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Renal Week 2 > ICL 2.4: Nephrotic Glomerular Diseases > Flashcards

ICL 2.4: Nephrotic Glomerular Diseases Flashcards

1
Q

which conditions are primary nephrotic syndrome?

A
  1. lipoid nephrosis
  2. focal glomerulosclerosis
  3. membranous glomerulonephritis
  4. membranoproliferative glomerulonephritis
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2
Q

which conditions are secondary nephrotic syndromes?

A
  1. DM

2. amyloidosis

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3
Q

what is the clinical presentation of nephrotic syndromes?

A
  1. edema
  2. proteinuira
  3. hypoproteinemia
  4. hyperlipidemia
  5. 3+ g protein/24 hours in urine
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4
Q

what causes the proteinuria, hypoproteinemia and edema seen in nephrotic syndromes?

A
  1. proteinuria due to leaky capillary basement membrane
  2. hypoproteinemia due to loss of proteins in urine
  3. edema due to decreased oncotic pressure
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5
Q

what causes hyperlipidemia seen in nephrotic syndromes?

A

we aren’t really sure…

possibly due to decreased oncotic pressure or increased lipoprotein synthesis in the liver since the liver is trying to replace all the lost proteins

this translates into lipiduria

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6
Q

what is an oval fat body?

A

tubular cell filled up with lipid droplets

when the patient has lipids in the urine, some of the lipids appear in the tubules and it’s associated with nephrotic syndrome most often

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7
Q

what is proteinuria? specifically what are they different grades?

A

1+ = 250-500 mg/24 hrs

2+ = 500-1000 mg/24 hrs

3+ = 1000-2000 mg/24 hrs

4+ = 2000+ mg/24hrs

nephrotic syndrome is 3+ g/24 hrs….so it’s always 4+

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8
Q

a 2 year old boy with recurrent nephrotic syndrome is found to have selective proteinuria

diagnosis?

renal biopsy findings?

prognosis?

A

lipoid nephrosis aka minimal change disease

it’s recurrent so it comes and goes

selective proteinuria is specific to this disease! it means it’s only spilling albumin!!

great prognosis; respond to steroids

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9
Q

what is incidence of lipoid nephrosis? what disease is it associated with?

A

it’s a primary nephrotic syndrome also known as minimal change disease

associated with Hodgkins lymphoma*

80% of cases in primary syndrome are in children and only 30% are in adults

more common males in children but equal risk in adults; peak age is from 1-5 years old

20-30% follow a URTI, 9% follow immunization while 70% have no preceding illness

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10
Q

what is lipoid nephrosis?

A

it’s a primary nephrotic syndrome

it’s caused by T cell-derived factor that cause podocyte damage and effacement = mechanism IV

clinical findings:
1. edema

  1. proteinuria; highly selective, mostly albumin –> good response to steroids

serologic tests for autoimmune disease are negative

responds to steroids but recurrent…

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11
Q

what is the course and prognosis of lipoid nephrosis?

A

responds to steroids

however, it’s recurrent…

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12
Q

what is the morphology of lipoid nephrosis?

A

LM shows no changes

IF is negative

EM will show podocyte fusion/effacement = no more projections; it’s just flat

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13
Q

what are the characteristics of focal glomerulosclerosis?

A
  1. hematuria
  2. nonselective proteinuria = albumin and globulin
  3. no response to steroids

nephrotic syndrome with associated hematuria = not good news; most progress to renal failure

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14
Q

what is clinical presentation of focal glomerulosclerosis?

A

it’s a primary nephrotic syndrome + nephritic

clinical findings:
1. 60-80% nephrotic syndrome

  1. 30% azotemia
  2. 25% HTN
  3. recurrence in renal allograft**

more likely to have non-selective proteinuria, hematuria, progression to chronic renal failure, and poor response to corticosteroid therapy

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15
Q

what is the morphology of focal segmental glomerulosclerosis?

A

LM shows juxtamedullary glomeruli with focal sclerosis –> you can’t take the superficial cortex biopsy you need the deep cortex to get the juxtamedullary glomeruli!

you can see an area of collagenous sclerosis that runs across the middle of the glomerulus in a trichrome stain (it’ll be blue)

you can also sometimes see fat droplets! which isn’t surprising because they have hyperlipidemia

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16
Q

what are the 2 types of focal segmental glomerulosclerosis?

