title: Cross-Sectional Analysis of Neurocognitive Function, Retinopathy, and Retinal Thinning by Spectral-Domain Optical Coherence Tomography in Sickle Cell Patients.
abstract: OBJECTIVE
  The purpose was to examine the relationship between neurocognitive function and two distinct forms of retinopathy in sickle cell disease.
  
  
  METHODS
  Patients with sickle cell disease (n = 44, age range: 19-56 years, 70% female) were prospectively recruited for this cross-sectional study. Retinopathy was characterized by: (1) Presence of focal retinal thinning on spectral domain optical coherence tomography and (2) determination of the sickle retinopathy stage on funduscopic exam based on Goldberg classification. Neurocognitive function was assessed using the Philadelphia Brief Assessment of Cognition (PBAC), a validated test of cognition. Univariate and multivariate analyses for PBAC score outcomes were performed. Retinal thinning and retinopathy stage were primary variables of interest and age, gender, genotype, education, and history of stroke were covariates.
  
  
  RESULTS
  Univariate analysis revealed associations with total PBAC score and age (P = 0.049), history of stroke (P = 0.04), and genotype (P < 0.001). Focal retinal thinning and Goldberg retinopathy stage were not associated with each other in this sample. Neither the presence of focal retinal thinning nor degree of retinopathy was associated with total PBAC score in univariate or multivariate analyses.
  
  
  CONCLUSIONS
  We find an association between lower cognitive function and older age, history of stroke and sickle cell genotype SS in patients with sickle cell disease. Our data do not provide evidence to support an association between cognitive function and retinopathy in sickle cell patients.
authors.affiliation: Department of Ophthalmology and Visual Sciences, University of Illinois at Chicago, Chicago, IL, USA; Department of Ophthalmology, Weill Cornell Medical College, New York, NY, USA.
authors.forename: Erica Z
authors.initials: EZ
authors.lastname: Oltra
authors.affiliation: Department of Ophthalmology and Visual Sciences, University of Illinois at Chicago, Chicago, IL, USA; Retinal Diagnostic Center, Campbell, CA, USA.
authors.forename: Clement C
authors.initials: CC
authors.lastname: Chow
authors.affiliation: Department of Ophthalmology and Visual Sciences, University of Illinois at Chicago, Chicago, IL, USA.
authors.forename: Thomas
authors.initials: T
authors.lastname: Wubben
authors.affiliation: Department of Ophthalmology and Visual Sciences, University of Illinois at Chicago, Chicago, IL, USA.
authors.forename: Jennifer I
authors.initials: JI
authors.lastname: Lim
authors.affiliation: Department of Ophthalmology and Visual Sciences, University of Illinois at Chicago, Chicago, IL, USA.
authors.forename: Felix Y
authors.initials: FY
authors.lastname: Chau
authors.affiliation: Department of Ophthalmology and Visual Sciences, University of Illinois at Chicago, Chicago, IL, USA.
authors.forename: Heather E
authors.initials: HE
authors.lastname: Moss
mesh_terms: D000328:Adult; D000755:Anemia, Sickle Cell / Q000145:classification / Q000175:diagnosis / Q000503:physiopathology*; D003072:Cognition Disorders / Q000175:diagnosis / Q000503:physiopathology*; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009887:Ophthalmoscopy; D012160:Retina / Q000503:physiopathology; D012164:Retinal Diseases / Q000175:diagnosis / Q000503:physiopathology*; D041623:Tomography, Optical Coherence / Q000379:methods; D055815:Young Adult
keywords: Neurocognitive Testing; Optical Coherence Tomography; Sickle Cell Disease; Sickle Retinopathy