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Glomerular diseases Flashcards

1
Q

Presence of Cast in urine indicates that

A

hematuria/pyuria is of glemerular or renal tubular origin

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2
Q

causes of hematuria with no casts (2)

A
  1. Bladder cancer

2. kidney stones

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3
Q

causes of pyouria with no cast (1)

A

acute cystitis

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4
Q

Cast in urine - types

A
  1. RBC casts
  2. WBC casts
  3. Fatty casts (oval fat bodies)
  4. Granular (“muddy brown”) casts
  5. Waxy casts
  6. Hyaline casts
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5
Q

RBC casts - seen in

A
  1. glomerulonephritis

2. malignant hypertension

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6
Q

WBC cast - seen in

A
  1. tubulointerstitial inflammation
  2. acute pyelonephritis
  3. trasnplant rejection
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7
Q

Fatty casts (oval fat bodies) - seen in

A

Nephrotic syndrome

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8
Q

Fatty casts (oval fat bodies) - associated with

A

Maltese-cross sign under polarized light

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9
Q

Fatty casts contain

A

lipid (eg. cholesterol)

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10
Q

Granular (“muddy brown”) casts - seen in

A

acute tubular necrosis

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11
Q

Waxy casts - seen in

A

ens-stage renal failure/chronic renal disease

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12
Q

Granular (“muddy brown”) casts vs waxy according to cellular or acellular

A

both acellular

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13
Q

Hyaline casts - seen in

A

nonspecific, can be normal finding, often in concentrated urine samples

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14
Q

Hyaline casts contain

A

protein (acellula)

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15
Q

casts with Maltese cross sign

A

Fatty casts (oval fat bodies)

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16
Q

muddy brown casts?

A

granular casts (acute tubular necrosis)

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17
Q

Nomenclature of glomerular disorders - focal (characteristcs and example)

A
  • less than 50% of glumeri are involved

- ex. focal segmental glomerulosclerosis

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18
Q

Nomenclature of glomerular disorders - diffuse (characteristcs and example)

A
  • more than 50% of glumeri are involved

- ex. diffuse proliferative glumerulonephritis

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19
Q

Nomenclature of glomerular disorders - proliferative (characteristcs and example)

A
  • hypercellular glomeruli

- membranoproliferative glomerulonephritis

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20
Q

Nomenclature of glomerular disorders - membranous (characteristcs and example)

A
  • thickening of glomerular basement membrane (GBM)

- membranous nephropathy

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21
Q

Nomenclature of glomerular disorders - primary glomerular disease (characteristcs and example)

A
  • a 1ry disease of the kidney specifically impacting the glomeruli
    2- eg. Minimal change disease
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22
Q

Nomenclature of glomerular disorders - secondary disease (characteristcs and example)

A
  • a systemic disease or disase of another organ system that also impacts the glomeruli
  • SLE, diabetic nephropathy
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23
Q

glomerulula diseases - groups

A
  1. nephritic syndrome
  2. nephrotic syndrome
  3. Nephritic-nephrotic syndrome
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24
Q

