Background: Extracellular matrix molecular components, previously linked to multisystem syndromes include collagens, fibrillins and laminins. Recently, we described a novel multisystem syndrome caused by the c.9418G>A p.(V3140M) mutation in the laminin alpha-5 (LAMA5) gene, which affects connective tissues of all organs and apparatus in a three generation family. In the same family, we have also reported a myopic trait, which, however, was linked to the Prolyl 4-hydroxylase subunit alpha-2 (P4HA2) gene. Results of investigation on vitreous changes and their pathogenesis are reported in the present study.Materials and Methods: Nineteen family individuals underwent complete ophthalmic examination including best-corrected visual acuity (BCVA), fundus examination, fundus photography, intraocular pressure measurement, axial length measurement using ocular biometry, Goldmann visual field examination, standard electroretinogram, SD-OCT. Segregation analysis of LAMA5 and P4HA2 mutations was performed in enrolled members.Results: The vitreous alterations fully segregated with LAMA5 mutation in both young and adult family members. Slight reduction of retinal thickness and peripheral retinal degeneration in only two patients were reported.Conclusions: In this work we showed that PVD is a common trait of LAMA5 multisystem syndrome, therefore occurring as an age-unrelated trait. We hypothesize that the p.(V3140M) mutation results in a reduction of retinal inner limiting membrane (ILM) stability, leading to a derangement in the macromolecular structure of the vitreous gel, and PVD. Further investigations will be necessary to elucidate the role of wild type and mutated LAMA5 in the pathogenesis of PVD. 相似文献
Retinal fundus photographs are employed as standard diagnostic tools in ophthalmology. Serial photographs of the flow of fluorescein and indocyanine green (ICG) dye are used to determine the areas of the retinal lesions. For objective measurements of features, the registration of the images is a necessity. In this paper, we employ optimization techniques for registration with the help of 2-parameter translational motion model of retinal angiograms, based on non-linear pre-processing (Wiener filtering and morphological gradient) and computation of the similarity criteria for the alignment of the two gradient images for any given rigid transformation. The optimization methods are effectively employed to minimize the similarity criterion.
The presence of noise, the variations in the background and the temporal variation of the fluorescence level pose serious problems in obtaining a robust registration of the retinal images. Moreover, local search strategies are not robust in the case of ICG angiograms, even if one uses a multiresolution approach.
The present work makes a systematic comparison of different optimization techniques, namely the minimization method derived from the optical flow formulation, the Nelder-Mead local search and the HCIAC ant colony metaheuristic, each optimizing a similarity criterion for the gradient images. The impact of the resolution and median filtering of gradient image is studied and the robustness of the approaches is tested through experimental studies, performed on macular fluorescein and ICG angiographies.
Our proposed optimization techniques have shown interesting results especially for high resolution difficult registration problems. Moreover, this approach seems promising for affine (6-parameter motion model) or elastical registrations. 相似文献
Background Intravitreal triamcinolone acetonide (TA) has been widely used as a therapeutic method for many ocular diseases, but a consensus on an appropriate safe therapeutic window of dosage for intravitreal injection, and whether vehicle of TA should be reduced or eliminated, has not yet been reached. The aim of this article is to investigate these issues.Methods Forty New Zealand white rabbits were divided into four experimental groups and one control group. Four or 25 mg TA, with vehicle either reduced or not, was injected into the vitreous cavity of rabbits in experimental groups. Rabbits in the control group received 0.2 ml intravitreal sterile saline solution. Intraocular pressures (IOP) were measured by a Tonopen tonometer. Values of lens density were measured by a Pentacam system. Soluble protein, total antioxidation capacity, reduced glutathione (GSH), glutathion peroxidase (GSH-px), and superoxide dismutase (SOD) in lens were measured by specific kits. ERG and pathological examinations, including light and electron microscopy of the retina, were also performed.Results Elevation of IOP was noted in all experimental groups after intravitreal TA (P<0.01, paired t-test). Significant increase of lens density was noticed at 1 week after intravitreal TA in the 25 mg vehicle-containing group (P<0.0001, paired t-test). Significant loss of GSH-px activity was noticed at the end of the study (P<0.05, paired t-test), while SOD activity increased (P<0.05, paired t-test). Amplitudes of ERG waves declined significantly in vehicle-containing groups (P<0.01, paired t-test) at the end of the study. Pathological examination showed obvious retinal toxicity in vehicle-containing groups.Conclusions Vehicle of TA should be eliminated or reduced before intravitreal injection to avoid potential retinal toxicity and transient increase in lens density.Presented at Chinese Academy of Fundus Disease Meeting, April, 2005.This study was supported by the 985 Fund of Peking University, Beijing.The authors have no financial interest in any instruments or drugs mentioned in this study. 相似文献
