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Progression of retinitis pigmentosa on multimodal imaging: the PREP‐1 study
摘要： Importance: It is important to establish reliable outcome measures to detect progression in retinitis pigmentosa (RP). Background: To evaluate progression of RP using multimodal imaging, including spectral-domain optical coherence tomography (SD-OCT), fundus autofluorescence (FAF) and microperimetry (MP). Design: Retrospective longitudinal study at a tertiary teaching hospital. Participants: 205 eyes of 106 patients with RP with 1-5 years of follow-up. Methods: Demographics and visual acuity (VA) were recorded, and each modality was graded at baseline and every annual follow-up. SD-OCT was graded for the width of ellipsoid zone (EZ), FAF was graded for the diameter and area of the hyperautofluorescent ring (if present), and MP was graded for mean, central and paracentral sensitivity. Spearman’s correlation was used to measure correlations at baseline. Mixed effects models were used to estimate the annual change of each parameter, adjusted for disease duration. Main outcome measures: Rate of progression. Results: The median VA at baseline was 75 letters and was positively correlated with mean and central sensitivity (r: 0.372 and 0.394; p=0.01 for both). All parameters (except paracentral sensitivity) were strongly correlated with each other (r: 0.673-0.991; p<0.001 for all). The annual rates of change for each parameter were as follows: VA, -2.3 letters (p<0.001); EZ, -151um (p<0.001); ring diameter, -132um (p<0.001); ring area, -0.4mm2 (p<0.001); mean sensitivity, -0.3 dB (p<0.001); central sensitivity, -0.7 dB (p<0.001); paracentral sensitivity, -0.4 dB (p<0.001). Conclusion and relevance: Structural and functional measures are well correlated in RP and can reliable measure disease progression within the course of a year.
关键词： Optical Coherence Tomography,Multimodal Imaging,Microperimetry,Fundus Autofluorescence,Disease Progression
更新于2025-09-04 15:30:14
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Radiation-responsive scintillating nanotheranostics for reduced hypoxic radioresistance under ROS/NO-mediated tumor microenvironment regulation
摘要： Abstract: Hypoxia-induced radioresistance is the primary reason for failure of tumor radiotherapy (RT). Changes within the irradiated tumor microenvironment (TME) including oxygen, reactive oxygen species (ROS) and nitric oxide (NO) are closely related to radioresistance. Therefore, there is an urgent need to develop new approaches for overcoming hypoxic radioresistance by incorporating TME regulation into current radiotherapeutic strategies. Methods: Herein, we explored a radiation-responsive nanotheranostic system to enhance RT effects on hypoxic tumors by multi-way therapeutic effects. This system was developed by loading S-nitrosothiol groups (SNO, a NO donor) and indocyanine green (ICG, a photosensitizer) onto mesoporous silica shells of Eu3+-doped NaGdF4 scintillating nanocrystals (NSC). Results: Under X-ray radiation, this system can increase the local dosage by high-Z elements, promote ROS generation by X-ray-induced photodynamic therapy, and produce high levels of NO to enhance tumor-killing effects and improve hypoxia via NO-induced vasodilation. In vitro and in vivo studies revealed that this combined strategy can greatly reinforce DNA damage and apoptosis of hypoxic tumor cells, while significantly suppressing tumor growth, improving tumor hypoxia and promoting p53 up-regulation and HIF1α down-regulation. In addition, this system showed pronounced tumor contrast performance in T1-weighted magnetic resonance imaging and computed tomography. Conclusion: This work demonstrates the great potential of scintillating nanotheranostics for multimodal imaging-guided X-ray radiation-triggered tumor combined therapy to overcome radioresistance.
关键词： X-ray induced photodynamic therapy,hypoxia-induced radioresistance,nanoscintillators,multimodal imaging,nitric oxide
更新于2025-09-04 15:30:14
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Intraoperative OCT imaging of the Argus II retinal prosthesis and its clinical significance
摘要： To report the clinical, multimodal imaging and genetic characteristics of patients with Enhanced-S-cone syndrome (ESCS) in a Tunisian cohort. Methods: Descriptive clinical and genetic study including patients with inherited retinal dystrophies who consulted the oculogenetics laboratory LR14SP01, at Hedi Rais Institute of ophthalmology. Results: A total of 9 patients from 6 consanguineous families were diagnosed with ESCS based on typical clinical and ERG abnormalities. All patients complaint from hemeralopia since childhood. Visual acuity ranged from light perception to 4/10. Fundus examination revealed variable abnormalities with radial yellow dots in the posterior pole in two patients, nummular yellow dots in one patient, minimal pigmentary changes along the arcades in five patients, nummular pigment clumping in 3 patients and peripheral retinal atrophy in one patient. One patient had macular atrophy and 8 had macular cysts confirmed by SS-OCT. Genetic analysis was contributive in 7 patients and revealed NR2E3 mutations in 5 patients and NR2E3 + RHO digenism in two patients from the same family. Conclusions: This is to our knowledge the first report on a Tunisian cohort with ESCS. The phenotype in ESCS is highly variable and multimodal imaging may help to the diagnosis in addition to electrophysiology results.
关键词： genetic characteristics,ESCS,multimodal imaging,Enhanced-S-cone syndrome,Tunisian cohort
更新于2025-09-04 15:30:14