The Second Team Haemophilia Education Meeting, 2016, Frankfurt, Germany

The first Team Haemophilia Education (THE) Meeting was held on 7-8 May 2015 in Amsterdam, The Netherlands. It aimed to promote the optimal care of patients with haemophilia through education of the multidisciplinary treatment team. This was achieved by reviewing the latest developments in haemophilia management, considering how these can be implemented in the clinic to improve patient care and providing a platform for networking and debate for all haemophilia treatment team members. The second THE Meeting was held on 19-20 May in Frankfurt, Germany, and participants included doctors, nurses, physiotherapists, patient representatives and data management staff from 20 different countries. Topics covered the role of the multidisciplinary team in delivering the best haemophilia care, challenges in the management of haemophilia across Europe, available clotting factor treatments, future treatments and the use of genetics in advising carriers of haemophilia. This report is a summary of the key developments in haemophilia care presented by various investigators and healthcare professionals at THE Meeting 2016.info:eu-repo/semantics/publishedVersio

RAGE Expression in Human T Cells: A Link between Environmental Factors and Adaptive Immune Responses

The Receptor for Advanced Glycation Endproducts (RAGE) is a scavenger ligand that binds glycated endproducts as well as molecules released during cell death such as S100b and HMGB1. RAGE is expressed on antigen presenting cells where it may participate in activation of innate immune responses but its role in adaptive human immune responses has not been described. We have found that RAGE is expressed intracellularly in human T cells following TCR activation but constitutively on T cells from patients with diabetes. The levels of RAGE on T cells from patients with diabetes are not related to the level of glucose control. It co-localizes to the endosomes. Its expression increases in activated T cells from healthy control subjects but bystander cells also express RAGE after stimulation of the antigen specific T cells. RAGE ligands enhance RAGE expression. In patients with T1D, the level of RAGE expression decreases with T cell activation. RAGE+ T cells express higher levels of IL-17A, CD107a, and IL-5 than RAGE− cells from the same individual with T1D. Our studies have identified the expression of RAGE on adaptive immune cells and a role for this receptor and its ligands in modulating human immune responses

The Role of Multifocal Electroretinography in the Assessment of Drug-Induced Retinopathy: A Review of the Literature

Multifocal electroretinography (mfERG) is an objective, noninvasive examination for the assessment of visual function. It enables the stimulation of multiple retinal areas simultaneously and recording of each response independently, providing a topographic measure of retinal electrophysiological activity in the central 40-50° of the retina. A clinical application of mfERG represents the assessment of retinal toxicity associated with systemic medications. Drug-induced retinopathy represents a disease that, although not common, requires early recognition: if not detected early, it may progress and cause irreversible retinal dysfunction with subsequent visual impairment. This review aims to evaluate the use of mfERG in the assessment of retinal dysfunction associated with various systemic pharmacological agents based on the currently available literature. The most commonly recognized systemic medications affecting retinal function are included, such as chloroquine and hydroxychloroquine, vigabatrin, deferoxamine, ethambutol, interferon-α, tamoxifen, digoxin, sildenafil, canthaxanthin, amiodarone and nefazodone. The role of mfERG in the early diagnosis of retinal toxicity and the evaluation of disease severity is reviewed, as well as its clinical value in monitoring disease progression or recovery after drug cessation. © 2016 S. Karger AG, Basel

Evaluation of macular function and morphology following accelerated collagen cross-linking in progressive keratoconus

Purpose: To assess any changes in macular function and morphology in patients with progressive keratoconus undergoing accelerated corneal cross-linking (CXL). Methods: This prospective case series included 9 eyes of 8 patients with progressive keratoconus undergoing CXL using a high intensity accelerated protocol (9 mW/cm2 for 14 min) with a total surface dose of 7.5 J/cm2. Visual acuity assessment, slit lamp biomicroscopy, dilated fundoscopy, corneal tomography, multifocal electroretinography (mfERG) and spectral domain optical coherence tomography scan were performed at baseline, 2 weeks and 6 weeks postoperatively. Results: Uncorrected and corrected distance visual acuity did not change significantly at 2 weeks and 6 weeks following accelerated CXL compared to baseline. Retinal response density (RRD) of mfERG significantly decreased at 2 weeks postoperatively compared to baseline (p = 0.008) but did not differ from the baseline value at 6 weeks postoperatively in the fovea (ring 1) (p = 0.95). Similarly, P1 latency significantly decreased at 2 weeks (p = 0.04) but did not change at 6 weeks (p = 1.00) postoperatively compared to baseline in the fovea. No changes in RRD or P1 latency were observed in the retinal rings surrounding the fovea (rings 2 to 5). Central foveal thickness did not change at 2 weeks and 6 weeks postoperatively compared to baseline (p = 0.53 and p = 0.93, respectively). Conclusions: A short-term reversible decrease in macular electrical activity without any structural changes seems to occur after accelerated CXL in patients with progressive keratoconus. The return of macular response to the preoperative values shows the safety of the CXL protocol. © The Author(s) 2022

