Nephrotic syndrome

Question 1

what is it a triad of

Answer 1

proteinuria (>3.5g/24 hour), hypoalbuminaemia (<25g/L), oedema

Question 2

what can cause nephrotic syndrome (primary)

Answer 2

minimal change disease; membranous nephropathy; focal segmental glomerulosclerosis, mesangiocapillary GN

Question 3

what are the secondary causes of nephrotic syndrome

Answer 3

hep B/C; SLE; diabetic nephropathy; amyloidosis; drug related

Question 4

what is the pathophysiology

Answer 4

injury to the podocyte which causes heavy protein loss

Question 5

what can patients present with

Answer 5

pitting oedema

Question 6

signs

Answer 6

urine dip shows ++++ protein, low albumin, bp normal or slightly high, renal function usually normal or mildly impaired

Question 7

differential diagnosis

Answer 7

CCF (incr JVP, pulm oedema, mild proteinuria) or liver disease (decr albumin)

Question 8

what is the most common cause in children

Answer 8

minimal change disease (lesion in the glomerulus) steroids used

Question 9

when is biopsy requires in children

Answer 9

if no response to steroids or signs point to another cause- age <1 year, family history, extra renal disease, renal failure, haematuria

Question 10

why is renal biopsy more difficult in patients with nephrotic syndrome

Answer 10

because of gross oedema and a hypercoagulable state

Question 11

complications

Answer 11

susceptibility to infection- cellulitis, strep, spont bacterial peritonitis (as Ig lost in urine and immunosuppressants); thromboembolism (incr clotting factors and platelet abnormalities); hyperlipidaemia ( incr chol and triglycerides)

Question 12

treatment to reduce oedema

Answer 12

loop diuretics- furosemide. fluid restriction to 1L/day and salt restrict whilst on diuretics

Question 13

treatment

Answer 13

reduce oedema, reduce proteinuria, reduce risk of complications, treat underlying cause

Question 14

treatment to reduce proteinuria

Answer 14

ACEi or ARB

Question 15

treatment to reduce risk of complications

Answer 15

anticoagulate if nephrotic range proteinuria, start statin to reduce chol, treat infections and vaccinate

Question 16

what % of nephrotic adults have minimal change disease

Answer 16

20%

Question 17

what can minimal change disease be due to in adults

Answer 17

idiopathic, in association with NSAIDS, or paraneoplastic

Question 18

if freq relapse minimal change disease what is the treatment

Answer 18

cyclophosphamide or ciclosporin/tacrolimus

Question 19

what accounts for 20-30% of nephrotic syndrome in adults

Answer 19

membranous nephropathy (2-5% in children)

Question 20

what can cause membranous nephropathy

Answer 20

mostly idiopathic, can be assoc with malignancy, NSAIDS, hep B, drugs (gold, penicillamine, NSAIDs), and autoimmunity (thyroid, SLE).

Question 21

what does biopsy show in membranous nephropathy

Answer 21

diffusely thickened GBM with IgG and C3 subepithelial deposits on IF

Question 22

treatment membranous nephropathy

Answer 22

underlying cause. ACE/ARB and diuretics.

Question 23

what is mesangiocapillary GN

Answer 23

mesangial and endocapillary proliferation a thickened capillary basement membrane

Question 24

treatment mesangiocapillary GN

Answer 24

underlying cause, ACE/ARB, immunosuppression with steroids and cyclophosphamide. poor prognosis

Question 25

focal segmental glomerulosclerosis- primary

Answer 25

idiopathic

Question 26

focal segmental glomerulosclerosis- secondary

Answer 26

IgA nephropathy, Alports syndrome, vasculitis, sickle cell

Question 27

focal segmental glomerulosclerosis presentation

Answer 27

nephrotic syndrome or proteinuria.

Question 28

biopsy focal segmental glomerulosclerosis

Answer 28

some glomeruli have scarring of some segments. IgM and C3 deposits IF

Question 29

treatment focal segmental glomerulosclerosis

Answer 29

corticosteroids in 30%. cyclophosphamide or ciclosporin

Question 30

prognosis focal segmental glomerulosclerosis

Answer 30

can progress to ESRF