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140 Cards in this Set

  • Front
  • Back
Granular IF
Type 3 rxn.
Linear, smooth IF
Anti-GBM Ab
Minimal change disease LM & IF
normal glomeruli
lipid droplet in proximal tubular epithelium

no deposit
Nephrotic syndrome in children 2-6 yrs.
minimal change disease
Test for minimal change disease
steroids
Minimal change disease EM
effacement of epithelial (podocytes) foot processes
Minimal change disease C/F & lab
proteinuria of albumin 4+
slightly cloudy urine
yellow urine
oval fat body

Lab:
high serum cholesterol
normal complement levels
Nephrotic syndrome in adults
& etiology
membranous nephropathy
Etiology:
idiopathic or genetic
Drug (penicillamine), renal transplantation, Heymann nephritis
SLE, DM, amyloidosis
Adenocarcinoma of lung and colon
Morphology of MGN
LM: H& E Stain--> diffuse thickening of capillary wall
Silver stain--> spikes
IF: granular deposits of IgG and C3
EM: Sub epithelial deposit along BM
Urinalysis and Lab of MGN
urine:
protein 4+
WBC/hpf = <2/hpf
Lab:
low complements
MGN C/F
HTN
hematuria
may progress to renal failure
Types of Acute glomerulonephritis
acute post streptococcal glomerulonephritis

non-streptococcal causes
Acute nephritic syndrome in a child
Acute post-streptococcal glomerulonephritis
ASO titer: high
Age: 2-4 years
Acute post-streptococcal glomerulonephritis

Etiology-->
Path-->
Hypersensitivity rxn-->
beta hemolytic group A strep infection of THROAT & SKIN

complement mediated tissue damage w/ the help of PMN

Type 3 (circulating IC)
Non-streptococcal glomerulonephritis

Causes-->
D/D-->
Test-->
pneumococcal pneumonia
Hep B, C
SLE, PAN
Malaria

Acute post-strep

ASO titre
Morpho Acute post-strep glomerulonephritis
LM: hypercellular, large glomeruli contain neutrophils
Tubules: RBC cast
IF: granular deposit of IgG, IgM, C3 in all glomerulous
EM: subepithelial humps
Urinalysis, C/F
Acute post-strep glomerulonephritis
Urine: smoky b/c of acid hemetin (due to hematuria), dysmorphic RBC (b/c RBC squeezing through glomerulus)

Serum complement low (C3 & C5). Complements used up by IC

ARF = acute nephritic syndrome

C/F: abrupt onset, malaise, slight fever, nausea
Past hx. of post strep glomerulonephritis?
pharyngitis, dermatitis, impetigo (honey-crusted lesions)
Complication of post strep glomerulonephritis
may progress to crescentric GN
may progress to chronic GN
rapidly occurring acute nephritic syndrome?

LM-->
C/F-->
CrGN

all types show glomerular crescent

hematuria and in 2-3 days ARF + uremia
Composition of crescent in CrGN
epithelial cells (podocytes)
fibrin
monocytes
Type 1 CrGN

C/F
D/D
1. AKA Anti-GBM DISEASE
2. AKA: Good pasture syndrome.
- Presence of Anti GBM antibody in serum: this react with alveolar capillary → pulmonary alveolar hemorrhage.
- Present as hematuria and hemoptysis
3. IF: Linear and smooth deposit of IgG, and C3 on GBM.

