- Caused by an inherited defect in type IV collagen
- Structural material that is needed for the normal function of different parts of the body – found in the ears, eyes, and kidneys
Alport syndrome is a triad of –
- Progressive renal failure,
- Hearing loss
- Ocular abnormalities.
What are the eye involvements in Alport syndrome ?
The absence of type IV collagen affects the basement membrane of cornea, lens capsule, and retina in the eye.
- Staircase foveopathy
- Dot-and-fleck retinopathy
- Lenticonus – cone-shaped lens
- Temporal retinal thinning