A

there are several types but here are the main 2

  1. C1q aka seronegative lupus

it is the most aggressive! bad prognosis and recurs in renal transplants as early as 2-3 weeks

  1. collapsing with HIV or IV drugs

glomerulus is shrunk in one part of Bowman’s space and there’s hyperplasia of podocytes

there is collapse of the entire glomerulus with podocyte hyperplasia associated with HIV or drugs –> poor prognosis

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17
Q

what is the prognosis of focal glomerulosclerosis?

A

downhill course that leads to renal failure….

there are persistent episodes of proteinuria, hematuria and nephrotic syndrome

recurrence in allograft** (C1Q type can reoccur as early as 2-3 weeks after transplant ):

doesn’t respond to steroids….

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18
Q

a 15 year old with a history of malignant lymphoma present with nephrotic syndrome

serologic tests, BUN and creatinine levels are all normal

urinalysis shows 4+ protein

differential diagnosis?

biopsy findings?

prognosis?

A

membranous glomerulonephritis

malignant lymphoma and now he has a nephrotic syndrome

no evidence of autoimmune disease

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19
Q

what are the predisposing factors for developing membranous glomerulonephritis?

A
  1. HepB (also in PAN but that’s nephritic and has high BUN, creatinine, HTN)
  2. tumor (GI, non-Hodgkins lymphomas)
  3. poison ivy (type IV hypersensitivity reaction too!)
  4. immune complex diseases like SLE
  5. infectious –> syphilis and malaria specifically
  6. renal vein thrombosis
  7. idiopathic
  8. drugs like penacillamine or GOLD therapy for RA
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20
Q

what is membranous glomerulonephritis?

A

a primary nephrotic syndrome caused by chronic immunocomplexes

chronic is key because it means the immune complexes are old and by the time they get deposited in the capillaries of the glomerulus they don’t activate much compliment so compliment levels are normal and deposits are small along the basement membrane (unlike post-strep nephritic syndrome which has huge immune complex deposits)

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21
Q

what is the morphology of membranous glomerulonephritis?`

A

LM will show thick basement membrane and capillary loops but no increased cellularity!

IF will show fine granular peripheral IgG and C3

EM shows 4 stages:
1. epimembranous deposits; flat! thick BM

  1. intramembranous deposits and spikes; deposits inside the membrane and on each side are deposits
  2. intramembranous deposits
  3. radiolucent areas where deposits used to be; deposits vanish
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22
Q

what is the course and prognosis of membranous glomerulonephritis?`

A

slow course

doesn’t usually recur in renal allograft

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23
Q

membranous glomerulonephritis associations?`

A

HepB, non-Hodgkins lymphoma

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24
Q

what are the 3 types of membranoproliferative glomerulonephritis?

