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@ARTICLE{YuWaiMan:268724,
author = {Yu-Wai-Man, Patrick and Carelli, Valerio and Newman, Nancy
J and Silva, Magda Joana and Linden, Aki and Van Stavern,
Gregory and Szaflik, Jacek P and Banik, Rudrani and
Lubiński, Wojciech and Pemp, Berthold and Liao, Yaping
Joyce and Subramanian, Prem S and Misiuk-Hojło, Marta and
Newman, Steven and Castillo, Lorena and Kocięcki, Jarosław
and Levin, Marc H and Muñoz-Negrete, Francisco Jose and
Yagan, Ali and Cherninkova, Sylvia and Katz, David and
Meunier, Audrey and Votruba, Marcela and Korwin, Magdalena
and Dziedziak, Jacek and Jurkutė, Neringa and Harvey,
Joshua P and La Morgia, Chiara and Priglinger, Claudia and
Llòria, Xavier and Tomasso, Livia and Klopstock, Thomas},
collaboration = {Group, LEROS Study},
title = {{T}herapeutic benefit of idebenone in patients with {L}eber
hereditary optic neuropathy: {T}he {LEROS} nonrandomized
controlled trial.},
journal = {Cell reports / Medicine},
volume = {5},
number = {3},
issn = {2666-3791},
address = {Maryland Heights, MO},
publisher = {Elsevier},
reportid = {DZNE-2024-00303},
pages = {101437},
year = {2024},
abstract = {Leber hereditary optic neuropathy (LHON) is a mitochondrial
disease leading to rapid and severe bilateral vision loss.
Idebenone has been shown to be effective in stabilizing and
restoring vision in patients treated within 1 year of onset
of vision loss. The open-label, international, multicenter,
natural history-controlled LEROS study (ClinicalTrials.gov
NCT02774005) assesses the efficacy and safety of idebenone
treatment (900 mg/day) in patients with LHON up to 5 years
after symptom onset (N = 199) and over a treatment period of
24 months, compared to an external natural history control
cohort (N = 372), matched by time since symptom onset. LEROS
meets its primary endpoint and confirms the long-term
efficacy of idebenone in the subacute/dynamic and chronic
phases; the treatment effect varies depending on disease
phase and the causative mtDNA mutation. The findings of the
LEROS study will help guide the clinical management of
patients with LHON.},
keywords = {Humans / Optic Atrophy, Hereditary, Leber: drug therapy /
Optic Atrophy, Hereditary, Leber: genetics / Optic Atrophy,
Hereditary, Leber: diagnosis / Antioxidants: therapeutic use
/ Ubiquinone: therapeutic use / Ubiquinone: genetics /
Ubiquinone: analogs $\&$ derivatives / Mutation / LHON
(Other) / Leber hereditary optic neuropathy (Other) /
idebenone (Other) / mitochondrial disease (Other) / mtDNA
(Other) / neuro-ophthalmology (Other) / optic atrophy
(Other) / optic neuropathy (Other) / retinal ganglion cells
(Other) / idebenone (NLM Chemicals) / Antioxidants (NLM
Chemicals) / Ubiquinone (NLM Chemicals)},
cin = {Clinical Research (Munich)},
ddc = {610},
cid = {I:(DE-2719)1111015},
pnm = {353 - Clinical and Health Care Research (POF4-353)},
pid = {G:(DE-HGF)POF4-353},
typ = {PUB:(DE-HGF)16},
pmc = {pmc:PMC10982982},
pubmed = {pmid:38428428},
doi = {10.1016/j.xcrm.2024.101437},
url = {https://pub.dzne.de/record/268724},
}