Nephritic syndrome vs nephrotic syndrome according to mechanism

A

Nephritic syndrome –> GBM disruption

Nephrotic syndrome –> podocyte disruption –> barier impaired

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25
Nephritic syndrome - symptoms and findings
1. Hypertension (salt secretion and periorbital edema) 2. increased BUN and creatinine 3. oliguria 4. hematuria (with RBCs in urine) 5. Proteinuria in Subnehrotic range (in severe cases may be in nephrotic range)
26
Nephritic syndrome - diseases?
1. acute poststreptococcal glomerulonephritis 2. Rapidly progressive glomerulonephritis 3. IgA nephropathy (Berger disease) 4. Alport syndrome 5. Membranoproliferative glomerulonephritis 6. Diffuse proliferative glomerulonephritis
27
Nephrotic syndrome - symptoms and findings
1. Massive proteinuria (>3.5g/day) with hypoalbuminemia (pitting edema in soft tissues) 2. hyperlipidemia (frothy urine with fatty casts) 3. edema 4. hypogammaglobulinemia (increased risk of infection) 5. loss of antithrombin III --> hypercoagulable state
28
Nephrotic syndrome - 1ry vs 2ry (according to mechanism)
1ry --> direct podocyte damage | 2ry --> podocyte damage from systemic process (diabetes)
29
Nephrotic syndrome - diseases (and if 1ry or 2ry)
1. focal segmental glomeulosclerosis (1ry or 2ry) 2. minimal change disease (lipoid nephrosis) (1ry or 2ry) 3. Membranous nephropathy (membranous glomerulonephritis) (1ry or 2ry) 4. Amyloidosis (2ry) 5. Diabetic glomerulonephropathy
30
Nephritic-nephrotic syndrome?
severe nephritic syndrome with profound GBM damage that may damages the glomerular filtration charge barrier (if damage to GBM is severe enougj to damage charger barrier) --> nephrotic-range proteinuria (>3.5g.day) and concominant features of nephrotic syndrome
31
Causes of Nephritic-nephrotic syndrome
can occur with any form of nephritic syndrome, but is most common seen with 1. diffuse proliferative glomerulonephritis 2. Membranoproliferative glomerulonephritis
32
Nephritic vs nephrotic vs Nephritic-nephrotic syndrome according to protein excreted per day
nephritic: Proteinuria in Subnehrotic range (less than in severe casees may be in nephrotic range nephrotic: Massive proteinuria (>3.5g/day) with hypoalbuminemia Nephritic-nephrotic syndrome: nephrotic-range proteinuria (>3.5g.day)
33
Nephritic syndrome - symptoms and findings
1. Hypertension (salt secretion and periorbital edema) 2. increased BUN (azotemia) and creatinine 3. oliguria 4. hematuria (with RBCs in urine) 5. Proteinuria in Subnehrotic range (less than in severe casees may be in nephrotic range
34
Acute poststreptococcal glomerulonephritis - age
Most frequently seen in children (may also occur in adults)
35
Acute poststreptococcal glomerulonephritis - mechanism
2-4 weeks after A streptococcal infection of of skin (impetigo) or pharynx--> immune complexes --> type III hypersensitivity reaction
36
Acute poststreptococcal glomerulonephritis - treatment
resolves spontaneously
37
Acute poststreptococcal glomerulonephritis - clinical presentation
1. peripheral and periorbital edema 2. cola-colored urine (hematuria) 3. hypertension 4. oliguria
38
Acute poststreptococcal glomerulonephritis - lab
1. + strep titers/serologies 2. low complement levels due to consumption 3. LM: glomeruli enlarged and hypecellular 4. IF: (starry sky) granular appearance (lumpy-bumpy) due to IgG, IgM and C3 deposotio along GBM and mesangium 5. EM: subepithelial immune complex (IC) humbs
39
causes of Rapidly progressive (crescentic) glomerulonephritis
several diseases processes may result in this pattern 1. Goodpasture - anti-GBM + anti alveolar BM 2. Granulomatosis with polyangiitis (Wegener) - PR3-ANCA/c-ANCA 3. Microscopic polyangitis, Churg strauus - MPO-ANCA/p-ANCA 4. poststreptococcal glomerulonephritis 5. Diffuse proliferative glomerulonephritis
40
Rapidly progressive (crescentic) glomerulonephritis - image
LM: crescent moon shape --> consist of fibrin and plasma proetins (eg. C3b) with glomerular parietal cells, monocytes, macrophages IF: a. Goodpasture --> linear b. wegener, Microscopic polyangitis --> negative IF (Pauci-immune --> no IG/C3 deposotion) c. if PSGN or Diffuse proliferative glomerulonephritis --> granular
41
Rapidly progressive (crescentic) glomerulonephritis - prognosis
poor --> rapidly deteriorating renal function (days to weeks)
42
Rapidly progressive (crescentic) glomerulonephritis - treatment
emergent plasmapheresis
43
Nephritic syndrome of SLE
Diffuse proliferative glomerulonephritis
44
Diffuse proliferative glomerulonephritis - mechanism
due to SLE or membranoproliferative glomerulonephritis
45
Diffuse proliferative glomerulonephritis - image
LM - wire looping of capillaries EM: subendothelial and sometimes intramembranous IgG-based ICs often with C3 deposotion IF: granular
46
IgA nephrpathy (Berger disasee) -mechanism/presentation
episodic gross hematuria that occurs concurrently with respiratory or GI tract infection (IgA is secreted by mucosal linings) --> IgA complex RENAL PATHOLOGY OF HENOCH-SCHONLEIN PURPURA
47
Henoch-Schonlein purpura - classic triad
1. Skin: palpable purpura on buttocks/legs 2. Arthralgias 3. GI: abdominal pain
48
