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A. Characteristics navigator

  1. Commonly called Lipoid Nephrosis
  2. Mainly occurs in young children, with male predominance
    1. 76% of nephrotic syndrome in children
    2. 26% of nephrotic syndrome in adults
  3. Generally selective proteinuria
    1. Albumin is preferentially lost into the urine
    2. This suggests that a glomerular epithelial cell charge defect is involved
  4. Relapsing course with chronic protein losses
    1. Progressive renal function decline is uncommon
    2. Essentially all morbidity is due to protein losses
  5. Hematuria or hypertension are also uncommon

B. Pathology navigator

  1. Minimal change on standard light microscopic evaluation
  2. Lipid droplets may be seen in tubular epithelial cells
  3. EM shows glomerular epithelial injury with widespread retraction of foot processes
  4. During remissions, foot processes return to normal

C. Pathophysiologynavigator

  1. Uncertain, but may be immune related
  2. T cell dysfunction has been suggested
    1. Increased production and circulation of T cell derived cytokines
    2. Some of these cytokines may be directly toxic to glomerular epithelial cells
    3. Result is retraction and fusion of glomerular epithelial foot processes
    4. This is believed to lead to heavy protein losses
  3. Various drugs may cause disease, including NSAIDs

D. Therapy navigator

  1. Glucocorticoids usually with cytotoxic therapy is nearly always effective
  2. Glucocorticoids
    1. Mainstay of therapy, with good initial response
    2. Typically 1.0-1.5mg/kg, maintain for 3-6 months
    3. Relapses in ~60% after tapering of therapy
    4. Longer term remission induction possible with cytotoxic drugs
  3. Cytotoxic Agents
    1. Chlorambucil or cyclophosphamide are used
    2. Two-3 month course of oral alkylating agents are generally recommended
    3. Cyclophosphamide 2.0-2.5mg/kg fpo qd for 8-12 weeks generally preferred
  4. Cyclosporine
    1. Good for glucocorticoid resistant disease
    2. Children treated with 5-6mg/kg with careful monitoring of renal function and levels
  5. Levamisole
    1. Antihelminthic, immunostimulatory agent
    2. May be used in frequent relapse disease
    3. May be as effective as cyclophosphamide
    4. Dose 2.5mg/kg on alternating days in children
    5. Side effects: leukopenia, hepatic dysfunction require monitoring

E. Prognosisnavigator

  1. Children usually do better
  2. Glucocorticoid responsive cases usually do better
  3. Disease will sometimes spontaneously remit
  4. Progressive renal functional decline is uncommon

References navigator

  1. Eddy AA and Symons JM. 2003. Lancet. 362(9384):629 abstract
  2. Mallick NP, Brenchley PE, Webb NJ. 1997. Kidney Int Suppl. 58:S80 abstract
  3. Langford CA, Klippel JH, Balow JE, et al. 1998. Ann Intern Med. 128(12):1021 abstract