Lipoid Nephrosis

 

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

Lipoid nephrosis

Lipoid nephrosis

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.

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