title: Retinal degeneration in children: dark adapted visual threshold and arteriolar diameter.
abstract: 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.affiliation: Department of Ophthalmology, Children's Hospital and Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115, USA.
authors.forename: Ronald M
authors.initials: RM
authors.lastname: Hansen
authors.forename: Susan E
authors.initials: SE
authors.lastname: Eklund
authors.forename: Ilan Y
authors.initials: IY
authors.lastname: Benador
authors.forename: Julie A
authors.initials: JA
authors.lastname: Mocko
authors.forename: James D
authors.initials: JD
authors.lastname: Akula
authors.forename: Yao
authors.initials: Y
authors.lastname: Liu
authors.forename: M Elena
authors.initials: ME
authors.lastname: Martinez-Perez
authors.forename: Anne B
authors.initials: AB
authors.lastname: Fulton
mesh_terms: D000293:Adolescent; D000328:Adult; D000375:Aging / Q000473:pathology / Q000502:physiology; D001160:Arterioles / Q000473:pathology*; D020788:Bardet-Biedl Syndrome / Q000473:pathology / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D003623:Dark Adaptation; D006801:Humans; D007223:Infant; D029242:Optic Atrophy, Hereditary, Leber / Q000473:pathology / Q000503:physiopathology; D012162:Retinal Degeneration / Q000473:pathology / Q000503:physiopathology*; D012171:Retinal Vessels / Q000473:pathology*; D012684:Sensory Thresholds; D052245:Usher Syndromes / Q000473:pathology / Q000503:physiopathology