Nephrotic syndromes

Question 1

features of adult minimal change dx

Answer 1

age>45 yrs
sudden onset of symptoms
hypertension (43%)

Question 2

laboratory data that support nephrotic syndrome

Answer 2

nephrotic range proteinuria >3g/day
serum albumin <2.2 g/dl
serum cholesterol is >420 mg/dl

see AKI (18%)

Question 3

when do we see adult minimal change dx

Answer 3

after upper resp infection and after some infections.

Question 4

what is seen on urinalysis with minimal change dx?

Answer 4

nothing on light microscopy

seen in minimal change dx
no RBCs or casts on UA

maintained GFR and normal BP

Question 5

electron microscopy of kidney with minimal change dx

Answer 5

see effacement of podocyte foot processes

Question 6

renal biopsy of minimal change dx

Answer 6

normal appearing glomeruli and no immune deposits by immunofluorescence

Question 7

does a pt who presents with nephrotic syndrome suggestive of minimal change dx need a renal biospy?

Answer 7

yes because clinical phenotype doesn’t accurately predict renal histopathology and so most adults with non diabetic nephrotic syndrome need renal biopsy to confirm diagnosis and guide therapy.

Question 8

nephrotic syndrome diagnostic criteria

Answer 8

24 hr urinary protein >3.5 g/day
edema
hypoalbuminemia <3g/dl
hyperlipidemia
lipiduria

Question 9

causes of nephrotic syndrome

Answer 9

minimal change dx
focal segmental glomerulosclerosis
membraneous nephropathy
amyloidosis
diabetic nephropathy

Question 10

complications of nephrotic syndrome

Answer 10

protein malnutrition
hypovolumia
VTE- especially the renal vein)
infection (pneumococcal)
proximal tubular dysfunction causing vitamin D deficiency

Question 11

Why are nephrotic syndrome pts at greater risk for VTE?

Answer 11

due to urinary loss of antithrombin 3 and plasminogen and increased levels of fibrinogen and platelet activation

Question 12

most common cause of nephrotic syndrome?

Answer 12

membranous nephropathy (seen in Hep B)

carries highest risk of VTE with up to 60% of pts developing renal vein thrombosis

Question 13

Who has highest risk for renal vein thrombosis?

Answer 13

pts who have >10 g/day of urinary protein and <2g/dl of serum albumin

seen in menbranous nephropathy pts

Question 14

chronic renal vein thrombosis presentation

Answer 14

can be chronic, asymptomatic, often not associated with worsening renal function or proteinuria and thrombosis can propagate to inferior vena cava and embolize to the pulmonary vasculature and cause PE (which can be only manifestation of chronic renal vein thrombosis)

Question 15

acute renal vein thrombosis presentation

Answer 15

pain hematuria and renal failure

Question 16

Diagnosis of renal vein thrombosis

Answer 16

selective venography, doppler ultrasound, CT scan or MRI

Question 17

should we routinely screen those with membranous nephropathy for renal vein thrombosis?

Answer 17

no.

Question 18

treatment of renal vein thrombosis?

Answer 18

anticoagulation

Question 19

what is Pickering syndrome?

Answer 19

flash pulmonary edema from bilateral renal ARTERY stenosis and this is associated with hypoxia, severe HTN and JVD.

Question 20

focal segmental glomerular sclerosis + HTN and edema should be treated with

Answer 20

ACE i because they have >1g/day of daily protein excretion into the urine which causes a more rapid decline in GFR than kidney function regardless of initial GFR or primary cause of renal dysfunction

Question 21

membranous nephropathy is

Answer 21

most common cause of nephrotic syndrome in NON diabetic pts

Question 22

prognosis of idiopathic membranous nephropathy

Answer 22

1/3 has spontaneous partial remission,

1/3 has partial remission <2g protein per day and 1/3 progressing to ESRD in 5-10 years

Question 23

risk factors for progressive idiopathic membraneous nephropathy is

Answer 23

male sex
age >50 yrs
nephrotic range proteinuria and increased GFR

Question 24

patients with idiopathic membraneous nephropathy and risk for becoming progressive

Answer 24

treat with cytotoxic or calcineurin inhibitors

cyclophosphamide with prednisone

Question 25

newly diagnosed pts with membraneous nephropathy are:

Answer 25

observed for 6 to 12 months while on conservative therapy to see if there’s spontaneous remission before initiating immunosuppression.

Question 26

most cases of membraneous nephropathy are:

Answer 26

primary 75%
25% - secondary causes SLE, hep B or C infection and solid tumors.

Pre eclampsia and sickle cell dx can have it

Question 27

Presentation of nephrotic syndrome on physical exam:

Answer 27

facial and lower extremity swelling
anasarca
ascites
pleural effusions
foamy urinalysis
hematuria and may see HTN

Question 28

when working someone up for nephrotic syndrome you should get:

Answer 28

UA
urine protein measurement
serum albumin
serological work up for infection and immune abnormalities
need renal u/s to eliminate the differential diagnosis

Question 29

nephrotic syndrome is classified by steroid:

Answer 29

steroid sensitive
steroid resistant
steroid dependent
frequently relapsing

maintstay of treatment is corticosteroids, cyclophosphamide and cyclosporine

Question 30

what medication can cause nephrotic syndrome?

Answer 30

NSAIDS over a long period of time.