Biomolecules · 2024 · 56 citations · 11 references
MedicineOphthalmologyPharmacotherapyGene TherapyOcular DiseaseOcular PharmacologyOcular Gene TherapyRetinal DiseaseRetinal DisordersRetinal PharmacotherapyCellular Gene TherapyCongenital Retinal DystrophyInherited Retinal Disease Gene TherapyInherited Retinal Dystrophy TherapyLong-term Safety Follow-upReal-world Safety DataRetinal Drug SafetyTherapeutic Trials
Voretigene neparvovec (VN) is the first available gene therapy for patients with biallelic RPE65-mediated inherited retinal dystrophy who have sufficient viable retinal cells. PERCEIVE is an ongoing, post-authorization, prospective, multicenter, registry-based observational study and is the largest study assessing the real-world, long-term safety and effectiveness of VN. Here, we present the outcomes of 103 patients treated with VN according to local prescribing information. The mean (SD) age was 19.5 (10.85) years, 52 (50.5%) were female, and the mean (SD) duration of the follow up was 0.8 (0.64) years (maximum: 2.3 years). Thirty-five patients (34%) experienced ocular treatment-emergent adverse events (TEAEs), most frequently related to chorioretinal atrophy (n = 13 [12.6%]). Eighteen patients (17.5%; 24 eyes [13.1%]) experienced ocular TEAEs of special interest, including intraocular inflammation and/or infection related to the procedure (n = 7). The mean (SD) changes from baseline in full-field light-sensitivity threshold testing (white light) at month 1, month 6, year 1, and year 2 were −16.59 (13.48) dB (51 eyes), −18.24 (14.62) dB (42 eyes), −15.84 (14.10) dB (10 eyes), and −13.67 (22.62) dB (13 eyes), respectively. The change in visual acuity from baseline was not clinically significant. Overall, the outcomes of the PERCEIVE study are consistent with the findings of VN pivotal clinical trials.
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Stephen R. Russell, Jean Bennett, Jennifer Wellman et al. · The Lancet · 2017 · 1.9K citations
Clemens Lange, Nicolas Feltgen, Bernd Junker et al. · Graefe s Archive for Clinical and Experimental Ophthalmology · 2008 · 629 citations