Nephrotic Syndrome Complete Flashcards
What are the most common secondary causes of FSGS in adults? (6)
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
_____________ people have a genetic predisposition to develop FSGS
African American
An important prognostic indicator of FSGS is ___________
Degree of proteinuria
What kind of renal disease could you expect to develop in someone someone had a partial nephrectomy or a congenital unilateral renal agenesis?
FSGS
Which renal disease causing nephrotic syndrome always has a secondary cause but mostly primary in adults?
Membranous nephropathy
Localization of immune complexes in the sub- epithelial zone is seen in ________
Membranous nephropathy
IC formation in situ / deposition of circulating IC’s is the favored theory for the deposition of IC’s in membranous nephropathy
IC formation in situ
What is the pathogenesis of the cause of nephrotic syndrome in membranous nephropathy
The antigen antibody complexes will activate complement → C5b- C9 insertion into podocyte cell membrane and that can cause:
- Detachment of podocyte due to alteration of cytoskeleton
- Activation of epithelial and mesangila cells causing GBM growth or stimulation of proteases, oxidants and cytokines
_______ antibody is present in 70% of patients with the primary form of membranous nephropathy
PLA2R
Major secondary causes of membranous nephropathy
- hepatitis B
- syphilis
- malaria
- GI and lung carcinomas
- LUPUS
What would you see on LM in someone with membranous nephropathy?
Diffuse thinking of GBM with a little ↑ in ceullaurty and spikes on silver stain
What would you see in IF in membranous nephropathy? (Contrast between primary and secondary)
Primary: fine granular deposits along GBM of IgG, C3
Secondary: IgG, IgA, IgM, C3, C1q (full house)
What would you see on EM for membranous nephropathy?
Subepithelial immune complex deposits and priliferationa Nd growth of new GBM → spikes
“Spikes” formation in the GBM on EM is seen in which renal disease?
Membranous nephropathy
What kind of deposits would you see on EM with membranous nephropathy: granular or fine?
Granular
___________ and ___________ are common features seen in diabetic nephropathy
Mesangial expansion and thickening of GBM (early lesion)
Different from membranous nepropathy because there are NO SPIKES IN DIABETIC NEPHROPATHY
What are some later lesions seen in diabetic nephropathy
Diffuse global glomerulosclerosis with ↑ in mesangial matrix and thinking of GBM
Kimmelsteil-Wilson nodules are seen in _________
Diabetic nephropathy and is indicative of nodular glomerulosclerosis because the nodules contain lipids and fibrin
Initially, hyperglycemia leads to __________ and ____________ but after 10-20 years there is _________
Hyperfiltration (↑ GFR) and ↑ glomerular hydrostatic pressure;
Persistent and progressive proteinuria with highly variable decline in GFR
Is there an immune or inflammatory response in amyloidosis?
NO
Organ damage and dysfunction in amyloidosis is due to __________
Infiltration by amyloid fibrin and replacement of normal organ architecture
What kind of patients would you expect to have AA amyloid in their kidneys?
Patients with long standing infection or inflammation such as RA, Tb, Behcet syndrome, crohn’s disease, osteomyelitis, RCC, Hodgkin’s lymphoma
In LM of amyloidosis in the kidneys will see nodular, amorphous hyaline material in the _______- and ________
Mesangium; capillary loops
EM shows subendotheilal and mesangial fibrils in the kidney, what is the disease?
Amyloidosis
Prognosis of amyloid in the kidneys
Very poor; many die of end organ failure from amyloid deposition
