Document Detail


A novel case of bilateral high myopia, cataract, and total retinal detachment associated with interstitial 11q deletion.
MedLine Citation:
PMID:  20450311     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
PURPOSE: Jacobsen syndrome, also known as 11q deletion syndrome, is a rare condition characterized by multiple anomalies, including developmental delay, cardiac abnormalities, blood dyscrasias, distal limb abnormalities, craniofacial anomalies, and variable ophthalmic manifestations. The syndrome's phenotype is due to a terminal deletion and is usually severely debilitating, frequently associated with fatality. Interstitial deletions, not involving the terminal end, have been associated with a more variable and less severe phenotype. METHODS: Herein, we describe a case of interstitial 11q deletion in a 16 year-old female with associated systemic and craniofacial abnormalities as well as a novel combination of ocular findings, specifically strabismus, high myopia, bilateral cataracts, and bilateral total retinal detachments. RESULTS: This case report highlights the necessity for a detailed ophthalmic examination of patients with both interstitial and terminal deletions of the long arm of chromosome 11.
Authors:
Reecha Sachdeva; Jonathan E Sears; Paul J Rychwalski
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Ophthalmic genetics     Volume:  31     ISSN:  1744-5094     ISO Abbreviation:  Ophthalmic Genet.     Publication Date:  2010 Jun 
Date Detail:
Created Date:  2010-05-10     Completed Date:  2010-06-10     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9436057     Medline TA:  Ophthalmic Genet     Country:  England    
Other Details:
Languages:  eng     Pagination:  84-8     Citation Subset:  IM    
Affiliation:
Cole Eye Institute, Cleveland Clinic, Cleveland, Ohio 44195, USA.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Cataract / genetics*
Chromosome Deletion*
Chromosomes, Human, Pair 11 / genetics*
Craniofacial Abnormalities / genetics
Female
Functional Laterality
Humans
Myopia / genetics*
Phenotype
Retinal Detachment / genetics*
Strabismus / genetics

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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