Roos Laura Sønderberg, Grønskov Karen, Jensen Hanne, Tümer Zeynep
Center for Applied Human Molecular Genetics, Kennedy Centret, Gamle Landevej 7, 2600 Glostrup, Denmark.
Ugeskr Laeger. 2012 Mar 12;174(11):713-6.
Anophthalmia and microphthalmia (AO/MO) are rare congenital eye malformations, in which the eyeball is apparently absent or smaller than normal, which causes various degrees of visual impairment. Over 200 different AO/MO-related syndromes have been described, but the genetic background is unknown in many cases. The aim of this article is to give an overview of AO/MO, focusing on the genetic background. It is illustrated that the future identification of new AO/MO related genes will benefit in the genetic counseling of AO/MO patients, and in the understanding of eye development and congenital eye malformations.
无眼畸形和小眼畸形(AO/MO)是罕见的先天性眼部畸形,其中眼球明显缺失或小于正常大小,可导致不同程度的视力损害。已经描述了200多种不同的与AO/MO相关的综合征,但在许多情况下,其遗传背景尚不清楚。本文旨在对AO/MO进行概述,重点关注其遗传背景。结果表明,未来对新的AO/MO相关基因的鉴定将有助于AO/MO患者的遗传咨询,以及对眼部发育和先天性眼部畸形的理解。