Objectives To observe the clinical characteristics of Leber congenital amaurosis (LCA) and analyze the features to differentiate LCA from other similar disorders.Methods Prospective study of 15 LCA patients which include 10 males and 5 females, aged from 2 to 31 years with the mean age 13.6 years. Medical history, family history, perinatal conditions, as well as complete ocular evaluations were well documented. Among the subjects, 12 patients underwent optometry check, 10 patients underwent ERG and 8 patients had OCT testing. Results All of the patients had nystagmus and sluggish pupillary reflex. The visual acuity distributed from light perception to 0.1. Fivepatients (33.3%) were presented with photophobia, while 7 patients (46.7%) had nyctalopia. Among 12 cases underwent refraction examination, 6 patients (50%) had spherical equivalent of ge;+5D；1 patient（8.3%）had spherical equivalent of ge;+5D; 2 patients (16.7%) had bilateral mild to moderate hyperopia；1 patient (8.3%) had one emmertropic eye and one mild myopic eye; 2 patients (16.7%) had moderate to high myopia. Eight patients (53.3%) had enophthalmus,4 patients (26.7%) had oculodigital sign. All of the 10 patients underwent ERG showed extinguished waveform. Under OCT assesement, 7 patients had decreased fovea thickness; 1 patient had increased fovea thickness, complicated by epiretinal membrane; mild abnormality of microstructure of the retina with diminished and disrupted IS/OS hyperreflectivity were found in 2 cases；while more pronounced disarrangement of the retinal layers were found in 6 cases，inner layers were better reserved in all patients.Conclusions Severe visual impairment or blindness, nystagmus, pupillary reflex, extinguished ERG and hyperopia are main clinical characteristics of LCA.
Objective Toinvestigate the influence of photocoagulation on macular function and morphous in patients with diabetic retinopathy (DR).Methods Forty eyes of thirty patients with severe nonproliferative diabetic retinopathy (NPDR) were examined by multifocal electroretinogram (mfERG) and optical coherence tomography (OCT) before and 2,7, and 14 days after photocoagulation. The results were statistically analyzed by using analysis of variance and t test; the changes of macular function and macular fovea thickness were detected and observed.Results P1 response densities of ring 1,3,and 5 were 131.79plusmn;50.92，37.50plusmn;17.27，24.07plusmn;11.49，respectively,2 days after photocoagulation; and were 212.96plusmn;53.75，46.70plusmn;15.89，and 30.91plusmn;10.78, respectively, before photocoagulation. The densities before and after photocoagulation differed much（t=7.910, 2.174, 2.205; Plt;0.05）. N1 response density of ring 4 was（60.39plusmn;20.69） and the prephotocoagulation corresponding response density was （107.11plusmn;44.63）; the difference was significant（t=5.375，Plt;0.01）. The latency of P1 of ring 4 was（41.83plusmn;3.41），which had significant statistically difference（t=-2.770，Plt;0.05) with that before photocoagulation（39.52plusmn;2.64); there was no significant changes in the latency of N1 (Pgt;0.05). The most significant changes of P1 and N1 response densities occurred in the central macular 5deg; area. Seven days after photocoagulation, the response density of P1 and N1 in the central macular 5deg; area seemed to be recoverd to some extend and increased to （179.70plusmn;47.10）and (81.11plusmn;34.18) respectively until 14 days after photocoagulation, which was still much lower than that before the photocoagulation（t=3.840, 2.746; P＜0.05）; the response densities of other areas had no significant differences (P＞0.05). Seven days after photocoagulation,the latency of P1 in ring 4 was delayed to（41.78plusmn;3.57）, which had significant difference（t=-3.144，P＜0.01）with that before the photocoagulation（39.52plusmn;2.64) ; but there was no significant difference between 14 days after photocoagulation and prephotocoagulation (t=-1.809，P＞0.05). The latency of N1 in ring 1 was（20.67plusmn;3.85）at seven days after photocoagulation, It had no significant difference (t=-1.171,P＞0.05) with that before the phtocoaguation（18.78plusmn;3.29）. Before and 2 days after photocoagulation, the macular fovea thickness were（224.42plusmn;122.88）and（274.85plusmn;108.20）respectively, and the difference was statistically significant（t=-2.420，P＜0.05）. Forteen days after photocoagulation，the macular fovea thickness was（236.29plusmn;70.45），It had no significant difference with that before the photocoagulation（t=-0.578，P＞0.05）. Before and seven days after photocoagulation, P1 response density had obvious negative correlation with corresponding macular fovea thickness（r=-0.755，Plt;0.01; r=-0.594，Plt;0.05）. Conclusions After photocoagulation in patients with DR，the macular function decreased in a certain degree，and the relationship of macular retinal function and macular morphology changes was close; combination of mfERG and OCT can evaluate macular function and macular morphology structure comprehensively and objectively.