C/F--> decrease C3
D/D--> wegener (+c-anca)
Ab to which antigens in CrGN
lung and GBM
Which type of hypersensitivity rxn is type 1 CrGN?
Type 2
Type 2 CrGN
Etiology: mainly SLE
IF: Granular deposit
Clinical : progress to renal failure.
Serum: ANA present

Type 3 hypersensitivity
C/F of SLE
butterfly rash over bridge of nose
photosensitivity
anemia
anti-smith Ab, dsDNA Ab
Type 3 CrGN
Aka: Pauci-immune (no immune reaction)
Disease association:
Wagner Granulomatosis, polyarteritis Nodosa
Serum:
Normal complements
Positive ANCA (c or p)
LM: glomerular crescent
IF and EM: no deposit
C/F PAN
malaise, HTN, fever, purpura in skin (ulcer)
C/F Type 3 CrGN
acute onset of hematuria, hemoptysis,
RPGN--> ARF, uremia
Prognosis of crescentric glomerulonephritis
depends upon the number of crescent in kidney: so biopsy is indicated
Causes of IgA elevation
longstanding-->
lung infection
GI disease (celiac sprue)
IgA elevation leads to deposits in...
kidney
blood vessels --> HSP
dermis--> dermetitis herpetiformis
recurrent hematuria in children and young adults
Berger's (IgA nephropathy)
Berger's
Syndrome: recurrent hematuria
This hematuria occur 1-2 days after upper respiratory tract infection.
May progress to Chronic renal Failure(25%-50%).
IgA deposit in skin- dermatitis herpetiformis.
Asso: Gluten enteropathy.

LM: focal proliferation of mesangial cells
IF: IgA is deposited mainly in mesangium.
Henoch-Schonlein Purpura
It is associated with
1.Skin purpuric Rash
2.Abdominal Pain
3.Arthritis
4.And Kidney change

Q: What is the similarity?
A: Both are caused by IgA deposition in Mesangium and skin deposit of IgA.
nephrotic syndrome in adults
MPGN type 1
MPGN Type 1
Etiology: Hepatitis B and C, HIV, SLE, chronic liver diseases, chronic Bacterial Infection.
LM:
H&E: hyper cellular glumeruli ( but no PMNs) and thick GBM.
Silver stain : Tram track
IF: Granular deposit.

Serum: low complements ( particularly C3)
Tram-tracking
double basement membranes b/c of basement membrane splitting

MPGN 1
Syndrome: Hematuria/ chronic renal failure-->
MPGN 2
MPGN 2
40% progress to end stage renal failure
IF: Dense deposit in GBM .
Aka dense deposit diseases.
Serum: C3NeF (C3 Nephritic Factor) autoantibody is Present.
Where are the dense deposits in MPGN 2?
C3 deposits in capillary walls and mesangium
--> bad prognosis. Why? deposition on mesangium is inescapable b/c of lack of blood supply


Granular
podocyte effacement
minimal change disease
MGN
MGN
hypercellular glomeruli
acute post-strep glomerulonephritis
subepithelial humps
acute post-strep glomerulonephritis
CrGN
Focal proliferation of mesangial cells
Berger's
IgA deposit in mesangium
Berger's
tram-tracking
MPGN 1
Proliferative
anasarca
nephrotic syndrome
granular
circulating immune complex
linear, smooth
anti-GBM
FSGS
Trichrome stain demonstrating blue, collagen deposits
FSGN
Non-selective proteinuria of nephrotic range
FSGS
nephrotic syndrome in child and adult
FSGN
proteins in non-selective proteinuria
albumin
fibrinogen
globulin
Morpho
FSGN
H&E: sclerosis of some glomeruli, w/ partial involvement
Trichrome: blue
Urinalysis of FSGS
frothy b/c of albumin
thick b/c of fibrinogen
D/x of FSGS
biopsy
FSGS
clinical
Clinical:
A. Poor response to corticosteroid
B. Hematuria, Hypertension
C. Progression to chronic renal failure
D. 50% develop End stage Renal failure within 10 years.
D/D of FSGS from minimal change disease
poor response to steroids
Secondary glomerulonephritis
Diabetes Mellitus
Systemic Lupus Erythematosus.
Amyloidosis
Goodpasture Syndrome
Wagner granulomatosis
Henoch-Schönlein Purpura
Bacterial Endocarditis
Complications of nephrotic syndrome
1. MI
2. repeated infection
3. DVT
An adult comes in with nephrotic syndrome, what is the order of possible causes you will suspect?
1. MGN
2. DM
3. SLE
4. Amyloid
Test for
DM?
SLE?
amyloidosis?
DM--> PAS positive, deposit composition
SLE --> anti-smith Ab, dsDNA Ab
amyloidosis--> congo red
D/x for lupus nephritis?
decrease in C1q
Which diseases have a normal looking glomerulus?
minimal change disease & (FSGS?) Type 3 CrGN?
LM of SLE
wire loop
IF of lupus nephritis
C1q deposits
Circulating IC nephritis (Type 3)
granular
All Type 3 hypersensitivity rxns (nephrotic) have anasarca, proteinuria, and lipiduria, but if oliguria is present also what will you suspect?
CrGN
1. CrGN= RPGN
2. Focal proliferative GN = ANS
3. Wire loop= NS
4. Mesangial lupus GN = NS
5. Normal glomerulus = NS
Lupus nephritis
Etiology of scleroderma and kidney
interlobular arteries of the kidney show intimal thickening (deposits on and w/in intima).
C/F of scleroderma and the kidney

