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AIM: To explore the multimodal imaging features of butterfly-like retinal pigment epithelial dystrophy(BPD)patients.METHODS: Retrospective analysis of the multimodal imaging of 18 BPD patients(36 eyes)from January 2016 to July 2019, including fundus color photography, infrared photography, autofluorescence, fundus fluorescein angiography, indocyanine green angiography, optical coherence tomography(OCT), and optical coherence tomography angiography(OCTA). RESULTS: A typical fundus color photography showed the appearance of a butterfly-like lesion caused by abnormal pigmentation. The appearance of the butterfly was not obvious after the lesion progressed, and the corresponding region shrinks; Infrared photography showed the yellow lesions clearly in the BPD patients, which are white highlight images; Autofluorescence showed patchy, spotted butterfly wing performance. When the lesion progressed, autofluorescence did not show typical butterfly changes, but it showed the damage of pigment epithelial cells; Fundus fluorescein angiography and choroidal angiography, in addition to showed butterfly lesions, can more accurately display vascular lesions, especially choroidal neovascularization(CNV); OCT showed lesions located between the retinal pigment epithelial(RPE)layer and the photoreceptors. As the lesion progressed, the pigment epithelium showed enlarged lesions correspondingly. Secondary CNV patients can be seen to break RPE; OCTA showed that the lesions were not obvious at the deep and superficial layers of the retina. But the choroidal blood flow signals were lost in some degrees in OCTA, and the blood flow images of CNV can be detected sensitively.CONCLUTION: Multimode imaging technology can provide imaging features of progression in BPD patients, which helps clinicians to understand the disease more deeply.

Familial exudative vitreoretinopathy(FEVR)is a severe clinically and genetically heterogeneous retinal disease which characterized by abnormal development of the peripheral retinal vessels. FEVR presents many clinical phenotypes, the main and typical feature is retinal folds. There are various inheritance modes with high genetic heterogeneity of FEVR including autosomal recessive, X-recessive, autosomal dominant recessive, and other scattered inheritance modes. So far, nine FEVR pathogenic genes have been reported: NDP, FZD4, LRP5, CTNNA1, TSPAN12, ZNF408, KIF11, CTNNB1, and JAG1 genes. These genes are mainly involved in signaling pathways such as Wnt, Notch, and Norrin-β-catenin. This article reviews the above nine FEVR pathogenic genes and their signaling pathways.

AIM: To study the effect of monocular form deprivation(MD)on the synaptic density in the visual cortex of the amblyopic rats during the critical period of visual development, the visual cortical synaptophysin(SYN)expression and significance of its expression, as well as the relationship between the synaptic density and the visual function. This research will provide mechanisms underlying the pathogenesis and clinical treatment of amblyopia.METHODS: Normal newborn Long Evans rats were randomly divided into normal control group and amblyopia model group, each with 16 rats. Both groups of rats were raised in the same environment. The normal control group did not receive any treatment. On the 13d after birth, the amblyopic model group received monocular suture to establish a classic monocular form deprived amblyopic model. Both groups of rats were received Visual evoked potential(F-VEP)detection on the 51d. Samples were taken immediately after the detection. The transmission electron microscope and Image J image analysis software were used to observe and analyse the synaptic density in the V1M of the primary visual cortex of the two groups of rats. Frozen sections of visual cortex were stained by immunofluorescence histochemical staining by bleaching method and the expression of SYN positive neurons was observed and quantitatively analyzed. RESULTS: F-VEP examination showed that compared with the normal control group, the P2 latency of the deprived eyes in the amblyopic group was significantly long, and the amplitude of P2 wave was significantly lower than that of normal eyes(P

AIM: To investigate the role of reactive oxygen species (ROS) and antioxidant mechanism underlying the metabolic memory of bovine retinal pericytes (BRPs) induced by high glucose. METHODS: Effects of high glucose levels and culture time on BRPs viability were evaluated by CCK-8. BRPs were grown in high-glucose media (30 mmol/L) for 4d followed by culture in normal glucose condition (5.6 mmol/L) for 4d in an experimental group. In contrast, in negative and positive control groups, BRPs were grown in either normal-glucose media or high-glucose media for 8d, respectively. The ROS levels, apoptosis, the expression and activity of manganese superoxide dismutase (MnSOD) in BRPs, as well as the protective effect of adeno-associated viral (AAV)-mediated over expression of MnSOD were determined separately by DCHFA, ELISA and Western blot. RESULTS: Comparing the result of cells apoptosis, activity and protein expression of MnSOD and caspase-3, the cell culture system that exposed in sequence in 30 mmol/L and normal glucose for 4d was demonstrated as a suitable model of metabolic memory. Furthermore, delivery of antioxidant gene MnSOD can decrease BRPs apoptosis, reduce activated caspase-3, and reverse hyperglycemic memory by reducing the ROS of mitochondria. CONCLUSION: Increased ROS levels and decreased MnSOD levels may play important roles in pericyte loss of diabetic retinopathy. BRPs cultured in high glucose for 4d followed by normal glucose for 4d could be an appropriate model of metabolic memory. rAAV-MnSOD gene therapy provides a promising strategy to inhibit this blinding disease.

Diabetic retinopathy (DR), a major micro-vascular complication of diabetes, has emerged as a leading cause of visual impairment and blindness among working adults in the worldwide. The pathobiology of DR involves multiple molecular pathways and is characterized chronic neurovascular degeneration. Current approaches to prevent or to treat DR are still far from satisfactory. Therefore, it is important to develop new therapeutic strategies for the prevention and treatment to DR. Pigment epithelium-derived factor (PEDF), a 50-kDa secreted glycoprotein, has been described as a multi-functional protein. Some emerging evidences indicate that PEDF are able to target multiple pathways exerting neurotropic, neuroprotective, anti-angiogenic, antivasopermeability, anti-inflammation, anti-thrombogenic and anti-oxidative effects in DR. In this review, we addressed the functions of PEDF in different pathways, which could lead to potential therapeutics on the treatment to DR.