TY - JOUR
T1 - Longitudinal Changes of Fixation Location and Stability Within 12 Months in Stargardt Disease
T2 - ProgStar Report No. 12
AU - ProgStar Study Group
AU - Schönbach, Etienne M.
AU - Strauss, Rupert W.
AU - Kong, Xiangrong
AU - Muñoz, Beatriz
AU - Ibrahim, Mohamed A.
AU - Sunness, Janet S.
AU - Birch, David G.
AU - Hahn, Gesa Astrid
AU - Nasser, Fadi
AU - Zrenner, Eberhart
AU - Sadda, Srini Vas R.
AU - West, Sheila K.
AU - Scholl, Hendrik P.N.
AU - Scholl, Hendrik P.N.
AU - Strauss, Rupert W.
AU - Wolfson, Yulia
AU - Bittencourt, Millena
AU - Shah, Syed Mahmood
AU - Ahmed, Mohamed
AU - Schönbach, Etienne
AU - Fujinami, Kaoru
AU - Traboulsi, Elias
AU - Ehlers, Justis
AU - Marino, Meghan
AU - Crowe, Susan
AU - Briggs, Rachael
AU - Borer, Angela
AU - Pinter, Anne
AU - Fecko, Tami
AU - Burgnoni, Nikki
AU - Sunness, Janet S.
AU - Applegate, Carol
AU - Russell, Leslie
AU - Michaelides, Michel
AU - Esposti, Simona Degli
AU - Moore, Anthony
AU - Webster, Andrew
AU - Connor, Sophie
AU - Barnfield, Jade
AU - Salchi, Zaid
AU - Alfageme, Clara
AU - McCudden, Victoria
AU - Pefkianaki, Maria
AU - Aboshiha, Jonathan
AU - Liew, Gerald
AU - Holder, Graham
AU - Robson, Anthony
AU - King, Alexa
AU - Wojciechowski, Robert
AU - Ervin, Ann Margret
N1 - Funding Information:
Funding/Support: The ProgStar studies are supported by the Foundation Fighting Blindness (FFB) Clinical Research Institute (Columbia, Maryland, USA) and a grant by the U.S. Department of Defense USAMRMC TATRC to FFB (Fort Meade, Maryland, USA; grant numbers W81-XWH-07-1-0720 and W81-XWH-09-2-0189). Rupert W. Strauss is supported by the Austrian Science Fund (Vienna, Austria; FWF; Project number: J 3383-B23) and the Foundation Fighting Blindness Clinical Research Institute (Columbia, Maryland, USA). Etienne M. Schönbach is supported by the German National Academy of Sciences Leopoldina, Grant Number LPDS 2015-14 (Halle, Germany) and the Foundation Fighting Blindness Clinical Research Institute (Columbia, Maryland, USA). Financial Disclosures: Etienne M. Schönbach is supported by the German National Academy of Sciences Leopoldina, Grant Number LPDS 2015-14 (Halle, Germany) and the Foundation Fighting Blindness Clinical Research Institute (Columbia, Maryland, USA). Rupert W. Strauss is supported by the Austrian Science Fund (Vienna, Austria; FWF; Project number: J 3383-B23) and the Foundation Fighting Blindness Clinical Research Institute (Columbia, Maryland, USA). Janet S. Sunness is a consultant for Genentech (San Francisco, California, USA) and is on the scientific advisory board of Acucela (Tokyo, Japan) and Apellis (Crestwood, Kentucky, USA) and on the data safety and monitoring committee for Cell Cure's OpRegen study (Jerusalem, Israel). David G. Birch is a consultant for AGTC (Alachua, Florida, USA), Ionis (Carlsbad, California, USA), Genentech (South San Francisco, California, USA), Nightstar (London, England), and Nacuity (Ft. Worth, Texas, USA), and is supported by the Foundation Fighting Blindness Clinical Research Institute (FFB CRI, Columbia, Maryland). SriniVas R. Sadda is financially supported by Allergan (Dublin, Ireland), Carl Zeiss Meditec (Jena, Germany), Genentech (San Francisco, California, USA), and Optos (Dunfermline, UK), and is a consultant for Allergan (Dublin, Ireland), CenterVue (Padova, Italy), Genentech (San Francisco, California, USA), Heidelberg Engineering (Heidelberg, Germany), Iconic Therapeutics, Inc (San Francisco, California, USA), NightstarX (London, UK), Novartis (Basel, Switzerland), Optos (Dunfermline, UK), Thrombogenics (Leuven, Belgium), and Topcon (Tokyo, Japan). Sheila K. West is a scientific technical advisory committee member for the Alcon Research Institute (Fort Worth, Texas), and for Research to Prevent Blindness (New York, New York). Dr. Scholl is a paid consultant of the following entities (not including the National Institutes of Health): Fovea Pharmaceuticals/Sanofi (Paris, France); Trevena Inc. (Chesterbrook, PA); Guidepoint Global, LLC (New York, NY); Gerson Lehrman Group (New York, NY); Shire (Dublin, Republic of Ireland). Dr. Scholl is a member of the Scientific Advisory Board of Vision Medicines, Inc. Hendrik P.N. Scholl is a member of the Data Monitoring Committee of the following entities (not including the National Institutes of Health): Genentech Inc (San Francisco, California, USA)/F. Hoffmann-La Roche Ltd (Basel, Switzerland); StemCells, Inc (Newark, California, USA); Genzyme Corp (Boston, Massachusetts, USA)/Sanofi (Paris, France); he also serves as a member of the Ophthalmic Devices Panel of the Medical Devices Advisory Committee, Food and Drug Administration (FDA, Silver Spring, Maryland, USA). These