Nephrotic Syndrome Flashcards
What is nephrotic syndrome?
Glomerular disorders characterized by proteinuria characterized by:
Hypoalbuminemia - pitting edema
Hypogammaglobulinemia - increased ris kof infection
Hypercoagulable state - due to loss of antithrombin III
Hyperlipidemia and hypercholesterolemia - may result in faty casts in urine
What is the most common cause of nephrotic syndrome in children?
Minimal change disease
What is the etiology of minimal change disease?
Idiopathic usually
May be associatedwith Hodgkin Lymphoma
What is seen on H&E stain, EM, and IF of MCD?
H&E: Normal glomeruli; Lipid may be seen in proximal tubule cells
EM: effacement of foot processes
IF: no immune complex deposits, negaitive IF
What kind of protein is lost in the urine in MCD?
Selective proteinuria for albumin. No loss of immunoglobulin
Tx for MCD
Excellent response to steroids (damage is mediated by cytokines from T cells)
What is focal segmental glomerulosclerosis (FSGS)?
MCC of nephrotic syndrome in hispanics, and African Americans
Etiology of FSGS
Usually idiopathic
May be associated with HIV, heroin use, and sickle cell disease
What is seen on the H&E stain, EM and IF of FSGS?
H&E stain - Focal (some glomeruli) and segmental (involving only part of the glomerulus) sclerosis
EM - effacement of foot processes
IF - negative IF (no immune complex deposits)
Tx for FSGS?
Poor response to steroids - usually progresses to chronic renal failure
MCC of nephrotic syndrome in Caucasian adults?
membranous nephropathy
Cause of membranous nephropathy?
Usually idiopathic
May be associated with Hep B/C, solid tumors, SLE or drugs
Does SLE normally develop nephrotic or nephritic syndrome?
SLe usually develops nephritic syndrome but when it does develop nephrotic, it will be membranous
What is seen on H&E stain, EM and If of membranous nephropathy?
H&E - thick glomerular BM on H&E
IF - immune complex deposition
EM - subepithelial deposits with “spike and dome” appearance
Does membranous nephropathy respond to steroids?
Poor response to steroids
Progresses to chronic renal failure
What proliferates in membranoproliferative glomerulonephritis?
Mesangial cells proliferate
What is seen on H&E stain and IF of membranoproliferative glomerulonephritis?
H&E - thick glomerular BM often with tram-track apperance (more often seen with Type I)
IF - granular due to immune complex deposits
What are the two types of membranoproliferative glomerulonephritis?
Type I - subendothelial deposits; associated with HBV and HCV
Type II (dense deposit disease) - intramembranous; associated iwth C3 nephritic factor
Does MPGN lead to nephritic or nephrotic syndrome?
Either or both
Does MPGN respond to steroids?
Poor response; progresses to chronic renal failure
How does DM lead to nephrotic syndrome?
High serum glucose causes nonenzymatic glycosylation of the vascular BM resulting in hyaline arteriolosclerosis
This affects the efferent arteriole more than the afferent arteriole leading to a high GFR
Eventually progresses to nephrotic syndrome due to sclerosis of mesangial cells
How is DM nephrotic ysndrome characterized?
Characterized by sclerosis of the mesangium with formation of Kimmelstiel-wilson nodules
Tx for DM nephrotic syndrome
ACE inhibitors
How does systemic amyloidosis cause nephrotic syndrome?
Kidney is the most commonly involved organ in systemic amyloidosis
Amyloid deposits in the mesangium, resulting in nephrotic syndrome
How is systemic amyloidosis nephrotic syndrome characterized?
Apple-green birefringence under polarized light after staining with congo red
what conditions cuse both nephritic and nephrotic syndrome?
Mebranoproliferative glomerulonephritis
Diffuse proliferative glomerulonephritis