nephritic or nephrotic?
shiny taught skin, increased dermal collagen, HTN, proteinuria +2 (non-nephrotic)
What is difference between CREST and Scleroderma?
CREST--> focal
Scleroderma--> diffuse, HTN, increased collagen
Composition of deposits inside glomerulus of diabetic kidney?

Nephrotic or nephritic?
increased glucose & subendothelial collagen

nephrotic
A diabetic kidney is at risk of....
infection
MI
thrombosis (DVT
What vascular change does a diabetic kidney undergo?
hyaline arteriosclerosis
Which test will not be useful in diagnosing a diabetic kidney?
IF
Amyloidosis of Kidney

Gross?
waxy pale surface

Pale b/c of lack of blood vessels
LM of amyloidosis of kidney
pink hyaline like deposit in mesangium
What are the diagnosing features of primary amyloidosis of the kidney?
Primary: (multiple myeloma)
M spike
Bence-Jones protein in urine
(amyloid light chain)
Where do you expect to find amyloid in the kidney?
acellular paraprotein deposits in the extracellular space causing atrophy
What is the secondary (reactive- in rheumatoid arthritis) type of amyloid?
AA
C/F of secondary type of amyloid of the kidney?
symmetrical joint pain in the morning, morning stiffness.
Hereditary recurrent hematuria in children?
Alport syndrome
Which organs are involved in Alport syndrome and what is the resulting effect?
ears--> nerve deafness
eye--> cataract, lens dislocation, corneal dystrophy
Sign of Alport syndrome?
family history of chronic renal failure
Inheritance of Alport syndrome?
X-linked autosomal recessive or dominant
What do we find in the tubules?
deposition of foamy cells (macrophages) containing mucopolysaccharide (MPS)

Seen on LM

Simulates a storage disease
What is the pathogenesis of Alport syndrome?
defective gene (alpha5) produces abnormal collagen type IV
Differential for alport syndrome and is there a genetic history with this differential disease?
thin membrane disease

No genetic hx. like Alport does.
What is found in the glomeruli of Alport syndrome under a light microscope?
irregular thickening b/c of collagen
Where do we find recurrent hematuria?
IgA nephropathy (Berger's disease)- kidney only

Henoch-Schonlein Purpura- systemic ( joint, abdomen, GIT, skin)

Alport syndrome (+ family hx. of hematuria)
Where do we find RPGN?
CrGN
What are the associates of CrGN?
1. Good pasture syndrome--> lungs
2. Wegner granulomatosis--> lungs
3. PAN--> kidney, skin
4. SLE--> multi organ (crescent, wire-looped, normal?)
C/F chronic glomerulos nephritis?
increasing BUN and creatinine, uremia, HTN, CRF, specific gravity= 1010, occasional polyuria unless in terminal stage
How long does it usually take to achieve CGN?
10 years
Which nephrotic syndromes are most likely to convert from acute Gn to chronic GN in 10years?
MGN & FSGS
Rx. for CGN?
30%-50% of all patients need hemodialysis and renal transplant
Gross of CGN?
cortical atrophy
LM of CGN?
Does this allow us to find the source of the problem?
non-specific findings (biopsy not useful)

