Path - Nephrotic Syndrome I Flashcards
Compare edema in Nephrotic vs Nephritic syndrome
Nephrotic - edema prominent
Nephritic - edema mild
Compare proteinuria in Nephrotic vs Nephritic syndrome
Nephrotic - prominent
Nephritic - mild
Compare urinary sediment in Nephrotic vs Nephritic syndrome
Nephrotic - inactive
Nephritic - active = dysmorphic RBCs and RBC casts (signs of inflammation)
Compare blood pressure in Nephrotic vs Nephritic syndrome
Nephrotic - normal
Nephritic - HTN
Compare creatinine in Nephrotic vs Nephritic syndrome
Nephrotic - normal or mild elevation in serum creatinine
Nephritic - elevated serum creatinine
Key cell involved in Nephrotic syndrome?
Visceral epithelial cell (aka podocyte)
Key cell involved in Nephritic Syndrome?
Endothelial cell
Is inflammation present in nephrotic or nephritic syndrome? What UA finding supports this
Nephritic syndrome = inflammation (presence of dysmorphic RBC and RBC casts)
Albumin & Lipid levels in Nephrotic Syndrome?
Hypoalbubinemia
Hyperlipidemia
Most common type of cast found in urine? Name 1 instance you would observe this?
Hyaline casts, seen commonly in dehydration
Buzz words. Foot process effacement/fusion?
Minimal change disease, focal segmental glomerulosclerosis
Buzz words. Spike and dome?
Membranous nephropathy
Buzz words. Subepithelial humps?
Post infectious (streptococcal usually) glomerulonephritis
Buzz words. Tram tracks?
Membranoproliferative glomerulonephritis
Buzz words. Basketweave?
Alport syndrome
Buzz words. Wire loops?
Lupus nephritis
Buzz words. Onion skin?
Hypertensive nephropathy (arterioles) or scleroderma (larger vessels)
Immunofluorescence is negative in what 2 nephrotic syndromes? Why?
Minimal change disease, focal segmental glomerularnephritis. No immune complex deposition, only foot processes effacement/fusion
Xanthelasma present on eyelids in nephrotic syndrome. Why?
Due to hypercholesterolemia/hyperlipidemia seen in nephrotic syndrome
Nephrotic syndrome. Albumin level in blood (hyper or hypo)?
Hypoalbuminemia
Nephrotic syndrome. Gammaglobuin level in blood?
hypogammaglobulinemia (increased risk for infection)
Nephrotic syndrome. Coaguable state? Why?
Hypercoaguable due to loss of antithrombin III
Hyperlipidemia in Nephrotic Syndrome results in what in urine?
Fatty casts in urine
Maltese cross pattern. What is it?
Fatty casts in urine that indicate presence of cholesterol in urine
Albumin radius is 3.6 nm. What is the significance of this?
Glomerular filtration barrier usually restricts molecules < 4 nm. Therefore, if not for the charge barrier on the membrane, albumin could leak through, producing hypoalbuminemia. KEY POINT: Charge is more important than size
Alpha-actinin 4 defect = ?
Autosomal dominant FSGS
Podocin defect = ?
Autosomal recessive steroid resistant FSGS
TRPC6 defect = ?
Autosomal dominant FSGS
NEPH 1 defect = ?
autosomal recessive Finish type, resembles Minimal Change Disease
Beta 2 microglobulin filtration status (in normal non-nephrotic syndrome)?
Almost completely unrestricted to filter through
Albumin filtration status (in normal non-nephrotic syndrome)?
Some albumin filtered through, but reabsorbed by proximal tubular cells
IgG filtration status (normally, in non nephrotic syndrome)?
Completely restricted, molecular radius large (nearly 1.5x the size of albumin)
Compare filtration status of small vs large dextran in nephrotic syndrome
lower excretion of small molecular weight dextran due to loss of filtration surface area and increased clearance of large molecular weight dextrans
IgG filtration status in nephrotic syndrome?
Increased excretion of IgG (neutral charge) due to loss of size barrier
Normal level of albumin excretion in healthy kidney?
40-80 mg/day
Urine dipstick measures what protein? What concentration of this protein must be present for dipstick to pick it up?
Albumin, >300 mg/day
Microalbuminuria = what level of albumin in urine? Significance of this?
= 30-300 mg/day; however, urine dipstick won’t pick it up since its below the dipstick threshold of 300 mg/day
normal spot urine albumin/creatinine ratio?
< 0.15
Urine albumin/creatinine ratio is good indicator of what?
Very good indicator of 24 hour urine profile for patient
Tamm-Horsfall mucoprotein normal excretion amount?
normal = 30-50 mg /day
Nephrotic range 24 hour urine proteinuria?
> 3.5 grams
Nephrotic range spot urine protein/creatinine ratio?
>3.5
2 main treatment methods of primary nephrotic syndrome?
- Provide supportive measures
- Provide disease modifiers
3 supportive measures of primary nephrotic syndrome?
Want to control HTN via:
low salt diet, ACEI, & ARB
2 main disease modifiers of primary nephrotic syndrome?
- Steroids
- Immunosuppresive drugs (cyclophosphamide, cyclosporin, mycophenolate mofeitil, tacrolimus)
Name the 4 immunosuppresive drugs used in primary nephrotic syndrome?
- Cyclophosphamide
- Cyclosporin
- Mycophenolate Mofeitil
- Tacrolimus
Treatment method for secondary nephrotic syndrome?
- Treat the cause
- provide supportive measures (same as primary nephrotic syndrome) with low salt diet, ACEI, ARB
Immunosuppresion therapy for secondary nephrotic syndrome?
None. Secondary nephrotic syndrome does not respond to steroids or immunosuppressants.
