Nephrotic Syndrome Complete

Question 1

What are the most common secondary causes of FSGS in adults? (6)

Answer 1

HIV, morbid obesity, chronic reflux nephropathy, heroin use, sickle cell and malignancy

• ↓ in renal mass will cause compensatory hypertrophy and hyperfiltration in the remaining glomeruli → intraglomerular HTN (hyperfiltration injury) → FSGS

Question 2

_____________ people have a genetic predisposition to develop FSGS

Answer 2

African American

Question 3

An important prognostic indicator of FSGS is ___________

Answer 3

Degree of proteinuria

Question 4

What kind of renal disease could you expect to develop in someone someone had a partial nephrectomy or a congenital unilateral renal agenesis?

Answer 4

FSGS

Question 5

Which renal disease causing nephrotic syndrome always has a secondary cause but mostly primary in adults?

Answer 5

Membranous nephropathy

Question 6

Localization of immune complexes in the sub- epithelial zone is seen in ________

Answer 6

Membranous nephropathy

Question 7

IC formation in situ / deposition of circulating IC’s is the favored theory for the deposition of IC’s in membranous nephropathy

Answer 7

IC formation in situ

Question 8

What is the pathogenesis of the cause of nephrotic syndrome in membranous nephropathy

Answer 8

The antigen antibody complexes will activate complement → C5b- C9 insertion into podocyte cell membrane and that can cause:

1. Detachment of podocyte due to alteration of cytoskeleton
2. Activation of epithelial and mesangila cells causing GBM growth or stimulation of proteases, oxidants and cytokines

Question 9

_______ antibody is present in 70% of patients with the primary form of membranous nephropathy

Answer 9

PLA2R

Question 10

Major secondary causes of membranous nephropathy

Answer 10

• hepatitis B
• syphilis
• malaria
• GI and lung carcinomas
• LUPUS

Question 11

What would you see on LM in someone with membranous nephropathy?

Answer 11

Diffuse thinking of GBM with a little ↑ in ceullaurty and spikes on silver stain

Question 12

What would you see in IF in membranous nephropathy? (Contrast between primary and secondary)

Answer 12

Primary: fine granular deposits along GBM of IgG, C3

Secondary: IgG, IgA, IgM, C3, C1q (full house)

Question 13

What would you see on EM for membranous nephropathy?

Answer 13

Subepithelial immune complex deposits and priliferationa Nd growth of new GBM → spikes

Question 14

“Spikes” formation in the GBM on EM is seen in which renal disease?

Answer 14

Membranous nephropathy

Question 15

What kind of deposits would you see on EM with membranous nephropathy: granular or fine?

Answer 15

Granular

Question 16

___________ and ___________ are common features seen in diabetic nephropathy

Answer 16

Mesangial expansion and thickening of GBM (early lesion)

Different from membranous nepropathy because there are NO SPIKES IN DIABETIC NEPHROPATHY

Question 17

What are some later lesions seen in diabetic nephropathy

Answer 17

Diffuse global glomerulosclerosis with ↑ in mesangial matrix and thinking of GBM

Question 18

Kimmelsteil-Wilson nodules are seen in _________

Answer 18

Diabetic nephropathy and is indicative of nodular glomerulosclerosis because the nodules contain lipids and fibrin

Question 19

Initially, hyperglycemia leads to __________ and ____________ but after 10-20 years there is _________

Answer 19

Hyperfiltration (↑ GFR) and ↑ glomerular hydrostatic pressure;

Persistent and progressive proteinuria with highly variable decline in GFR

Question 20

Is there an immune or inflammatory response in amyloidosis?

Answer 20

NO

Question 21

Organ damage and dysfunction in amyloidosis is due to __________

Answer 21

Infiltration by amyloid fibrin and replacement of normal organ architecture

Question 22

What kind of patients would you expect to have AA amyloid in their kidneys?

Answer 22

Patients with long standing infection or inflammation such as RA, Tb, Behcet syndrome, crohn’s disease, osteomyelitis, RCC, Hodgkin’s lymphoma

Question 23

In LM of amyloidosis in the kidneys will see nodular, amorphous hyaline material in the _______- and ________

Answer 23

Mesangium; capillary loops

Question 24

EM shows subendotheilal and mesangial fibrils in the kidney, what is the disease?

Answer 24

Amyloidosis

Question 25

Prognosis of amyloid in the kidneys

Answer 25

Very poor; many die of end organ failure from amyloid deposition