ANGIOID STREAKS ASSOCIATED WITH ABETALIPOPROTEINEMIA

Citation
Mb. Gorin et al., ANGIOID STREAKS ASSOCIATED WITH ABETALIPOPROTEINEMIA, Ophthalmic genetics, 15(3-4), 1994, pp. 151-159
Citations number
NO
Categorie Soggetti
Genetics & Heredity",Ophthalmology
Journal title
ISSN journal
13816810
Volume
15
Issue
3-4
Year of publication
1994
Pages
151 - 159
Database
ISI
SICI code
1381-6810(1994)15:3-4<151:ASAWA>2.0.ZU;2-I
Abstract
Angioid streaks were observed in two patients with abetalipoproteinemi a. The progression of the angioid streaks was minimal over the years t hat these patients received vitamin A and E supplementation, though in one patient the development of subretinal neovascular membranes withi n the angioid streaks was the cause of rapid central visual loss. The simultaneous appearance of two rare entities in unrelated individuals strengthens the relationship between these two disorders that has been suggested by previous case studies. The authors propose a common meta bolic pathway involving trace element deficiencies that may account fo r this relationship as well as the association of angioid streaks with other rare disorders such as Paget's disease, hypoparathyroidism, lea d poisoning, hyperphosphatemia, and a number of hemoglobinopathies. Th eir study of these two patients underscores the need for further inves tigations as to the role of copper, zinc and omega-3 fatty acids in th e pathogenesis of retinopathy in abetalipoproteinemia,