ObjectiveTo observe the effects of penetrance, different time of onset and mutation sites on retinal nerve fiber layer (RNFL) and macular thickness in patients with Leber's hereditary optic neuropathy (LHON).MethodsThis was a cross-sectional observational study. A total of 88 patients with LHON and 1492 relatives of the maternal relatives (gene carriers) who received treatment in People’s Liberation Army General Hospital from 2015 to 2017 were included in the study. Among the 1492 family members, there were 694 males and 798 females. Peripheral venous blood was extracted from all subjects for mitochondrial DNA testing, and penetrance was calculated. A total of 117 patients underwent BCVA and SD-OCT examinations, including 82 patients and 35 gene carriers. The BCVA examination was performed using the Snellen visual acuity chart, which was converted into logMAR visual acuity. The thickness of RNFL, ganglion cell complex (GCC) and inner limiting membrane (ILM)-RPE were measured with OCT instrument. The mean follow-up was 50.02±86.27 months. The disease course was divided into 6 stages including ≤3 months, 4-6 months, 7-12 months and >12 months. The thickness of RNFL, GCC and ILM-RPE in patients with different time of onset and mutation sites were comparatively analyzed by covariance analysis. Categorical variables were expressed as a percentage, and the χ2 test was used for comparison among multiple groups.ResultsAmong the 1492 family members, 285 were diagnosed with LHON and highly suspected clinical manifestations (19.10%), including 190 males (21.98%) and 95 females (11.90%). The total penetrance rates of 11778, 14484 and rare mutation sites were 19.84% (228/1149), 20.50% (33/161), and 13.19% (24/182) respectively; male penetrance rates were 28.87% (153/530), 27.28% (20/72), and 18.48% (17/92) and female penetrance rates were 12.12% (75/619),14.61% (13/89) and 7.78% (7/90). There was no significant difference in total (χ2=4.732), male (χ2=4.263) and female (χ2=4.263) penetrance between different mutation sites (P=0.094, 0.110, 0.349). Compared with non-pathogenic carriers, the thickness of the RNFL, GCC and ILM-RPE were all different in the four stages ( ≤3months, 4-6 months, 7-12 months and >12 months). The thickness of RNFL, GCC and ILM-RPE decreased with the time of onset (P=0.000). There were significant differences in the thickness of each of the GCC and ILM-RPE layers in the macular area of LHON patients with different mutation sites (P＜0.05). Among them, the site 11778 and 3460 had the most severe damage in all quadrants of macular GCC and ILM-RPE layer, followed by 14484 site, and the rare site had the least damage in all quadrants.ConclusionsThe penetrance of LHON patients is 19.10%. With the extension of the onset time (within 1 year), the RNFL layer of the optic disc and all quadrants of the macular GCC and ILM-RPE layer gradually thinned. Compared with 11778 and rare site, 14484 site, and the rare site had the lighter damage on the thickness of RNFL, GCC and ILM-RPE.
