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37 Cards in this Set

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IF and EM: “ribbon-like” zone of increased density within the GBM and mesangial matrix

associated with?
Type II MPGN: dense deposit disease
proliferations of the pariatal glomerulous are known as crescents... what is this seen in?
Type II MPGN: Dense Deposit Disease

also type I, RPGN, and Anti-GBM
In Type II MPGN: Dense Deposit Disease, what antibody to what complement protein is typical for membranoproliferative glomerulonephritis.
C3

can help distinguish Type II from type I MPGN
Lupus is what kind of disease? What is the one of the more common features?
Autoimmune

Nephritis
in SLE ______ form to in situ antigens or circulating antigens; deposit in variety of glomerular locations
Immune complexes
what is a treatment for Lupus?
immunosupression (corticosteroids)
wire loop histo appearance =
Lupus
Most common form of GN in the world
?
IgA Nephropathy (Berger Disease)
who is most affected by IgA Nephropathy (Berger Disease)
young men
to be nephrotic, what criteria has to be met?
>3.5 grams protein in urine per 24 hours
Deposition of IgA-dominant immune complexes, with high levels of circulating IgA
IgA Nephropathy (Berger Disease)
in IgA Nephropathy (Berger Disease), where is IgA deposited
the mesangium
What is Goodpasture's disease? what is one of the problems normally seen in this?
Kidney + pulmonary disease

kidney problem is Anti-GBM GN
what do you treat Goodpasture's with?
high-dose immunosuppressants
what does Anti-GBM GN often follow?
URI
what happens in Anti-GBM GN
Autoantibody directed against portion of type IV collagen molecule present in GBM
what will be your major LM finding of Anti-GBM GN
glomerular crescents >90%... feature of rapidly progressive glomerulonephritis
if you see IF findings of diffuse linear staining of GBM for IgG what do you likely have?
Anti-GBM GN
what is the hallmark of Rapidly Progressive GN (RPGN)
Crescents on light microscopy
what are the 3 types of Rapidly Progressive GN (RPGN)
TYPE I (ANTI-GBM ANTIBODY)
---Goodpastures

TYPE II (Immune complex)
--Lupus
--IgA nephropathy (Henoch-Schonlein purpura)

TYPE II (Pauci-immune)
pts with Hereditary Nephridites present with?
hematuria
what are the 2 types of Hereditary Nephridites
Thin basement membrane disease

Alport syndrome
what is the most common cause of Hereditary Nephridites
thin membrane disease

this is usually asymptomatic
in Hereditary Nephridites, you have mutations in genes coding for alpha chains of ______
type IV collagen
EM shows what in Hereditary Nephridites
thinning of the GBM
pt has hematuria with progression to CRF (also nerve deafness, eye disorders)
Alport syndrome
what is the genetics underlying Alport syndrome
Majority are X-linked (males express full form; females limited to benign hematuria)

Defective assembly of collagen type IV (GBM, BM of eye lens, cochlea)
the EM finding of alternating thinning and thickening of GBM is seen in
Alport syndrome
Progressive renal failure
Oliguria, proteinuria, edema, azotemia, uremia, death
Anemia (deficient EPO), anorexia, general malaise
Most patients are hypertensive with cardiac and CNS effects

clinical symptoms of?
Chronic Glomerulosclerosis (CGN)
ppl who survive acute phase of RPGN generally develop....
Chronic Glomerulosclerosis (CGN)
Shrunken kidney with diffuse granular cortical surface is descriptive of?
Chronic Glomerulosclerosis (CGN)
arteriolar sclerosis, tubular atrophy, renal osteodystrophy are signs of?
Chronic Glomerulosclerosis (CGN)
12 yr old presents with periorbital, abdominal, and ankle swelling. She had been in good health until several months ago when she gained some weight and noted swelling in her legs. A Chest xray shows bilateral pleural effusions without evidence or lung disease. a urinalysis reveals heavy proteiunuria (8kg/24hr) there is no hematuria. a percutaneous needle biospy of the kidney reveals no morphological abnormality via LM. which of the following best describes the most likely medical condition responsible:

Amyloid nephropathy
Focal segmental glomerulosclerosis
Hereditary nephritis
Membranous glomerulophay
Minimal change disease
her lipids would probably be?
high, and found in urine

answer: Minimal change disease
2 conditions associated with amyloid nephrophathy
multiple myeloma (AL)

Chronic inflammatory process (AA)
---think auto immune causing this
Focal segmental glomerulosclerosis
African American adult
An 8 year old girl presents with headaches dizziness and malaise. PE shows edema. Her PB is 180/110 and 24 hr urine reveals oliguria, hematuria, and 2.7g protein. A kidney biopsy shows electron-dense sub-epithelial humps. What would be the most likely additional finding in this girl:

Kidney biopsy with no appreciable findings by LM and IF

Bilateral nerve deafness

serology positive for nephritic factor

a history of antecedent pharyngitis

progression to chronic renal failure despite aggressive corticosteroid therapy
she probably has: Post infectious glomerulonephritis

Answer: a history of antecedent pharyngitis

a) associated with: minimal change disease or focal segmental glomerular sclerosis (FSGS)---not correct
b)Alports syndrome (x linked recessive, if a girl has it, it would be benign)
EM finding of minimal change disease?
podocyte foot process effacement