Alport's syndrome Hereditary nephritis

  • Facts:
    Anti-GBM glomerulonephritis ( nephritic syndrome )
    Hereditary, common in boys 5-20 years old
    X-linked recessive defect in alpha 5 chain of collagen type IV (COLA4A5)
  • History / PE:
    Hematuria
    Nerve deafness
    Eye disorders (posterior cataracts, lens dislocation)
    Family h/o renal failure
  • Diagnosis:
    GBM splitting on electron miscroscopy
  • Complications:
    Renal failure
    Anti-GBM nephritis may recur after transplant
  • See Also:
    Nephrotic syndrome
  • Differential Diagnosis:
    Goodpasture's syndrome (hemoptysis, linear anti-GBM deposits)
    Jervell-Lange-Nielson syndrome (congenital deafness)



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August 3rd 2010