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Path Exam 3 > Renal: Glomerular Diseases > Flashcards

Renal: Glomerular Diseases Flashcards

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1
Q

nephrotic syndrome is characterized by:

A

heavy proteinuria (>3.5 mg/day)

  • hypoalbuminemia (bc of heavy proteinuria)
  • edema
  • hyperlipidemia & lipiduria
  • normal complement levels
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2
Q

why is there hypercoagulability in nephrotic syndrome?

A

loss of anti-thrombin III

ATIII breaks up thrombin and coagulation factors so that you cannot make thrombin; therefore loss of ATIII = increased coagulation risk

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3
Q

list the renal diseases associated with nephrotic syndrome based on immunoglobulin deposition

A
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4
Q

____ is the most common cause of nephrotic syndrome in children

A

minimal change disease is the most common cause of nephrotic syndrome in children

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5
Q

describe the EM image seen in the condition in the image

A

minimal change disease

PAS-stained image = normal

EM: arrow = effacement of foot processes and absence of deposits

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6
Q

the condition seen in the image is associated with ____ (another condition)

A

the condition seen in the image is associated with Hodgkin’s lymphoma

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7
Q

describe what is seen on physical exam in the condition seen in the image

A

normal BP, edema (periorbital and pedal)

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8
Q

>90% of children have complete remission proteinuria in the condition seen in the image within 8 weeks of ____ therapy

A

>90% of children have complete remission proteinuria in the condition seen in the image within 8 weeks of steroid therapy

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9
Q

in the condition seen in the, there is autoimmune destruction of ____ cells, facilitated by ____ cells

A

in the condition seen in the, there is autoimmune destruction of epithelial cells (podocytes), facilitated by T cells

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10
Q

in the condition seen in the image, there is diffuse effacement of ____

A

in the condition seen in the image, there is diffuse effacement of foot processes of podocytes

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11
Q

the injury in the condition seen in the image results in increased ____ and subsequent massive ____

A

the injury in the condition seen in the image results in increased permeability and subsequent massive proteinuria

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12
Q

what condition shows the following results:

A

minimal change disease

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13
Q

the condition seen in the image can be secondary to:

A
  • secondary to:
    • HIV
    • morbid obesity
    • chronic reflux nephropathy
    • heroin use
    • malignancies (lymphoma)
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14
Q

the prognosis of the condition in the image depends on ____

A

the prognosis of the condition in the image depends on the degree of proteinuria

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15
Q

if a patient has HIV or does heroin and then develops nephrotic syndrome, which disease is most likely?

A

FSGS

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16
Q

describe the pathogenesis of the condition seen in the image

A

decreased renal mass

↓

compensatory hyperfiltration

↓​

intraglomerular HTN and hyperfiltration injury

↓

non-selective proteinuria

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17
Q

which condition shows the following lab results:

LM: segmental hyalinosis of some glomeruli

IF: negative (or non-specific IgM & C3)

EM: patchy fusion of the foot processes & effacement

A

FSGS

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18
Q

in minimal change disease, there is ___ fusion of the foot processes and effacement

whereas

in FSGS, there is ___ fusion of the foot processes and effacement

A

in minimal change disease, there is diffuse fusion of the foot processes and effacement

whereas

in FSGS, there is patchy fusion of the foot processes and effacement

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19
Q

describe presentation of the condition seen in the image

A

present with nephrotic syndrome

microscopic hematuria & HTN

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20
Q

the condition seen in the image does NOT respond to ___ and ultimately progresses to ____

A

the condition seen in the image does NOT respond to steroids and ultimately progresses to end-stage renal failure (ESRF)

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21
Q

describe what is seen in the EM image

A

membranous glomerulopathy

EM showing electron-dense deposits (arrow) along the epithelial side of the basement membrane (B); note the effacement of foot processes overlying deposits

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22
Q

the condition seen in the image is caused by ___ deposition in the ____ zone

A

the condition seen in the image is caused by immune-complex deposition in the subepithelial zone

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23
Q

describe the pathogenesis of the condition seen in the image

A

membranous glomerulopathy

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24
Q

in the 2 conditions with the word “membranous”, they are characterized by thickening of ____ due to ____ deposition

A

membranous nephropathy

membranoproliferative glomerulonephritis

in the 2 conditions with the word “membranous”, they are characterized by thickening of the membrane due to immune-complex deposition

