Prognosis of Alport Syndrome

Alport syndrome causes progressive damage to the kidneys through the gradual replacement of normal kidney structures (glomeruli and tubules) by scar tissue. This process is known as fibrosis. All boys with X-linked Alport syndrome eventually develop kidney failure. Dialysis or transplantation often becomes necessary by adolescence or early adulthood, but kidney failure may be delayed until 40-50 years of age in some men with Alport syndrome. Most girls with X-linked Alport syndrome do not develop kidney failure. However, as woman with Alport syndrome age the risk of kidney failure increases.

All boys and girls with autosomal recessive Alport syndrome develop kidney failure, usually by their teens or young adult years. People with autosomal dominant Alport syndrome are usually well into middle age before kidney failure develops.

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