Alport's syndrome

Abstract

Alport's syndrome (hereditary nephropathy) is a progressive nephropathy characterized by focal and segmental thickness of the basement membrane with cleft formation in the lamina densa of the basement membrane, sensorineural deafness, lenticular opacities, corneal and retinal abnormalities. The course of the disease ins rapid in males and father to son transmission does not occur. The commonest mode of transmission is X linked though autosomal mode of inheritance is well described. Patients with Alport's syndrome don't get Goodpasture syndrome unless they are transplanted because they lack goodpasture antigen.