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25
if a patient has hepatitis B, which nephrotic syndrome are they at risk of developing?
**membranous nephropathy**
26
if a patient has SLE, which nephrotic syndrome are they at risk of developing?
**membranous nephropathy**
27
which condition shows the following lab results:
28
on EM of the condition seen in the image, there are subepithelial immune-complex deposits w/ alternating ___ and \_\_\_\_
on EM of the condition seen in the image, there are subepithelial immune-complex deposits w/ alternating **spikes** and **domes**
29
on immunofluorescence of the condition seen in the image, there are subepithelial deposits of ___ and \_\_\_\_
on immunofluorescence of the condition seen in the image, there are subepithelial deposits of **IgG** and **C3**
30
describe complications of the condition seen in the image
**infections, hypercoagulability (ischemic heart disease), renal failure**
31
what would indicate a poor prognosis of the condition seen in the image
**males, \>50 yrs old, \>10 gm of proteinuria**
32
what is the FIRST change seen in diabetic nephropathy?
high serum glucose leads to **non-enzymatic glycosylation (NEG) of vascular basement membranes**, resulting in **hyaline arteriosclerosis** arteriosclerosis preferentially in efferent arterioles → increased backpressure in glomerulus →
33
in diabetic nephropathy, initially hyperglycemia leads to \_\_\_\_
in diabetic nephropathy, initially hyperglycemia leads to **hyperfiltration**
34
the earliest lesions seen in diabetic nephropathy is expansion of ____ and thickening of \_\_\_\_
the earliest lesions seen in diabetic nephropathy is expansion of **mesangial matrix** and thickening of **GBM**
35
later lesions seen in diabetic nephropathy is diffuse global ____ with: diffuse increase in \_\_\_\_ and diffuse thickening of \_\_\_\_
later lesions seen in diabetic nephropathy is diffuse global **glomerulosclerosis** with: diffuse increase in **mesangial matrix** and diffuse thickening of **GBM**
36
in diabetic nephropathy, ____ nodules can be seen which contain ___ & \_\_\_\_
in diabetic nephropathy, **Kimmelstiel-Wilson** nodules can be seen which contain **lipids** & **fibrin**
37
amyloid fibrils are composed of various normal soluble proteins that have undergone a misfolding event resulting in formation of abnormal protein with ____ structures
amyloid fibrils are composed of various normal soluble proteins that have undergone a misfolding event resulting in formation of abnormal protein with **B-pleated sheet** structures
38
in amyloidosis, organ damage & dysfunction is due to ____ and replacement of normal organ architecture with consequent loss of cellularity
in amyloidosis, organ damage & dysfunction is due to **infiltration by amyloid fibrils** and replacement of normal organ architecture with consequent loss of cellularity
39
which condition shows the following lab results:
**amyloidosis**
40
describe what condition is seen in the images
41
describe what is seen in the urine in nephritic syndrome
* RBCs and/or RBC casts * granular casts * variable proteinuria * possibly WBC
42
list the normal complement level nephritic disorders (4)
* IgA nephropathy/Henoch-Schonlein purpura * Alport's syndrome (hereditary nephritis) * SLE (class I, II, V) * benign hematuria
43
list the low complement level nephritic disorders (4)
* PSGN * membranoproliferative glomerulonephritis * SLE (class III, IV) * bacterial endocarditis/infected ventriculoatrial shunt * cryoglobulinemia
44
\_\_\_\_ is the most common cause of nephritic syndrome
**mesangioproliferative glomerulonephritis (IgA nephropathy/Berger's disease)** is the most common cause of nephritic syndrome
45
list the conditions associated with IgA nephropathy
* hepatic cirrhosis * gluten enteropathy * HIV infection * minimal change disease * others: membranous, Wegener's, ankylosing spondylitis, small cell ca.
46
the condition seen in the image in children is part of \_\_\_\_
the condition seen in the image in children is part of **Henoch-Schonlein Purpura Nephritis** * kidneys = IgA nephropathy * skin = non-blanching purpuric rash * GI = mesenteric vasculitis → red infarct * joints = arthralgia
47
the condition seen in the image presents in adults 1-3 days after \_\_\_\_
the condition seen in the image presents in adults 1-3 days after **respiratory (or GI) infection** **resp. + GI are lined by mucous membranes and therefore secrete IgA**
48
the condition seen in the image is associated with \_\_\_\_
the condition seen in the image is associated with **liver cirrhosis, Celiac's disease, HIV and minimal change disease**
49
