Thin basement membrane disease

Thin basement membrane disease

Thin basement membrane disease includes familial benign hematuria, familial hematuric nephritis, benign hereditary nephritis

  • Most common cause of persistent hematuria in both children and adults; affects about 1% of population 
  • Renal impairment is usually not associated with disease, which is a lifelong and nonprogressive disorder; family history is often positive for disease
  • Presents similarly with glomerular origin hematuria and minimal proteinuria, often after exercise or during infection; hematuria is usually microscopic but may be macroscopic in up to about 20% of patients 
  • Differentiate by clinical presentation and course; hypertension, edema, and declining renal function do not develop
  • May be diagnosed by characteristic biopsy and electron microscopy findings (uniformly thinned glomerular basement membranes) if confirmatory diagnosis is needed 
    • In particular, may need to be distinguished from Alport syndrome, as both conditions share similar features early in course

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