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Renal - Gupta > Nephritic Syndrome > Flashcards

Nephritic Syndrome Flashcards

1
Q

What 2 types of Nephritic Syndrome Fall under the category of:
Visceral Epithelial Injury: Podocyte Disorder?

A
  • Minimal Change Disease

* Focal Segmental Glomerulosclerosis

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2
Q

What 4 types of Nephritic Syndrome Fall under the category of:
Immune Complex Formation?

A
  • Membranous Nephropathy (subepithelial) - really this is a nephrotic syndrome
  • Post-infectious Glomerulonephritis (subepithelial)
  • Membranoproliferative Glomerulonephritis (SubENDOthelial)
  • IgA nephropathy (mesangial)
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3
Q

What 3 types of Nephritic Syndrome Fall under the category of:
Glomerular Basement Membrane Disease

A
  • Anti-glomerular Basement Membrane Disease
  • Alport’s Syndrome
  • Thin Basement Membrane
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4
Q

What 2 types of Nephritic Syndrome Fall under the category of:
Vascular Injury Syndromes?

A
  • ANCA-associated glomerulonephritis/pauci-immune glomerulonephritis
  • Hemolytic Uremic Syndrome (HUS) and Thrombotic Thrombocytopenia Purpura (TTP)
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5
Q

What are some important Side Effects seen with most Nephritic Syndromes?

A

Hematuria
• microscopic or macroscopic
• Red Blood Cell Casts

HTN
Oliguria
Edema (moderate)
Mild to moderate proteinuria
Azotemia
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6
Q

Where are the immune deposits located in:
• Membranoproliferative GN
• IgA nephropathy

A

Membranoproliferative GN = SUBENDOTHELIAL

IgA Nephropathy = MESANGIAL

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7
Q

What are 4 diseases associated with SubENDOthelial and mesangial immune DEPOSITS?
• what particle/process is causing damage in these diseases?

A
  • MEMBRANOPROLIFERATIVE GN
  • IgA Nephropathy (mesangial)
  • Lupus Nephritis
  • Post-Infectious GN

*Diseases are associated with circulating immune complexes that deposit on the basis of size, charge, etc.

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8
Q

What are some diseases that cause actual ENDOTHELIAL CELL INJURY?
• how?

A
  • Thombotic Microangiopathies

* Immune complexes/PARAproteins

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9
Q

What is the most common causes of Paraprotein entrapment that leads to Endothelial cell injury?

A

Multiple Myeloma

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10
Q

What causes endothelial damage nephritic syndrome?

A

INFLAMMATORY RESPONSE that results from…

CYTOKINES and Autacoids are released which UPREGULATE Adhesion Molecules on Endothelial and circulating immune cells

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11
Q

T or F: HEMATURIA is the clinical indication that nephritic syndrome is occuring.

A

True

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12
Q

What are the two forms of Membranoproliferative Glomerulonephritis?
• Prevalence
• Deposition location?

A

Type I: MOST COMMON

Type II: Dense Deposit Disease

Deposition Location: SubENDOthelial

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13
Q

Membranoproliferative Glomerulonephritis Type I
• light microscopic changes
• KEY thing to recognize visually
• Important Lab Value

A

Light Microscopic Changes:
• Hypercellularity of Glomerular Tuft
• Increased mononuclear cells within the expanded mesangium and in capillary lumens

KEY:
• Tram-Track Appearance

Lab Value:
• Reduced C3 levels

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14
Q

What is frequently the cause of Membranoproliferative Glomerulonephritis Type I in adults?

A

Hepatitis C

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15
Q

How do people of all ages typically present with MPGN (membranoproliferative glomerulonephritis) type I?

A
  • Microscopic Hematuria
  • Nephrotic range proteinuria and Nephrotic Syndrome is possible
  • Acute Nephritic Syndrome
  • Rapidy progressive glomerulonephritis (PRGN)
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16
Q

What is the typical prognosis of MPGN type I?

• treatment?