describe the presentation of the condition seen in the image
episodic gross hematuria w/ a background of persistent hematuria, oliguria
50
describe what is seen on microscopy of the urine in the condition seen in the image
**RBC casts**
51
blood investigations in the condition seen in the image would show normal ____ levels, elevated ____ levels and \_\_\_\_\_
blood investigations in the condition seen in the image would show normal **complement** levels, elevated **IgA** levels and **azotemia**
52
describe what is seen on LM in the condition seen in the image
segmental areas of increased mesangial matrix & hypercellularity
53
describe what is seen on IF in the condition seen in the image
coarse granular (lumpy-bumpy) with mesangial & subendothelial IgA and C3
54
describe what is seen on EM in the condition seen in the image
**mesangial and subendothelial IgA & C3**
55
describe the complication of the condition seen in the image
can progress to **Type II RPGN**
56
the condition seen in the image can occur 10 days after ____ or 3 weeks after \_\_\_\_
the condition seen in the image can occur 10 days after **pharyngitis** or 3 weeks after **impetigo**
57
diagnosis of the condition seen in the image is with elevated titers of ___ or ____ in association with low \_\_\_\_
diagnosis of the condition seen in the image is with elevated titers of **anti-streptolysin O Ab** or **anti-DNAse B** in association with low **complement levels**
58
in the condition seen in the image, initially, there are ____ deposits which then become ____ which is responsible for \_\_\_
in the condition seen in the image, initially, there are **subendothelial deposits** which then become **subepithelial humps** which is responsible for **epithelial cell damage & proteinuria**
59
on LM of the condition seen in the image, what is seen?
* hypercellular glomeruli: neutrophils + monocytes * proliferation of: mesangial, endothelial, epithelial cells * process is diffuse (entire lobules of all glomeruli) * closure of capillary loops due to proliferation & swelling of endothelial cells & leukocytes infiltration
60
in the condition seen in the image, there is closure of \_\_\_\_
in the condition seen in the image, there is **closure** **of capillary loops due to proliferation & swelling of endothelial cells & leukocytes infiltration**
61
describe what is seen on IF in the condition seen in the image
coarse, granular, (lumpy bumpy) deposits of IgG & C3 in mesangium & along capillary walls
62
in the EM of the condition seen in the image, there are \_\_\_
in the EM of the condition seen in the image, there are **electron-dense deposits in subepithelial space; humps**
63
a complication of the condition in the image is that it can progress to \_\_\_\_
a complication of the condition in the image is that it can progress to **type II RPGN**
64
the outcome of most cases of the condition seen in the image is \_\_\_\_
the outcome of most cases of the condition seen in the image is **complete resolution**
65
on LM of the condition seen in the image, there is ___ expansion and \_\_\_\_
on LM of the condition seen in the image, there is **mesangial expansion** and **hypercellularity**
66
what is a characteristic finding in the condition seen in the image?
duplication of the GBM = "tram-track" appearance
67
describe what is seen on EM in type I of the condition seen in the image
type I = **subendothelial deposits (C3**, +/- IgG, C1q, C4)
68
describe what is seen on EM in type II of the condition seen in the image
deposition of dense material **along GBM** (**complement deposition**, no immune complexes)
69
describe what is seen on EM in type III of the condition seen in the image
subendothelial, mesangial, subepithelial deposits (C3 +/- IgG)
70
describe what is seen in the image
MPGN: type I note the discrete, electron-dense deposits (arrows) incorporated into the glomerular capillary wall between duplicated (split) BMs (double arrows)
71
describe what is seen in the image
MPGN: type II dense-deposit disease (type II MPGN) there are dense homogenous deposits within the BM
72
in type I of the condition seen in the image, there is ___ activation via the ___ pathway
in type I of the condition seen in the image, there is **complement** activation via the **classical** pathway
73
in type II of the condition seen in the image, there is activation of the ___ pathway which leads to depressed ____ levels
in type II of the condition seen in the image, there is activation of the **alternate** pathway which leads to **depressed C3 levels**
74
list the 4 ways the condition seen in the image can present
