Glomerular Disease 3

Question 1

What are nephrotic syndromes that are under primary glomerular diseases.

Answer 1

Minimal Change disease
FSGS (focal segmental glomerulosclerosis)
Membranous GN
Others - IgA nephropathy, mesangial

Question 2

What are secondary glomerular diseases that would be classified under nephrotic syndrome?

Answer 2

DM
amyloidosis
drugs - NSAIDs, Pencillamine, street heroin
Infections - malaria, syphilis, HBV, HCV
Malignancies - carcinoma, lymphoma
Miscellaneous - bee sting allergy, hereditary nephritis

Question 3

What is the most frequent cause of nephrotic syndrome in children?

Answer 3

Minimal Change Disease

Question 4

Another name for Minimal Change Disease?

Answer 4

lipoid nephorsis

Question 5

What is the age for the peak incidences of minimal change disease?

Answer 5

2-6 years of age

Question 6

What is the best treatment for minimal change disease?

Answer 6

corticosteroid therapy (dramatic response)

Question 7

Prognosis of Minimal Change Disease?

Answer 7

excellent prognosis

Question 8

How does the kidney appear in Light microscopy of one with minimal change disease?

Answer 8

normal

Question 9

How do the kidneys appear upon EM of one with Minimal Change disease?

Answer 9

diffuse effacement of foot processes

Question 10

What are the mechanisms of proteinuria in minimal change disease?

Answer 10

loss of glomerular polyanions causing defects in the charge barrier

Question 11

What is Congenital nephrotic syndrome (Finnish type) with minimal change disease.

Answer 11

mutation in nephron gene leading to loss of proteins

Question 12

On PAS of an individual with minimal change disease what may be observed?

Answer 12

tubular lipoid cells - lipoid nephrosis

Question 13

Does minimal change disease resolve?

Answer 13

yes; resolves as child reaches puberty

Question 14

What is an association of MCD in adults?

Answer 14

association with Hodgkin disease and other leukemias and lymphomas NSAID therapy

Question 15

Describe what may be seen upon light microscopy of individual with FSGS?

Answer 15

sclerosis involving a portion of the few glomerulus involvement of juxtamedullary glomeruli

Question 16

What can be seen upon EM of a person with FSGS?

Answer 16

diffuse effacement of foot processes
may also be focal detachment of epithelial cells

Question 17

What is the most common cause of nephrotic syndrome in adults?

Answer 17

FSGS

Question 18

What are some secondary causes of FSGS?

Answer 18

HIV nephropathy
heroin nephropathy
sickle cell disease

Question 19

Is there a higher chance of hematuria in minimal change disease of FSGS?

Answer 19

FSGS

Question 20

Is there higher incidence of reduced GFR in FSGS or minimal change disease?

Answer 20

FSGS

Question 21

Is there a higher incidence of HTN in FSGS or minimal change disease?

Answer 21

FSGS

Question 22

Is proteinuria in FSGS selective or nonselective?

Answer 22

nonselective

Question 23

Describe response of FSGS to corticosteroid therapy?

Answer 23

poor

Question 24

Describe prognosis too FSGS and how it progresses.

Answer 24

poor prognosis

progression to chronic glomerulosclerosis with at least 50% end-stage renal disease within 10 years

Question 25

Why is IF -ve in FSGS?

Answer 25

The proportion of glomeruli are not enough to be seen There is nonspecific deposition ("trapping") of IgM and C3 in sclerotic segment

Question 26

Which age group does better with FSGS?

Answer 26

children

Question 27

What is the second most common cause of NS in adults?

Answer 27

membranous GN

Question 28

Describe capillary wall of one with membranous GN?

Answer 28

uniform, diffuse thickening

Question 29

On silver stain what is characteristic features of membranous GN?

Answer 29

spike and dome pattern

Question 30

Is membranous GN IF +ve or IF -ve?

Answer 30

IF +ve

Question 31

Primary cause of membranous GN?

Answer 31

idiopathic (may be autoab against the phospholipase A2 on podocytes.

Question 32

Secondary causes of membranous GN?

Answer 32

Infections (HBV, HCV, etc) solid tumors SLE Drugs( penicillamine, gold, NSAIDS)

Question 33

Is a feature of membranous GN slow onset or rapid onset of nephrotic syndrome?

Answer 33

slow onset

Question 34

Is proteinuria selective or non selective in membranous GN?

Answer 34

non selective

Question 35

Does membranous GN respond well to corticosteroid therapy?

Answer 35

no

Question 36

Prognosis of membranous GN?

Answer 36

poor prognosis

Question 37

Is membranous GN associated with sclerosis of glomeruli and increased BUN and HTN?

Answer 37

yes

Question 38

There are cases of spontaneous remission of membranous GN in what groups?

Answer 38

mostly in women and in those with non-nephrotic range proteinuria

Question 39

Before you claim an idiopathic cause of membranous GN, what must you rule out?

Answer 39

secondary causes