A

Px: USUALLY - slow progression to end stage renal disease; RARELY spontaneous remission may occur

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17
Q

Dense Deposit Disease (Membranoproliferative glomerulonephritis type II).
• KEY lab terms (pathophysiology)
• KEY TERM for histology

A

Key Lab Terms:
• LOW C3 is often present because 80% of ppl. ohave C3 NEPHRITIC FACTOR which binds and stabiliizes C3

Key Histo Terms:
• Dark SubENDOthelial ribbons on Electron Microscopy (aka electron dense deposits)

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18
Q

What causes Tram Track appearance?

• associated disease?

A

Tram Tracks:
• Splitting of the GBM is associated with Tram Track Appearace is due to MESANGIAL cells (which have macrophage-like function) PENETRATE the Basement Membrane in pursuit of Immune Complexes

19
Q

What does the glomerulus look like in Membranoproliferative Glomerulonephritis and Dense Deposit Disease?
• Basement Membrane
• Cells
• Overall Appearance

A

Glomeruli = Large and Lobular

  • All Cell Types have proliferated - Mesangial, Endothelial, and Leukocytes
  • GBM has thickened
20
Q

What differences exist between MPGN type I and II on immunofluorescence?

A

MPGN type I:
• Granular C3 positive Basement Membrane

Dense Deposit Disease:
• BRIGHT C3 DEPOSITS in MESANGIUM and some in the capillary wall

21
Q

T or F: in some cases MPGN can be associated with MONOCLONAL GAMMOPATHIES

A

True

22
Q

MPGN vs. DDD:

• Pathophysiology

A

MPGN:
Immune Complexes in GBM induced CLASSICAL COMPLEMENT PATHWAY*

Immune Complexes in GBM induce ALTERNATIVE COMPLEMENT PATHWAY

• this leads to damage endothelial cellls and basement membrane by leukocyte recruitment

23
Q

What is the Most Common Primary GN worldwide?

• what are some secondary causes of this disease?

A

• IgA Nephropathy - Berger Disease

Secondary Causes: 
• Henoch - Schoenlein Purpura
• Ankylosing Spondylitis 
• Dermatitis Herpetiformis
• Celiac Disease
• Inflammatory Bowel Disease
• Cirrhosis 
• Psoriasis
24
Q

What is the location of Immune Deposits in IgA nephropathy?

• aka what is the pattern of Injury

A

IgA containing Immune complexes are found mostly in the MESANGIUM

Pattern of Injury:
• Mesangioproliferative Pattern of Injury

25
Q

What is Classically the trigger of IgA nephropathy?

A

IgA nephropathy:
• Often Triggered by Upper Respiratory Tract infection

IMPORTANT: sediment forms in a SYNPHARYNGITIC manner i.e. within 1 to 2 days of infection (not after infection)*

26
Q

What is the problem with the IgA formed in IgA nephropathy (Berger’s disease)?
• Where do these immune complexes form?
• why do they form?

A

GALACTOSE DEFICIENT IgA1 circulating immune complexes deposit in the MESANGIUM***

WHY:
• underglycosylation of O-linked glycans that terminate with N-acetylgalactosamine instead of galactose

• OTHER IgG or IgA antibodies recognize this unglycosylated region and form IMMUNE COMPLEX

27
Q

How does IgA nephropathy present?

A

SUPER VARIABLE:

  • Asymptomatic
  • Microscopic Hematuria
  • Intermittent Gross Hematuria
  • Synpharyngitic Hematuria
  • Nephrotic (15%) or non-nephrotic proteinuria
  • Acute Glomerulonephritis
  • Rapidly Progressive Glomerulonephritis
28
Q

T or F: patients must have elevated to serum IgA levels to have IgA nephropathy.

A

FALSE, only ~50% of patients with this have increased serum IgA levels

29
Q

What are some Poor Prognostic Indicators of IgA nephropaty?

