Kidney Path Flashcards
(84 cards)
nephrotic syndrome
severe proteinuria hypoalbuminemia generalized edema hyperlipidemia lipiduria
nephritic syndrome
hematuria azotemia oliguria hypertension proteinuria
causes of nephrotic syndrome
Minimal Change Disease (MCD) Membranous Glomerulonephritis (MGN) Focal Segmental Glomerulosclerosis (FSGS) Membranoproliferative Glomerulonephritis (MPGN)
Minimal Change Disease (MCD)
n most frequent cause of the nephrotic
syndrome in children
n glomeruli have normal appearance under
the light microscope, but electron
microscopy reveals diffuse loss of visceral
epithelial foot processes
what is universal to nephrotic syndrome
fusion of podocytes
Minimal Change Disease (MCD) path
current evidence points to a disorder of T cells
-elaborate factors (IL-8, TNF, etc.?) that affect
nephrin synthesis
Minimal Change Disease (MCD) gold standard for diagnosis
EM
Minimal Change Disease (MCD) immunofluorescence?
no
Minimal Change Disease (MCD) clinical course
Insidious development of nephrotic syndrome in
otherwise healthy child (peak age, 2-6)
-Usually follows respiratory infection or prophylactic
immunization; assoc. w/atopic disorders
Selective proteinuria (mainly albumin)
Good prognosis
>90% respond to short course of corticosteroids
<5% develop chronic renal failure after 25 years
Membranous Glomerulonephritis
MGN
most common cause of nephrotic syndrome in adults (peak age, 30-50) n glomeruli appear normal early in the disease, but develop diffuse thickening of the capillary walls
Membranous Glomerulonephritis
(MGN) path
form of chronic immune complex nephritis
-idiopathic forms (85% of cases) are mainly autoimmune
*antibodies react to antigens in the glomerulus components, or to
antigens that have become trapped there
-may also be due to circulating complexes of known exogenous
or endogenous antigen
the membrane attack complex of complement (C5b-C9)
activates mesangial and epithelial cells, causing them to
liberate proteases and oxidants that damage glomerular
capillaries
Membranous Glomerulonephritis
(MGN) key to dianosis
silver stain
detects collagen in BM
Membranous Glomerulonephritis
(MGN) clinical course
Insidious development of nephrotic syndrome
Proteinuria is nonselective and does not respond
to corticosteroid therapy
Variable course
40% suffer progressive disease ending in renal failure
after 2-10 years
10-30% follow benign course with partial or complete
remission
Focal Segmental
Glomerulosclerosis (FSGS)
characterized by sclerosis affecting some
but not all glomeruli and involving only
segments of each glomerulus
most well-known association is with HIV
patients and IV drug users, but occurs in
all ages (more common in African-
American populations)
Focal Segmental
Glomerulosclerosis (FSGS) path
unknown (some have suggested it is an
aggressive variant of minimal change
disease)
circulating mediator is likely responsible
-proteinuria recurs soon after renal transplant
Focal Segmental
Glomerulosclerosis (FSGS) diagnosis
IF usually isn’t done, but detects IgM and
C3 in sclerotic segments
Focal Segmental
Glomerulosclerosis (FSGS) clinical course
May be a primary disease or result from other
forms of GN (esp. IgA nephropathy)
Proteinuria is nonselective and has poor
response to corticosteroid therapy
Higher incidence of hematuria and hypertension
(more likely to evolve from nephritic syndromes)
Prognosis is poor, with children faring slightly
better than adults
-50% suffer renal failure after 10 years
Membranoproliferative
Glomerulonephritis (MPGN)
may be nephritic, nephrotic, or mixed
characterized by alterations in the GBM
and mesangium plus proliferation of
glomerular cells
Membranoproliferative
Glomerulonephritis (MPGN) Type I
(2/3rds of cases)
Circulating immune complexes of unknown antigen
-Associated with hepatitis B and C, SLE, infected
atrioventricular shunts, chronic lymphocytic leukemia
Membranoproliferative
Glomerulonephritis (MPGN) Type II
Less clear, but serum of patients has a factor called C3
nephritic factor (C3NeF)
Activates the alternative complement pathway by stabilizing
C3 convertase
hypocomplementemia, but normal C1, C2, C4
Membranoproliferative
Glomerulonephritis (MPGN) key to diagnosis
“doubled” GBM on silver stain
Membranoproliferative Glomerulonephritis (MPGN) EM varies by type
Type I: subendothelial deposits Type II: dense deposits in center of GBM and under endothelium
Causes of Nephritic Syndrome
Acute Proliferative (Poststreptococcal) Glomerulonephritis (PGN) Rapidly Progressive (Crescentic) Glomerulonephritis (RPGN or CrGN) IgA Nephropathy (Berger Disease)
Acute Proliferative (Poststreptococcal) Glomerulonephritis (PGN)
Typically caused by immune complexes of
exogenous or endogenous antigens
-Prototype exogenous pattern = 1-4 weeks after a
streptococcal infection
*Only “nephritogenic” strains of group A β-hemolytic
streptococci associated
*Most cases follow skin or pharynx infection
Children affected more frequently than adults