| One Family of Aniridia. |
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Young Ja Hwang, Byong Ho Kim, Hyun Bong Bae, Wan Seop Shim |
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Department of Ophthalmology, Chung-Nam National University Hospital, Taejoen, Korea. |
| 한가족 6명에 발생한 선천성 무홍채증 |
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황영자(Young Ja Hwang),김병호(Byong Ho Kim),배현봉(Byung Bong Bae),신완섭(Wan Seop Shim) |
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| Abstract |
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Aniridia is a congenital lack of iris and rudimentary iris tissue, almost always bilateral, occuring as a dominant characteristic by an autosomal gene with high penetrance and variable expression. The syndrome of congenital aniridia may be composed of four phenotypes. The authors have experienced one an iridic family: mother, one daughter and four sons. The most family members have ectopia lentis. cataract. corneal pannus and glaucoma as well as aniridia, so they were expected the first phenotype of aniridia due to a failure in development of retinal ectoderm. Two sons of them were operated in Chungnam National University Hospital for control of glaucoma and visual impairment due to cataractous ectopic lens. In one case the intraocular pressure was not controlled after trabeculectomy and in another case lens extraction resulted in visual improvement. The review was made of relating literatures for the case of aniridia family, breifly. |
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