<oai_dc:dc xmlns:dc="http://purl.org/dc/elements/1.1/" xmlns:oai_dc="http://www.openarchives.org/OAI/2.0/oai_dc/" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" xsi:schemaLocation="http://www.openarchives.org/OAI/2.0/oai_dc/ http://www.openarchives.org/OAI/2.0/oai_dc.xsd">
  <dc:creator>Schönbach EM</dc:creator>
  <dc:creator>Strauss RW</dc:creator>
  <dc:creator>Ibrahim MA</dc:creator>
  <dc:creator>Janes JL</dc:creator>
  <dc:creator>Birch DG</dc:creator>
  <dc:creator>Cideciyan AV</dc:creator>
  <dc:creator>Sunness JS</dc:creator>
  <dc:creator>Muñoz B</dc:creator>
  <dc:creator>Ip MS</dc:creator>
  <dc:creator>Sadda SR</dc:creator>
  <dc:creator>Scholl HPN</dc:creator>
  <dc:date>2020</dc:date>
  <dc:description xmlns:ns0="xml" ns0:lang="en">PURPOSE
Mean sensitivity (MS) derived from a standard test grid using microperimetry is a sensitive outcome measure in clinical trials investigating new treatments for degenerative retinal diseases. This study hypothesizes that the functional decline is faster at the edge of the dense scotoma (eMS) than by using the overall MS.


DESIGN
Multicenter, international, prospective cohort study: ProgStar Study.


METHODS
Stargardt disease type 1 patients (carrying at least 1 mutation in the ABCA4 gene) were followed over 12 months using microperimetry with a Humphrey 10-2 test grid. Customized software was developed to automatically define and selectively follow the test points directly adjacent to the dense scotoma points and to calculate their mean sensitivity (eMS).


RESULTS
Among 361 eyes (185 patients), the mean age was 32.9 ± 15.1 years old. At baseline, MS was 10.4 ± 5.2 dB (n = 361), and the eMS was 9.3 ± 3.3 dB (n = 335). The yearly progression rate of MS (1.5 ± 2.1 dB/year) was significantly lower (β = -1.33; P &lt; .001) than that for eMS (2.9 ± 2.9 dB/year). There were no differences between progression rates using automated grading and those using manual grading (β = .09; P = .461).


CONCLUSIONS
In Stargardt disease type 1, macular sensitivity declines significantly faster at the edge of the dense scotoma than in the overall test grid. An automated, time-efficient approach for extracting and grading eMS is possible and appears valid. Thus, eMS offers a valuable tool and sensitive outcome parameter with which to follow Stargardt patients in clinical trials, allowing clinical trial designs with shorter duration and/or smaller cohorts.</dc:description>
  <dc:format>application/pdf</dc:format>
  <dc:identifier>https://sonar.ch/global/documents/93543</dc:identifier>
  <dc:language>eng</dc:language>
  <dc:relation>info:eu-repo/semantics/altIdentifier/doi/10.1016/j.ajo.2020.03.020</dc:relation>
  <dc:relation>info:eu-repo/semantics/altIdentifier/pmid/32222369</dc:relation>
  <dc:rights>info:eu-repo/semantics/openAccess</dc:rights>
  <dc:source>American journal of ophthalmology. - 2020</dc:source>
  <dc:subject xmlns:ns1="xml" ns1:lang="en">ATP-Binding Cassette Transporters</dc:subject>
  <dc:subject xmlns:ns2="xml" ns2:lang="en">Adolescent</dc:subject>
  <dc:subject xmlns:ns3="xml" ns3:lang="en">Adult</dc:subject>
  <dc:subject xmlns:ns4="xml" ns4:lang="en">Female</dc:subject>
  <dc:subject xmlns:ns5="xml" ns5:lang="en">Fluorescein Angiography</dc:subject>
  <dc:subject xmlns:ns6="xml" ns6:lang="en">Humans</dc:subject>
  <dc:subject xmlns:ns7="xml" ns7:lang="en">Male</dc:subject>
  <dc:subject xmlns:ns8="xml" ns8:lang="en">Middle Aged</dc:subject>
  <dc:subject xmlns:ns9="xml" ns9:lang="en">Prospective Studies</dc:subject>
  <dc:subject xmlns:ns10="xml" ns10:lang="en">Retina</dc:subject>
  <dc:subject xmlns:ns11="xml" ns11:lang="en">Scotoma</dc:subject>
  <dc:subject xmlns:ns12="xml" ns12:lang="en">Sensitivity and Specificity</dc:subject>
  <dc:subject xmlns:ns13="xml" ns13:lang="en">Stargardt Disease</dc:subject>
  <dc:subject xmlns:ns14="xml" ns14:lang="en">Tomography, Optical Coherence</dc:subject>
  <dc:subject xmlns:ns15="xml" ns15:lang="en">Visual Acuity</dc:subject>
  <dc:subject xmlns:ns16="xml" ns16:lang="en">Visual Field Tests</dc:subject>
  <dc:subject xmlns:ns17="xml" ns17:lang="en">Visual Fields</dc:subject>
  <dc:subject xmlns:ns18="xml" ns18:lang="en">Young Adult</dc:subject>
  <dc:title xmlns:ns19="xml" ns19:lang="en">Faster Sensitivity Loss around Dense Scotomas than for Overall Macular Sensitivity in Stargardt Disease: ProgStar Report No. 14.</dc:title>
  <dc:type>http://purl.org/coar/resource_type/c_6501</dc:type>
</oai_dc:dc>
