PMID- 35816046
OWN - NLM
STAT- MEDLINE
DCOM- 20220713
LR  - 20220721
IS  - 1552-5783 (Electronic)
IS  - 0146-0404 (Print)
IS  - 0146-0404 (Linking)
VI  - 63
IP  - 8
DP  - 2022 Jul 8
TI  - Choroideremia Carriers: Dark-Adapted Perimetry and Retinal Structures.
PG  - 4
LID - 10.1167/iovs.63.8.4 [doi]
LID - 4
AB  - PURPOSE: In choroideremia (CHM) carriers, scotopic sensitivity was assessed by 
      dark adapted chromatic perimetry (DACP) and outer retinal structure was evaluated 
      by multimodal imaging. METHODS: Nine carriers (18 eyes) and 13 healthy controls 
      (13 eyes) underwent DACP testing with cyan and red stimuli. Analysis addressed 
      peripapillary (4 test locations closest to the optic disc), macular (52 
      locations), and peripheral (60 locations outside the macula) regions. Responses 
      were considered to be rod-mediated when cyan relative to red sensitivity was 
      >5 dB. Fundus imaging included spectral domain optical coherence tomography 
      (SD-OCT), short-wavelength (SW-AF), near-infrared (NIR-AF), ultrawide-field (200 
      degrees) pseudocolor fundus imaging, and quantitative (qAF) fundus 
      autofluorescence. RESULTS: Detection of the cyan stimulus was rod mediated in 
      essentially all test locations (99.7%). In the macular and peripheral areas, DACP 
      sensitivity values were not significantly different from healthy eyes. In the 
      peripapillary area, sensitivities were significantly decreased (P < 0.05). SD-OCT 
      imaging ranged from hyper-reflective lesions and discontinuities of the outer 
      retinal bands to hypertransmission of signal. SW-AF and NIR-AF images presented 
      with peripapillary atrophy in seven patients (14 eyes). Mosaicism was detectable 
      in SW-AF images in seven patients and in NIR-AF images in five patients. Frank 
      hypo-autofluorescence was visible in eight patients with distinct 
      chorioretinopathy in seven patients. The qAF values were below the 95% confidence 
      interval (CI) of healthy age-matched individuals in 12 eyes. CONCLUSIONS: Rod 
      mediated scotopic sensitivity was comparable to that in control eyes in macular 
      and peripheral areas but was decreased in the peripapillary area where changes in 
      retinal structure were also most severe.
FAU - Parmann, Rait
AU  - Parmann R
AD  - Departments of Ophthalmology, Columbia University, New York, NY, United States.
FAU - Greenstein, Vivienne C
AU  - Greenstein VC
AD  - Departments of Ophthalmology, Columbia University, New York, NY, United States.
FAU - Tsang, Stephen H
AU  - Tsang SH
AD  - Departments of Ophthalmology, Columbia University, New York, NY, United States.
AD  - Departments of Pathology and Cell Biology, Columbia University, New York, NY, 
      United States.
FAU - Sparrow, Janet R
AU  - Sparrow JR
AD  - Departments of Ophthalmology, Columbia University, New York, NY, United States.
AD  - Departments of Pathology and Cell Biology, Columbia University, New York, NY, 
      United States.
LA  - eng
GR  - R01 EY024091/EY/NEI NIH HHS/United States
GR  - R01 EY018213/EY/NEI NIH HHS/United States
GR  - R01 EY009076/EY/NEI NIH HHS/United States
GR  - R01 EY024698/EY/NEI NIH HHS/United States
GR  - P30 EY019007/EY/NEI NIH HHS/United States
PT  - Journal Article
PT  - Research Support, N.I.H., Extramural
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - Invest Ophthalmol Vis Sci
JT  - Investigative ophthalmology & visual science
JID - 7703701
SB  - IM
MH  - *Choroideremia/diagnosis/pathology
MH  - Fluorescein Angiography/methods
MH  - Fundus Oculi
MH  - Humans
MH  - Retina/diagnostic imaging/pathology
MH  - Tomography, Optical Coherence/methods
MH  - Visual Field Tests
PMC - PMC9284471
COIS- Disclosure: R. Parmann, None; V.C. Greenstein, None; S.H. Tsang, Abeona 
      Therapeutics, Inc. (F), Emendo Bio, Inc. (F), Rejuvitas (O), Nanoscope 
      Therapeutics (S); J.R. Sparrow, None
EDAT- 2022/07/12 06:00
MHDA- 2022/07/14 06:00
PMCR- 2022/07/11
CRDT- 2022/07/11 09:33
PHST- 2022/07/11 09:33 [entrez]
PHST- 2022/07/12 06:00 [pubmed]
PHST- 2022/07/14 06:00 [medline]
PHST- 2022/07/11 00:00 [pmc-release]
AID - 2783465 [pii]
AID - IOVS-22-35082 [pii]
AID - 10.1167/iovs.63.8.4 [doi]
PST - ppublish
SO  - Invest Ophthalmol Vis Sci. 2022 Jul 8;63(8):4. doi: 10.1167/iovs.63.8.4.