Cite this page: Pernick N. Lens and vitreous: Alport syndrome. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/eyelensalport.html. Accessed July 21st, 2019.
Definition / general
- Also called hereditary nephritis
- Inherited disorder of type IV collagen in basement membranes
- Hematuria, progressive nephritis with proteinuria and declining renal function, deafness (55%), ocular abnormalities (15 - 30%)
- Anterior lenticonus (forward central protrusion of anterior surface of lens due to weakness in type IV collagen, relatively specific for Alport)
- Also keratoconus, spherophakia (small, spherical lens), myopia, retinal flecks, cataracts, retinitis pigmentosa, amaurosis (blindness without an apparent ocular cause)