ObjectiveTo observe the full-field ERG (ff-ERG) characteristics of patients with acute regional occult outer retinopathy (AZOOR).MethodsA retrospective observational study. From June 2017 to June 2019, 62 eyes of 42 patients (AZOOR group) who were diagnosed with AZOOR in the Department of Ophthalmology of West China Hospital of Sichuan University were included in the study. All patients had no obvious localized disease on the fundus. Among 62 eyes, BCVA of 16 eyes were＜0.1, BCVA of 27 eyes were ≤0.5, and BCVA of 19 eyes were＞0.5. From June 2018 to January 2019, 40 normal volunteers (80 eyes) who attended the outpatient clinic of West China Hospital of Sichuan University and passed detailed ophthalmological examination to exclude all eye diseases including refractive errors were selected as the normal control group. All the examined eyes were tested with ff-ERG using the German Roland visual electrophysiological inspection system. The peak times and amplitudes of the waveforms induced by each response of dark adaptation 0.01 ERG, dark adaptation 3.0 ERG, dark adaptation 3.0 ERG, light adaptation 3.0 ERG, and light adaptation 30 Hz flicker ERG were recorded, respectively. The peak time and amplitude of each ff-ERG response between the two groups were compared by independent sample t test. The peak time and amplitude of each ff-ERG response between different BCVA eyes in the AZOOR group were compared by variance test.ResultsCompared with the normal control group, 0.01 ERG b wave of the dark adaptation of AZOOR group (t=3.601, －6.120), 3.0 ERG a wave and b wave of dark adaptation (t=2.627, －4.263, 3.719, －5.866), 3.0 Oscillation potential P2 wave of dark adaptation (t=－6.625), 3.0 ERG a wave and b wave of bright adaptation (t=3.762, －3.612, 3.648, －3.739) and 30 Hz flicker ERG P wave of bright adaptation (t=－3.832), all peak time of those were significantly delayed, the amplitude decreased, and the difference was statistically significant (P＜0.05). Comparison of different BCVA eyes in the AZOOR group showed that 0.01 ERG b wave amplitude of dark adaptation (F=3.950), 3.0 ERG a peak and b wave amplitude of dark adaptation (F=4.408, 4.876), oscillation potential P2 wave amplitude of dark adaptation (F=4.295), 3.0 ERG b wave amplitude of bright adaptation (F=4.344) and 30 Hz flicker ERG P wave amplitude of bright adaptation (F=4.483) of differences were statistically significant (P＜0.05). There was no statistically significant difference in waveform peak time and amplitude of the other reactions (P＞0.05). Pairwise comparison results showed that, compared with those with 0.1≤BCVA≤0.5 and BCVA＞0.5, those with BCVA＜0.1 dark adaptation to 0.01 ERG b wave, dark adaptation 3.0 ERG b wave, dark adaptation oscillation potential P2 wave, and light adaptation 3.0 ERG b wave and light adaptation 30 Hz scintillation ERG P wave amplitude were significantly reduced, and dark adaptation to 3.0 ERG a peak was significantly delayed, the difference was statistically significant (P＜0.05).ConclusionsThe ff-ERG of patients with AZOOR show delayed peak time and decreased amplitude of each response. The worse BCVA are accompanied by the more obvious decrease of each response amplitude of ff-ERG.
Objective：To observe the protective effect of ginkgo bilo ba extrac t (EGb 761), a free radical scavenger, on the photoreceptor cells after lighti nduced retinal damage. Methods：Seventytwo female SpragueDa wley (SD) rats we re randomly divided into 4 groups: normal control group, lightinduced retinal da m age model group, model+physiological saline group, and model+EGb 761 group, with 18 rats in each group. All of the rats except the ones in the control group were exposed to white light at (2740plusmn;120) lx for 6 hours after the dark adap tation for 24 hours to set up the lightinduced retinal damage model. Rats in m o del + physiological saline group and model+EGb 761 group were intraperitoneall y injected daily with physiological saline and 0.35% EGb 761 (100 mg/kg), respec tively 7 days before and 14 days after the light exposure. Apoptosis of photorec eptor cells was detected 4 days after light exposure; 7 and 14 days after light exposure, histopathological examination was performed and the layer number of ou ter nuclear layers (ONL) on the superior and inferior retina was counted. Results：Four days after light exposure, the apoptosis of photorecep tor cells was fou nd on ONL in model, model+ physiological saline and model+EGb 761 group, and w as obviously less in model + EGb 761 group than in model and model+physiologic al saline group. Seven days after light exposure, the layers of ONL on the super ior retina were 3 to 4 in model and model+physiological saline group, and 7 to 8 in model+EGb 761 group; the mean of the layer number of ONL in model+EGb 761 group (6.92plusmn;0.82) was less than that in normal control group (8.40plusmn;0.95) (t=-1.416, P＜0.05), but significantly more than that in model (5.96 plusmn;1.36 ) and model+physiological saline group (5.90plusmn;1.40）（t=1.024, 1.084; P＜0.05). Fourteen days after light exposure, the layers of ONL on the superior retina were 0 to 1 in model and model+physiological saline group, and 3 to 4 i n model+EGb 761 group. The mean of the layer number of ONL in model+EGb 761 group (5.5 2plusmn;1.06) was significantly more than that in model (3.44plusmn;2.15) and model + physiological saline group (3.37plusmn;1.91) (t=2.082, 2.146, P＜0.05). Conclusion：EGb 761 can partially inhibit the apoptosis of pho toreceptor cells, thus exert protective effect on photoreceptor cells.
