Glomerular Disease 3 Flashcards
What are nephrotic syndromes that are under primary glomerular diseases.
Minimal Change disease
FSGS (focal segmental glomerulosclerosis)
Membranous GN
Others - IgA nephropathy, mesangial
What are secondary glomerular diseases that would be classified under nephrotic syndrome?
DM
amyloidosis
drugs - NSAIDs, Pencillamine, street heroin
Infections - malaria, syphilis, HBV, HCV
Malignancies - carcinoma, lymphoma
Miscellaneous - bee sting allergy, hereditary nephritis
What is the most frequent cause of nephrotic syndrome in children?
Minimal Change Disease
Another name for Minimal Change Disease?
lipoid nephorsis
What is the age for the peak incidences of minimal change disease?
2-6 years of age
What is the best treatment for minimal change disease?
corticosteroid therapy (dramatic response)
Prognosis of Minimal Change Disease?
excellent prognosis
How does the kidney appear in Light microscopy of one with minimal change disease?
normal
How do the kidneys appear upon EM of one with Minimal Change disease?
diffuse effacement of foot processes
What are the mechanisms of proteinuria in minimal change disease?
loss of glomerular polyanions causing defects in the charge barrier
What is Congenital nephrotic syndrome (Finnish type) with minimal change disease.
mutation in nephron gene leading to loss of proteins
On PAS of an individual with minimal change disease what may be observed?
tubular lipoid cells - lipoid nephrosis
Does minimal change disease resolve?
yes; resolves as child reaches puberty
What is an association of MCD in adults?
association with Hodgkin disease and other leukemias and lymphomas NSAID therapy
Describe what may be seen upon light microscopy of individual with FSGS?
sclerosis involving a portion of the few glomerulus involvement of juxtamedullary glomeruli
What can be seen upon EM of a person with FSGS?
diffuse effacement of foot processes
may also be focal detachment of epithelial cells
What is the most common cause of nephrotic syndrome in adults?
FSGS
What are some secondary causes of FSGS?
HIV nephropathy
heroin nephropathy
sickle cell disease
Is there a higher chance of hematuria in minimal change disease of FSGS?
FSGS
Is there higher incidence of reduced GFR in FSGS or minimal change disease?
FSGS
Is there a higher incidence of HTN in FSGS or minimal change disease?
FSGS
Is proteinuria in FSGS selective or nonselective?
nonselective
Describe response of FSGS to corticosteroid therapy?
poor
Describe prognosis too FSGS and how it progresses.
poor prognosis
progression to chronic glomerulosclerosis with at least 50% end-stage renal disease within 10 years
Why is IF -ve in FSGS?
The proportion of glomeruli are not enough to be seen
There is nonspecific deposition (“trapping”) of IgM and C3 in sclerotic segment
Which age group does better with FSGS?
children
What is the second most common cause of NS in adults?
membranous GN
Describe capillary wall of one with membranous GN?
uniform, diffuse thickening
On silver stain what is characteristic features of membranous GN?
spike and dome pattern
Is membranous GN IF +ve or IF -ve?
IF +ve
Primary cause of membranous GN?
idiopathic (may be autoab against the phospholipase A2 on podocytes.
Secondary causes of membranous GN?
Infections (HBV, HCV, etc)
solid tumors
SLE
Drugs( penicillamine, gold, NSAIDS)
Is a feature of membranous GN slow onset or rapid onset of nephrotic syndrome?
slow onset
Is proteinuria selective or non selective in membranous GN?
non selective
Does membranous GN respond well to corticosteroid therapy?
no
Prognosis of membranous GN?
poor prognosis
Is membranous GN associated with sclerosis of glomeruli and increased BUN and HTN?
yes
There are cases of spontaneous remission of membranous GN in what groups?
mostly in women and in those with non-nephrotic range proteinuria
Before you claim an idiopathic cause of membranous GN, what must you rule out?
secondary causes
