PMID- 30834176
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20220408
IS  - 2164-2591 (Print)
IS  - 2164-2591 (Electronic)
IS  - 2164-2591 (Linking)
VI  - 8
IP  - 2
DP  - 2019 Mar
TI  - Cross-Sectional and Longitudinal Assessment of the Ellipsoid Zone in 
      Childhood-Onset Stargardt Disease.
PG  - 1
LID - 10.1167/tvst.8.2.1 [doi]
LID - 1
AB  - PURPOSE: To evaluate the reliability of ellipsoid zone (EZ) loss width and area 
      measurements from spectral-domain optical coherence tomography (SD-OCT) images 
      and track disease progression in childhood-onset Stargardt disease (STGD1). 
      METHODS: Children with molecularly confirmed STGD1 (n = 46, mean age 12.4 years) 
      underwent SD-OCT for the measurement of the transverse (width) loss of the EZ and 
      en face analysis to quantify the area of EZ loss. All scans were analyzed twice 
      by two graders to evaluate reliability. The annual rate of EZ width and area loss 
      were calculated. RESULTS: The intra- and intergrader reliability of transverse EZ 
      loss and area of EZ loss measurements at baseline for both graders was 0.99. The 
      mean annual rate of transverse EZ loss (+/-standard deviation) was 279.5 +/- 259.9 
      mum/y. The mean rate of area of EZ loss (+/-standard deviation) was 1.20 +/- 1.29 
      mm(2)/y. The percentage transverse EZ loss was 10.2 +/- 9.9%/y, which was 
      significantly lower than the area of EZ loss at 19.4 +/- 16.3%/y. High degree of 
      interocular symmetry was observed. CONCLUSIONS: This is a prospective study on 
      the quantification of EZ loss in children with STGD1 and highlights the 
      reliability of SD-OCT in measuring EZ loss. High intra- and intergrader 
      reliability was observed, with good ability to detect changes over time. 
      TRANSLATIONAL RELEVANCE: Measuring the area of EZ loss was more sensitive 
      compared with transverse EZ width loss measurements and will be valuable for 
      natural history studies and clinical trials requiring sensitive and reliable 
      structural endpoints.
FAU - Tanna, Preena
AU  - Tanna P
AD  - UCL Institute of Ophthalmology, University College London, London, UK.
AD  - Moorfields Eye Hospital, London, UK.
FAU - Georgiou, Michalis
AU  - Georgiou M
AD  - UCL Institute of Ophthalmology, University College London, London, UK.
AD  - Moorfields Eye Hospital, London, UK.
FAU - Strauss, Rupert W
AU  - Strauss RW
AD  - UCL Institute of Ophthalmology, University College London, London, UK.
AD  - Moorfields Eye Hospital, London, UK.
AD  - Departments of Ophthalmology, Johannes Kepler University and Medical University 
      Graz, Austria.
FAU - Ali, Naser
AU  - Ali N
AD  - UCL Institute of Ophthalmology, University College London, London, UK.
AD  - Moorfields Eye Hospital, London, UK.
FAU - Kumaran, Neruban
AU  - Kumaran N
AD  - UCL Institute of Ophthalmology, University College London, London, UK.
AD  - Moorfields Eye Hospital, London, UK.
FAU - Kalitzeos, Angelos
AU  - Kalitzeos A
AD  - UCL Institute of Ophthalmology, University College London, London, UK.
AD  - Moorfields Eye Hospital, London, UK.
FAU - Fujinami, Kaoru
AU  - Fujinami K
AD  - UCL Institute of Ophthalmology, University College London, London, UK.
AD  - Moorfields Eye Hospital, London, UK.
AD  - Division of Vision Research, National Institute of Sensory Organs, National 
      Hospital Organization, Tokyo Medical Center, Tokyo, Japan.
AD  - Department of Ophthalmology, Keio University School of Medicine, Tokyo, Japan.
FAU - Michaelides, Michel
AU  - Michaelides M
AD  - UCL Institute of Ophthalmology, University College London, London, UK.
AD  - Moorfields Eye Hospital, London, UK.
LA  - eng
GR  - Wellcome Trust/United Kingdom
PT  - Journal Article
DEP - 20190301
PL  - United States
TA  - Transl Vis Sci Technol
JT  - Translational vision science & technology
JID - 101595919
PMC - PMC6397016
OTO - NOTNLM
OT  - ABCA4
OT  - EZ
OT  - OCT
OT  - STGD1
OT  - Stargardt disease
OT  - clinical trials
OT  - ellipsoid zone
OT  - endpoints
OT  - retina
EDAT- 2019/03/06 06:00
MHDA- 2019/03/06 06:01
PMCR- 2019/03/01
CRDT- 2019/03/06 06:00
PHST- 2018/10/18 00:00 [received]
PHST- 2018/12/12 00:00 [accepted]
PHST- 2019/03/06 06:00 [entrez]
PHST- 2019/03/06 06:00 [pubmed]
PHST- 2019/03/06 06:01 [medline]
PHST- 2019/03/01 00:00 [pmc-release]
AID - TVST-18-1188 [pii]
AID - 10.1167/tvst.8.2.1 [doi]
PST - epublish
SO  - Transl Vis Sci Technol. 2019 Mar 1;8(2):1. doi: 10.1167/tvst.8.2.1. eCollection 
      2019 Mar.