PURPOSE:To measure the epidermal growth factor (EGF)contents in vitreous and serum samples in normal subjects and patients with proliferative retinal diseases.
METHODS: Using radioreceptor assay(RRA)to measure the EGF contents in vitreous and serum in patients with proliferative diabetic retinopathy (PDR) 16 cases, proliferative vitreoretinopathy (PVR) 20 cases, central retinal vein occlusion (CRVO)16 cases,other retinal vascular diseases 5 cases,and controls 10 cases.
RESULTS:The EGF levels in vitreous of the patient group were apparently higher than those of the controls (Plt;0. 001). Among patient group,the EGF contents in vitrectomy fluid was lower than that of original vitreous, reflecting about 60~ 63~ EGF level in original vitreous, Both showed positive correlation. To compare the EGF contents in serum of patients and controls,the EGF contents in serum of PDR group increased significantly. In CRVO group the EGF contents also increased,while in PVR group,the EGF contents were lower than those of the controls.
CONCLUSIONS: The increased EGF contents in vitreous of patient group may play a role in the pathogenesis of proliferative retinal diseases. The RRA is a sensitive method for quantitative measurement of growth factor.
（Chin J Ocul Fundus Dis,1996,12: 91- 93）
Retinal degeneration mainly include age-related macular degeneration, retinitispigmentosa and Stargardt’s disease. Although its expression is slightly different, its pathogenesis is photoreceptor cells and/or retinal pigment epithelial (RPE) cel1 damage or degeneration. Because of the 1ack of self-repairing and renewal of retinal photoreceptor cells and RPE cells, cell replacement therapy is one of the most effective methods for treating such diseases.The stem cells currently used for the treatment of retinal degeneration include embryonicstem cells (ESC) and various adult stem cells, such as retinal stem cells (RSC), induced pluripotent stem cells (iPSC). and mesenchyma1 stem cells (MSC). Understanding the currentbasic and clinical application progress of ESC, iPSC, RSC, MSC can provide a new idea for the treatment of retinal degeneration.
Pyroptosis is a newly discovered form of cell death. Through the activation of inflammasome complexes, pyroptosis induces the production of interleukin (IL) -1β and IL-18, and the osmotic swelling of cells, thus induces cellular rupture and death. It plays a role in the pathological process of a variety of human diseases. The death of retinal cells including photoreceptor cells and retinal pigment epithelium (RPE) cells is the main reason leading to visual dysfunction in the pathogenesis in ocular fundus diseases. Researches have demonstrated that pyroptosis is closely related to the onset and progression of various retinal diseases. In age-related macular degeneration, pyroptosis directly causes apoptosis of RPE cells and upregulation of pro-inflammatory factors, enhancing toxic effect of lipofuscin. For retinitis pigmentosa, pyroptosis is the leading manner of death of secondary cone photoreceptor cells. In cytomegalovirus retinitis, pyroptosis is the main responding way to infection. This review presented the molecular mechanism of pyroptosis and its role in age-related macular degeneration, retinitis pigmentosa and cytomegalovirus retinitis and other retinal diseases.
Objective
To observe the characteristics of multiple evanescent white dot syndrome (MEWDS) with modern multimodal imaging modalities.
Methods
This was a retrospective case study. Eleven patients (11 eyes) diagnosed with MEWDS were enrolled. There were 10 females and 1 male, mean age was 27.6 years (range 15-41 years). The period between disease onset and visiting to the hospital was between 2 to 13 days, the average time was 4.7 days. All the patients underwent examinations of best corrected visual acuity, slit-lamp biomicroscope, indirect ophthalmoscope, fundus color photography, fundus autofluorescence (FAF), fundus fluorescein angiography (FFA), indocyanine green angiography (ICGA) and spectral domain optical coherence tomography (SD-OCT). The mean follow up duration was 3.2 months. The imaging characteristics were compared.
Results
Fundus color photography showed foveal orange-red granularity in all eyes. FAF showed strong autofluorescence with a vague boundary. FFA showed a variable number of highly fluorescent fine needle-like dots arranged in a ring in the early stage, and fluorescence remained in the late stage. ICGA showed advanced lesions of vague boundary merged into a large plaque of deep retinal hypofluorescence. SD-OCT showed the hyperreflectant material deposit over the retinal pigment epithelium and extending anteriorly through the interdigitation zone, ellipsoid layer, and toward the external limiting membrane. At the site of extrafoveal lesions, SD-OCT revealed the presence of discontinuities or disruptions centered on the ellipsoid zone to include the interdigitation.
Conclusions
In MEWDS patients, fundus photography showed foveal orange-red granularity; FFA showed early fluorescent dots distributed in a ring pattern; ICGA showed hypofluorescent lesions in the later stage; SD-OCT showed disruption of the interdigitation zone and ellipsoid zone and accumulations of hyperreflective material that was of variable size and shape; FAF showed strong autofluoresce areas that correlated to spots observed with FFA and ICGA.
