.
Purpose: Fabry Disease (FD) is a rare X‐linked metabolic disorder characterized by diffuse deposition of sphingolipids in many tissues. Retinal vessel tortuosity is a common ocular manifestation in FD and may represent a useful marker for the disease. Unfortunately its clinical evaluation is poorly reproducibile and alternative means of evaluation may be of interest. We tested a new semi‐automatic software measuring retinal vessel tortuosity from eye fundus digital images in a group of FD patients.
Methods: Observational case‐control study evaluating four mathematical parameters describing tortuosity (relative length, sum of angle metric [SOAM], product of angle distance [PAD], triangular index) obtained from fundus pictures of 35 FD patients and 35 age‐matched controls. Only the right eye was considered in order to reduce bias. Mann–Whitney test was used to compare the FD group versus the control group, males versus females and patients with versus without clinically identified retinal vessels tortuosity in the FD group. Linear regression analysis was performed on a subgroup of patients to evaluate the possible association of retinal vessels tortuosity parameters with age and with markers of systemic disease’s progression.
Results: Three parameters (SOAM, PAD and triangular index) were significantly higher in FD patients in comparison with the controls (p < 0.0001, p = 0.001, p = 0.002 respectively). In the FD group the same three parameters showed higher values in hemizygous males than in heterozygous females ((p < 0.0001, p = 0.002, p < 0.0001 respectively).
Conclusion: A computer assisted analysis of retinal vasculature demonstrated an increased vessels tortuosity in FD patients. The technique might be useful to establish disease severity and monitor its progression.
Although the review found increased risk of open-angle glaucoma with pars plana vitrectomy, the studies were heterogenous or inconsistent regarding ocular hypertension and intraocular pressure increase. Larger studies should be conducted in homogenous cohorts of patients undergoing macular surgery, excluding complex conditions such as retinal detachment or diabetic retinopathy.
Antibiotic prophylaxis does not reduce the rate of endophthalmitis following IVI and might potentially be associated with an increased risk of post-operative infection.
Macular serous retinal detachment was first described in 1998 as an uncommon complication of tilted disc syndrome showing angiographic and OCT features similar to a chronic central serous chorioretinopathy. In contrast to this pathology, in our patients treatment with PDT or intravitreal bevacizumab did not succeed, probably because of a different pathogenesis of macular serous detachment. Further investigations are needed to clarify the proper therapy of this disease.
Background: Stargardt's disease (STGD) and Retinitis Pigmentosa (RP) are inherited retinal degenerations that may be affected, in opposite way, by diet. Methods: Dietary profile was assessed in 24 patients with STGD and in 56 patients with RP. We documented in only 6 out of 24 (25 %) STGD patients a daily intake of vitamin A within the recommended range while 14/24 (58.3 %) reported a high daily intake and 4/24 (16.7 %) showed a low daily intake. With regard to RP, 4/56 (7.1 %) reported to be within the recommended range, 37/56 (66.1 %) reported high daily intake and 15/56 (26.8 %) showed low daily intake of vitamin A. Results: Interestingly, STGD patients with low vitamin A intake (<600 µg RAE/day) showed significantly better visual acuity with respect to those introducing higher intake of vitamin A. Conclusion: The present study suggests insuitable nutrient intakes among patients with STGD and RP, especially for daily intake of vitamin A. The results may be used to provide tailored nutritional interventions in these patients.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.