Serum anti-retinal autoantibodies (ARA) are a group of autoantibodies that bind to retinal auto-antigens with significant biological importance in pathological processes such as retinal degeneration, inflammatory microenvironment formation, and tissue destruction. In recent years, the expression of serum anti-retinal antibodies has been found to be upregulated in patients with various blinding retinal diseases such as age-related macular degeneration, autoimmune retinopathy, and retinitis pigmentosa, closely correlated with the progression of diseases. However, current researches on ARA are incomplete, lacking animal experiments and large randomized controlled clinical trials. As a result, the exact mechanism of ARA is not well understood. Although several studies have demonstrated that serum ARA has an important diagnostic value in hereditary, autoimmune, and degenerative retinal diseases, there still lacks recognized laboratory tests and laboratory indicators with high specificity and sensitivity. Clinical symptoms should be considered when making definitive diagnosis of the diseases. Therefore, clarifying the mechanisms of ARA in retinal dystrophies provides new ideas in early diagnosis and treatments of retinal diseases, which is clinically and scientifically important for the maintenance of visual functions.
Objective To observe the change of diffusion upper limit of macromol ecules through pathological retina and the difference between the layers of retina. Methods Retinal edema was emulated by establishing branch retinal vein occlusion (RVO) model in miniature pig eyes under photodynamic method. Two days later, the retinas of both eyeballs were peeled off. The diffusion test apparatus was designed by ourselves. FITC-dextrans of various molecular weights (4.4, 9.3, 19.6, 38.9, 71.2 and 150 kDa) and Carboxyfluorescein (376 Da) were dissolved in RPMI1640 solutions and diffused through inner or outer surface of retina. The rate of transretinal diffusion was determined with a spectrophotometer. Theoretical maximum size of molecule (MSM) was calculated by extrapolating the trend-linear relationship with the diffusion rate. In separate experiments to determine the sites of barrier to diffusion, FITC-dextrans were applied to either the inner or outer retinal surface, processed as frozen sections, and viewed with a fluores cence microscope. Results FITC-dextrans applying to inner retinal surface, 4.4 kDa dextrans were largely blocked by inner nuclear layer (INL); 19.6,71.2 kDa dextrans were blocked by the nerve fiber layer (NFL) and inner plexiform layer; 15.0 kDa dextrans were blocked by NFL. FITC-dextrans applying to outer retinal surface, most dextrans with various molecular weights were blocked before outer nuclear layer (ONL). No matter applying to the inner or outer surface, Carboxyfluore scein can diffuse through the whole retina and aggregate at INL and ONL. After RVO, the inner part of retina became edema and cystoid, loosing the barrier function. Compared with the normal retina, the MSM in RVO tissues increased (6.5plusmn;0 39nm Vs 6.18plusmn;0.54nm, t=4.143, P=0.0001). Conclusions A fter RVO, the barrier function of inner part of retinal is destroyed and the upper limit of diffusion macromolecule size increased, which is nevertheless limited. ONL acts as bottle-neck barriers to diffusion, if the outer part of retina is damaged, the change of the diffusion upper limit will be prominent. （Chin J Ocul Fundus Dis，2008，24：197-201）
Objective To investigate the clinical features, etiological classification and staging of epiretinal macular membrane(MEM)． Methods Clinical materials of 194 cases of MEM diagnosed by fundus fluorescein angiography in outpatient department of eye clinic in this hospital from 1983 to 2000 were retrospectively analyzed． Results There were typical clinical symptoms and signs of MEM in all of this 222 eyes of 194 patients． Etiological classification revealed that 4 cases were congenital(2．12%), 22 cases were secondary(11．34%), and 168 cases were idio pathic(86．60%)． Staging of course of disease indicated that 119 eyes were in early stage(53．60%), 72 eyes were in middle stage(32．43%), and 31 eyes were in late stage(13．96%)． Conclusion MEM may be classified as congenital, secondary and idiopathic type according to its pathogenesis , as early, middle and late stage according to the clinical course of disease．This can be helpful in treating the disease． (Chin J Ocul Fundus Dis, 2001,17:210-213)
