Patients with Alport syndrome present with hematuria in early childhood. Proteinuria develops during teenage years, with nephrotic syndrome in 30%-40%. Male patients with X-linked Alport progress to end-stage kidney disease, usually in adulthood. Female carriers of X-linked classic Alport syndrome usually have only hematuria, but may develop progressive kidney disease as middle-aged or older adults. Carriers of rare autosomal recessive variants of Alport syndrome also have hematuria. Gradual deafness develops in about half of adult men with Alport syndrome, with eye defects in about one-third, most commonly anterior lenticonus.