Objective
To investigate the effect of suppression of ischemia-induced retinal neovascularization by VEGF antisense oligodeoxyribonucleotides.
Methods
Mouse models of hyperoxia-induced ischemic retinopathy were established. Retrobulbar injections were performed with VEGF antisense oligodeoxyribonucleotides or NS in 4 groups:normal control and various doses respectively. The nuclei of new vessel buds extending from the retina into the vitreous in differ ent groups were counted and compared under the light microscope.
Results
There were plenty of new vessel buds in the eyes of mice in hyperoxic condition., while the number of the nuclei of new vessel buds is less in the murine eyes with retrobulbar injection of VEGF antisense oligodeoxyribonucleotides,especially the nuclei were redused with 59.3% in eyes with large dose.
Conclusion
The proliferation of retinal new vessel may be suppressed by using the retrobulbar injection of VEGF antisense oligodeoxyribonucleotides.
(Chin J Ocul Fundus Dis, 2001,17:141-143)
Objective
To observe the imaging features of ultra-wide field short wave fundus autofluorescence (SW-FAF) in eyes with multiple evanescent white dot syndrome (MEWDS), and analysis the correspondence to conventional images.
Methods
It was a retrospective case series study. Thirteen patients (14 eyes) diagnosed with MEWDS were enrolled. There were 12 females and 1 male, aged from 22 to 57 years, mean age was 34.5 years. All the eyes underwent fundus color photography, optical coherence tomography (OCT) and ultra-wide field autofluorescence (FAF). Simultaneous fundus fluorescein angiography (FFA) and indocyanine green angiography (ICGA) were performed in 6 eyes. The characteristic changes of SW-FAF in studied eyes were observed and compared with the images of FFA and ICGA. All the eyes were followed up every 1 to 2 weeks, with an average of 16.7 weeks. The characteristic images of SW-FAF and corresponding OCT were studied during follow up.
Results
MEWDS presented with numerous multiple hyper-autofluorescent spots, sized from 50-500 μm, with a vague boundary in ultra-wide field SW-FAF. These spots located mainly at the peripapillary area and the posterior pole with a confluent pattern. The lesions extended to the mid-peripheral retina as well and became more scattered. The distribution of the hyper-autofluorescent lesions in SW-FAF corresponded roughly to that of the greyish-white spots seen in color photograph and the hyper-fluorescent spots detected by FFA. It was consistent with the distribution of hypo-fluorescent spots in late-phase ICGA as well. But the number of the spot showed in FAF is much more than that in FFA, and slightly less than that in ICGA. The OCT scans through the hyper-autofluorescent lesions in SW-FAF showed impairment of outer retina. After the recovery, the hyper-autofluorescent spots disappeared with the outer retina structure repaired completely.
Conclusions
MEWDS presented with numerous multiple hyper-autofluorescent spots which located mainly at the peripapillary area in ultra-wide field SW-FAF. The distribution of the hyper-autofluorescent lesions in SW-FAF corresponded roughly to color photograph, FFA and ICGA in late-phase. The OCT scans through the hyper-autofluorescent lesions in SW-FAF showed impairment of outer retina.
Objective To observe the clinical features of congenital hypertrophy of retinal pigment epithelium （CHRPE）. Methods The clinical data of 13 CHRPE patients including visual acuity, slit-lamp microscope examination, indirect ophthalmoscope examination and fundus fluorescein angiography (FFA) were retrospectively analyzed. The patients, 9 males and 4 females, with the mean age of 27.8 years. Results All patients were unilateral, without systemic diseases and no subjective symptoms in majority. Only 30.77% of initial diagnosis was correct, other diagnosis include choroidal nevi, old chorioretinopathy or no diagnosis. The round or oval black lesion was found in ocular fundus of all patients, 7.69% was located on the optic disk, 46.15% was located on the inferior temporal retina, 30.77% was located on the superior temporal retina, 15.39％ was located on the inferior nasal retina. 92.31％ was pigmented CHRPE and 7.69％ was non-pigmented CHRPE. FFA showed blocked fluorescence and transmitted fluorescence in the lesion, few eyes were found dilated capillary vessel and fluorescent leakage on the late stage of FFA, most eyes had normal retinal vessels. Conclusion The isolated CHRPE is round or oval black lesion in ocular fundus which lack of subjective symptoms, mostly located on the peripheral retina; the FFA characteristics showed blocked fluorescence and transmitted fluorescence, and CHRPE often misdiagnosed as other disease, it should be combine the ocular fundus manifestation with the FFA to diagnose properly.