A

type I = mesangocapillary

type II = dense deposit disease

type III = mesangial proliferative

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25
a 5 year old female with nephrotic syndrome, HTN, and hematuria for the last 6 months has a history of hearing problems. her uncle died at 22 with CKF. HB is 10, BUN 20, creatinine 3, 2+ blood and 4+ protein and RBC casts in urine diagnosis?
mesangiocapillary membranoproliferative nephritis (type I) HTN and hematuria = nephritic and nephrotic!! bad news.... hearing problems which are often associated with hearing problems not surprised she's anemic because she's losing blood in the urine! this is anemia of chronic disease!! so it's a normocytic anemia hearing problems and family history and CKD could be membranoproliferative or Alport syndrome! but alport if familial disease that has hearing, hematuria, HTN but no nephrotic syndrome!
26
what is the incidence of mesangiocapillary membranoproliferative nephritis?
type I membranoproliferative nephritis 5-20% of primary nephrotic syndrome cases female > male no preceding illnesses
27
what is the pathogenesis of mesangiocapillary membranoproliferative nephritis?
immune complexes that activate the complement pathway so when you look at serum complement it'll be decreased C3, C4 and C50 post-strep nephritis and SLE also have decreased complement
28
what is the morphology of mesangiocapillary membranoproliferative nephritis?
LM shows: 1. lobular pattern* --> looks like an orchid flower 2. membrane changes and cell changes 3. mesagnial matrix expansion 4. thick BM silver stain shows splitting of basement membrane IF shows C3 deposits in a peripheral lobular distribution EM shows mesangialization which is mesangial cells that have made their way into the basement membrane so they split the BM!
29
what is the course and prognosis of mesangiocapillary membranoproliferative nephritis?
slow course recurs in renal allograft
30
which conditions recur in renal allografts?
1. IgA nephrotpathy 2. focal segmental glomerulosclerosis 3. mesangiocapillary membranoproliferative nephritis
31
what is the incidence of dense deposit disease?
this is type II membranoproliferative nephritis it's only 0.5-2.5% of chronic glomerulonephritis cases so it's rare mean age is 16 years old but no sex predilection infectious disease is sometimes a preceding illness; mainly strep and pneumonia
32
what is the pathogenesis of dense deposit disease?**
alternate complement pathway activation (3rd mechanism) so you'll have low C3 BUT normal C4 and you'll see serum C3 nephritic factor** this is type II membranoproliferative nephritis
33
what is the clinical presentation of dense deposit disease?
this is type II membranoproliferative nephritis 1. proteinuria (100%) 2. nephrotic (80%) 3. HTN (60%) 4. hematuria (50%) = nephrotic and nephritic 5. decreased serum C3
34
what is the morphology of dense deposit disease?
LM shows the same as type I mesangiocapillary membranoproliferative nephritis: 1. lobular pattern* 2. membrane changes and cell changes 3. mesagnial matrix expansion 4. thick BM IF shows *discontinuous* linear deposits of C3 peripherally (Goodpasture show's continuous linear deposits) EM shows ribbon-like deposits in lamina densa
35
how is DM associated with renal disease?
the development of renal disease is associated with the duration of the DM, the severity and whether it's controlled or not DM is one of the most common causes of renal failure! the lesions in DM are nodular the two lesions associated with DM and renal failure: 1. diffuse glomerular scarring, no nephrotic syndrome 2. Kimmelstiel-Wilson disease; KW lesions associated with nephrotic syndrome
36
what is Kimmelstiel-Wilson disease?
microangiopathy everywhere in the whole body ischemic lesions everywhere! associated with DM aka diabetic nephropathy from uncontrolled DM
37
what is the morphology Kimmelstiel-Wilson disease?
LM shows: 1. nodular sclerosis (KW lesions)* 2. hyalinization of both afferent and efferent arterioles* 3. hyaline droplets that look like fibrin caps in the BM and capsular drops in the Bowman's capsule 4. vacuoles in tubular epithelium 5. diffuse sclerosis** 6. hyalinized vessels 7. fibrotic interstitium 8. thickening of tubular BM IF will show variable trapping of IgG and EM will shows microangiopathy GO LOOK IT'LL BE ON STEP
38
what are the types of amyloidosis? when do they present?
familial present in the 3rd decade secondary presents in the 4th decade primary presents in the 6th decade they all have nephrotic syndrome!
39
what is the morphology of amyloidosis?
LM will show nodular lesions!! apple-green birefringence with congo red stain IF will show positive light chains, mostly lambda light chain EM shows non-branching fibrin in BM, mesangium and vessel walls = diagnostic!
40
what is the course and prognosis of amyloidosis?
slow downhill course most end up with renal failure don't respond to steroids
