Document Detail


Retinal degeneration in children: dark adapted visual threshold and arteriolar diameter.
MedLine Citation:
PMID:  17765282     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
To assess the condition of the retina in children with retinal degeneration due to Bardet-Biedl syndrome (BBS, n=41), Leber congenital amaurosis (LCA, n=31), or Usher syndrome (USH, n=13), the dark adapted visual threshold (DAT) and arteriolar diameters were measured. Compared to controls, the initial DATs of nearly all (83/85) were significantly elevated, and in 26/62 with serial DATs, significant progressive elevation occurred. Arteriolar diameters were significantly attenuated and narrowed with age in BBS and USH, but not LCA. Higher DATs were associated with narrower arterioles. Such non-invasive procedures can document the natural history of these retinal diseases and have the potential to assess response to future treatment.
Authors:
Ronald M Hansen; Susan E Eklund; Ilan Y Benador; Julie A Mocko; James D Akula; Yao Liu; M Elena Martinez-Perez; Anne B Fulton
Publication Detail:
Type:  Journal Article     Date:  2007-08-31
Journal Detail:
Title:  Vision research     Volume:  48     ISSN:  0042-6989     ISO Abbreviation:  Vision Res.     Publication Date:  2008 Feb 
Date Detail:
Created Date:  2008-02-06     Completed Date:  2008-04-11     Revised Date:  2011-09-26    
Medline Journal Info:
Nlm Unique ID:  0417402     Medline TA:  Vision Res     Country:  England    
Other Details:
Languages:  eng     Pagination:  325-31     Citation Subset:  IM    
Affiliation:
Department of Ophthalmology, Children's Hospital and Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115, USA.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Aging / pathology,  physiology
Arterioles / pathology*
Bardet-Biedl Syndrome / pathology,  physiopathology
Child
Child, Preschool
Dark Adaptation*
Humans
Infant
Optic Atrophy, Hereditary, Leber / pathology,  physiopathology
Retinal Degeneration / pathology,  physiopathology*
Retinal Vessels / pathology*
Sensory Thresholds
Usher Syndromes / pathology,  physiopathology
Grant Support
ID/Acronym/Agency:
R01 EY010597-13/EY/NEI NIH HHS; S10 RR019318-01/RR/NCRR NIH HHS
Comments/Corrections

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