Alport syndrome pathophysiology
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Pathophysiology
Microscopic Pathology
Basement membranes are thin, sheet-like structures that separate and support cells in many tissues. When mutations prevent the formation of type IV collagen fibers, the basement membranes of the kidneys are not able to filter waste products from the blood and create urine properly, which allows blood and protein to enter into the urine.
The abnormalities of type IV collagen in the glomerular basement membrane cause gradual scarring of the kidneys, eventually leading to chronic renal failure in many people with the disease.
Associated Conditions
Some associated conditions that occur with Alport syndrome are chronic kidney failure, and proteinuria.