Purpose: To compare the effects of accelerated corneal collagen cross-linking (CXL) in progressive keratoconus (KCN) patients via epithelium removal and transepithelial techniques, using Daya Disruptor (Duckworth and Kent, Hertfordshire, UK). Methods: This study is a double-blinded, randomized clinical trial. Patients with documented bilateral progressive KCN were randomized into two groups: one eye underwent epithelium removal (Group 1), and the fellow eye underwent epithelium disruption (Group 2). The primary outcomes were best corrected visual acuity (BCVA) and uncorrected visual acuity (UCVA), Scheimpflug-extracted keratometric indices, and anterior segment-optical coherence tomography-derived epithelial thickness profiles. These parameters were evaluated before and 12 months after CXL. Results: Sixty-four eyes from 34 patients with progressive KCN (34 eyes in the epithelium-removal group and 30 eyes in the epithelium-disruption group) were included. The mean ± standard deviation (SD) of age was 23.4 ± 3.8 years in the epithelium-removal group and 23.2 ± 3.5 years in the epithelium-disruption group. The mean ± SD of the preoperative spherical equivalent (SE), front maximum keratometry (K-max), back K-max, thickness of thinnest point, and corneal apex thickness were − 2.9 ± 3.0 diopter (D) and − 3.7 ± 3.1 D ( P = 0.183), 53.8 ± 5.15 D and 54.4 ± 5.53 D ( P = 0.653), −6.63 ± 2.40 D and − 6.68 ± 2.48 D ( P = 0.131), 459.2 ± 37.4 μm and 460.8 ± 32.7 μm ( P = 0.708), 470.5 ± 37.7 μm and 469.7 ± 33.1 μm ( P = 0.679), and 55.4 ± 4.97 μm and 54.6 ± 7.16 μm ( P = 0.767) in the epithelium-removal and epithelium-disruption groups, respectively. The mean ± SD changes of the UCVA and BCVA 12 months after CXL were − 0.1 ± 0.11 and − 0.02 ± 0.18 and − 0.04 ± 0.12 and − 0.02 ± 0.14 in the epithelium-removal and epithelium-disruption groups, respectively. No statistically significant improvement was observed in the UCVA and BCVA between the two groups ( P = 0.868 and P = 0.937, respectively). The mean ± SD changes of the SE, superior epithelial thickness, corneal apex thickness, and thickness of thinnest point 12 months after CXL were − 0.21 ± 1.1 D and + 0.32 ± 1.6 D ( P = 0.0001), −0.08 ± 0.26 μm and + 0.03 ± 0.33 μm ( P = 0.028), −23 ± 11 μm and − 2 ± 6 μm ( P = 0.0001), and − 25 ± 8 μm and − 3 ± 7 μm ( P = 0.0001) in the epithelium-removal and epithelium-disruption groups, respectively. Conclusions: This study showed that the epithelium-disruption CXL using Daya has a similar potential for halting KCN progression as the epithelium-removal CXL. However, regarding the 12-month changes, the epithelium-di...

Purpose: To compare epithelium-removal and epithelium-disruption corneal crosslinking (CXL) methods in Fourier analysis of keratometric data and clinical outcomes. Methods: In this double-masked randomized clinical trial, each eye of 34 patients with bilateral keratoconus was randomly allocated to either the epithelium-removal or epithelium-disruption CXL treatment groups. Ocular examination, refraction, uncorrected and best spectacle-corrected visual acuity (UCVA and BSCVA, respectively) measurements, and Pentacam imaging (keratometry, pachymetry, and Fourier analysis) were performed at baseline and at six-month follow-up period. Results: Patients’ mean age was 23.3 ± 3.6 years. The preoperative thickness of the thinnest point was 459.20 ± 37.40 μm and 455.80 ± 32.70 μm in the epithelium removal and epithelial-disruption CXL groups, respectively (P = ?). The corresponding figures were 433.50 ± 33.50 μm and 451.90 ± 39.70 μm, respectively, six months after the treatment (P = 0.0001). The irregularity component was 0.030 ± 0.016 μm in the epithelium-removal group and 0.028 ± 0.011 μm in the epithelium-disruption group preoperatively (P = ?). This measurement was 0.031 ± 0.016 μm and 0.024 ± 0.009 μm, respectively at month 6 (P = 0.04). The epithelium-disruption CXL group had better results in terms of the thickness of the thinnest point and the irregularity component as compared to the epithelium-removal group. The two study groups were comparable in spherical equivalent, mean keratometry, UCVA, BSCVA, or other Fourier analysis components (spherical R min, spherical eccentricity, central, peripheral regular astigmatism, and maximum decentration) (P > 0.05). Conclusion: This study shows that epithelium-disruption CXL is superior to epithelium removal CXL regarding the short-term changes in pachymetry and corneal irregularity. Other evaluated parameters were comparable between the two techniques within the six-month follow-up period.

The neuromuscular junction is a highly specialized cholinergic synapse, essential for initiating nerve-evoked muscle contractions by means of neuromuscular transmission. Loss or dysfunction of any component of this junction might affect synaptic performance. Congenital Myasthenic Syndromes (CMSs) are rare heterogeneous disorders of autosomal inheritance caused by genetic defects affecting neuromuscular transmission that results in skeletal muscle weakness and abnormal fatigability on exertion. The onset is usually from birth to childhood. CMSs are more uncommon than autoimmune myasthenia gravis. CMSs are classified based on their genetic and clinical presentations into presynaptic, synaptic basal lamina, and postsynaptic CMSs. To date, mutations in more than 25 genes have been implicated in the pathogenesis of CMSs. In this review article, different CMSs diagnostic procedures are investigated, and the genetic, clinical, and molecular aspects of CMSs are outlined.

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Background: Descemet stripping and automated endothelial keratoplasty (DSAEK) has gained widespread popularity in the treatment of corneal endothelial dysfunction. In the present study, we evaluated the outcome of DSAEK in our center. Methods: This retrospective cross-sectional study was conducted on 60 patients, who had undergone DSAEK at least 12 months before in Khalili Hospital, Shiraz University of Medical Sciences. On follow-up, best spectacle-corrected visual acuity (BSCVA), refractive errors and specular microscopy parameters were assessed. Results: The majority of the studied eyes (49.2%) had a BSCVA within the range of 20/40 to 20/30. BSCVA was worse than 20/200 in 8 (13.3%) patients; 6 (75%) patients showed graft failure and 2 (25%) patients had graft rejection. Mean post-operative astigmatism was 0.29 ± 1.88 diopters. The mean refractive hyperopic shift was 1.30 ± 1.34 diopters. Specular microscopy indicated that 3 (5.1%) patients had cell density of less than 700 cells/mm 2 , but in none of the patients cell density was less than 500 cells/mm 2. Furthermore, hexagonality of endothelial cells in 50% of the eyes was 54% to 67%. Mean coefficient of variant was 27.76%. Abnormal variations in specular microscopy findings were mostly confined to endothelial cell density, sparing other parameters. Conclusions: In general, results of this study demonstrated acceptable BSCVA in the average duration of one-year post-DSAEK regarding late complications such as graft failure, graft rejection and epithelial downgrowth.