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Renal Physiology > Nephrotic Syndrome II - Nichols > Flashcards

Nephrotic Syndrome II - Nichols Flashcards

1
Q

What are the two main categories of nephrotic syndromes?

A

Podocytopathies and Membranous Nephropathy

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2
Q

What are the two major podocytopathies?

A

Minimal Change Disease

Focal Segmental Glomerulosclerosis

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3
Q

Where is the primary affected site in Membranous Nephropathy?

A

Subepithelial layer of the basement membrane

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4
Q

What is a similar disorder to membranous nephropathy but is considered a nephritic syndrome?

A

Post-strep glomerulonephritis, which also has subepithelial deposits.

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5
Q

Who is primarily affected by minimal change disease?

A

the very young and the very old typically white and male

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6
Q

What percentage of nephrotic syndromes are caused by minimal change in children? in adults?

A

75% in children butonly 25% in adults

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7
Q

What can minimal change disease be secondary to?

A

Hodgkins disease, thymoma,t-cell leukemia, use of non-steroidal anti-inflammatory drugs of interferone-alpha use

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8
Q

Describe the proteinuria in minimal change disease?

A

highly selective, largely allowing only passage of albumin

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9
Q

What is the damage seen in minimal change disease?

A

effacement (shown by electron microscopy) and detachment of podocyte foot processes

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10
Q

How is steroid use for tx with minimal change disease?

A

great (90%) in children
adults have slow response

If not responsive to steroids, think FSGS

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11
Q

What is the general definition of a nephrotic syndrome?

A

Results from an increased permeability of the GBM, with effacement of podocytes and detachment of foot processes, allowing proteinuria

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12
Q

What are several key findings in nephrotic syndrome?

A 
massive proteinuria
edema
hypoalbunemia
hyperlipidemia and lipiduria
hypercoagulability (due to Anti-Thrombin III loss)
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13
Q

What is focal segment glomerulosclerosis characterized by?

A

deposition of basement membrane-like material associated with podocyte injury, not affecting all glomeruli and only segments of each affected glomerulus, but progressing to sclerosis

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14
Q

Who typically is seen with FSGS?

A

adults males and blacks

FSGS causes 64% of nephrotic syndromes in blacks

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15
Q

What can fsgs be secondary to?

A

hiv, parvovirus b19 infxn, injecting drug use (heroin), lithium, pamidronate, obesity, reflux nephropathy or sickle cell nephropathy

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16
Q

What are various mutations that can lead to fsgs?

A

podocin, alpha-actinin-4 and transient receptor potential cation channel 6

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17
Q

what mutation to african-americans have that lead to increased fsgs and arterionephrosclerosis?

A

a mutation in the apoL1 gene

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18
Q

Explain that mechanism of suPAR?

A

soluble urokinase-type pasminogen activator receptor is a circulating factor that binds to B3-integrin on podocytes. High levels of suPAR are predictive of recurence in transplanted kidneys and a reduction in suPAR levels in blood by plasmapheresis induces disease remission.

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19
Q

Explain the pathology of fsgs

A

increased mesangial matrix, occluded capillary lumens, and depos of proteinaceous material (hyalinosis) ((which goes on to become sclerotic))

20
Q

Initially, what glomeruli are affected by fsgs? what glomeruli become affected in later fsgs?

A

initially only juxtamedullary, later cortical

21
Q

What are the two primary spots that fsgs localizes in the glomerulus?

A

the vascular pole of the glom

the urinary pole of the glom

22
Q

When localization of fsgs is in the vascular pole, what is commonly seen in pts?

A

htn

23
Q

when localization of fsgs is to the urinary pole of the glom, what is commonly seen in pts?

A

heavier proteinuria, more common in whites

24
Q

Explain the significance of collapsing type of fsgs?

A

the glomerular tufts collapse, leading to heavier proteinuria and worst renal survival.
more common in black children

associated with drug toxicity or hiv infection

25
Q

what do people with fsgs usually present wiht?

A

proteinuria (nonselective compared to minimal change disease)
microscopic hematuria
renal insufficiency
hypertension

26
Q

how do you treat fsgs?

A 
corticosteroids (with poor response)
calcinuerin inh (cyclosporine and tacrolimus)
27
Q

What is the prognosis of fsgs?

A

half develop end-stage renal disease within 10 yrs of dx

28
Q

How can you differentiate between fsgs and minimal change disease?

A

FSGS suggestive features:

  • older age
  • hematuria
  • htn
  • non-selective proteinuria
  • poor response to steroids

Immunofluorescence will be neg. with minim. change. disease but can be positive in fsgs for igm

29
Q

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

A

membranous nephropathy (aka membranous glomerulopathy)

30
Q

What is membranous nephropathy?

A

chronic, slowly-progressive disease characterized by gGBM thickening, subepithelial immune complex deposits and effacement of foot processes

31
Q

when does one typically seen membranous nephropathy?

A

40s and up white males

32
Q

What are secondary causes of membranous nephropathy?

A 
AI diseases ie sle
sickle cell
carcinoma
complement deficiencys 
hep b
syphilis
malaria
33
Q

Describe the general (next question is specifics) mechanism of membranous nephropathy?

A

autoimmune complexes formed from binding of filtered autoantibody to incrinsic, locally generated antigen

34
Q

What is NEP?

A

neutral endopeptidase is a target antigen in congenitla membranous nephropathy

35
Q

What do the majority of membranous nephropathy pts have antibodies against?

A

an epitode in phopholipase a2 receptor (PLA2R) which is co-localized with IgG4 in glomeruli. There is increased deposition of C3 along the GBM, leading to complement activation and podocyte injury

36
Q

When looking at membranous nephropathy through light microscopy, what is the only you cans ee?

A

increased thickening of the gbm

37
Q

What does electron microscopy show in membranous nephropathy?

A

thickening due in part to abundant subepithelial deposits, forming a “spike and dome” appearance. also effacement of overlying foot processes.

38
Q

What does immunofluorescence show in membranous nephropathy?

A

granular subepithelial deposits of IgG and C3 along gbm, no inflammation because complement is activated where there are not circulating inflammatory cells.

39
Q

What are signs and symptoms of membranous nephropathy

A

variable proteinuria, microscopic hematuria and non-selective proteinuria,

40
Q

how do you treat membranous nephropathy?

A

ACEi or Angiotensin receptor blockade to lower bp

steroids for progressive cases

41
Q

what are risk factors for progression of membranous nephropathy?

A

male, severe proteinura (>10g/day), htn, azotemia, tubulointerstitial fibrosis and glomerulosclerosis.

42
Q

What is the prognosis for membranous nephropathy?

A

spontaneous resolution in 40% progressive failure in 30% and renal dysfunction in 30%

43
Q

Is post-infectious glomerulonephritis related to nephrotic syndrome

A

nope

44
Q

What is post-infxs glomerulonephritis?

A

acute immune comlex glom.nephrit. with proliferation of glomerular cells and infiltration by neutrophils.

45
Q

What is the typical presentation of post-infxs glomerulonephritis?

A

proteinuria, edema azotemia, htn, gross hematuria (smoky or tea colored), oliguria, low c3 but normal c4 (alternative activation).

46
Q

What does immunofluorescence reveal in post-infxs glomerulonephritis?

A

diffuse granular deposits of IgG and C3 in capillary walls and masngium. Dome-shaped subepithelial electron-dense deposits referred to as humps

47
Q

How do you treat post-infxs glomerulonephritis?

A

aggressive mgmt of htn, careful loop diuretics

have good prognosis

Renal Physiology (26 decks)

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