Nephrotic Syndrome 1 Flashcards
What are some key features of nephrotic syndrome?
edema, proteinuria, hypoalbuminemia, hyperlipidemia, non- inflammatory, inactive urinary sediment, normal or mild elevation of serum creatine
What is the key cell involved in nephrotic syndrome?
visceral epithelial cells – podocytes
What are some key features of nephritic syndrome?
acive urinary sediment, dysmorphic RBCs, RBC casts in urine, Inflammation, HTN, elevated serum creatinine, crescents on kidney biopsy
What is the key cell involved in nephritic syndrome?
endothelial cell
What two diseases are associated w/ podocyte injury?
minimal change disease and focal segmental golmerulosclerosis
What is involved w/ subepithelial space immune complex formation and complement activation?
membranous nephropathy and late stage of post-infectious glomerulonephritis
What is associated w/ glomerular capillary wall depostion diseases?
amloidosis, light chains deposition disease, diabetic nephropathy
What is involved w/ subendothelial space or mesangial immune complex formation and complement activation? Nephritic syndrome
- post-infectious glomerulonephritis
- IgA nephropathy
- lupus nephritis
What is a disorder that has Ab’s directed at the glomerular BM?
goodpastures
What can lead to necrotizing injury and inflammation of vascular and GCW?
ANCA diseases
What is associated w/ IgA nephropathy?
macroscopic hematuria
What are the different clinical presentations of glomerular disease?
asymptomatic, macroscopic hematuria, nephrotic syndrome, nephritic syndrome, rapidly progressive glomerulonephritis, and chronic glomerulonephritis
What causes immune complexes to form and get trapped in glomeruli?
- high plasma flow rate
- high intraglomerular pressure
- high glomerular hydraulic conductivity
What is the spectrum of immune complex disease dependent on?
nature of Ag involved and site of immune complex
If there is generalized edema, what should on look for next?
proteinuria
What can been seen in urinary sediment?
oval fat bodies and maltese cross
T or F. One can see xanthelasma in nephrotic syndrome.
T
How can one make sense of nephrotic and nephritic syndrome if that have some common findings?
- Nephrotic syndrome - abnormal glomerulus w/out inflammation
- Nephritic syndrome – abnormal golmerulus w/ inflammation
What are the 3 purposes of a kidney biopsy?
- diagnosis
- prognosis
- guide therapy
Buzz word - foot process effacement = ?
minimal change disease (but not specific)
spike and dome = ?
membranous nephropathy
subepithelial humps =?
post-infectious glomerulonephritis
tram tracks = ?
membranoproliferative glomerulonepritis
basketweave = ?
alport syndrome
wire loops = ?
lupus nephritis
onion-skin = ?
hypertensive nephropathy or scleroderma
What are 3 things that are filtration in glomerulus depends on?
- charge
- size
- shape of substance
Why don’t we normally filter albumin even though it is the right size to be filtered?
the podocytes are negatively charged which repels albumin which is also negatively charged
What is found in the BM of the glomerulus?
- laminin
- COLLAGE IV
- heparan sulfate PGs
- integrin
- agrin
What is the size restriction for filtration?
- uncharged macromolecules 4nm completely restricted
What proteins of the slit diaphragm are involved in proteinuria?
- podocin
- TRPC6
- Actinin-4
- NEPH-1
- Cadherine
- FAT
- Nephrin
What happens to the glomerular permeability in nephrotic syndrome?
- lower excretion of small MW substances
- increased clearance of large MW substances
- increased excretion of IgG due to loss of size barrier
What is seen w/ glomerular proteinuria?
albumin is dominany protein in urine
What is seen w/ tubular proteinuria?
low MW proteins
What protein does urine dipstick measure?
albumin
How much protein is normally excreted by a healthy kidney?
40-80mg/day, 150mg/day is the upper range of normal
What is the normal urine albumin/creatinine ratio?
<30 mg/g
What is an abnormal urine dip stick reading?
1+, 2+, 3+
What is the standard proteinuria test?
24-hr urine collection, normal <150mg
What is the general management for primary nephrotic syndrome?
Goal is to preserver kidney function
How do you treat the proteinuria?
- Supportive measures involved controling the HTN
- Steroids
- Immunosuppressive drugs
What can be used to control HTN?
- low salt diet
- ACE inhibitor
- Angiotensein receptor blocker
What is the function of the glomerular capillary wall?
selective permeability barrier
What 3 layers make up the glomerular capillary wall?
- endothelium
- glomerular BM
- podoctyte visceral epithelial cell layer
