Glomerular- Pathology Flashcards by Daniel Guck

This is the term where is increased nitrogenous compounds in the blood (BUN and creatinine).

Azotemia

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What happens to the GFR in azotemia?

it ↓

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What causes prerenal aztoemia?

hypoperfusion of the kidneys (↓ blood flow)

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True or False: there is parenchymal damage in prerenal azotemia,

FALSE. there is impairment of renal fxn but no parenchymal damage.

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What causes postrenal azotemia?

a blockage past the kidney

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This is the term when azotemia is associated with a bunch of other signs and Sx, like failure of renal exretory fxn, metabolic and endocrine alterations, and 2o involvement of other systems.

Uremia

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This is the rapid and frequently reverible deteroration of renal fxn dominated by oliguria or anuria and a recent onset of azotemia.

Acute Renal Failure (ARF)

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What are the 4 stages of chronic renal fialure?

Diminished renal reserve
Renal insufficiency
3, chronic renal failure
end-stage renal disease

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In the diminshed renal reserve stage of chronic renal failure, how much is hte GFR reduced as compared to normal?

50% of normal

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In the renal insufficiency stage of chronic renal failure, what is the GFR % of normal?

GFR 20-50% of normal

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In the renal insufficiency stage of chronic renal failure, what are the other Sx in addition to azotemia??

anemia, HTN, polyuria and nocturia

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In the chronic renal failure stage, the GFR is < 20%, and kidney fxn really sucks, causing what Sx?

Edema, metabolic acidosis, hyperkalemia, and overt uremia

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What % of GFR is at the end-stage renal disease of chronic renal failure?

< 5%

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This is the glomerular syndrome where there is an acute onset of hematuria, azotemia, mild/moderate proteinuria, oliguria, edema, and HTN.

Nephritic syndrome

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This is the glomerular syndrome where there is acute nephritis, proteinuria, and acute renal failure.

Rapidly progressing glomerulonephritis (RPGN)

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This is the glomerular syndrome where there is heavy proteinuria (>3.5 g/day), hypoalbuminemia, hyperlipidemia, lipiduria, and severe edema.

Nephrotic syndrome

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What are the 2 components of the glomerular basement membrane?

type IV collagen and glycoproteins

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These are cells that are similiar to vascular smooth muscle cells and pericytes and lie between the capillaries and support the entire glomerular tuft.

Mesangial cells

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What is the fxn of mesangial cells?

They’re mesenchymal in origin, contractile, phagocytotic, proliferative, and secrete biologically active mediators.

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Hypercellularity in the glomerularnephropathies causes cellular proliferation and leukocyte infiltration, leading to what formation in the glomerulus?

crescents

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Crescents from hypercellualrity is from the leakage of what component into the urinary space?

fibrin

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In glomerulonephropathies, the deposition of amorphous, electron-dense material and increased synthesis of the GBM’s proteins causes what?

GBM thickening

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What mechanisms underlie most glomerulonephropathy?

immune mechnaisms

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If immune complexes are deposited in the glomeruli, what do they look like on immunflourescence (IF)?

Granular

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If antibodies are directed against antigens in the GBM, what does it look like on IF?

Diffuse linear pattern

Which syndrome gives a diffus linear parrtern of immune complexes on IF?

Goodpasture syndrome

When circulating immune complexes get deposited and cause glomerulonephritis, there is leukocyte infiltration and proliferation of what 2 cells?

Mesangial and endothelial cells.