Objctive To explore the relationship between the expression of Fas/FasL and the apoptosis occurs in retinal ischemia/reperfusion injury of rats , as well as the therapeutic effects of bFGF on the ischemic retina.Methods Th emodels of retinal ischemia/reperfusion injury was made by transient elevating introcular pressure. A total of 28 rats were divided into normal and operation group.The latter were subdivided into 1 hour, 6, 12, 24, 48 and 72 hours after reperfusion group, in which the left eyes of the rats were in the ischemia/reper fusion groups and the right ones were in the treatment groups (bFGF intracameral injection). Apoptosis was assessed by the terminal deoxynucleotidyl transferase-mediated dUTP-biotin nick-end labelling (TUNEL) method, and the expression of Fas and Fas ligand was studied by strept avidin-biotin complex （SABC）immunohistochemistry. Results No positive cells were observed in the normal rats′retinae, but there was a significant number of TUNEL positive cells in 6-24 hours after transient ischemia followed by a decrease at the 48th hour. The number of TUNEL positive cells reached a maximum at the 24th hour after ischemia. The expression of Fas gradually increased as early as when it was at the 6th hour, reached a peak at the 24th hour, and then decreased at the 48th hour. Similarly, the expression of Fas ligand was at peak in 24-48 hours in GCL and INL of retina. Conclusions Retinal ischemia-reperfusion after transient elevated IOP induced apoptosis of cells in the retina. Fas/FasL may play an important role in the early events of the apoptotic pathways. bFGF can rescue RGCs from retinal ischemia/reperfusion injury through downregulation of the expression of Fas/FasL and may represent an important mechanism for therapeutic neuroprotection. (Chin J Ocul Fundus Dis，2003，19：160-163)
Objective
To investigate the mutations of the gene in Chinese patients with X linked juvenile retinoschisis (XLRS), and to provide the genetic diagnosis and consultation of heredity for the patients and their families.
Methods
Genomic DNA was isolated from leukocytes of 29 male patients with XLRS, 38 female carriers and 100 normal controls (the patients and the carriers were from 12 families). All 6 exons of XLRS1 gene were amplified by polhism (SSCP) assay. The positions and types of XLRS1 gene mutations were determined by direct sequencing.
Results
Eleven different XLRS1 mutations were identified in these 12 families, including one frameshift mutation due to base loss of the first exon: c.22delT（L9CfsX20）, one nonsense mutation due to base loss of the first exon (Trp163X), one splice donor site mutation(c.52+2 Trarr;C; IVS1+2T to C), and eight missense mutation due to base replacement（Ser73Pro, Arg102Gln, Asp145His, Arg156Gly, Arg200Cys, Arg209His, Arg213Gln, and Cys223Arg). No gene mutation was detected in the control group. Four new mutations included frmaeshift mutation（L9CfsX20）and mutations of Asp145His, Arg156Gly, and Trp163X at the fifth exon. A newly discovered non-disease-related polymorphism (NSP) was the c.576C to T (Pro192Pro) change at the sixth exon.
Conclusion
Eleven different XLRS1 mutations were detected, which is the cause of XLRS in Chinese people. The detection of gene mutations may provide the guidance of genetic diagnosis and the consultation of family heredity for the patients and their families.
(Chin J Ocul Fundus Dis, 2006, 22: 77-81)
ObjectiveTo investigate the clinical characteristics and surgical management of the familial exudative vitreoretinopathy (FEVR)-associated rhegmatogenous retinal detachment (RRD).
MethodsRetrospective noncomparative case series. Thirty-three eye of 32 patients were diagnosed FEVR-associated RRD by Fluorescein fundus angiography. There were 26 males and 6 females. The male to female ratio is 4.3:1 with an average age of 19.35±8.83 years. The detection of best corrected visual acuity (BCVA), refraction status, fundus photograph and fluorescein fundus angiography(FFA)were underwent in all patients. FEVR was confirmedby FFA and positive family history. The BCVA, refraction status, morphology of retinal detachment, location, size and shape of retinal hole, presence and grade of proliferative vitreoretinopathy (PVR), and subretinal proliferation were recorded.
ResultsAs for the refractive status, the scope of refraction was +2.0 D to-13.0 D andthe BCVA were range from light perception to 0.7. Atrophy holes which located at the temporal half were responsible for retinal detachment in all cases. Besides, horseshoe tears were noted in 6 eyes (18.18%), while macular tears were noted in 2 eyes of RRD (6.06%). The PVR greater than stage C2 was noticed in 10 eyes (30.30%), while subretinal proliferation was presented in 23 eyes (69.70%).
ConclusionsMale predominance, juvenile onset and associated with moderate to high myopia are the main characteristics in FEVR-associated RRD. Atrophy holes at the temporal half and the subretinal proliferations were most commonly in FEVR-associated RRD. Detailed fuduns and FFA examination of the fellow eye should be undergone to avoid misdiagnosis.
