Nephrotic Syndrome

Question 1

Key characteristics of nephrotic syndrome

Answer 1

1. Proteinuria (>3.5 g/day)
2. Hypoalbuminemia
3. Edema
4. Hyperlipidemia

Associated with hyper coagulable state due to ATIII loss in urine

Question 2

Minimal change disease

Answer 2

Only see abnormalities (podocyte effacement) through EM, IF: -

Only nephrotic syndrome that has excellent response to corticosteroids

Associated with Hodgkin Lymphoma

Due to cytokine release that damages foot processes

Question 3

FSGS

Answer 3

Associated with HIV, sickle cell, and heroin abuse

EM: effacement of foot processes; IF: -

Does not respond to steroids

Progression of MCD if not responsive to steroids

Question 4

Membranous Nephropathy

Answer 4

Assoc with Caucasians, SLE (if they present with nephrotic syndrome vs. DPG if present with nephritic), Hep B and C

Immune complex deposition (membranous = thickening of membrane due to immune complexes)

IF: granular; EM: spike and dome appearance (due to immune complexes in sub epithelial region)

Question 5

Membranoproliferative Glomerulonephritis

Answer 5

Membranous = immune complexes, granular IF appearance

Can cause nephrotic or nephritic syndrome (or both)

Tram tracking (due to mesangial cells that divide the sub endothelial or sub BM deposits)

Type I (sub-endo): Assoc with HBV and HCV; tram-tracking

Type II (within BM): C3 nephritic factor (auto-antibody against C3 convertase; over activation and depletion of C3)

Question 6

Nephrotic Syndrome categorization

Answer 6

1. Effacement of foot processes: MCD and FSGS
2. Immune complex deposits: Membranous and Membranoproliferative
3. Systemic diseases: DM and Amyloidosis

Question 7

DM glomerulonephropathy

Answer 7

Hyaline arteriolosclerosis (generally in efferent arteriole) via due to non-enzymatic glycosylation (NEG)

NEG is the first change seen in diabetes

Kimmelstein-Wilson nodules: regions (nodules) of sclerosis that are pathognomonic for DM nephrotic syndrome

ACE-Is (e.g. Lisinopril) are good for DM because it blocks angiotensin II (which also normally constricts the efferent arteriole)

Question 8

Systemic Amyloidosis

Answer 8

Kidney is most often affected

Amyloid deposits

Apple-green birefringence under polarized light