What are the 2 major histologic characteristics of progressive renal damage?

``` Focal Segmental Glomerulosclerosis (FSGS) Tubulointerstitial fibrosis (TIF) ```

Is FSGS a nephritic or nephrotic syndrome?

Nephrotic syndrome

Is the protein content in urine higher or lower than 3.5g/day in nephrotic syndrome?

> 3.5 g/day

In FSGS, where to proteins accumulate when there is endothelila and epithelial are injures causing the increased glomerular permeability to proteins?

Accumulation in mesangial matrix

What happens to the foot processes in FSGS?

They undergo effacement (thinning)

In FSGS, the accumulation of proteins in the mesangium of the glomerulus caues what change of the remaining glomeruli?

Compensatory hyperplasia

The compensatory hyperplasia then causes what change in FSGS?

segmental glomerulosclerosis

Though FSGS is usually idiopathic, what are the 3 high yield assocaitions with its etiology?

HIV, heroin use, and sickle cell disease

True or False: in FSGS, there is immune complex deposition causing a granular appearence on IF.

FALSE

True or False: FSGS patients do not respond to steroid treatment.

True.

In TIF, what causes direct injury to and activation of tubular cells?

Proteinuria

The proteinuria damage in TIF cuases activation of what mediators to cause the interstitial fibrosis?

cytokines, chemokines, and GFs

This syndrome is characterized by the inflammation of the glomeruli, and involves hematuria*, red cell casts in urine, azotemia, oliguria, and mild/moderate HTN.

Nephritic syndrome | PHAROH

This nephritic syndrome occurs 1-4 weeks after a streptococcal infection.

Poststreptococcal Glomerulonephritis (PSGN)

Which patients are at most risk for PSGN?

Kids 6-10 (adults can get it too)

Which protein on the strep bug causes the cross linking to self Ag's?

M protein

True or False: all nephritic syndromes involve immune-complex deposition.

true

There are enlarged, hypercellular glomeruli in PSGN because of the the diffuse infiltration of what cells?

Leukocytes

What are seen in the subepithelium in PSGN as a result of Ag-Ab complex depostion?

Subepithelial humps

True or False: treatment for PSGN is usually supportive.

True

True or False: kids rarely progress to RF in PSGN

True

What syndrome can adults progress to in PSGN?

Rapid Progressive Glomerulonephritis (RPGN)

True or False: Postinfectious (nonstrep) GN causes pretty much the same characteristics as PSGN.

True

This is the nephritic syndrome that progresses to renal failrue in weeks-months.

RPGN

What are in the bowmans space in RPGN?

Crescents

What are the 2 components of the crescents in RPGN?

Fibrin and macrophages (inflammatory debris)

You find a young male with a linear IF pattern complaining of hemoptysis and hematuria. What is the Dx?

Goodpastures syndrome

Why is Goodpastures the only RPGN to cause a linear IF?

because Ab's are directed against the collagen in the glomerular and alveolar BM.

Which 2 diseases give a Granular IF pattern in RPGN?

PSGN | Diffuse proliferative Glomerulonephritis (DPGN)

DPGN are subendothelial deposits and is the msot common cause of renal disease in which patients?

SLE

What test do u do next if you get a negative IF (pauci-immune) for RPGN?

ANCA

A patient who presents with RPGN, hemoptysis, and sinusitis problems will show which ANCA pattern?

c-ANCA | Wegeners

A patient with granulomatous inflammation, eosinophilia, and asthma will show which ANCA pattern?

p-ANCA | Churg-Strauss

This is the syndrome from derangement in glomerular capillary walls resulting in proteinuria (>3.5 g/day), hypoalbulinemia, edema, and hyperlipidemia and lipiduria.

Nephrotic syndrome | nephrOtic with ↑ prOteins makes u look like an O

This is a common cause of nephrotic syndrome in adults and is a form of chronic immune complex-mediated disease.

Membranous nephropathy

Though membranous nephropathy is mostly idiopathic, what are some high yield associations

HBV and HCV Solid tumors SLE*** Drugs (NSAIDS or penicillamine)

What causes the glomeruli to become leaky (and thus prOteinuria) in membranous nephropathy?

immune-complexes against a renal autoantigen and resultant inflammation

In membranous nephropathy, there are subepithelial immune deposits which appear with what pattern on EM?

Spike and dome

What shows up on IF for membranous nephropathy?

grnaular deposits along the GBM

This is the most common nephrOtic syndrome in kids ages 2-6.

Minimal change disease

What usually precedes minimal change disease?

A respiratory tract infection and prophylactic immunizaiton

True or False: there is immune-complex deposition in minimal change disease.

False. there are no immune complexes

what is the hihg yield association with minimal change disease in adults?

Hodkins lymphoma

Which mediators damage the visceral epithelial cells causing proteinuria in minimal change disease?

cytokines

What happens to the foot processes in minimal change disease?

Effacement

True or False: in minimal change disease, there is selective proteinuria with usually no HTN or hematuria.

true | minimal change ya hearrrr

What is a good treatment for minimal change disease?

steroids

What happens in the PCT in minimal change?

They get fatty (lipoid nephrosis)

In FSGS, the entrapment of proteins and increased ECM deposition cause which change?

Healinosis and sclerosis

HIV-infected individuals are likely to develop which form of FSGS?

Severe collapsing FSGS

This is the nephrotic syndrome where there are alteration in the GBM, proliferation of glomerular cells, and leukocyte infiltration.

Membranoproliferative Glomerulonephritis (MPGN)

True or False: there are immune complex deposits in MPGN.

True!

Where are the deposits in type I MPGN?

subendothelium

What are the appearance of the immune deposits on H&E for MPGN?

Tram-track appearance

What are the 2 infectious associations with type I MPGN?

HBV and HCV

Where are the immune deposits in type II MPGN?

within the basement membrane

What do the patients with type II MPGN have that stabilizes C3 convertase --> overactivation of complement?

C3 nephritic factor

Which form of MPGN can arise from chronic immune complex disordrs, A1AT deficiency, malignant disease, or hereditary deficiencies?

Secondary MPGN

This is the nephritic syndrome where there is prominent IgA deposits in the mesangial regions.

IgA nephropathy (berger syndrome)

There is increased IgA in the serum in Berger syndrome usually following which infections?

Mucus infections

Who typically gets Berger syndorme?

older children and young adults

What is the most common presentation of Bergers syndrome post infection?

gross hematuria

This is the nephritic syndrome from an inherited defect (X-linked) in type IV collagen causing thinning and splitting of the GBM.

Alport syndrome

What are the 3 main Sx to Alport syndrome?

hematuria, deafness, eye disorders

This is the syndrome that is characterized by diffuse thinning of the GBM and asymptomatic hematuria that kinda looks like Alport syndrome.

Benign familial hematuria

What Sx in benign familial hematuria differentiate it from Alport syndrome?

There is NO hearing loss/vision problems/family Hx

What is the gross morphology of the kidneys in chronic renal failure (CRF)?

symmetrically contracted kidneys | thinned cortex

What happens to the glomeruli in CRF?

obliteration of the glomeruli that turn into acellular collagen masses (like scarring)

CRF patients with arterial intimal thickening, focal calcification, deposition of calcium oxalate crystals, cysts, and cancer are receiving what?

Dialysis

What is the Tx for CRF?

Transplant or dialysis

These are purpuric skin lesions on the extremities with GI and renal Sx.

Henoch-Schonlein Purpura (HSP)

What is deposited in the mesangium causing mesangial proliferation and/or endocapillary to crescentric glomerulonephritis in HSP?

IgA

This is the constion where there is immune complex nephritis from complexes of baterial Ag and Ab, causing crescents, FSGN, and DPGN.

Bacterial Endocarditis-Associated Glomerulonephritis

What happens in diabetes to cause hyaline arteriolosclerosis?

Nonenzymatic glycosylation of GBM

Which arteriole is more affected in diabetes to cause ↑ GFR?

Efferent arteriole

Which drugs help the diabetes associated nephrotic syndrome?

ACEi's

These are the things formed by diabetes from sclerosis of the mesangium,

Kimmelsteil-Wilson nodules

In systemic amyloidosis, there are amyloid deposits in the mesangium leading to what characteristics under polarized light?

Apple-green birefringence

Clean-up: What are the nephrotic syndromes?

