Thin Basement Membrane Disease



Thin Basement Membrane Disease


Helen Liapis, MD

Joseph Gaut, MD, PhD










This PAS stained slide from a woman with thin basement membrane disease (TBMD) shows a normal glomerulus, typical of TBMD by light microscopy. The GBM averaged 230 nm by electron microscopy.






Electron microscopy in TBMD reveals a diffusely thin but otherwise normal GBM of < 200 nm (normal is 320 ± 50 nm in females, 370 ± 50 nm in males). Morphometry is recommended to assess the thickness of the GBM.


TERMINOLOGY


Abbreviations



  • Thin basement membrane disease (TBMD)


Synonyms



  • Benign familial hematuria (BFH)


Definitions



  • Autosomal dominant disorder presenting with asymptomatic microscopic hematuria characterized by diffuse GBM thinning


ETIOLOGY/PATHOGENESIS


Genetics

Jul 7, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Thin Basement Membrane Disease
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