PMID- 37979600
OWN - NLM
STAT- MEDLINE
DCOM- 20240226
LR  - 20240503
IS  - 1879-1891 (Electronic)
IS  - 0002-9394 (Linking)
VI  - 259
DP  - 2024 Mar
TI  - Progression of PROM1-Associated Retinal Degeneration as Determined by 
      Spectral-Domain Optical Coherence Tomography Over a 24-Month Period.
PG  - 109-116
LID - S0002-9394(23)00472-5 [pii]
LID - 10.1016/j.ajo.2023.11.010 [doi]
AB  - PURPOSE: To evaluate the progression of atrophy as determined by spectral-domain 
      optical coherence tomography (SD-OCT) in patients with molecularly confirmed 
      PROM1-associated retinal degeneration (RD) over a 24-month period. DESIGN: 
      International, multicenter, prospective case series. METHODS: A total of 13 eyes 
      (13 patients) affected with PROM1-associated RD were enrolled at 5 sites and 
      SD-OCT images were obtained at baseline and after 24 months. Loss of mean 
      thickness (MT) and intact area were estimated after semi-automated segmentation 
      for the following individual retinal layers in the central subfield (CS), inner 
      ring, and outer ring of the ETDRS grid: retinal pigment epithelium (RPE), outer 
      segments (OS), inner segments (IS), outer nuclear layer (ONL), inner retina (IR), 
      and total retina (TR). RESULTS: Statistically significant losses of thickness of 
      RPE and TR were detected in the CS and inner ring and of ONL and IS in the outer 
      ring (all P < .05); a statistically significant decrease in the intact area of 
      RPE and IS was observed in the inner ring, and of ONL in the outer ring (all P < 
      .05); the change in MT and the intact area of the other layers showed a trend of 
      decline over an observational period of 24 months. CONCLUSIONS: Significant 
      thickness losses could be detected in outer retinal layers by SD-OCT over a 
      24-month period in patients with PROM1-associated retinal degeneration. Loss of 
      thickness and/or intact area of such layers may serve as potential endpoints for 
      clinical trials that aim to slow down the disease progression of PROM1-associated 
      retinal degeneration.
CI  - Copyright (c) 2023 The Authors. Published by Elsevier Inc. All rights reserved.
FAU - Grosspotzl, Manuel
AU  - Grosspotzl M
AD  - Department of Ophthalmology (M.G., G.S., A.W., G.S., R.W.S), Medical University 
      Graz, Graz, Austria.
FAU - Riedl, Regina
AU  - Riedl R
AD  - Institute for Medical Informatics, Statistics and Documentation (R.R.), Medical 
      University Graz, Graz, Austria.
FAU - Schliessleder, Gernot
AU  - Schliessleder G
AD  - Department of Ophthalmology (M.G., G.S., A.W., G.S., R.W.S), Medical University 
      Graz, Graz, Austria.
FAU - Hu, Zhihong Jewel
AU  - Hu ZJ
AD  - Doheny Eye Institute (Z.J.H., S.V.S.), David Geffen School of Medicine at 
      University of California Los Angeles, Los Angeles, California, USA.
FAU - Michaelides, Michel
AU  - Michaelides M
AD  - Moorfields Eye Hospital (M.M., R.W.S.), NHS Foundation Trust, London, United 
      Kingdom; UCL Institute of Ophthalmology (M.M., R.W.S.), University College 
      London, London, United Kingdom.
FAU - Sadda, SriniVas
AU  - Sadda S
AD  - Doheny Eye Institute (Z.J.H., S.V.S.), David Geffen School of Medicine at 
      University of California Los Angeles, Los Angeles, California, USA.
FAU - Birch, David
AU  - Birch D
AD  - Retina Foundation of the Southwest (D.B.), Dallas, Texas, USA.
FAU - Charbel Issa, Peter
AU  - Charbel Issa P
AD  - Department of Ophthalmology (P.C.I.), University of Bonn, Bonn, Germany; Oxford 
      Eye Hospital (P.C.I.), Oxford University Hospitals NHS Foundation Trust, Oxford, 
      United Kingdom; Nuffield Laboratory of Ophthalmology (P.C.I.), Department of 
      Clinical Neurosciences, University of Oxford, Oxford, United Kingdom.
FAU - Wedrich, Andreas
AU  - Wedrich A
AD  - Department of Ophthalmology (M.G., G.S., A.W., G.S., R.W.S), Medical University 
      Graz, Graz, Austria.
FAU - Seidel, Gerald
AU  - Seidel G
AD  - Department of Ophthalmology (M.G., G.S., A.W., G.S., R.W.S), Medical University 
      Graz, Graz, Austria.
FAU - Scholl, Hendrik P N
AU  - Scholl HPN
AD  - Institute of Molecular and Clinical Ophthalmology Basel (H.P.N.S.), Basel, 
      Switzerland; Department of Ophthalmology (R.W.S.), University of Basel, Basel, 
      Switzerland.
FAU - Strauss, Rupert W
AU  - Strauss RW
AD  - Department of Ophthalmology (M.G., G.S., A.W., G.S., R.W.S), Medical University 
      Graz, Graz, Austria; Moorfields Eye Hospital (M.M., R.W.S.), NHS Foundation 
      Trust, London, United Kingdom; UCL Institute of Ophthalmology (M.M., R.W.S.), 
      University College London, London, United Kingdom; Institute of Molecular and 
      Clinical Ophthalmology Basel (H.P.N.S.), Basel, Switzerland; Wilmer Eye Institute 
      (R.W.S.), Johns Hopkins University, Baltimore, Maryland, USA. Electronic address: 
      r.strauss@medunigraz.at.
LA  - eng
GR  - R21 EY029839/EY/NEI NIH HHS/United States
PT  - Journal Article
PT  - Multicenter Study
DEP - 20231117
PL  - United States
TA  - Am J Ophthalmol
JT  - American journal of ophthalmology
JID - 0370500
RN  - 0 (PROM1 protein, human)
RN  - 0 (AC133 Antigen)
SB  - IM
MH  - Humans
MH  - Tomography, Optical Coherence/methods
MH  - *Retinal Degeneration/diagnosis
MH  - Retina
MH  - Retinal Pigment Epithelium
MH  - *Macular Degeneration
MH  - AC133 Antigen
EDAT- 2023/11/19 09:42
MHDA- 2024/02/26 06:44
CRDT- 2023/11/18 19:26
PHST- 2023/09/04 00:00 [received]
PHST- 2023/11/06 00:00 [revised]
PHST- 2023/11/10 00:00 [accepted]
PHST- 2024/02/26 06:44 [medline]
PHST- 2023/11/19 09:42 [pubmed]
PHST- 2023/11/18 19:26 [entrez]
AID - S0002-9394(23)00472-5 [pii]
AID - 10.1016/j.ajo.2023.11.010 [doi]
PST - ppublish
SO  - Am J Ophthalmol. 2024 Mar;259:109-116. doi: 10.1016/j.ajo.2023.11.010. Epub 2023 
      Nov 17.