Thin basement membranes and Alport's syndrome

Abstract

Classical Alport's syndrome is an X-linked disease and is the most common form of Alport's syndrome (90% of patients), with an overall incidence of Alport's syndrome in the United States of 1:5000 to 1:10,000 (1-4). Patients show hematuria in childhood with progressive hearing loss in one third, and ocular defects and progression to renal failure in 30% to 40% by early adulthood. Anterior lenticonus is the most common eye defect. © 2007 Springer New York.