Scarring of Glomeruli, bowmen’s space
Hyalinization of glomeruli.
Interstitial fibrosis.
Tubular atrophy
Thickening of the small and medium sized arteries.

NO!
GFR of ESKD?
less than 5% of the normal

Use GFR to tell when the kidney reaches the end stage
What is the appearance of the glomeruli in CGN?
all sclerosed
Test for proteinuria?
24 hour urine collection
dipstick
What is the cause of nephritic syndrome in an adult?
infections:
1. Hepatitis
2. malaria
3. SLE
What is the cause of nephritic syndrome in a child?
post-strep
What is the name of type 2 hypersensitivity reaction?
in-situ IC nephritis
What is the cause of hyperlipiduria and lipiduria?
decreased albumin in blood triggers lipoprotein synthesis
How does lipiduria present?
oval fat body in urine
What does hyperlipiduria do in the kidney?
steatosis of tubular epithelial cells causing increased permeability of GBM to lipoproteins
C/F of acute nephritic syndrome
anuria or oliguria
proteinuria 2+ 3+
hematuria
azotemia
HTN
Onset of nephritic syndrome
weeks
Diseases where circulating immune complex nephritis (Type 3- B-cell activation) is seen?
1. SLE
2. Strep
3. Hep B
4. Treponema pallidum (syphillis)
5. Malaria

IF--> granular deposits
Antibody reacting to in-situ antigen in glomeruli (Type 2)?
Anti-GBM Ab

Ex. Good pasture syndrome (CrGn type 1)

IF--> linear, smooth deposits
Primary glomerular diseases
Minimal change disease
Membranous glomerulonephritis
Acute glomerulonephritis
Crescentic glomerulonephritis
Berger's disease (IgA nephropathy)
Membrenoproloferative GN
Alport syndrome
What stain is useful for minimal change disease?
Oil O-red stain, b/c of fat in tubular epithelium hence the name lipoid nephrosis
Mucin deposits from which complication is one of the causes of MGN?
adenocarcinoma of lung and colon
Acute nephritic syndrome is hand in hand with....
ARF b/c of the decrease in GFR caused by the proliferation/ hypercellularity
What is responsible for the proliferation in acute glomerulonephritis?
neutrophils
How would you distinguish post-strep GN from non-strep GN?
ASO titer
D/D for post-strep GN?
pyelonephritis, but WBC casts will signify pyelonephritis
Humpy-dumpy lesions- large IC
Acute post-strep GN
c-anca (+)
Wegner's
Type 3 CrGN
p-anca (+)
PAN
Type 3 CrGN
Where would IgA deposit in the skin (dermatitis herpetiformis)?
papillary dermis
Treatment for IgA nephropathy or elevation?
stop primary source of infection
SLE (lupus nephritis)
LM: wire loop
Diabetic Kidney
LM: nodular hyaline deposit
PAS positive
Kimmelstiel-Wilson disease or
Nodular glomerulosclerosis
DM kidney
hyaline arteriosclerosis
Amyloidosis of kidney
LM: pink hyaline like deposit in mesangium
CGN
"contracted kidney"
coarse granular surface
CGN
hyalinized glomeruli
IF
amyloidosis
amyloidosis
LM: congo red stain- brick red
LM
normal glomerulus
MPGN type 2
Dense deposit
These bright deposits are of C3 in capillary walls and in the mesangium.
SLE
lupus nephritis
IF: C1q deposits everywhere
granular, Type 3 rxn.