Background The Retinal Thickness Analyser (RTA) is intended to detect glaucomatous changes as well as macular pathologies at the posterior pole. We determined the diagnostic accuracy for eyes with manifest glaucoma or macular diseases.Methods We examined 71 eyes with long-term, established eye conditions. Included were 28 eyes with glaucoma, 21 with different macular diseases and 22 normal eyes. All examinations were evaluated in a blind-test by RTA experts without any clinical information on the patients. After comparison of the RTA interpretation with the clinical diagnosis, we determined sensitivity, specificity, positive and negative predictive values.Results Of 71 examinations, 15 (21%) were not interpretable. If these results are excluded, the following diagnostic accuracy values were calculated for glaucoma and macular disorders respectively: sensitivity 75 and 59%, specificity 55 and 97%, positive predictive value 48 and 90% and negative predictive value 80 and 84%. These values were not significantly different when both eyes of each patient were included in the final analysis (n=133).Conclusion The diagnostic values of the RTA determined in this case control study were not satisfactory. However, no clinical information was used in the assessment. The extent to which additional clinical information increases the diagnostic value remains to be determined. 相似文献
The development of the eye was investigated in the mouse embryo following a single administration of ethanol plus [3H]thymidine to the dam on day 13 of gestation. After 1 hr there was no difference in the number of labelled cells/100 micron 2 in the neural layer of the retina compared to controls, but there was an alcohol-related reduction in labelling density. After 24 hr there was an increase in the numbers of both pyknotic cells and mitotic figures, breaks occurred in the inner surface of the retina and cell debris was being extruded into the posterior chamber. At 48 hr the increase in pyknotic cells persisted, but there was less evidence of cell debris and the borders had been repaired. The estimated cell cycle time in the neural progenitor cells following maternal alcohol administration was increased 7-fold compared to controls. Morphometric analysis revealed that after 48 hr there were significant alcohol-related reductions in the width and depth of the eye, in the thickness of the neural layer and in the interocular distance. It appears that many of the ophthalmic abnormalities reported in human fetal alcohol syndrome can be produced in the mouse embryo following a single episode of acute maternal intoxication during a critical period of ocular ontogeny, and that they evolve primarily from disturbances in the normal patterns of recruitment and loss of neural progenitor cells in the developing retina. 相似文献
Background A recombinant form of the α2(IV)NC1 domain of type IV collagen has been shown to have potent anti-angiogenic activity although
this peptide has not been studied in the context of proliferative retinopathies. In the current investigation we examined
the potential for α2(IV)NC1 to regulate retinal microvascular endothelial cell function using a range of in vitro and in vivo
assay systems.
Materials and methods α2(IV)NC1 at concentrations between 0.1 and 1 μg/ml was added to retinal microvascular endothelial cells (RMECs) followed
by assessment of cell attachment, proliferation and survival. This agent was also tested within a novel in vitro three-dimensional
retinal angiogenesis assay and the number of angiogenic sprouts quantified. α2(IV)NC1 was also delivered intra-vitreally to
mice with oxygen-induced proliferative retinopathy (OIR) and neovascularisation evaluated in comparison with vehicle-treated
controls.
Results RMECs treated with α2(IV)NC1 (0.1, 0.5 and 1 μg/ml) showed delayed attachment at 3 h post-seeding, although this deficit had
been restored at the 6-h time point. BrdU assay of DNA replication revealed that confluent RMECs treated with α2(IV)NC1 showed
no measurable response in comparison with vehicle-treated controls. By contrast, proliferation of sub-confluent RMECs was
significantly reduced by α2(IV)NC1 at 0.5 μg/ml (P<0.01). α2(IV)NC1 also induced apoptosis in RMECs and inhibited angiogenesis of pre-existing retinal vascular networks in
vitro (P<0.001). Intra-vitreal injection of α2(IV)NC1 in the OIR model significantly inhibited pre-retinal neovascularisation compared
with vehicle-treated controls (P<0.001).
Conclusion α2(IV)NC1 inhibits angiogenesis in the retinal microvasculature. This recombinant protein has potential for the treatment
of neovascularisation in proliferative retinopathies.
BioStratum Inc. did not sponsor this research in any way. None of the authors are paid consultants with this company. 相似文献