نتایج جستجو برای: anophthalmia

تعداد نتایج: 586  

2009
Kulvinder Kaur Nicola K. Ragge Jiannis Ragoussis

PURPOSE Haploinsufficiency through mutation or deletion of the forkhead transcription factor, FOXC1, causes Axenfeld-Rieger anomaly, which manifests as a range of anterior segment eye defects and glaucoma. The aim of this study is to establish whether mutation of FOXC1 contributes toward other developmental eye anomalies, namely anophthalmia, microphthalmia, and coloboma. METHODS The coding s...

2010
Ryan Chao Linda Nevin Pooja Agarwal Jan Riemer Xiaoyang Bai Allen Delaney Matthew Akana Nelson JimenezLopez Tanya Bardakjian Adele Schneider Nicolas Chassaing Daniel F. Schorderet David FitzPatrick Pui-yan Kwok Lars Ellgaard Douglas B. Gould Yan Zhang Jarema Malicki Herwig Baier Anne Slavotinek

Anophthalmia and microphthalmia are important birth defects, but their pathogenesis remains incompletely understood. We studied a patient with severe unilateral microphthalmia who had a 2.7 Mb deletion at chromosome 18q22.1 that was inherited from his mother. In-situ hybridization showed that one of the deleted genes, TMX3, was expressed in the retinal neuroepithelium and lens epithelium in the...

Journal: :Journal of medical genetics 2014
Taraneh Esmailpour Hamidreza Riazifar Linan Liu Sandra Donkervoort Vincent H Huang Shreshtha Madaan Bassem M Shoucri Anke Busch Jie Wu Alexander Towbin Robert B Chadwick Adolfo Sequeira Marquis P Vawter Guoli Sun Jennifer J Johnston Leslie G Biesecker Riki Kawaguchi Hui Sun Virginia Kimonis Taosheng Huang

INTRODUCTION Lenz microphthalmia syndrome (LMS) is a genetically heterogeneous X-linked disorder characterised by microphthalmia/anophthalmia, skeletal abnormalities, genitourinary malformations, and anomalies of the digits, ears, and teeth. Intellectual disability and seizure disorders are seen in about 60% of affected males. To date, no gene has been identified for LMS in the microphthalmia s...

2011
Stefano Pensiero Paolo Cecchini Paola Michieletto Gloria Pelizzo Maurizio Madonia Fulvio Parentin

INTRODUCTION The complete absence of the chiasm (chiasmal aplasia) is a rare clinical condition. Hypoplasia of the optic nerve and congenital nystagmus are almost invariably associated characteristics. Microphthalmos or anophthalmos are common features in chiasmal aplasia, while central nervous system abnormalities are less frequent. Esophageal atresia can be isolated or syndromic. In syndromic...

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