Disease Information for Nephritis, hereditary (Alports): Definition

  • Alport Syndrome synonyms: Hematuria-Nephropathy Deafness

    Hemorrhagic Familial Nephritis; Hereditary Deafness and Nephropathy; Hereditary Nephritis; Hereditary Nephritis With Sensory Deafness; Nephritis and Nerve Deafness, Hereditary; Nephropathy and Deafness,Hereditary.

    Subdivisions: Autosomal Dominant Alport Syndrome (ADAS);

    Autosomal Recessive Alport Syndrome (ARAS); X-Linked Alport Syndrome (XLAS).

    Discussion: Alport syndrome is a group of hereditary disorders characterized by progressive deterioration of parts of the kidney known as basement membranes; This deterioration may lead to chronic kidney (renal) disease. Eventually, severe renal failure (end-stage renal disease or ESRD) may develop; Some types of Alport syndrome may also affect vision and hearing; Most cases of Alport syndrome have an X-linked pattern of inheritance.[NORD 2005]