12.3 Nephrotic Syndrome Flashcards
which nephrotic syndrome is the only one with an effective treatment?
- minimal change disease
- tx with steroids (damage by cytokines of T cells)
Focal Segmental Glomerulosclerosis (FSGS)
-etiology, what associatd with?
-usu idiopathic. associated with HIV, heroin, sickle cell
diabetic nephropathy
-what drug slows its progression, how?
- ACE-I, b/c it inhibits ATII’s constrictive effect on efferent arteriole.
- remember, efferent arteriole is more affected by the hyaline arteriosclerosis of NEG
what is most common nephrotic and nephritic syndromes for SLE?
nephrotic: membranous nephropathy
nephritic (most common for SLE overall): Diffuse proliferative glomerulonephritis (causes rapidly progressive glomerulonephritis)
Focal Segmental Glomerulosclerosis (FSGS)
- clinical findings
- histology findings? IF?
- nephrotic syndrome
- effacement of podocytes (similar to MCD. if MCD does not respond to steroids, disorder progresses to FSGS)
- focal (only certain glomeruli)
- segmental (only part of glomerulus–becomes sclerosed)
- negative IF (no immune deposits)
what is most common cause of nephrotic syndrome in whites?
Membranous nephropathy
Membranoproliferative glomerulonephritis
-tx
poor response to steroids, progresses to chronic renal failure
Amyloidosis on the kidney
-what happens
- amyloid deposits in the mesangium, causing nephrotic syndrome
- kidney is most commonly involved organ in systemic amyloidosis
Membranoproliferative glomerulonephritis
-what is each type associated with?
type 1: subendothelial
-assoc with HBV, HCV. (“tram track appearance”)
type 2: intramembranous (“dense deposit disease)
-assoc with C3 nephritic factor (autoAb that stabilizes C3 convertase, leading to overactivation of complement, inflammation, and low levels of circulating C3)
Nephrotic syndrome
-main clinical findings and their symptoms (5)
- proteinuria >3.5 g/day (liver makes 10-15 g/day, but tubular cells keep much of the protein that enters the tubules)
- hypoalbuminuria (pitting edema)
- hypogammaglobulinemia (increased infection risk)
- hypercoagulable state (loss of ATIII)
- hyperlipidemia, hypercholesterolemia (may result in fatty casts in urine)
Focal Segmental Glomerulosclerosis (FSGS)
-tx
-poor response to steroids, progresses to chronic renal failure
what is most common nephrotic syndrome in children?
minimal change disease
Membranous nephropathy
- mech
- what do you see on histology? (H&E, EM, IF)
- immune complex deposition–subepithelial
- H&E: thickened basement membrane
- EM: ‘spike and dome’ appearance b/c BM grows over subepithelial deposits
IF: granular deposits
diabetic nephropathy
-what do you see on histology
Sclerosis of mesangium, (kimmelstiel-wilson nodules)
Membranoproliferative glomerulonephritis
-histology appearance
- tram track appearance
- mesangial cells are proliferating and slitting the immune complex depositions into two.
minimal change disease
- clinical findings
- tx
- nephrotic syndrome
- effacement of podocytes
- selective proteinuria (albuminuria, not immunoglobulins)
- Steroids, excellent response b/c damaged is from cytokines from T cells
diabetic nephropathy
-stages of mech (3)
- eventually nephrotic syndrome
1. nonenzymatic glycosylation (NEG) of vascular BM, results in hyaline arteriosclerosis
2. efferent arteriole more affected, leading hyperfiltration (microalbuminuria)
3. eventually nephrotic syndrome from hyperfiltration damage - sclerosis of mesangium (kimmelstiel-wilson nodules)
Nephrotic syndromes: List them (6)
remember by groups of 2:
- minimal change disease (MCD)
- focal segmental glomerulosclerosis (FSGS)
- Membranous nephropathy
- Membranoproliferative glomerulonephritis
- DM2
- Systemic amyloidosis
minimal change disease
- what do you see on histology (H&E stain, and electron microscopy)
- what do you see in immunoflourescence (IF)
- H&E light microscopy: normal glomeruli (may see thin filtration layer)
- electron microscopy: effacement (flattening) of podocytes
- IF: negative b/c no immune complex deposits
Membranous nephropathy
-tx
poor response to steroids, progresses to chronic renal failure
minimal change disease
- etiology
- mechanism
- usu idiopathic, associated with Hodgkin’s Lymphoma
- mech: damage to podocytes by cytokines from T cells
C3 nephritic factor
-autoAb that stablizes C3 convertase, leading to overactivation of complement, inflammation, and low levels of circulating C3.
C3–> C3a + C3b
C3 convertase catalyzes this.
C3 nephritic factor stabilizes C3 convertase, overactivating it
what is most common cause of nephrotic syndrome in Hispanics and blacks?
-focal segmental glomerulosclerosis (FSGS)
Membranoproliferative glomerulonephritis
- mech
- types
- mech: immune complex deposition (granular IF)
- 2 types based on deposit location:
Type 1: subendothelial
Type 2: intramembranous
Membranous nephropathy
- etiology
- associated with what other problems? (4)
- usu idiopathic
- associated with:
1. Hep B, C
2. solid tumors
3. SLE
4. drugs (NSAIDs, penicillamine)
what is associated with C3 nephritic factor?
Type 2 Membranoproliferative glomerulonephritis
