Lenticonus anterior and Alport's syndrome.
Six patients, from five families, five males and one female, suffering from lenticonus anterior are presented. Furthermore, all suffered from chronic nephritis and nerve deafness and must be considered cases of Alport's syndrome. As all cases with lenticonus anterior which have been published within the last 13 years likewise were affected with nerve deafness and/or chronic nephritis, the assumption is submitted that lenticonus anterior exists exclusively as a part of Alport's syndrome. It is pointed out that four patients had a weakened bilateral macula reflex with changes in pigmentation. Attention is drawn to the fact that patients with lenticonus anterior often have a cornea astigmatism of 2-3 D. A complete table of all reported patients with anterior lenticonus is presented.
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D006312:Hearing Loss, Bilateral / Q000235:genetics; D006319:Hearing Loss, Sensorineural / Q000235:genetics; D006801:Humans; D007223:Infant; D007908:Lens, Crystalline / Q000002:abnormalities*; D008297:Male; D008875:Middle Aged; D009394:Nephritis, Hereditary / Q000235:genetics; D013577:Syndrome