Objective To investigate the characteristics of optical coherence tomography (OCT) images in idiopathic macular hole. Methods OCT、color photography and fundus fluorescein angiography were performed in 65 cases(70 eyes) of macular holes and which were then graded by connecting to their clinical characteristics. Results Among the 70 eyes the number of 1～4 stages of macular holes were 11,12,36 and 11 eyes respectively.In eyes of stage 1 OCT images showed flattening or disappearing of fovea and minimally reflective space within or beneath the neurosensory retina;stage 2 showed a fullthickness hole with an attached operculum and surrounding edema;stage 3 displayed a full-thickness hole with surrounding edema and stage 4 showed a full-thickness hole and a complete separation of the poterior hyaloid membrane from the retina.The dimeter of the macular holes in stage 2,3 and 4 were (241.75plusmn;107.08),(699.78plusmn;160.99), (631.36plusmn;243.46)mu;m,respectively. Conclusions OCT can display the characteristics of idiopathic macular holes and measure the diameters of holes quantitatively. (Chin J Ocul Fundus Dis, 1999, 15: 205-208)
ObjectiveTo compare the clinical effects of urokinase thrombolytic therapy for optic artery occlusion (OAO) and retinal artery occlusion (RAO) caused by facial microinjection with hyaluronic acid and spontaneous RAO.MethodsFrom January 2014 to February 2018, 22 eyes of 22 patients with OAO and RAO caused by facial microinjection of hyaluronic acid who received treatment in Xi'an Fourth Hospital were enrolled in this retrospective study (hyaluronic acid group). Twenty-two eyes of 22 patients with spontaneous RAO were selected as the control group. The BCVA examination was performed using the international standard visual acuity chart, which was converted into logMAR visual acuity. FFA was used to measure arm-retinal circulation time (A-Rct) and filling time of retinal artery and its branches (FT). Meanwhile, MRI examination was performed. There were significant differences in age and FT between the two groups (t=14.840, 3.263; P=0.000, 0.003). The differecens of logMAR visual acuity, onset time and A-Rct were not statistically significant between the two groups (t=0.461, 0.107, 1.101; P=0.647, 0.915, 0.277). All patients underwent urokinase thrombolysis after exclusion of thrombolytic therapy. Among the patients in the hyaluronic acid group and control group, there were 6 patients of retrograde ophthalmic thrombolysis via the superior pulchlear artery, 6 patients of retrograde ophthalmic thrombolysis via the internal carotid artery, and 10 patients of intravenous thrombolysis. FFA was reviewed 24 h after treatment, and A-Rct and FT were recorded. Visual acuity was reviewed 30 days after treatment. The occurrence of adverse reactions during and after treatment were observed. The changes of logMAR visual acuity, A-Rct and FT before and after treatment were compared between the two groups using t-test.ResultsAt 24 h after treatment, the A-Rct and FT of the hyaluronic acid group were 21.05±3.42 s and 5.05±2.52 s, which were significantly shorter than before treatment (t=4.569, 2.730; P=0.000, 0.000); the A-Rct and FT in the control group were 19.55±4.14 s and 2.55±0.91 s, which were significantly shorter than before treatment (t=4.114, 7.601; P=0.000, 0.000). There was no significant difference in A-Rct between the two groups at 24 h after treatment (t=1.311, P=0.197). The FT difference was statistically significant between the two groups at 24 h after treatment (t=4.382, P=0.000). There was no significant difference in the shortening time of A-Rct and FT between the two groups (t=0.330, 0.510; P=0.743, 0.613). At 30 days after treatment, the logMAR visual acuity in the hyaluronic acid group and the control group were 0.62±0.32 and 0.43±0.17, which were significantly higher than those before treatment (t=2.289, 5.169; P=0.029, 0.000). The difference of logMAR visual acuity between the two groups after treatment was statistically significant (t=2.872, P=0.008). The difference in logMAR visual acuity before and after treatment between the two groups was statistically significant (t=2.239, P=0.025). No ocular or systemic adverse reactions occurred during or after treatment in all patients. ConclusionsUrokinase thrombolytic therapy for OAO and RAO caused by facial microinjection with hyaluronic acid and spontaneous RAO is safe and effective, with shortening A-Rct, FT and improving visual acuity. However, the improvement of visual acuity after treatment of OAO and RAO caused by facial microinjection with hyaluronic acid is worse than that of spontaneous RAO.
