Nephrotic Syndrome 2 Flashcards
What are the 2 podoctyopathies?
minimal change disease and focal segmental glomerulosclerosis
Who usually gets minimal change disease?
very young and very old - bimodal distribution
What is the most common cause of nephrotic syndrome in kids?
minimal change disease
What other clinical findings are common in minimal change disease?
- insidious onset of edema
- normal blood pressure
- usually normal renal function
- highly selective proteinuria - albumin
What is thought to be the pathogenesis for minimal change disease?
not clear but thought to be mediated by a T cell that secretes a circulating permeability factor that causes podocyte damage
What is seen w/ minimal change disease for pathology?
effacement and detachment of foot processes
What is the treatment for minimal change disease?
oral glucocorticoids
Who responds well to steriods in minimal change disease?
kids
If there is a poor response to steroids what should be done?
- look for other cause (especially in children)
2. unsampled focal segmental glomerulosclerosis
What are some clinical findings for focal segmental glomerulosclerosis?
- high BP
2. low albumin –> proteinuria
How many of the pts w/ focal segmental glomerulosclerosis develop end stage kidney disease?
50%
What is Supar?
the circulating factor that binds to beta2 integrin and causes effacement of podocytes
What are some secondary causes of focal segmental glomerulosclerosis?
- familial – mutation in genes alpha-actinin-4, podosin, TRCP6, and APOL1
- infection - HIV or Parvo virus
- drugs - pamidronate, heroin, Li
- Loss of nephron mass
Why are people of African descent susceptible to getting focal segmental glomerulosclerosis?
b/c 40% of them have mutations in APOL1 making them susceptible to African sleeping sickness
Why does hyalinosis occur in FSG?
accumulation of leaked plasma proteins and lipids
T or F. Adhesion of involved segment to Bowman Capsule does occur in FSG?
T
What are subtypes of FSG?
- collapsing
- cellular
- tip
- Perhiliar
- not otherwise specified (42%)
Which of the subtypes of FSG will most likely obtain remission?
Tip
What is found in the collapsing type of FSG?
collapsed BM and adhesions common
What shows up on immunofluoresence for FSG?
nothing really