arrangements have been reviewed and approved by the Johns Hopkins University in accordance with its conflict of interest policies. Hendrik P.N. Scholl is principal investigator of grants at The Johns Hopkins University sponsored by the following entity (not including the National Institutes of Health): QLT, Inc (Vancouver, Canada). Grants to investigators at Johns Hopkins University are negotiated and administered by the institution (such as the School of Medicine) which receives the grants, typically through the Office of Research and Administration. Individual investigators who participate in the sponsored project(s) are not directly compensated by the sponsor but may receive salary or other support from the institution to support their effort on the project(s). The following authors have no financial disclosures: Xiangrong Kong, Beatriz Muñoz, Mohamed A. Ibrahim, Gesa-Astrid Hahn, Fadi Nasser, and Eberhart Zrenner. All authors attest that they meet the current ICMJE criteria for authorship.
Publisher Copyright:
© 2018 Elsevier Inc.
PY - 2018/9
Y1 - 2018/9
N2 - Purpose: To investigate the natural history of Stargardt disease (STGD1) using fixation location and fixation stability. Design: Multicenter, international, prospective cohort study. Methods: Fixation testing was performed using the Nidek MP-1 microperimeter as part of the prospective, multicenter, natural history study on the Progression of Stargardt disease (ProgStar). A total of 238 patients with ABCA4-related STGD1 were enrolled at baseline (bilateral enrollment in 86.6%) and underwent repeat testing at months 6 and 12. Results: Outcome measures included the distance of the preferred retinal locus from the fovea (PRL) and the bivariate contour ellipse area (BCEA). After 12 months of follow-up, the change in the eccentricity of the PRL from the anatomic fovea was −0.0014 degrees (95% confidence interval [CI], −0.27 degrees, 0.27 degrees; P =.99). The deterioration in the stability of fixation as expressed by a larger BCEA encompassing 1 standard deviation of all fixation points was 1.21 degrees squared (deg 2 ) (95% CI, −1.23 deg 2 , 3.65 deg 2 ; P =.33). Eyes with increases and decreases in PRL eccentricity and/or BCEA values were observed. Conclusions: Our observations point to the complexity of fixation parameters. The association of increasingly eccentric and unstable fixation with longer disease duration that is typically found in cross-sectional studies may be countered within individual patients by poorly understood processes like neuronal adaptation. Nevertheless, fixation parameters may serve as useful secondary outcome parameters in selected cases and for counseling patients to explain changes to their visual functionality.
AB - Purpose: To investigate the natural history of Stargardt disease (STGD1) using fixation location and fixation stability. Design: Multicenter, international, prospective cohort study. Methods: Fixation testing was performed using the Nidek MP-1 microperimeter as part of the prospective, multicenter, natural history study on the Progression of Stargardt disease (ProgStar). A total of 238 patients with ABCA4-related STGD1 were enrolled at baseline (bilateral enrollment in 86.6%) and underwent repeat testing at months 6 and 12. Results: Outcome measures included the distance of the preferred retinal locus from the fovea (PRL) and the bivariate contour ellipse area (BCEA). After 12 months of follow-up, the change in the eccentricity of the PRL from the anatomic fovea was −0.0014 degrees (95% confidence interval [CI], −0.27 degrees, 0.27 degrees; P =.99). The deterioration in the stability of fixation as expressed by a larger BCEA encompassing 1 standard deviation of all fixation points was 1.21 degrees squared (deg 2 ) (95% CI, −1.23 deg 2 , 3.65 deg 2 ; P =.33). Eyes with increases and decreases in PRL eccentricity and/or BCEA values were observed. Conclusions: Our observations point to the complexity of fixation parameters. The association of increasingly eccentric and unstable fixation with longer disease duration that is typically found in cross-sectional studies may be countered within individual patients by poorly understood processes like neuronal adaptation. Nevertheless, fixation parameters may serve as useful secondary outcome parameters in selected cases and for counseling patients to explain changes to their visual functionality.
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U2 - 10.1016/j.ajo.2018.06.003
DO - 10.1016/j.ajo.2018.06.003
M3 - Article
C2 - 29890160
AN - SCOPUS:85049539585
SN - 0002-9394
VL - 193
SP - 54
EP - 61
JO - American journal of ophthalmology
JF - American journal of ophthalmology
ER -