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)