IgA nephrpathy (Berger disasee) - image
LM - mesangial proliferation EM - mesangial IC depositis IF - IgA-based IC deposits in mesangium
49
Alport syndrome - mechanism
Mutation in type IV collagen --> thinning and splitting of glomerular basement membrane MC X-linked dominant
50
Alport syndrome - mode of inheritance
MC X-linked dominant
51
Alport syndrome - manifestation
1. Eye problem (eg. retinopathy, lens dislocation) 2. glomerulonephritis --> isolated hematuria 3. sensorineural deafness
52
Alport syndrome - image
"Basket-weave" appearance on EM
53
Mebranoproliferative glomerulonephritis (MPGN) - mechanism
``` type 1 --> 2ry to hepatitis B or C infection, May also be idiopathic type 2 (dense deposit disease) --> associated with C3 nephritic factor (autoantibody that stabilize C3 convertase) --> low serum C3 levels ```
54
Mebranoproliferative glomerulonephritis (MPGN) - prognosis
poor response to steroids --> progress to chronic renal failure
55
Mebranoproliferative glomerulonephritis (MPGN) type I - image
subendothelial immune complex (IC) deposits with granular IF tram track appearance on PAS stain and H&E stain due to GBM splitting caused by mesangial ingrowth
56
Mebranoproliferative glomerulonephritis (MPGN) type I vs type to according to location of deposits
type 1 --> subendothelial | type 2 --> intramembranous
57
Nephrotic syndrome - symptoms and findings
1. Massive proteinuria (>3.5g/day) with hypoalbuminemia (pitting edema in soft tissues) 2. hyperlipidemia (frothy urine with fatty casts) 3. edema 4. hypogammaglobulinemia (increased risk of infection) 5. loss of antithrombin III --> hypercoagulable state
58
MCC of nephrotic syndrome in children
Minimal change disease (lipoid nephrosis)
59
Minimal change disease (lipoid nephrosis) - mechanism
1. often 1ry (idiopathic) and my be triggered by recent infection, immunization, immune stimulus 2. Rarely, may be secondary to lymphoma (eg. cytokine mediated damage
60
primary minimal change disease can be triggered by
1. recent infection 2. immunization 3. immune stimulus
61
Minimal change disease (lipoid necrosis) - treatment
excellent response to corticosteroids
62
Minimal change disease (lipoid necrosis) - image
LM - normal glomeruli, lipid may be seen in PCT cells IF - normal EM - effacement of foot process
63
MCC of nephrotic syndrome in African and Hispanics
Focal segmental glomerulosclerosis
64
causes of focal segmental glomerulosclerosis
1. Can be 1ry (idiopathic) 2. 2ry to other conditions --> a. HIV infection b. Sickle cell anemia c. heroin abuse d. massive obesity e. interferon treatment f. chronic kidney disease due to congenital malformations
65
focal segmental glomerulosclerosis - course and treatment
may progress to chronic renal disease | 1ry disease has incosistent response to steroids
66
focal segmental glomerulosclerosis - image
LM - segmental sclerosis and hyalinosis IF - often (-), but may be + for nonspecific focal deposits of IgM, C3, C1... EF - effacement of ffot process similar to minimal change
67
MC cause of 1ry nephrotic syndrome in Caucasian adults
Membranous nephropathy (membranous glomerulonephritis)
68
causes of Membranous nephropathy (membranous glomerulonephritis)
1. primary (antibodies to phospholypase A2 receptor) | 2. 2ry to: a. drugs (eg. NSAIDs, penicillamine) b. infections (HBV, HCV) c. SLE d. solid tumors
69
causes of Membranous nephropathy (membranous glomerulonephritis) - course and treatment
may progress to chronic renal disease | 1ry disease has poor response to steroids
70
Membranous nephropathy (membranous glomerulonephritis) - image
LM - diffuse capillary and BM thickenning IF - granular (immune complex) EM - "spike and done" appearance with subepithelial deposits
71
the most commonly involved organ in amyloidosis
kidney
72
amyloidosis - common types
1. AL (primary) 2. AA (secondary) 3. dialysis-related 4. heritable 5. age related (senile) systemic 6. organ specific
73
nephrotic syndrome - amyloidosis - image
LM - congo red stain shows apple green birefringence under polairzed light due to amyloid deposition in the mesangium
74
MCC of end-stage renal disease in US
Diabetic glomerulonephropathy
75
Diabetic glomerulonephropathy - mechanism
Nonenzymatic glycosylation of GBM --> increased permeability, thickening Nonenzymatic glycosylation of efferent arterioles --> increased GFR --> mesangial expansion
76
Diabetic glomerulonephropathy - image
LM - mesangial expansion, GBM thickening, eosinphilic nodular glomerulosclerosis (Kimmelstiel-Willson)
77
eosinphilic nodular glomerulosclerosis
Kimmelstiel-Willson
78
HCV, HBV nephritis - types
nephrotic --> Membraous nephropathy (membranous glomerulonephritis) nephritic --> membranoproliferative glomerulonephritis
79
Lupus nephritis - types
nephritic - diffuse proliferative glomerulonephritis | nephrotic - membranous glomerulonephritis
80
heroin nephritis
focal segmental glomerulosclerosis
81
MC nephropathy worldwide
IgA nephropathy (Berger disease)
82
glomerular disease with subepithelial deposits
1. Acute poststreptococcal glomerulonephritis | 2. Membranous nephropathy (membranous glomerulonephritis)

Renal (15 decks)

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