``` Minimal Change FSGS Membranous nephropathy MPGN Diabetes Amyloidosis ```

Clean-up: what are the 2 nephrotic syndrome that have effacement of foot processes?

Minimal change + FSGS

Clean-up: What 2 nephrotic syndromes have immune complex deposition?

Membranous + MPGN

Clean-up: What 2 nephrotic syndromes are systemic in origin?

Diabetes + amyloidosis

Clean-up: Which disease responds to steroids: minimal change or FSGS?

Minimal change

Clean-up: Which NEPHRIOTIC syndrome is assocaited with SLE?

Membraneous nephropathy

Clean-up: Which NEPHRITIC syndrome is assoacited with SLE?

DPGN

Clean-up: What are the nephritic syndromes?

PSGN, Alport, IgA neuropathy, RPGN "PAIR"

Clean-up: What are the Sx to nephritic syndromes?

PHAROH | Proteinuria under 3.5, Hematiuria, Azotemia, RBC casts, oliguria, and HTN

Clean-up: In the nephrotic syndromes, which disease causes deposits in the Subendothelium?

Type I MPGN

Clean-up: In the nephrotic syndromes, which disease causes deposits in the BM?

Type II MPGN

Clean-up: In the nephrotic syndromes, which disease causes deposits in the Subepithelium?

Membranous nephropathy

Glomerular- Pathology Flashcards

(117 cards)