Glomerular Diseases

Question 1

What glomerular nephropathies are primarily pediatric diseases

Answer 1

minimal change disease and post-striptococcal muriel and nephritis

Question 2

What are the histologic characteristics of minimal change disease

Answer 2

Normal glomerular membrane with lipoid nephrosis

Question 3

collapsing glomerulo nephropathy is associated with what

Answer 3

Remember the 4-H’s: Hiv, heroin, hemonopathies, heavy

Question 4

Compare and contrast the histologic characteristics of collapsing and idiopathic FSGS

Answer 4

collapsing: Very poor prognosis; Epithelioid hyperplasia
Idiopathic: Prognosis is variable; degeneration of the glomerular epithelium

Question 5

What are common diseases and drugs that cause secondary Membranous nephropathies

Answer 5

Thrombotic vascular diseases; NSAIDs; penicillamine; carcinomas of the lung, colon, & melanoma

Question 6

Describe the histological characteristics of FSGS

Answer 6

focal Sclerosis and hyalinosis With the destruction of potocytes

Question 7

Describe the pathogenesis of minimal change disease

Answer 7

cell cytokine storm triggers damage to the foot processes of the potocytes

Question 8

70% of Membranous neuropathies are associated with deposits of IgG immune complexes. What is the pathogenesis Of these IGG deposits?

Answer 8

Antibodies against phospholipase A2 receptors Activate compliment C5b-C9 Which triggers the deposition of IgG complexes in the sub endothelial layer of the glomerular membrane leading to membrane thickening

Question 9

describe the histologic features of memberness nephropathy

Answer 9

granular deposits in a spike in dome pattern

Question 10

What is the pathogenesis of diabetic glomerulonephropathy

Answer 10

Extensive glycosylation of the renal vessels Leads to arterial or membrane thickening; this increases the pressure within the glomeruli and expands the mesangium; this leads to subsequent sclerosis and glomerular hypertrophy

Question 11

Describe the histologic characteristics of diabetic glomerulonephropathy

Answer 11

arterial hyalinosis; kimmelstiel-wilson nodules in mesangial matrix; Gbm thickening

Question 12

diabetic glomerulonephroth also causes what complication

Answer 12

4 collagen microangiopathy

Question 13

What are the unique histologic features of amyloidosis neuropathy

Answer 13

Amyloid deposits within the mesangium; Apple Green Bifringements with bence Jones proteins

Question 14

Membranous neuropathy is a generic term That can be associated with any disease that causes what?

Answer 14

thickening of the glomerular membrane Isolated to the sub epithelial layer

Question 15

What nephropathies are isolated to just the mesangium and not the glomerular membrane

Answer 15

Amyloidosis neuropathy And IgA nephropathy; Class I & II SLE nephritis

Question 16

What nephropathies are isolated to just the glomerular membrane and do not affect the mesagnium

Answer 16

FSGS; Membranous Nephropathy; Alport syndrome And thin basement membrane nephropathy; Class V & VI SLE nephritis

Question 17

Membrano proliferative comarial nephritis Can cause either a nephronic or nephrotic syndrome. What do Both subtypes of this disease have in common

Answer 17

Both produce a granular pattern along the GBM And mesangium; both will have low serum levels of C3

Question 18

What is the pathogenesis of type 1 Member no proliferative glomerulonephritis

Answer 18

strong association with hepatitis C; Immune complex deposits mediated by classical complement

Question 19

What is the pathogenesis of type 2 membrano proliferative glomerulonephritis

Answer 19

Antibody mediated by alternative compliment; Antibodies bind to C3 convertase preventing its degradation and Subsequently overactivating complement

Question 20

compare and contrast the histologic characteristics of type 1 and type 2 membrano proliferative glomerulonephritis

Answer 20

one: Deposits in the sub endothelial layer of the basement membrane and in the mesangianm
two: intra memberous deposits within the gbm in a ribbon like fashion

