Path - Nephrotic Syndrome I

Question 1

Compare edema in Nephrotic vs Nephritic syndrome

Answer 1

Nephrotic - edema prominent

Nephritic - edema mild

Question 2

Compare proteinuria in Nephrotic vs Nephritic syndrome

Answer 2

Nephrotic - prominent

Nephritic - mild

Question 3

Compare urinary sediment in Nephrotic vs Nephritic syndrome

Answer 3

Nephrotic - inactive

Nephritic - active = dysmorphic RBCs and RBC casts (signs of inflammation)

Question 4

Compare blood pressure in Nephrotic vs Nephritic syndrome

Answer 4

Nephrotic - normal

Nephritic - HTN

Question 5

Compare creatinine in Nephrotic vs Nephritic syndrome

Answer 5

Nephrotic - normal or mild elevation in serum creatinine

Nephritic - elevated serum creatinine

Question 6

Key cell involved in Nephrotic syndrome?

Answer 6

Visceral epithelial cell (aka podocyte)

Question 7

Key cell involved in Nephritic Syndrome?

Answer 7

Endothelial cell

Question 8

Is inflammation present in nephrotic or nephritic syndrome? What UA finding supports this

Answer 8

Nephritic syndrome = inflammation (presence of dysmorphic RBC and RBC casts)

Question 9

Albumin & Lipid levels in Nephrotic Syndrome?

Answer 9

Hypoalbubinemia

Hyperlipidemia

Question 10

Most common type of cast found in urine? Name 1 instance you would observe this?

Answer 10

Hyaline casts, seen commonly in dehydration

Question 11

Buzz words. Foot process effacement/fusion?

Answer 11

Minimal change disease, focal segmental glomerulosclerosis

Question 12

Buzz words. Spike and dome?

Answer 12

Membranous nephropathy

Question 13

Buzz words. Subepithelial humps?

Answer 13

Post infectious (streptococcal usually) glomerulonephritis

Question 14

Buzz words. Tram tracks?

Answer 14

Membranoproliferative glomerulonephritis

Question 15

Buzz words. Basketweave?

Answer 15

Alport syndrome

Question 16

Buzz words. Wire loops?

Answer 16

Lupus nephritis

Question 17

Buzz words. Onion skin?

Answer 17

Hypertensive nephropathy (arterioles) or scleroderma (larger vessels)

Question 18

Immunofluorescence is negative in what 2 nephrotic syndromes? Why?

Answer 18

Minimal change disease, focal segmental glomerularnephritis. No immune complex deposition, only foot processes effacement/fusion

Question 19

Xanthelasma present on eyelids in nephrotic syndrome. Why?

Answer 19

Due to hypercholesterolemia/hyperlipidemia seen in nephrotic syndrome

Question 20

Nephrotic syndrome. Albumin level in blood (hyper or hypo)?

Answer 20

Hypoalbuminemia

Question 21

Nephrotic syndrome. Gammaglobuin level in blood?

Answer 21

hypogammaglobulinemia (increased risk for infection)

Question 22

Nephrotic syndrome. Coaguable state? Why?

Answer 22

Hypercoaguable due to loss of antithrombin III

Question 23

Hyperlipidemia in Nephrotic Syndrome results in what in urine?

Answer 23

Fatty casts in urine

Question 24

Maltese cross pattern. What is it?

Answer 24

Fatty casts in urine that indicate presence of cholesterol in urine

Question 25

Albumin radius is 3.6 nm. What is the significance of this?

Answer 25

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

Question 26

Alpha-actinin 4 defect = ?

Answer 26

Autosomal dominant FSGS

Question 27

Podocin defect = ?

Answer 27

Autosomal recessive steroid resistant FSGS

Question 28

TRPC6 defect = ?

Answer 28

Autosomal dominant FSGS

Question 29

NEPH 1 defect = ?

Answer 29

autosomal recessive Finish type, resembles Minimal Change Disease

Question 30

Beta 2 microglobulin filtration status (in normal non-nephrotic syndrome)?

Answer 30

Almost completely unrestricted to filter through

Question 31

Albumin filtration status (in normal non-nephrotic syndrome)?

Answer 31

Some albumin filtered through, but reabsorbed by proximal tubular cells

Question 32

IgG filtration status (normally, in non nephrotic syndrome)?

Answer 32

Completely restricted, molecular radius large (nearly 1.5x the size of albumin)

Question 33

Compare filtration status of small vs large dextran in nephrotic syndrome

Answer 33

**lower excretion** of small molecular weight dextran due to loss of filtration surface area and increased clearance of large molecular weight dextrans

Question 34

IgG filtration status in nephrotic syndrome?

Answer 34

Increased excretion of IgG (neutral charge) due to loss of size barrier

Question 35

Normal level of albumin excretion in healthy kidney?

Answer 35

40-80 mg/day

Question 36

Urine dipstick measures what protein? What concentration of this protein must be present for dipstick to pick it up?

Answer 36

Albumin, \>300 mg/day

Question 37

Microalbuminuria = what level of albumin in urine? Significance of this?

Answer 37

= 30-300 mg/day; however, urine dipstick won't pick it up since its below the dipstick threshold of 300 mg/day

Question 38

normal spot urine albumin/creatinine ratio?

Answer 38

\< 0.15

Question 39

Urine albumin/creatinine ratio is good indicator of what?

Answer 39

Very good indicator of 24 hour urine profile for patient

Question 40

Tamm-Horsfall mucoprotein normal excretion amount?

Answer 40

normal = 30-50 mg /day

Question 41

Nephrotic range 24 hour urine proteinuria?

Answer 41

\> 3.5 grams

Question 42

Nephrotic range spot urine protein/creatinine ratio?

Answer 42

\>3.5

Question 43

2 main treatment methods of primary nephrotic syndrome?

Answer 43

1. Provide supportive measures 2. Provide disease modifiers

Question 44

3 supportive measures of primary nephrotic syndrome?

Answer 44

Want to control HTN via: low salt diet, ACEI, & ARB

Question 45

2 main disease modifiers of primary nephrotic syndrome?

Answer 45

1. Steroids 2. Immunosuppresive drugs (cyclophosphamide, cyclosporin, mycophenolate mofeitil, tacrolimus)

Question 46

Name the 4 immunosuppresive drugs used in primary nephrotic syndrome?

Answer 46

1. Cyclophosphamide 2. Cyclosporin 3. Mycophenolate Mofeitil 4. Tacrolimus

Question 47

Treatment method for secondary nephrotic syndrome?

Answer 47

1. Treat the cause 2. provide supportive measures (same as primary nephrotic syndrome) with low salt diet, ACEI, ARB

Question 48

Immunosuppresion therapy for secondary nephrotic syndrome?

Answer 48

None. Secondary nephrotic syndrome does not respond to steroids or immunosuppressants.