OPHTHALMIC FINDINGS in LATE STAGE SJOGREN-LARSSON SYNDROME

Tavish Nanda; Jaclyn L. Kovach

doi:10.1097/ICB.0000000000000583

OPHTHALMIC FINDINGS in LATE STAGE SJOGREN-LARSSON SYNDROME

Tavish Nanda, Jaclyn L. Kovach

Ophthalmology

Research output: Contribution to journal › Article › peer-review

5 Scopus citations

Abstract

Purpose:To report spectral domain optical coherence tomography and fundus autofluorescence documentation of late stage macular findings associated with Sjogren-Larsson Syndrome in three adult siblings.Methods:Three adult siblings with Sjogren-Larsson Syndrome underwent ophthalmic examination and imaging.Results:Crystalline maculopathy and subretinal deposits, presumably lipofuscin accumulation, with macular atrophy were present in varying degrees in all three adult siblings.Discussion:In adults with Sjogren-Larsson Syndrome, crystalline retinopathy can progress to macular atrophy and the appearance of lipofuscin accumulation.

Original language	English (US)
Pages (from-to)	251-254
Number of pages	4
Journal	Retinal Cases and Brief Reports
Volume	13
Issue number	3
DOIs	https://doi.org/10.1097/ICB.0000000000000583
State	Published - Jun 1 2019

Keywords

Muller cell
Sjogren-Larsson Syndrome
crystalline retinopathy
fundus autofluorescence
spectral domain optical coherence tomography

ASJC Scopus subject areas

Ophthalmology

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title = "OPHTHALMIC FINDINGS in LATE STAGE SJOGREN-LARSSON SYNDROME",

abstract = "Purpose:To report spectral domain optical coherence tomography and fundus autofluorescence documentation of late stage macular findings associated with Sjogren-Larsson Syndrome in three adult siblings.Methods:Three adult siblings with Sjogren-Larsson Syndrome underwent ophthalmic examination and imaging.Results:Crystalline maculopathy and subretinal deposits, presumably lipofuscin accumulation, with macular atrophy were present in varying degrees in all three adult siblings.Discussion:In adults with Sjogren-Larsson Syndrome, crystalline retinopathy can progress to macular atrophy and the appearance of lipofuscin accumulation.",

keywords = "Muller cell, Sjogren-Larsson Syndrome, crystalline retinopathy, fundus autofluorescence, spectral domain optical coherence tomography",

author = "Tavish Nanda and Kovach, {Jaclyn L.}",

note = "Funding Information: Funding was provided by NIH center grant P30-EY014801 and by an unrestricted grant to the University of Miami from Research to Prevent Blindness, New York, NY. The sponsor or funding organization had no role in the design or conduct of this research.",

year = "2019",

month = jun,

day = "1",

doi = "10.1097/ICB.0000000000000583",

language = "English (US)",

volume = "13",

pages = "251--254",

journal = "Retinal Cases and Brief Reports",

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T1 - OPHTHALMIC FINDINGS in LATE STAGE SJOGREN-LARSSON SYNDROME

AU - Nanda, Tavish

AU - Kovach, Jaclyn L.

N1 - Funding Information: Funding was provided by NIH center grant P30-EY014801 and by an unrestricted grant to the University of Miami from Research to Prevent Blindness, New York, NY. The sponsor or funding organization had no role in the design or conduct of this research.

PY - 2019/6/1

Y1 - 2019/6/1

N2 - Purpose:To report spectral domain optical coherence tomography and fundus autofluorescence documentation of late stage macular findings associated with Sjogren-Larsson Syndrome in three adult siblings.Methods:Three adult siblings with Sjogren-Larsson Syndrome underwent ophthalmic examination and imaging.Results:Crystalline maculopathy and subretinal deposits, presumably lipofuscin accumulation, with macular atrophy were present in varying degrees in all three adult siblings.Discussion:In adults with Sjogren-Larsson Syndrome, crystalline retinopathy can progress to macular atrophy and the appearance of lipofuscin accumulation.

AB - Purpose:To report spectral domain optical coherence tomography and fundus autofluorescence documentation of late stage macular findings associated with Sjogren-Larsson Syndrome in three adult siblings.Methods:Three adult siblings with Sjogren-Larsson Syndrome underwent ophthalmic examination and imaging.Results:Crystalline maculopathy and subretinal deposits, presumably lipofuscin accumulation, with macular atrophy were present in varying degrees in all three adult siblings.Discussion:In adults with Sjogren-Larsson Syndrome, crystalline retinopathy can progress to macular atrophy and the appearance of lipofuscin accumulation.

KW - Muller cell

KW - Sjogren-Larsson Syndrome

KW - crystalline retinopathy

KW - fundus autofluorescence

KW - spectral domain optical coherence tomography

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OPHTHALMIC FINDINGS in LATE STAGE SJOGREN-LARSSON SYNDROME

Abstract

Keywords

ASJC Scopus subject areas

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