Question 21

Because biopsies are expensive and invasive, wood lab can be used to differentiate type 1 and type 2 membrano proliferative glomerulonephritis

Answer 21

C4 serum levels
Type 1: low
Type 2: normal

Question 22

Iga nephropathy typically becomes symptomatic in which decade of life

Answer 22

second and third decade

Question 23

Having a history of what diseases are strongly associated with IGA nephropathy AKA burger disease

Answer 23

Complicated respiratory infections; in stage liver and celiac disease

Question 24

Describe the pathogenesis of IGA nephropathy

Answer 24

Thought to be due to immunological stresses and insults in the 1st and 2nd decades of life:
Respiratory tract infections can activate expression of GD-IgA; This triggers an autoimmune response against IG-IgA isotypes Which deposit in the mesangium; Subsequent activation of alternative complement causes damage to the glomeruli

Question 25

What are the histologic features of IGA neuropathy

Answer 25

The sangrial cell hyperplasia And protein deposits within the mesangianm

Question 26

Describe the pathogenesis Of PSGN

Answer 26

virulent factor: M protein Allows s. pyogenes to persist and survive inside Lukiosites And cause reactivation of infection elsewhere in the body; When these leuke sites deposit and the kidney it activates complement And causes subsequent damage to the glomerulus and the mesandium

Question 27

describe the distinct histologic characteristics of psgn

Answer 27

PMN Hyperplasia in the mesangium; granular deposits along the eclomycular capillaries Creating a starry Sky Lumpy Bumpy appearance; +IgG & C3

Question 28

Compare and contrast the different clinical manifestations of good pastures disease and granulomatosis with polyingitis

Answer 28

good pastures is primarily isolated to the lungs and kidneys; Whereas granulomatosis with polyangitis is a vascular disease that affects multiple systems and patients will typically present with complaints of nasal congestion due to nasal septum defects; They also have different antibodies: ANCA for GMP and anit GM for GP

Question 29

GP Antibodies affect what Protein

Answer 29

alpha 3 chain of type 4 collagen; IgG Type 2 hypersensitivity

Question 30

For good pasteur syndrome what we'll be seeing with immunofluorescence that will not be seen with PMG

Answer 30

linear deposits

Question 31

what are similarities between gp and gmp

Answer 31

Both will present with Crescent shaped glomerulo capillaries; hemoptysis Both are categorized as subtypes of rapidly progressive glomerulonephritis

Question 32

What are hallmarks of PSGN

Answer 32

Peri orbital edema; Recent history of a upper respiratory infection; decreased serum levels of C3 and normal serum levels of C4

Question 33

lab work of Lupus Induced nephritis Low serum levels of both C3 and C4. Therefore which complement pathway has been activated

Answer 33

the classical pathway

Question 34

what classes of sle nephritis are associated with glomerular crescents

Answer 34

classes 3 and 4

Question 35

Describe the histologic characteristics of class 3 and 4 lupus induced nephritis

Answer 35

wire looping, crescents, mesangial and sub endothelial electron dense deposits

Question 36

What antibodies are associated with sle

Answer 36

anti-ANA and anti-dsDNA

Question 37

What is the pathogenesis of Alport syndrome?

Answer 37

X linked dominant inheritance pattern; mutation of the alpha 3 subtype of collagen 4

Question 38

what are the clinical manifestations associated with Alport syndrome

Answer 38

Symptomatic onset 5-20 yrs. of age; hearing loss; orbital anomalies

Question 39

what are the unique histologic characteristics Of Alport syndrome

Answer 39

Interstitial foam cells; Splitting and lamination of the lamina densa Giving a basket like appearance

Question 40

Thin basement membrane neuropathy pathogenesis is Very similar to that of Alport syndrome. Difference between the two

Answer 40

thin basement membrane nephropathy is not associated at all with the clinical manifestations of Alport syndrome and the prognosis is very very good in comparison