“Double scleral tunnel in tandem” technique for glaucoma drainage tube implants

Purpose: To describe a new technique of Ahmed glaucoma valve implantation in patients with refractory glaucoma and present the long-term results. Methods: Twenty-eight eyes of 28 patients with refractory glaucoma participated in the study. “Double scleral tunnel in tandem” technique was used for glaucoma valve implantation in all patients. The technique consists of the formation of two scleral tunnels in tandem so as to completely cover the extraocular part of valve’s tube and facilitate a parallel to the iris placement of the intraocular part of the tube. Results: Postoperative hypotony developed in two (7.1%) cases which resolved within 3 days in all cases. One (3.6%) eye manifested postoperative hyphema that resolved without complications within a week, and two (7.1%) cases presented with exposure of the tube. The average highest postoperative intraocular pressure (IOP) during the follow-up period was 19 mmHg (range 8–38 mmHg) without medications, and the average IOP at the last follow-up visit was 14.5 mmHg (range 8–22 mmHg). Additional treatment for IOP control was necessary in 11 (39.3%) cases. The average follow-up period was 60 months. Conclusion: This novel technique was found to be easy to master, effective and with low cost and rate of complications. © 2017, Springer Science+Business Media B.V

Effect of carotenoids dietary supplementation on macular function in diabetic patients

Abstract Background Diabetic retinopathy is a major cause of visual impairment and blindness among working-age people worldwide. The aim of our study was to investigate the effects of a carotenoid supplementation on retinal thickness and macular function of patients with diabetes using optical coherence tomography (OCT) and multifocal electroretinography (mfERG). Methods A retrospective study of one hundred and twenty eyes of sixty patients age between 40 and 60 years with non-insulin dependent type 2 diabetes mellitus without diabetic retinopathy who underwent OCT and mfERG and took vitamin supplements for a period of two years. Patients received a carotenoid supplement containing lutein (10 mg), zeaxanthin (2 mg) and meso-zeaxanthin (10 mg) once a day for two years. The thickness of the fovea was evaluated using OCT and the macular function was tested by mfERG. Results OCT showed an increase in the central foveal thickness and mfERG revealed increased retinal response density within the central 13° surrounding the fovea (rings 1 to 3) at two years after the onset of carotenoids supplement intake. Conclusion The use of carotenoid supplements may be of benefit for improving visual function of type 2 diabetes patients. However, further study is needed to assess the treatment’s long-term efficacy

Intravitreal cilium associated with retinal detachment 40 years following penetrating eye injury: A case report

Background: The presence of an intraocular cilium is very rare and the response of the eye to the cilium is variable. We present the case of a patient with a cilium found in the vitreous cavity during vitrectomy for rhegmatogenous retinal detachment 40 years following penetrating eye injury. To our knowledge, this is the longest reported presence of a cilium in the vitreous cavity. Case presentation: A 70-year-old Caucasian woman presented to the emergency department of our hospital complaining of sudden visual impairment and floaters of her right eye initiated 2 weeks earlier. Ophthalmic history included a penetrating injury of the right eye with a sharp metallic object 40 years ago and an uncomplicated phacoemulsification surgery in the same eye 2 years earlier. Fundoscopy revealed an inferior macula off rhegmatogenous retinal detachment. No inflammation was present. During vitrectomy and under scleral indentation at 5-o'clock position, a cilium was found at far retinal periphery. One end of the cilium was embedded in the retina, whereas the other end floated freely in the vitreous. The cilium was removed through the pars plana sclerotomy with intraocular foreign body forceps. The procedure was completed without any complications. Conclusion: Penetrating eye injury is the most possible cause of cilium entrance in vitreous cavity in this case, which suggests that cilium can be well tolerated in vitreous cavity for as long as 40 years. © 2015 Dettoraki et al.; licensee BioMed Central