*Thin Basement Membrane Disease

Chapter: Thin Basement Membrane Disease
McMaster Section Editor(s): Christine M. Ribic, Karen C.Y. To
Section Editor(s) in Interna Szczeklika: Franciszek Kokot, Robert Drabczyk
McMaster Author(s): Matthew Miller
Author(s) in Interna Szczeklika: Marian Klinger, Robert Drabczyk
Additional Information

Definition, EtiologyTop

Thin basement membrane disease (TBMD) (also called benign familial hematuria) is a congenital glomerulopathy characterized by thinning (<250 nm) of the glomerular basement membrane. Patients with familial TBMD have autosomal dominant inheritance, although they may not have a known family history, given the asymptomatic nature of the condition.

Clinical FeaturesTop

Patients develop microscopic hematuria, which is frequently detectable as early as in childhood. Typically proteinuria and hypertension are absent and patients do not develop renal failure. Hematuria is not aggravated by comorbidities and extrarenal symptoms do not occur.

DiagnosisTop

Diagnosis is based on electron microscopy examination demonstrating thin basement membranes without other pathologies.

TreatmentTop

Specific treatment is neither available nor necessary.

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