0bjective To explore the effect of endothelin(ET)、nitrioxide (NO) in plasma on retinopathv in the pregnancy-induced hypertension(PIH)． Methods The 1evel of ET and NO in plasma of 75 cases of in-patient women with PIH and 20 cases of women with the full terms and normal pregnancy before and after delivery was determined by radioimmunoassay．The retinopathy of the patients with FIH before and after delivery was detected by appointed doctor．The levels of ET and N0 in both groups were compared and the relationship between ET and N0 in plasma and the retinopathy before and after the delivery was detected.Results The levels of ET[(145．oo±54.41)ng／L] in serious PIH patients were much higher than that in the control[(81．50±43．80)ng／L]，the minor[(85.30±33.33)ng／L]and middling PIH group[(90．20±39．25)ng／L]．The levels of ET in plasma before and after pregnancy were not changed in PIH patients [(118．70±33．44)ng／L]，but were higher than that in the control group. The levels of plasma NO in serious[(87．56±35．58)ng／L]and middling[(78.11±28.96)ng／L] PIH group were both higher than that in the control group[(46．70±32.64)ng/L]，and the levels in minor(52．56±28．35)ng／L]and middling PIH group were lower than that in the serious PIH group.The level of N0 in plasma of PIH patients after the delivery was much lower than that before the delivery，while higher than that in the control．The positive correlation between levels of ET and NO and retinopathy was found in PIH patients.Conclusions The 1evels of plasma ET and N0 in PIH patients are related to the extent of the disease，and the level of ET in plasma is highly related to the retinopathy in PIH patients, ET and NO might be played an important role in pathogenesis of retinopathy and ET might be a good index in reflecting the rank of retinopathy in PIH.(Chin J Ocul Fundus Dis，2004，20：12-15)
Objective To investigate the changes of ocular hemodynamics in patients with retinal vein occlussion(RVO)． Methods The hemodynamic parameters(PSV,EDV,PI,Vmax)of central retinal artery(CRA)and central retinal vein(CRV)were measured in the involved eyes(n=48) with RVO and the contralateral clinically healthy eyes(n=39) and in the control eyes(n=40) by color Doppler imaging (CDI)(ATLHDI3000). Results Peak systolic velocity (PSV) and end diastolic velocity (EDV) were significantly lower in the CRA of involved eyes and clinically healthy eyes of patients with RVO compared with control eyes,and pulsatility index(PI)was significantly higher in the CRA of involved eyes of patients with RVO compared with control eyes．PSV were significantly lower in the CRA of involved eyes of patients with RVO compared with their clinically healthy eyes．Pulsatility index(PI)was significantly higher in the CRA of involved eyes of patients with RVO compared with their clinically healthy eyes．Maximun vein velocity (Vmax) was significantly lower in the CRV of involved eyes and clinically healthy eyes of patients with RVO compared with control eyes． Conclusion The changes of hemodynamics in CRA,CRV of involved eyes of patients with RVO may invade their clinically healthy eyes．CDI may be helpful to early diagnosis for RVO． (Chin J Ocul Fundus Dis,1998,14:111-113)