Common clinical features of unilateral retinal pigment epithelium dysgenesis and combined hamartoma of the retina and retinal pigment epithelium.

BACKGROUND: Herein, we report two cases of unilateral retinal pigment epithelium dysgenesis (URPED) in Chinese patients and explore the relationship between URPED and combined hamartoma of the retina and retinal pigment epithelium (CHRRPE). CASE PRESENTATION: The lesion margins in the two cases showed pathognomonic clinical features of URPED, namely, a scalloped reticular margin in hyperplastic retinal pigment epithelium and mild fibrosis. The hypoautofluorescence observed by fundus autofluorescence was inverted compared with that observed by fundus fluorescence angiography. A large amount of fibroglial proliferation and disorganization of the retina involving the whole layer, which are also found in peripapillary CHRRPE, were found in the lesions. CONCLUSIONS: URPED appears to share some clinical features with CHRRPE, and the relationship between URPED and CHRRPE needs further study.

The response of anti-VEGF therapy and tamoxifen withdrawal of tamoxifen-induced cystoid macular edema in the same patient.

BACKGROUND: Numerous cases with ocular toxicity secondary to tamoxifen have been reported, and became more apparent with keratopathy, cataract, optic neuritis, macular holes, crystalline retinopathy with or without cystoid macular edema (CME). Withdrawing tamoxifen with the approval of the oncologist is the major treatment for cases with tamoxifen-induced retinopathy. CASE PRESENTATION: We herein reported a patient with a two-year history of painless and reduced visual acuity in both eyes who received tamoxifen therapy for 6 years. Tamoxifen-induced rentinopathy with CME showed significant development even though the patient has already discontinued tamoxifen treatment for 6 months. Anatomic improvements after intravitreal ranibizumab injection in both eyes were significant but were temporary. Surprisingly, CME in both eyes has been resolved spontaneously after 10 months in the penultimate visit without any therapy. CONCLUSION: Intravitreal ranibizumab injection temporarily improved the anatomy of the eyes in a case with tamoxifen-induced CME, and only tamoxifen withdrawal can bring a sustained effect.

Cyclic RGD Peptide Targeting Coated Nano Drug Co-Delivery System for Therapeutic Use in Age-Related Macular Degeneration Disease.

Vascular endothelial growth factor (VEGF) expression increased significantly in the pathogenesis of age-related macular degeneration, which induced the formation of pathological blood vessels. Dexamethasone is an exogenous anti-angiogenic drug while bevacizumab is an endogenous anti-angiogenic drug. They both have been widely used in ophthalmology. However, independent administration is not enough to completely block the development of choroidal neovascularization (CNV), and the number of eyes vitreous injections is limited. Reasonable combination of drugs may produce significantly better therapeutic effect than single drug treatment. The cyclic RGD (cRGD) peptide has a particularly high affinity with retinal pigment epithelial cells, where VEGF secretes from. In this study, we prepared nanoparticles of bevacizumab and dexamethasone with cRGD peptide as the target (aBev/cRGD-DPPNs). The particle size of the aBev/cRGD-DPPNs was 213.8 ± 1.5 nm, SEM results showed that the nano-carriers were well dispersed and spherical. The cell uptake study demonstrated the selectivity of the aBev/cRGD-DPPN to ARPE-19 with αVß3 over expressed. The aBev/cRGD-DPPNs had a better apoptosis induction effect and an obvious inhibitory effect on migration, invasion, and capillary-like structures formation of human umbilical vein epithelial cells. The fluorescein fundus angiography study, immunohistochemistry and histopathological evaluation showed the aBev/cRGD-DPPNs greatly reduced the development of CNV on a rabbit model.

Complete recovery of the retinal pigment epithelium layer after spontaneous large serous retinal pigment epithelial tear.

INTRODUCTION: The objective of this study is to report a case of complete recovery of the retinal pigment epithelium (RPE) layer after spontaneous large serous RPE tear and explore the possible RPE repair mechanism. CASE DESCRIPTION: A 63-year-old male patient developed serous detachment of the RPE in the macula of his left eye. During the follow-up period, an oval-shaped RPE tear spontaneously occurred in the macular area, and fundus autofluorescence showed that the torn area was 6.59 mm2. Spectral-domain optical coherence tomography (OCT) revealed that the subretinal fluid had been absorbed and that the continuity of the RPE layer was gradually restored from the periphery to the center over the course of 9 months. Moreover, visual acuity (VA) in the left eye was restored to 20/20, and there was no significant difference in macular function between the two eyes, as measured by multifocal electroretinography, at the last follow-up. In addition, the patient received no special treatment during the entire follow-up period. CONCLUSION: The integrity of the RPE layer can be restored completely after extensive RPE tear, and recovered RPE may allow the recovery of macular function.

Five-year follow-up of a spontaneously progressive retinal racemose hemangioma.

