Alport Syndrome, also known as hematuria nephropathy deafness, hemorrhagic familial nephritis, hereditary deafness, nephropathy, or hereditary nephritis with sensory deafness, is a rare form of progressive kidney disease characterized by damage to the glomeruli and abnormalities of the inner ear and the eye. It is subdivided into three types: Autosomal Dominant Alport Syndrome (ADAS), Autosomal Recessive Alport Syndrome (ARAS), and X-linked Alport Syndrome (XLAS).
Epidemiology One in every 50,000 live births worldwide (USA: 1 in every 5000 people; Europe: 1 in 100,000 people to 1 in every 11,000) Approximately 0.2% of adults and 3% of children in the US with end-stage kidney disease About 85% of Alport syndrome cases are XLAS, about 15% are ARAS, and only a few cases are ADAS (no percentages available). In the case of XLAS, males are affected more than females, and in autosomal forms,…
