Document Detail


Stickler syndrome, ocular-only variants and a key diagnostic role for the ophthalmologist.
MedLine Citation:
PMID:  21921955     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The entity described by Gunnar Stickler, which included hereditary arthro-ophthalmopathy associated with retinal detachment, has recently been recognised to consist of a number of subgroups, which might now more correctly be referred to as the Stickler syndromes. They are the most common clinical manifestation of the type II/XI collagenopathies and are the most common cause of inherited rhegmatogenous retinal detachment. This review article is intended to provide the ophthalmologist with an update on current research, subgroups, and their diagnosis together with a brief overview of allied conditions to be considered in the clinical differential diagnosis. We highlight the recently identified subgroups with a high risk of retinal detachment but with minimal or absent systemic involvement--a particularly important group for the ophthalmologist to identify.
Authors:
M P Snead; A M McNinch; A V Poulson; P Bearcroft; B Silverman; P Gomersall; V Parfect; A J Richards
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review     Date:  2011-09-16
Journal Detail:
Title:  Eye (London, England)     Volume:  25     ISSN:  1476-5454     ISO Abbreviation:  Eye (Lond)     Publication Date:  2011 Nov 
Date Detail:
Created Date:  2011-11-14     Completed Date:  2012-01-05     Revised Date:  2012-01-10    
Medline Journal Info:
Nlm Unique ID:  8703986     Medline TA:  Eye (Lond)     Country:  England    
Other Details:
Languages:  eng     Pagination:  1389-400     Citation Subset:  IM    
Affiliation:
Vitreoretinal Service, Molecular Genetics and Medical Genetics, Cambridge, UK. mps34@cam.ac.uk
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MeSH Terms
Descriptor/Qualifier:
Arthritis / complications,  diagnosis*,  genetics
Collagen Type II / genetics
Collagen Type XI / genetics
Connective Tissue Diseases / complications,  diagnosis*,  genetics
Diagnosis, Differential
Hearing Disorders / etiology
Hearing Loss, Sensorineural / complications,  diagnosis*,  genetics
Humans
Jaw Abnormalities / etiology
Phenotype
Retinal Detachment / complications,  diagnosis,  etiology*,  genetics
Vitreous Body / chemistry
Chemical
Reg. No./Substance:
0/COL2A1 protein, human; 0/Collagen Type II; 0/Collagen Type XI

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


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