1. hematuria or proteinuria discovered on urinalysis 2. acute nephritic syndrome with hematuria, HTN and edema 3. recurrent episodes of gross hematuria 4. insidious onset of edema and nephrotic syndrome
75
on LM of the condition seen in the image, there is proliferative GN with prominent ____ formation +/- segmental \_\_\_\_
on LM of the condition seen in the image, there is proliferative GN with prominent **crescent** formation +/- **segmental** **necrosis**
76
describe type I of the condition seen in the image; what kind of staining is seen on IF?
**linear staining** on IF **Anti-GBM** disease, such as **Goodpasture's syndrome**
77
describe type II of the condition seen in the image; what kind of staining is seen on IF?
**granular staining** on IF immune-complex disease; found in **SLE, post-infectious, IgA, Henoch-Schonlein Purpura**
78
describe type III of the condition seen in the image; what kind of staining is seen on IF?
**no staining** on IF; **pauci****-immune GN** Wegener's (c-ANCA) microscopic polyangitis (p-ANCA) Churg-Strauss (p-ANCA)
79
what are the crescents in the image composed of?
crescents = fibrin + macrophages + proliferating parietal cells
80
the best prognosis of the condition seen in the image is with patients with \_\_\_
the best prognosis of the condition seen in the image is with patients with **treatable underlying disorders (such as SLE) or one that spontaneously remits (such as post-strep)**
81
describe what is seen in the image
type I RPGN; anti-BM disease, such as **Goodpasture's**
82
on IF examination of lupus nephritis, it is positive for ____ which is also called a \_\_\_\_
on IF examination of lupus nephritis, it is positive for **IgG, IgA, C3, IgM** which is also called a **full house**
83
describe class I of lupus nephritis
**minimal mesangial lupus nephritis** * LM = normal * IF & EM = mesangial immune deposits
84
describe class II lupus nephritis
**mesangial proliferative lupus nephritis** * mesangial immune deposits resulting in expansion & hypercellularity * clinical: mild disease, microscopic hematuria, proteinuria, nephrotic syndrome
85
describe class III lupus nephritis
**focal segmental proliferative lupus nephritis** * \<50% glomeruli affected on LM * **subendothelial & mesangial IC deposits;** complement activation, influx of inflam. cells * clinical: hematuria, nephrotic syndrome, HTN, renal failure
86
describe class IV of lupus nephritis
**diffuse proliferative lupus nephritis** * \>50% glomeruli affected on LM * **marked deposition of IC in subendothelial and mesangium** * **crescents and necrotizing lesions** * clinical: **most common and severe form**; hematuria, proteinuria, nephrotic syndrome, renal failure, low complements, high anti-DNA levels
87
describe class V lupus nephritis
**membranous lupus nephritis** * **subepithelial** immune complex deposits * diffuse **thickening of GBM** * clinical: same as idiopathic membranous; nephrotic syndrome, normal C3/C4, negative anti-DNA * IC deposits in blood vessels
88
describe class VI lupus nephritis
**advanced sclerosing lupus nephritis** * **global sclerosis of \>90% of glomeruli** * advanced interstitial fibrosis and tubular atrophy * represents healing of prior inflammatory injury, advanced stages of chronic class III, IV, V lupus nephritis
89
disease activity of lupus nephritis can be monitored by serial measurements of ____ (4 things)
disease activity of lupus nephritis can be monitored by serial measurements of: * complements * anti-dsDNA Ab * ESR * CRP
90
the condition seen in the image is also called \_\_\_\_\_
the condition seen in the image is also called **inherited nephritic syndrome**
91
the condition seen in the image has a defect in \_\_\_\_
the condition seen in the image has a defect in **α-5 subunit of type IV collagen**
92
the genes associated with the condition seen in the image are....
**COL4A3, COL4A4, COL4A5** **COL4 = collagen 4**
93
describe the presentation of the condition seen in the image
* hematuria, periorbital edema, oligura * **cataracts** * **sensorineural deafness**
94
describe the blood levels of the condition seen in the image
NORMAL complements levels, azotemia (increased BUN + increased creatinine without uremic symptoms)
95
describe what is seen on LM in the condition in the image
initially normal → hyperfiltration → FSGS
96
describe what is seen on EM in the condition in the image
**basket-weave appearance** due to alternating **thickening and thinning of lamina** **densa** **in BM**
97
list complications of the condition seen in the image
* **can progress to nephrotic FSGS** → anasarca & 3.5g of protein & fatty casts * **deafness, blindness**

Path Exam 3 (9 decks)

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