41
minimal change
selective proteinuria recurrent respond to steroids
42
focal segmental sclerosis types
C1q SLE because it's aggressive collapsing because it's associated with HIV and IV drug use
43
membranous glomerulonephritis associations
HepB non-Hodgkins lymphomoa poison ivy
44
which conditions recur in transplant?
1. focal segmental sclerosis 2. IgA nephrropathy 3. membranoproliferative type I
45
which conditions have nodular lesions?
1. amyloidosis 2. diabetes 3. light chain (occurs with multiple myeloma)
46
which conditions have low C3?
1. post-strep 2. membranoproliferative type I and II 3. SLE 4. cryoglobulinemia (associated with HepC)
47
A 5 year old boy presents with an itchy vesicular rash and foamy urine for 6 days. Urinalysis reveals 4+ protein and oval fat bodies 7gm/24 hrs diagnosis?
membranous nephritis itchy rash is poison ivy! foamy urine = nephrotic syndrome itchy vesicular rash
48
a 40 year old presents with hemoptysis, hematuria and acute renal failure for 3 days diagnosis? tests? renal biopsy findings?
Goodpasture syndrome it's not Wegner because that's chronic! this is acute you would want to run anti-GBM test you'd find linear deposits on IF and LM would show necrosis and crescents
49
IV drug user with skin rash, hematuria, hypertension, eosinophilia diagnosis?
PAN IV drug user is HepB positive probably! renal morphology will show all stages of inflammation!
50
25 year old asymptomatic male notices blood in his urine diagnosis?
IgA nephropathy
51
10 year old with palpable red rash on buttocks, abdominal pain and arthralgia diagnosis? skin biopsy findings? serological tests?
Henoch-Schonlein purpura skin IF will shows IgA deposits in the skin and kidney serologic test will show increased IgA
52
A year old boy presents with hematuria and hypertension and BUN and creatinine are elevated he has RBCs casts in his urine what will you ask mom? diagnosis? tests? biopsy results?
did you have a sore throat?this is classic post-strep nephritic syndrome presentation! LM will show mesangial cell proliferation and necrosis IF will show rocks; lumpy bumpy EM will show humps
53
45 year old female presents with tight skin on her face and dysphagia. Her hands swell when she reaches in her freezer. She is also hypertensive. diagnosis? tests? biopsy findings?
scleroderma run anti-SCL70 test biopsy will show scarring everywhere including the kidney
54
A 58 year old female with cervical lymphadenopathy that is painful when she drinks alcohol presents with bilateral pedal edema and a serum cholesterol of 400. UA shows 4+ protein diagnosis? renal biopsy?
minimal change she has hodgkins lymphoma plus hyperlipidemia and nephrotic syndrome hodgkins = lymphadenopathy that's painful when you drink alcohol biopsy will show fusion of podocytes
55
A 45 year old surgeon with a history a hepatitis C presents with nephritic syndrome. diagnosis? biopsy findings?
mixed essential cryoglobulinemia
56
full house IF
SLE
57
low C3
SLE and post strep
58
IgG deposits in tubular basement membrane
SLE
59
dose not in involve the lungs
PAN
60
schistocytes + hematuria
1. HUS 2. DIC 3. TTP 4. malignant HTN because of fibrinoid necrosis of vessels so cells get shredded when going through
61
fibrin
crescents
62
HepB
PAN membranous
63
cANCA
granulomatous polyangitis
64
palpable rash
HS purpura
65
Antibodies to Alpha 3 chain of type IV collagen
Goodpasture
66
subepithelial humps
post-strep
67
Berger disease
IgA nephropathy
68
IgA depostis in the skin
HS purpura
69
low C3 and C4
1. post-strep 2. SLE 3. membranoproliferative type I
70
eosinophilia
PAN
71
linear IgG
Goodpasture
72
impetigo
strep infection of the skin with honey crust lesions!
73
humps
post strep
74
recurs in renal transplant
1. IgA nephropathy 2. focal segmental sclerosis 3. membranoprolifeartive type I
75
increased serum IgA
HS purpura
76
normal bleeding time
HS purpura
77
RBC casts
nephritic syndrome
78
sub endothelial deposits
SLE
79
oval fat body
nephrotic syndrome made of tubular cells with lipids in them
80
corticomedullary glomeruli
1. focal segmental sclerosis | 2. familiar medullary cystic kidney
81
HIV
focal segmental sclerosis; collapsing type!
82
Cytokines damage to podocytes (visceral epithelial cells)
minimal change
83
foamy ruine
nephrotic syndrome from proteinuria
84
spikes
membranous
85
apple green birefringence
amyloidosis
86
HepC
1. membranoproliferative | 2. cryoglobulinemia
87
HepB
membranous nephrtitis = nephrotic nephritic is PAN
88
pulmonary fibrosis
scleroderma
89
anti smith
SLE
90
selective proteinuria
minimal change
91
nephrotic + nephritic
1. focal segmental sclerosis | 2. membranoprolierative I and II
92
KW lesions
DM
93
mesangializaiton
membranoproliferative type I
94
ribbon like deposits in lamina dense
membranoproliferative type II
95
malignant lymphoma non-hodgkins
membranous

Renal Week 2 (10 decks)

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