PURPOSE: To report the progression of a patient with retinal racemose hemangioma after five years of follow-up. METHODS: Observational case report. RESULTS: A 14-year-old girl was diagnosed with an isolated retinal racemose hemangioma with retinal arteriovenous malformation in the posterior pole of the left eye fundus. At the fifth year of follow-up, a spontaneous enlargement of the previously normal vessel was found above the original lesion. The patient's vision remained stable and no complication was observed. CONCLUSIONS: Retinal racemose hemangioma was once considered stable and non-progressive, but progression and expansion of the lesion may also occur, so long-term follow-up is necessary.

Lentiviral-mediated overexpression of KCTD12 inhibits the proliferation of human uveal melanoma OCM-1 cells.

Human potassium channel tetramerization domain containing 12 (KCTD12, also known as Pfetin) is a member of the KCTD family which consists of 26 members. It has been reported that KCTD12 regulates agonist potency and kinetics of GABAB receptor signaling. Proteomic analysis indicates that KCTD12 may be a potential biomarker for the diagnosis and prognosis of gastrointestinal stromal tumors. However, little has been reported concerning the role of KCTD12 in the other tumor types. In the present study, we designed and subcloned N-terminally Flag-tagged human KCTD12 into the pLVX­Puro vector. We then generated a human uveal melanoma cell line (OCM-1) stably expressing KCTD12. Using this stable cell line, we performed a series of experiments including colony formation, invasion, migration and wound-healing assays, flow cytometry and western blotting. Based on the experimental results, we first demonstrated that KCTD12 effectively suppressed the proliferation of OCM-1 cells and limited the spread of OCM-1 cells. In the flow cytometric analysis, prolongation of the progression of G2/M to G1 phase in the KCTD12-overexpressing OCM-1 cells was observed. In addition, inhibition of KCTD12-overexpressing OCM-1 cell xenograft growth in nude mice was observed. Taken together, KCTD12 may serve as a novel therapeutic target for patients with uveal melanoma.

Agmatine protects Müller cells from high-concentration glucose-induced cell damage via N-methyl-D-aspartic acid receptor inhibition.

Neural injury is associated with the development of diabetic retinopathy. Müller cells provide structural and metabolic support for retinal neurons. High glucose concentrations are known to induce Müller cell activity. Agmatine is an endogenous polyamine, which is enzymatically formed in the mammalian brain and has exhibited neuroprotective effects in a number of experimental models. The aims of the present study were to investigate whether agmatine protects Müller cells from glucose-induced damage and to explore the mechanisms underlying this process. Lactate dehydrogenase activity and tumor necrosis factor-α mRNA expression were significantly reduced in Müller cells exposed to a high glucose concentration, following agmatine treatment, compared with cells not treated with agmatine. In addition, agmatine treatment inhibited glucose-induced Müller cell apoptosis, which was associated with the regulation of Bax and Bcl-2 expression. Agmatine treatment suppressed glucose-induced phosphorylation of mitogen-activated protein kinase (MAPK) protein in Müller cells. The present study demonstrated that the protective effects of agmatine on Müller cells were inhibited by N-methyl-D-aspartic acid (NMDA). The results of the present study suggested that agmatine treatment protects Müller cells from high-concentration glucose-induced cell damage. The underlying mechanisms may relate to the anti-inflammatory and antiapoptotic effects of agmatine, as well as to the inhibition of the MAPK pathway, via NMDA receptor suppression. Agmatine may be of use in the development of novel therapeutic approaches for patients with diabetic retinopathy.

Clinical manifestations of syphilitic chorioretinitis: a retrospective study.

BACKGROUND: Syphilitic chorioretinitis can produce severe vision loss. However, the clinical manifestations of syphilitic chorioretinitis are still unclear, particularly during different stages. Herein, we will present our diagnostic technique for syphilitic chorioretinitis. METHODS: This retrospective study recruited 109 cases; we performed a clinical evaluation including case history, serology analysis, fundus photography, fluorescein fundus angiography with or without indocyanine green angiography, auto-fluorescence, and optical coherence tomography. RESULTS: 109 were diagnosed with acute syphilitic posterior placoid chorioretinitis by fundus photograph that revealed filthy, yellowish-white lesions. For autofluorescence, during early-stage syphilitic chorioretinitis, hyperfluorescence could be observed. During the convalescence stage, the fluorescence became hypofluorescence or disappeared. Fluorescein fundus angiography indicated early-stage transmitted fluorescence or hypofluorescence. During the venous stage, the lesion area had fluorescent leakage, mostly accompanied by retinal vasculitis. During the late stage, speckle staining was observed with optic disc fluorescence. Hypofluorescence or undistinguishable fluorescence was seen at an early stage with indocyanine green angiography. At an advanced stage, the lesion had obvious hypofluorescence. Optical coherence tomography indicated various inner segment/outer segment damage, accompanied by retinal pigment epithelium impairment. The inner segment/outer segment alteration could be lessened with treatment. CONCLUSIONS: The clinical manifestations of syphilitic chorioretinitis include impaired vision, shadow blocking, or photopsia of one or both eyes. Fundus photography, fluorescein fundus angiography with or without indocyanine green angiography, autofluorescence, and optical coherence tomography could be useful accessory examinations. Autofluorescence and optical coherence tomography could be the main examinations for monitoring disease progression.