mouse ears”)

A

Acanthocytes

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9
Q

Characteristics of GLOMERULAR hematuria

A
  1. acanthocytes
  2. RBC casts
  3. mild to moderate proteinuria
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10
Q

Characteristics of NON-GLOMERULAR hematuria

A
  1. bright red or pink urine
  2. normal RBC morphology
  3. no RBC casts
  4. blood clots
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11
Q

What are the common causes of nephritic syndrome? Name 4

A
  1. Post-streptococcal glomerulonephritis (PSGN)
  2. IgA nephropathy (Berger disease)
  3. Goodpasture disease (anti-GBM disease)
  4. Alport syndrome (hereditary nephritis)
  5. Pauci-immune diseases
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12
Q

What are the common causes of nephrotic syndrome? Name 3.

A
  1. Minimal change disease
  2. Focal segmental glomerulosclerosis
  3. Membranous nephropathy
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13
Q

A clinical syndrome that includes any type of glomerulonephropathy in which rapid deterioration of renal function occurs overs weeks to months leading to renal failure and ESRD.

A

RPGN

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14
Q

Rapid rise in BUN and Cr in a patient with nephritic sediment leading to decreased urine output with days to weeks. May have hemoptysis

A

RPGN

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15
Q

Nephritic sediment with rapidly rising serum creatinine levels is suspicious for?

A

RPGN

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16
Q

Crescent formation (moon-shaped) of plasma proteins (C3) and fibrin on light microscopy

A

RPGN

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17
Q

What are some common causes of both nephritic-nephrotic syndrome (mixed picture)? Name 2

A
  1. Membranoproliferative glomerulonephritis
  2. Diffuse proliferative glomerulonephritis
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18
Q

Glomerular injury characterized by splitting of the GBM (double-contour or tram-track appearance) on light microscopy caused by immune complex deposition or complement activation.

A

MPGN

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19
Q

Disease characterized by deposition of antibodies or complement factors in the mesangium and along capillary walls (usually associated with Hep. C infection).

A

MPGN

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20
Q

Subendothelial and mesangial IgG immune complex deposits with granular appearance on electron microscopy.

A

MPGN type 1

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21
Q

Intramembranous C3 deposits (dense deposit disease) on basement membrane on electron microscopy.

A

MPGN type 2

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22
Q

Disease characterized by increased cellularity in more than half of the glomeruli on light microscopy.

A

Diffuse proliferative glomerulonephritis

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23
Q

Most common and severe manifestation of lupus nephritis in SLE

A

Diffuse proliferative glomerulonephritis

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24
Q

X-linked dominant disease caused by mutation in the gene encoding type IV collagen

A

Alport syndrome (Hereditary nephritis)

