Nephrotic Syndrome II Flashcards
What is an ApoL1 mutation?
mutations that cause truncations of apolipoprotein L1 which confers resistance to trypanosomiasis (african sleeping sickness) but leads to susceptibility to focal segmental glomerulosclerosis
What in the name of the good lord is soluble urokinase-type plasminogen activator receptor?
a circulating factor that binds to the podocyte anchor beta3-integrin and could be a common cause of focal segmental glomerulosclerosis.
What is M-type phospholipase A2 receptor?
podocyte membrane component that forms immune complexes with autoantibodies probably causing most primary membranous nephropathy
What are the two categories of Nephrotic syndromes?
- ) podocytopathies- those that occur on visceral epithelium
- ) Membranous nephropathy- immune complex deposition
Two different podocytopathies?
- ) Minimal Charge
2. ) Focal segmental glomerulosclerosis
Two different types of membranous nephropathy?
- ) Endogenous antigen immune complex
2. ) Filtered antigenimmune complex
What are some highlights of the epidemiology of minimal change disease?
Affects the very young and very hold. 75% of nephrotic syndrom in children. 25% in adults. more common in whites than blacks. Children = 2:1 male to female.
Minimal change disease is a nephrotic syndrome so it involves what
Proteinuria, ding dong
What type of protein is predominant in minimal change disease?
Albumin
How do you definitively diagnose minimal change disease?
renal biopsy….ight microscopy is negative but electron microscopy shows effacement of the podocyte foot processes
How do you treat minimal change disease?
glucocorticoids.
So if you see a kid with nephrotic syndrome it is most likely what?
Minimal change disease
What is focal segmental glomerulosclerosis?
Involves deposition of a basement membrane like material associated with podocyte injury only in certain glomeruli (hence focal) and only parts of that glomerulus (hence segmenal)
What does FSG progress to>
Sclerosis
Epidemiology of FSG?
Most common in adults, blacks, males. 64% of nephrotic syndrome in black adults
What mutation in African Americans puts them at risk for FSG?
ApoL1
Can FSG be both secondary and Primary?
Yes…can follow things like glomerulonephritis, HIV, drug use, etc…
What circulating factor could be a cause of FSG?
suPAR…soluble urokinase-type plasminogen activator binds to the podocyte anchor beta 3 integrin and can cause focal segmental glomerulosclerosis
What is the type of FSG with the worst prognosis?
collapsing glomerulopathy
What are the different subtypes of FSG>
Collapsing glomerulopathy, vascular pole, urinary pole, Glomerular tip (white ppl)
Collapsing glomerulopathy is most predominant among what population?
African Americans, particularly kids
Collapsing glomerulopathy is often associated with what?
HIV and drug use
treatment of FSG?
corticosteroids
Prognosis is worse in FSG or minimal change>?
FSG…importatn to differntiate between them
