The lens pathology of bilateral progressive anterior lenticonus in familial hemorrhagic nephritis (Alport's syndrome) is reported in this paper. The lens pathology consists of thinning of the lens capsule, decrease of the number of lens epithelial cells, bulging of lens substance, and minimal subcapsular cataract at the anterior pole of both lenses.
Alport's syndrome has been known since 1912 as "hereditary familial congenital hemorrhagic nephritis."1,2,3 The earliest reports of the syndrome found were by Guthrie in 1897 and 1902.4,5 In its initial description it was thought to include only progressive nephritis with albuminuria and hematuria and progressive congenital nerve deafness. The nephritis characteristically leads to early death in males while females usually have a normal life expectancy.2,6,7 Lens involvement with anterior lenticonus, spherophakia, anterior polar cataract, and posterior cortical cataract has later been described in association with the syndrome.6 It is interesting to note that lens
BROWNELL RD, WOLTER JR. Anterior Lenticonus in Familial Hemorrhagic Nephritis: Denmonstration of Lens Pathology. Arch Ophthalmol. 1964;71(4):481–483. doi:10.1001/archopht.1964.00970010497007
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