Nephrotic syndrome

Nephrotic syndrome results from loss of plasma proteins in the urine and characterized by hypoalbuminemia, hyperalbuminuria, hyperlipidemia, and edema. It may be caused by primary (idiopathic) renal disease or by a variety of secondary causes.

Clinical presentation

Patients present with marked edema, proteinuria, hypoalbuminemia, and often hyperlipidemia ¹.

Complications

• venous thromboembolism ^1,2

  • renal vein thrombosis ^1,7

• acute renal failure ¹

• superimposed bacterial infection ¹

Pathology

It may be classified as congenital or acquired ¹:

• congenital

  • nephrotic syndrome of Finnish type

  • focal and segmental hyalinosis (FSH)

  • neonatal glomerulonephritis

  • diffuse mesangial sclerosis

  • Alport syndrome

  • Pierson syndrome

  • Nail-patella syndrome

  • Denys-Drash syndrome

• acquired

  • primary

    • minimal-change glomerular disease (MCGD)

    • focal segmental glomerulosclerosis (FSGS)

    • membranous glomerular disease

    • membranoproliferative glomerular disease

  • secondary

    • diabetes mellitus

    • systemic lupus erythematosus

    • amyloidosis

    • multiple myeloma

    • drugs, e.g. NSAIDs

    • infections

  • when no cause is identified the term adult idiopathic nephrotic syndrome is used

Radiographic features

Nephrotic syndrome per se may produce non-specific appearances due to marked edema and hypoalbuminemia, e.g. anasarca, bowel wall thickening, etc. Chyluria may be seen due to the heavy losses of chyle/fats in the urine.

Treatment and prognosis

Treatment of most patients should include fluid and sodium restriction, oral or intravenous diuretics, and angiotensin-converting enzyme inhibitors ^1,4-6. Some adults with nephrotic syndrome may benefit from corticosteroid treatment, cyclosporine A, although research data are limited ^1,4-6. Intravenous albumin, prophylactic antibiotics, and prophylactic anticoagulation are not currently recommended ^1,4-6.