1- Biology:
A- Definition:
Massive proteinuria> 50 mg / kg / day (40 mg / m² / h); clear
Hypoproteinemia and hypoalbuminemia (<30 g / L)
Hyperlipidemia and hypercholesterolemia
B- Protein:
α2-globulins still high (-> 30% plasma protein)
Gamma globulins are low
High coagulation factors
Reduce protein vector => anemia (decreased transferrin) hypothyroidism …
C- Electrolytes:
Natriuresis is always reduced (aldosteronism 2aire)
Normal or low serum sodium; normal or increased serum potassium
Decreased total serum calcium (decreased albumin).
D- Blood:
Hypovolemia
Thrombocytosis and anemia (discrete); Leukocytosis
Elevated ESR
Hyperlipidemia is due to the decrease of orosomucoid (factor of lipoprotein lipase)
Lipoid nephrosis -> 3 microscopic type:
Minimal glomerular lesions
Injury focal segmental glomerulosclerosis
Mesangial proliferation
Urinary symptoms: frequent microscopic hematuria; oliguria
Functional renal impairment is rare, associated with significant oliguria
The decrease in gamma globulin covers IgG whereas IgM are increased
C3 fraction of complement is normal
2- Complications:
A- Infectious:
Pneumococcal disease (pneumonia, meningitis, peritonitis)
Skin infections (pyoderma …) …
B- Hydro-sodées:
Fluid retention: hyponatremia, headache and convulsion
By sudden collapse hypovolemia (beware diuretics)
C- Thrombo-embolism:
Renal vein thrombosis …
Pulmonary embolism
D- Digestive:
Diarrhea
Abdominal pain: serous edema, ascites
E- Other:
Malnutrition; hypothyroidism
Convulsion (hyponatremia, hypocalcemia); tetany
Congenital nephrotic syndrome of the Finnish-type (before 1 year, severe)
Secondary SN: Berger’s disease (IgA deposition); HSP; hepatitis B …
Blood pressure is usually normal
Symptomatic treatment: without strict salt diet edema phase; normal drink (water restriction and diuretics should be avoided)
For large edema => albumin + furosemide
Total length of treatment with prednisone => 4 months and ½
The disappearance of the clinical SN and proteinuria after 1 continuous attack treatment months defines the cortico-sensitivity
Corticosteroid: relapse after stopping corticosteroids or when decreasing doses
Healing: absence of clinical and biological signs for 2 years without corticosteroids.
