nephrotic syndrome

Question 1

what are the basic principles of nephrotic syndrome

Answer 1

glomerular disorders characterized by loss of protein >3.5g/day. hypoalbuminemia (pitting edema), hypogammaglobinemia (increased risk of infection), hypocoaguable state (due to the loss of ATIII), hyperlipemia and hypercholesterolemia (fatty casts in urine)(liver dumping).

Question 2

minimal change disease MCD characteristics?

Answer 2

most common nephrotic in children.

Question 3

what is the most common cause of minimal change disease?

Answer 3

usually idiopathic, may be associated with hogkins lymphoma.

Question 4

what do the glomeruli look like on the HE stain for MCD?

Answer 4

normal. there maybe some lipid in the proximal tubule.

Question 5

what test will determine MCD and what does it look like?

Answer 5

EM. look for effacement of the foot processes of the podocytes.

Question 6

can IF be used to determine MCD?

Answer 6

NO. there will be no Ig deposits and thus negative IF.

Question 7

what is another common symptom that helps to distinguish MCD?

Answer 7

there will be selective hypoalbuminuria. NO gammaglobinuria. there is an excellent response to steroids.

Question 8

what does MCD progress to if it does?

Answer 8

focal segmental glomerulonephritis.

Question 9

focal segmental glomerulosclerosis characterisitics?

Answer 9

nephrotic syndrome type. usually idiopathic, but does have some associations. effacement of podocyte foot processes on EM. NO immune complex deposition.

Question 10

who common gets FSGS?

Answer 10

HIV, hispanics, african americans, drug addicts (heroin), sickle cell disease.

Question 11

is there immune complex deposition in FSGS?

Answer 11

NO. IF is negative.

Question 12

does FSGS respond to steroids?

Answer 12

no. there is a poor response. usually progresses to chronic renal failure.

Question 13

membranous nephropathy characterisitics

Answer 13

most common cause of nephrotic in caucasian males. usually idiopathic but can be associated. it has a thick basement membrane on HE. this is caused by immune deposition.

Question 14

what is membranous associated with?

Answer 14

SLE, hep B/C, solid tumors, drugs (NSAIDs and penicillamine).

Question 15

what is the appearance of membranous on IF?

Answer 15

typically granular.

Question 16

what is the EM finding for membranous nephropathy?

Answer 16

there will be sub epithelial deposits that cause a spike and dome appearance.

Question 17

how is membranous treated?

Answer 17

there is a POOR response to steroids. usually progresses to chronic renal failure.

Question 18

membranoproliferative glomerulonephritis characterisitics

Answer 18

thick basement membrane on HE with a tram-track appearance. this is due to immune complex deposition (granular IF). there are two types.

Question 19

type I membranoproliferative glomerulonephritis characteristics

Answer 19

subendothelial. associated with Hep B/C

Question 20

type II membranoproliferative glomerulonephritis characteristics

Answer 20

also called dense deposit disease. this is intramembranous. associated with C3 nephritic factor.

Question 21

what is C3 nephritic factor?

Answer 21

this is autoantibody that stabilizes a C3 convertase. this leads to an over activation of complement and thus hypocomplementemia of C3.

Question 22

how to treat membranoproliferative glomerulonephritis

Answer 22

there is a poor response to steroids and it usually progresses to renal failure.

Question 23

diabetes mellitus characterisitics

Answer 23

high serum glucose leads to non enzymatic glycosylation of vascular basement membrane resulting in hyaline arteriolosclerosis. the glomerular efferent arteriole is preferentially affected leading to hyper filtration injury. this leads to microalbuminuria that eventually leads to nephrotic syndrome. there is sclerosis of the mesangium and formation of kimmelstiel-wilson nodules.

Question 24

how do we treat diabetic nephropathy?

Answer 24

ACEi will slow the progression of the hyper filtration injury.

Question 25

systemic amyloidosis characteristics

Answer 25

kidney is the most commonly involved organ in systemic amyloidosis. amyloid will deposit in the mesangium resulting in nephrotic syndrome. characterized by apple-green birefringence and polarized light after congo-red stain

Question 26

MCD by light microscopy?

Answer 26

no lesion.

Question 27

MCD via IF?

Answer 27

no fluorescence, no change, no immune deposits

Question 28

MCD by EM?

Answer 28

no immune deposition, but there will be podocytes effacement and foot process fusion.

Question 29

FSGS by light microscopy

Answer 29

focal and segmental glomerular scarring or consolidation possibly some glomerular hypertrophy.

Question 30

FSGS by IF?

Answer 30

there are no immune deposits in FSGS

Question 31

FSGS by EM?

Answer 31

there is no deposition, but there will be foot process effacement, hyalinosis, and lipid vacuolization.

Question 32

membranous GN by light microscopy

Answer 32

diffuse, global capillary wall thickening.

Question 33

membranous GN by IF

Answer 33

diffuse capillary wall immunoglobulin staining

Question 34

membranous GN by EM

Answer 34

diffuse, supepithelial dense deposits

Question 35

membranoproliferative GN by light microscope.

Answer 35

capillary wall thickening and endocapillary hypercellurity.

Question 36

membranoproliferative GN by IF

Answer 36

diffuse capillary wall complement

Question 37

membranoproliferative GN by EM

Answer 37

type I: subendothelial dense deposits; type II: intramembranous dense deposits.