Nephrotic syndrome

Nephrotic syndrome pathogenesis:

 -ve charge in capillary wall
 + permeability
Clinical picture:
 Oliguria
 Oedema
 Proteinuria > 40 mg/m2/hr
 Hypoalbuminemia < 2.5 g/dl
 Hypercholesterolemia > 250 mg/dl
Typical nephrotic syndrome:
 No hypocomplementemia
 No persistent hematuria
 No renal insufficiency
 No hypertension
Types or causes of nephrotic syndrome:
 Primary
 Secondary
Primary nephrotic syndrome:
 Congenital nephrotic syndrome
 Minimal change nephrotic syndrome
 Focal segmental glomerulo-sclerosis
 Membrano-proliferative nephrotic syndrome
Secondary nephrotic syndrome: DADI
Diseases:
Renal:
 Alport syndrome
 Finnish nephrosis
 Ig A nephropathy
 Hemolytic uremic syndrome
 Rapidly progressive glomerulonephritis
Autoimmune:
 Systemic lupus erythematosus
 Henoch-Schonleen purpura
 Diabetes mellitus
 Amyloidosis
Allergy:
 Insect sting
 Serum sickness
Drugs:
 Penicillamine
 Captopril Infections:
 Gold salts  Malaria
 Mercury  SBE
Minimal change nephrotic syndrome MCNS:  HBV
Occurs in 2-7 years old  HCV
90% of these are MCNS HCV
Complications of nephrotic syndrome:
Male predominance
 Loss of protein=loss of immunity
10% other types
 More body fluid=more bacterial growth
Pathogenesis of NS:  Hyperlipidemia=blood vessel thrombosis
Increase renal permeability  Interstitial fluid=loss of intravascular blood volume
 Low protein& high steroid=less signs of inflammation
Protein loss in urine

Hypoalbuminemia

Edema

Hyperlipidemia

Investigations:
Urine:
 Protein in urine collection over 24 hours > 2 g/day
 Proteinuria 3+ or more by dipstick
 Protein/creatinine ratio > 3
Serum:
 Cholesterol > 250 mg/dl
 Albumin < 2 g/dl
 Low calcium
CXR:
 Pleural effusion
Renal biopsy if:
 Persistent hematuria
 Persistent proteinuria
 Persistent hypertension
Management:
 Supportive& specific
 Hospitalization
Supportive therapy:
 Protein Water Salt
 Stress Infection Diuretics
Specific therapy = Steroid therapy:
1. Predenisolone tab 60 mg/m2/day for 4 weeks
2. Until protein in urine is –ve or trace for 5 successive days
3. Change to 40 mg/m2/day alternative days for 4 weeks
4. Gradual tapering over 2-3 months
Side effects of steroid therapy:
 Gastritis
 Headache
 Osteoporosis
 Cushinoid face
 Hyperglycemia
 Liability to infections
 Delayed wound healing
 Adrenal gland suppression
During therapy you may face atypical response:
 Frequent relapses
 Steroid resistant
 Steroid dependent
Other drugs:
 Cyclophosphamide for resistant NS
 Mycophenolate for frequent relapses NS
 Cyclosporins for dependent& resistant NS
 Thyroxin& indomethacin for congenital NS