Alport Syndrome and Thin Basement Membrane Nephropathy: Causes, Symptoms, Diagnosis & Treatment
HEREDITARY
NEPHRITIS (ALPORT SYNDROME)/THIN
BASEMENT MEMBRANE NEPHROPATHY
Hereditary nephritis (HN, also known as Alport
syndrome) and thin basement membrane nephropathy (TBMN) are both inherited
disorders that feature structural defects in type IV collagen, an integral
component of the glomerular basement membrane. Both conditions present in
childhood with persistent microscopic hematuria.
HN is rare, affecting 1 in 50,000
individuals, and often progresses to end stage renal disease (ESRD). TBMN, in
contrast, affects 1 in 20 to 100 individuals and typically does not have a
progressive course.









