LENTICONUS IN ALPORT'S SYNDROME

Abstract

A South Indian family with 3 well documented cases of Alport''s syndrome with anterior lenticonus was reported. Clinical features of the syndrome including ocular and laboratory findings were presented and discussed. Macular pigmentation, 5 cases, subcapsular opacity and nephrotic syndrome, 1 case each, observed in the present series were of great interest and are quite rare in patients with Alport''s syndrome. Critical analysis of the family pedigree revealed autosomal dominance with incomplete penetrance as the possible mode of genetic transmission of the disease.