Nephrotic Syndrome
http://pediatrics.uchicago.edu/chiefs/nephro/documents/Peds_Nephro_for_Residents_Nov2005.pdf
Glomerular disease
Glomerulonephritis
Nephrotic Syndrome =
Associated triad:
Hypoalbuminaemia (<25g/L)
Gradual onset oedema (protein loss, transudate of fluid from IV space, reduced renal perf pressure, RAS/ADH, Na resorption from PT, H2O resorb from CD)
Hypercholesterolaemia
Exclude other causes of oedema
Exclude other non idiopathic causes of NS
Exclude suggestions of nephritic syndrome
Also immunocomprimised: loss of Ig, complement in urine
Hypercoagulable: vascular stasis, increased clotting factors, loss of serum anticoagulant, acute phase plt ↑, vast deplete so ↑Hct
Can get HTN and haematuria
Etiology (See Nelson's)
Genetic
Pathophys: Glomerular capillary permeability
Ix for steroid resistant
Renal Biopsy if Steroid Resistant (i.e. after 8weeks of pred)
GFR
?24 hour urine protein
Rx
Nephrologist
Prednisolone 2mg/kg (max 60mg) PO daily for 6 weeks
then 1.5mg (max 40mg) PO alternate days tapering for total course of 12 w
For relapse > restart on 2mg/kg/day and continue until at least 3 days without proteinuria
Then 1.5mg/kg for at least 4 weeks
Frequent relapsing and steroid dependent
same as for relapse but Rx for 3 months at lowest dose to remain in remission
(aim for less than 0.75mg/kg to avoid growth suppression)
Go to daily dosing (same dose as alternate) during URTIs
if side effect alkylating agents, cyclophosphamide or chlorambucil as steroid sparing agents
Steroid resistant
6m trial of cyclosporin, stop if no response
12m if full/partial response
Combine with low dose prednisolone
Pneumococcal vaccine for children
Influenza vaccine for children and household
No live vaccine until <1mg/kg (or 20mg) daily OR <2mg/kg (or 40mg) alternate days
No live vaccines with steroid sparing agents
Live vaccines to household but no contact with boy fluids for 6 weeks
ZIG if varicella contact
?Antithrombolytics
Complete remission = nil protein dipstick for 3 days OR uPCR <200mg/g
Partial remission = uPCR 200-2000mg/g AND 50% reduction from admission
Initial responder = Complete within 4 weeks
Steroid resistant aka non-responder= not complete within 8 weeks
Late non responder = relapse not remitted after 4 weeks of Rx
Frequent relapser = 2 within 6months of remission or 4 in any 12 month
Steroid dependent = 2 consecutive relapses during or within 2 weeks of cessation of steroid therapy
Immunosuppressors
Calcineurin inhibitors
non calcineurin
alk
Complications
Hypovolaemia
BP increases with BMI
Ambulatory BP monitoring
normotension
prehypertension
hypertension
severe hypertension
isolated nocturnal hypertension
non-dippers
INH and NonDipping is due to excess sympathetic drive
Glomerular disease
Glomerulonephritis
Nephrotic Syndrome =
- >40mg/kg/hr on 24 hour urine
- OR Pr:Cr >0.2g/mmol on first morning urine
- OR +++/++++ on dipstick
Associated triad:
Hypoalbuminaemia (<25g/L)
Gradual onset oedema (protein loss, transudate of fluid from IV space, reduced renal perf pressure, RAS/ADH, Na resorption from PT, H2O resorb from CD)
Hypercholesterolaemia
- hypoalbuminaemia stimulates hepatic protein synthesis (inc lipoproteins, fibrinogen and clotting factors)
- lipoprotein lipase is lost in urine > reduced lipid catabolism
Exclude other causes of oedema
- Liver disease (poor alb production): LFT/BSL/Coags
- Protein loosing enteropathy: stool alpha1AT
- CCF: CXR
Exclude other non idiopathic causes of NS
- <1y, >12y
- fever, rash, joint pain (?SLE)
Exclude suggestions of nephritic syndrome
- Macroscopic haematuria, red cell casts
- HTN
- Raised Cr
Also immunocomprimised: loss of Ig, complement in urine
Hypercoagulable: vascular stasis, increased clotting factors, loss of serum anticoagulant, acute phase plt ↑, vast deplete so ↑Hct
Can get HTN and haematuria
Etiology (See Nelson's)
Genetic
- Alports (can be nephrotic or itic)
- Minimal change disease
- Focal segmental GN
- Membranous nephropathy aka membranous GN
- Membranous N aka GN secondary to Hep B
- SLE
- HSP
Pathophys: Glomerular capillary permeability
Ix for steroid resistant
Renal Biopsy if Steroid Resistant (i.e. after 8weeks of pred)
GFR
?24 hour urine protein
Rx
Nephrologist
Prednisolone 2mg/kg (max 60mg) PO daily for 6 weeks
then 1.5mg (max 40mg) PO alternate days tapering for total course of 12 w
For relapse > restart on 2mg/kg/day and continue until at least 3 days without proteinuria
Then 1.5mg/kg for at least 4 weeks
Frequent relapsing and steroid dependent
same as for relapse but Rx for 3 months at lowest dose to remain in remission
(aim for less than 0.75mg/kg to avoid growth suppression)
Go to daily dosing (same dose as alternate) during URTIs
if side effect alkylating agents, cyclophosphamide or chlorambucil as steroid sparing agents
Steroid resistant
6m trial of cyclosporin, stop if no response
12m if full/partial response
Combine with low dose prednisolone
Pneumococcal vaccine for children
Influenza vaccine for children and household
No live vaccine until <1mg/kg (or 20mg) daily OR <2mg/kg (or 40mg) alternate days
No live vaccines with steroid sparing agents
Live vaccines to household but no contact with boy fluids for 6 weeks
ZIG if varicella contact
?Antithrombolytics
Complete remission = nil protein dipstick for 3 days OR uPCR <200mg/g
Partial remission = uPCR 200-2000mg/g AND 50% reduction from admission
Initial responder = Complete within 4 weeks
Steroid resistant aka non-responder= not complete within 8 weeks
Late non responder = relapse not remitted after 4 weeks of Rx
Frequent relapser = 2 within 6months of remission or 4 in any 12 month
Steroid dependent = 2 consecutive relapses during or within 2 weeks of cessation of steroid therapy
Immunosuppressors
Calcineurin inhibitors
- Cyclosporin
- Tacrolimus
non calcineurin
- Sirolimus (found on Rapanui)
alk
- Cyclophosphamide
Complications
Hypovolaemia
- 20% albumin 5ml/kg (1g/kg) over 4 hrs IV
- Give frusemide 1mg/kg (max 40mg) mid infusion
BP increases with BMI
Ambulatory BP monitoring
normotension
prehypertension
hypertension
severe hypertension
isolated nocturnal hypertension
non-dippers
INH and NonDipping is due to excess sympathetic drive