Nephrotic syndrome Pathology (different from above) Flashcards
- ) Nephrotic Syndrome is characterized by what 4 things (the last lecture had 5 if you’re confused)?
- ) How many do you need to diagnose nephrotic syndrome?
- ) What is nephrotic syndrome in a phrase?
- ) -proteinuria > 3.5
- hypoalbuminemia
- edema
- hyperlipidemia - ) 3
- ) protein leak caused by increased glomerular permeability
T or F BM does not separate endo cell from from mesangial cell
T. This means that fluid and immune complexes can get into the mes. cell from cap.
what are some major complications of nephrotic syndrome?
Increased infections
Malnutrition
Thrombosis (ATIII deficiency)
Where are you especially susceptible to get thrombosis? why?
renal v. bc ATIII level is lowest at the area leaving the kidney
What is epithelial cell detachment and how does it apply to podocytes?
normally podocytes are attached to BM and have an overall negative charge. Toxins, antibodies etc. can cause damage to the BM and affect the podocyte. The podocyte then secretes stuff and takes off its feet (called detachment or retraction). causes holes and leakiness.
what primary glomerular nephrotic disease is most common in children. What about in adults?
children- minimal change disease
Adults- membranous glomerulopathy and focal segmental glomerulosclerosis
What are good questions to ask yourself when looking at a histo slide of the glomerulus?
Is it hyper cellular? Are there open cap loops? are there Inflammatory Cells? Is BM of tubule the same size as BM on cap? Scarring?
Foot process effacement is a(n) _______ change best seen on ____
ultrastructural
EM
What protein deficiencies do you get in nephrotic syndrome and what are the consequences?
This is a long one but they all pretty much make sense. Besides, all you have to do is remember HAGAI from the Bible (just don’t spell it with two G’s like it does in the Bible).
Protein Deficiency:
HD lipoprotein= Accel atherogenesis
Antithrombin III, etc. = Clotting tendency especially Renal vein thrombosis
General protein loss= Protein malnutrition, Growth retardation
Albumin= Edema, hypotension, Drug toxicity
Immunoglobulin/complement= Susceptibility to infections
Also you can get Hypercholesteremia, hyponatremia/hypokalemia, and progression to CRF but we don’t totally understand how these three work.
T or F Nephrotic diseases are primarily non-inflammatory?
T. nephritic is mostly inflammatory.
In nephrotic, circulating factors bind to and can mess with podocytes (also called Glomerular epithelial cell) membranes without fixing complement (as in MCD). Or they can act by fixing complement via anti-GEC Ab’s (as in membranous nephropathy).
minimal change nephropathy (or disease)
- what is its classic presentation?
- what morphologic data is seen?
- What pathogenic data is seen?
- what therapy is given?
- prognosis?
Focus on the cards of this type mostly The learning objectives focused on these.
- nephrotic syndrome in YOUNG kids.
- nothing really or only minimal change (name comes from this)
- unknown- some circ. factor:
- EM: foot process effacement
- IF: negative - steroids
- good
Focal Sclerosis (FSGS- focal segmental glomerular sclerosis)
- what is its classic presentation?
- what morphologic data is seen?
- What pathogenic data is seen?
- what therapy is given?
- prognosis
- nephrotic syndrome most common in young African American adults or associated with HIV/IV drug use
- focal segmental glomerular sclerosis
- caused by circ. factor (unknown which), hyperfiltration, and genetics.
- steroids
- some progress to ESRD (this is the answer to every nephrotic disease prognosis except MCD).
there are various forms of FSGS. What are they
- idiopathic
- HIV associated nephropathy
- heroin nephropathy
- secondary FSGS (this is hyperfiltration nephropathy which occurs with any disease that knocks out a bunch of nephrons and leaves the rest to ‘hyperfiltrate’ or do the work of the dead nephrons which basically leads to nephron burnout and proteinuria.)
Membranous Glomerulopathy
- what is its classic presentation?
- what morphologic data is seen?
- What pathogenic data is seen?
- what therapy is given?
- prognosis?
- nephrotic syndrome (typically occurs in adults).
- GMB thickened (has thickened looks with ‘spikes’ but it is leaky).
- chronic immune complex:
- EM: subepithelial deposits
- IF: Granular GBM - steroids
- some progress to ESRD
Is Membranous Glomerulopathy a primary or a secondary disease?
While this disease can be primary or idiopathic, it can also be a secondary disease of lupus or some other systemic disorder. So you have to do the work to see if there’s anything else (look for “bugs, drugs, tumors, and rheum”).