A
  • Heavy Proteinuria
  • Decreased GFR at onset of disease
  • Older age at Onset
  • Uncontrolled HTN
  • Cresents and/or tubulointerstitial fibrosis/atrophy
30
Q

T or F: immune deposits are found in the subendothelium in IgA glomerulonephropathy

A

FALSE, IgA nephropathy has Immune Deposits in the MESANGIUM

31
Q

What two diseases involve the basement membrane?

A
  • Anti-GBM disease

* Hereditary Nephritis

32
Q

What causes anti-GBM disease?
• what is another name for this disease?
• Disease progression?

A

Anti-GBM
Cause:
• IgG Autoantibodies against a noncollagenous portion of the ALPHA-3 SUBUNIT of type IV collagen

Development:
• Disease is rapidly progressing!!! - days to weeks until these people develop renal failure

33
Q

What is the appearance of anti-GBM antibodies in immunofluorescence?
• Appearance on H and E?
• correlation between concentration of anti-GBM in the membrane and disease activity.

A

Appearance:
• LINEAR pattern on Immunofluorescence - PATHOpneumonic for Goodpasture’s

• H and E = CRESCENT formation (focal or diffuse); Glomerulonephritis

Anti-GBM conc.:
• NO correlation between anti-GBM titers and disease activity

34
Q

What other antibody besides Anti-alpha-3 collagen antibodies are present in Goodpastures?

A

MPO-ANCA

35
Q

What besides renal dysfunction, what are some other symptoms/problems associated with Goodpastures?

A

Pulmonary Problems:
• Cough, dyspnea, crackles, hemoptysis (may precede or coincide with renal dysfunction)
• Smoking makes this worse

Blood Problems:
• Anemia out of proportion to renal insufficiency
• NORMOTENSIVE with decreased renal function (late complication)

Bone/joint Problems:
• Arthritis/Arthralgias

36
Q

What is the treatment for Goodpasture’s (anti-GBM) disease?

A
  • Corticosteroids
  • Plasmapheresis
  • Cytotoxic Agents
37
Q

What happens to the glomerulus in light of the formation of crescents in Goodpasture’s syndrome?

A

• Glomerular Tuft is compressed by PROLIFERATING EPITHELIAL CELLS in the crescent

38
Q

What is the progression of disease severity in crescent formation?
• when can you classify a disease as a crescentric glomerulonephritis?

A

Segmental Proliferative and Necrotizing Lesions => Cellular Crescents => Fibrocellular Crescents => Fibrous Crescents

Classification:
• Referred to as a diffuse crescentric glomerulonephritis if greater then 50% glomeruli are involved under light microscopy

39
Q

What is the etiology of crescent formation in the kidney?

A

• May be due to severe damage ot capillary walls that tear and necrose the GBM

Result:
• RBCs, WBCs, fibrinogen, and plasma constituents enter Bowman’s space and cause proliferation of mononuclear cells and parietal epithelial cells

40
Q

In Rapidly progressive glomerulonephritis what is seen on Immunofluorescence?

A
  • FIBRINOGEN stains into GREEN CRESCENTS
41
Q

Alport Syndrome
•Cause?
• Who is most often affected?
• Associated Symptoms?

A

Cause:
•X-linked (most often) defects in ALPHA-5 COLLAGEN TYPE IV

Who is affected:
• Because its X-linked MALES are most often affected

Associated symptoms:
• Sensorineural Hearing Loss
• Lens abnormalities, Platelet Defects
• Esophageal Leiomyomas

42
Q

Alport Syndrome
• Key terms
• microscopy used in dx?

A

BASKET WEAVE APPEARANCE

EM is where you see basket weave appearance, but early in the disease you don’t see anything

43
Q

Thin Basement membrane Disease
• cause
• severity of Disease
• appearance

A

Cause:
• Defects in ALPHA-3 or ALPHA-4 collagen type IV

Severity:
• Heterozygotes = Benign
• Homozygotes/Compound Heterozygotes = Affected

Appearance:
• GBM appears at 1/2 thickness

Renal - Gupta (17 decks)

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