25
Hematuria, sensorineural hearing loss and abnormalities of the eye (cataracts, retinopathy and lens dislocation)
Alport syndrome (Hereditary nephritis)
26
Diagnostic tests for the underlying cause of nephritic syndrome
- U/A microscopy - Serologic markers of system disease - Renal biopsy, if etiology is unclear
27
Name the three etiologies of glomerulonephritis
1. Anti-GBM Ab associated 2. Immune complex mediated 3. ANCA-associated (pauci-immune)
28
Condition where autoantibodies form against basement membranes in the kidneys and lungs against type IV collagen
Goodpasture disease (Anti-GBM disease)
29
Linear deposition of Ig along the glomerular and alveolar based membranes on immunofluorescence microscopy
Goodpasture disease (Anti-GBM disease)
30
What are the immune complex mediated nephritic diseases?
31
Which nephritic syndrome has a characteristic low serum C3 level?
PSGN
32
Dome-shaped, sub-epithelial immune complex deposits on electron microscopy
PSGN
33
Periorbital and peripheral edema, HTN, tea or cola colored urine in a child after Group A beta-hemolytic strep pharyngitis or skin infection.
PSGN
34
Lab findings in PSGN
- positive anti streptococcal antibodies (ASO, ADB) - decreased serum C3 levels due to consumption
35
Adult with asymptomatic microhematuria and intermittent gross hematuria after URI, GI infections or strenuous exercise
IgA nephropathy (Berger disease)
36
Lab findings in IgA nephropathy (Berger disease)
- increased serum IgA - normal C3 complement levels
37
Negative immunofluorescence microscopy pattern
ANCA-associated glomerulonephritis (pauci-immune glomerulonephritis)
38
What are the three ANCA-associated glomerulonephritis?
1. Granulomatosis with polyangiitis 2. Microscopic polyangiitis 3. Eosinophilis granulomatosis with polyangiitis
39
Granulomatous vasculitis, chronic sinusitis, nasal ulcers, perforation of the nasal septum, hemoptysis, pulmonary nodules, c-ANCA/PR3-ANCA
Granulomatosis with polyangiitis (Wegner granulomatosis)
40
Granulomatous vasculitis, asthma, allergic rhinitis, peripheral neuropathy, p-ANCA/MPO-ANCA, peripheral eosinophilia
Eosinophillic granulomatosis with polyangiitis (Churg-Strauss syndrome)
41
Necrotizing vasculitis, dyspnea, cough, hemoptysis, p-ANCA/MPO-ANCA
Microscopic polyangiitis
42
What are the etiologies of nephrotic syndrome?
1. Primary (idiopathic) forms 2. Secondary forms
43
What are the idiopathic forms of nephrotic syndrome?
1. Minimal change disease 2. Focal segmental glomeruloscerlosis 3. Mebranous nephropathy
44
Cytokine-mediated damage of podocytes
Minimal change disease
45
- No changes on light microscopy. - Effacement of the podocyte foot processes on electron microscopy
Minimal change disease
46
Which nephrotic disease is NOT associated with immune complex deposition?
Focal segmental glomerulosclerosis
47
What conditions are associated with focal glomuerlosclerosis?
- heroin use - HIV infection - Sickle cell disease - obesity - congenital malformations (Charcot-Marie-Tooth syndrome)
48
Sclerosis of the glomeruli causing damage and loss of podocytes
Focal segmental glomerulosclerosis
49
- Segmental sclerosis and hyalinosis on light microscopy. - Effacement of the podocyte foot processes on electron microscopy.
Focal segmental glomerulosclerosis
50
Antiphospholipase A2 receptor antibodies bind to PLA2R auto-antigen in the podocytes forming immune complexes that activate the complement system and injure the podocytes.
Membranous nephropathy
51
Secondary causes of membranous nephropathy
- Infection (HBV, HCV, malaria, syphilis) - Autoimmune (SLE) - Tumors - Medications (NSAIDs, penicillamine, gold)
52
- Diffuse thickened glomerular capillary loops and basement membrane on light microscopy. - Granular subepithelial deposits of IgG and C3 (dense deposits) giving a "spike and dome" appearance on electron microscopy. - Granular sub-epithelial deposits of immune complexes and complement on immunofluorescence microscopy.
Membranous nephropathy
53
What are the secondary forms of nephrotic disease?
1. Diabetic nephropathy 2. Amyloid nephropathy 3. Lupus nephritis
54
Thickening of the glomerular basement membrane and eosinophilic nodular glomerularsclerosis on light microscopy.
Diabetic nephropathy
55
What is the name for the eosinophillic nodular glomerulosclerosis?
Kimmelstiel-Wilson nodules
56
- Mesangial proliferation, sub-endothelial or sub-epithelial immune complex deposition, thickening of the capillary walls ("wire loops") on light microscopy. - Apple birefringence under polarized light on Congo red stain on light microscopy.
Amyloid nephropathy
57
Mesangial and/or subendothelial deposition of immune complexes causing expansion and thickening of mesangium, capillary walls and/or GBM. Presents with nephritic or nephrotic syndrome.
Lupus nephritis
58
Which nephrotic syndrome can be induced by heroin?
Focal segmental glomerulosclerosis
59
Which mixed nephritic-nephrotic syndrome can be induced by heroin?
Membranoproliferative glomerulonephritis - immune complex deposition