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Fundus autofluorescence and ellipsoid zone (EZ) line width can be an outcome measurement in RHO-associated autosomal dominant retinitis pigmentosa

  • Retinal Disorders
  • Published:
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Abstract

Purpose

To evaluate the progression of retinitis pigmentosa (RP) due to mutations in rhodopsin (RHO) by measuring the short-wavelength autofluorescence (SW-AF) increased autofluorescence ring and ellipsoid zone (EZ)-line width.

Methods

Fundus autofluorescence (FAF) and spectral domain optical coherence tomography (SD-OCT) images were obtained from 10 patients with autosomal dominant RP due to mutations in the RHO gene. Measurements of ring area on FAF images, as well as the EZ line width on SD-OCT images and horizontal, vertical diameter, were performed by two independent masked graders.

Results

The ring area decreased by a rate of 0.6 ± 0.2 mm2 per year. We observed that the EZ line width decreased by an average of 152 ± 37 μm per year, while the horizontal and vertical diameters decreased by 106 ± 35 μm and 125 ± 29 μm per year, respectively. Progression rates were similar between eyes.

Conclusions

We observed SW-AF ring constriction and a progressive loss of EZ line width over time.

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Funding

The Jonas Children’s Vision Care and Bernard & Shirlee Brown Glaucoma Laboratory are supported by the National Institutes of Health [P30EY019007, R01EY018213, R01EY024698, R01EY026682, R21AG050437], National Cancer Institute Core [5P30CA013696], Foundation Fighting Blindness [TA-NMT-0116-0692-COLU], the Research to Prevent Blindness (RPB) Physician-Scientist Award, and unrestricted funds from RPB, New York, NY, USA. S.H.T. is a member of the RD-CURE Consortium and is supported by ARVO/Genentech, Kobi and Nancy Karp, the Crowley Family Fund, the Rosenbaum Family Foundation, the Tistou and Charlotte Kerstan Foundation, the Schneeweiss Stem Cell Fund, New York State [C029572], and the Gebroe Family Foundation.

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Authors and Affiliations

  1. Department of Ophthalmology, Columbia University, New York, NY, USA

    Vitor K. L. Takahashi, Júlia T. Takiuti, Jose R. L. Carvalho-Jr, Christine L. Xu & Stephen H. Tsang

  2. Jonas Children’s Vision Care, and the Bernard & Shirlee Brown Glaucoma Laboratory, Departments of Ophthalmology, Pathology & Cell Biology, Columbia Stem Cell Initiative, Institute of Human Nutrition, Columbia University, New York, NY, USA

    Vitor K. L. Takahashi, Júlia T. Takiuti, Jose R. L. Carvalho-Jr, Christine L. Xu & Stephen H. Tsang

  3. Department of Ophthalmology, Federal University of São Paulo, Sao Paulo, Brazil

    Vitor K. L. Takahashi & Jose R. L. Carvalho-Jr

  4. Division of Ophthalmology, University of São Paulo Medical School, Sao Paulo, Brazil

    Júlia T. Takiuti

  5. Empresa Brasileira de Servicos Hospitalares (EBSERH) - Hospital das Clinicas de Pernambuco (HCPE), Departament of Ophthalmology, Federal University of Pernambuco (UFPE), Recife, Brazil

    Jose R. L. Carvalho-Jr

  6. Department of Biostatistics, Columbia University, New York, NY, USA

    Jimmy K. Duong

  7. Omics Laboratory, Department of Ophthalmology, Byers Eye Institute, Stanford University School of Medicine, Palo Alto, CA, USA

    Vinit B. Mahajan

  8. Veterans Affairs Palo Alto Health Care System, Palo Alto, CA, USA

    Vinit B. Mahajan

  9. Department of Pathology & Cell Biology, Stem Cell Initiative (CSCI), College of Physicians and Surgeons, Institute of Human Nutrition, Columbia University, New York, NY, USA

    Stephen H. Tsang

  10. Columbia University Medical Center, Harkness Eye Institute, 635 West 165th Street, Box 212, New York, NY, 10032, USA

    Stephen H. Tsang

Authors
  1. Vitor K. L. Takahashi
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Corresponding author

Correspondence to Stephen H. Tsang.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

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Takahashi, V.K.L., Takiuti, J.T., Carvalho-Jr, J.R.L. et al. Fundus autofluorescence and ellipsoid zone (EZ) line width can be an outcome measurement in RHO-associated autosomal dominant retinitis pigmentosa. Graefes Arch Clin Exp Ophthalmol 257, 725–731 (2019). https://doi.org/10.1007/s00417-018-04234-6

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  • DOI: https://doi.org/10.1007/s00417-018-04234-6

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