Functional retinal changes in Gaucher disease.

In Gaucher disease, sphingolipid glucosylceramide is accumulated in cells of the reticulo-endothelial system. This leads to the formation of "Gaucher cells," which are enlarged macrophages. In the eye, circumscript preretinal deposits are a pathognomonic feature. We describe the case of a 23-year-old woman with no subjective complaints but a reduced ERG response, leading to the suspicion that in addition to circumscript deposition diffuse accumulation of metabolic endproducts might be present in the retina in Gaucher patients.

Preservative-free tafluprost 0.0015% in the treatment of patients with glaucoma and ocular hypertension.

INTRODUCTION: The objective of this study was to evaluate efficacy, local tolerability, and safety of this first-in-class preservative-free prostaglandin preparation in patients with ocular hypertension and glaucoma. METHODS: Patients with glaucoma or ocular hypertension who required a change of medication or were naïve to treatment were included in this noninterventional and observational study. Noninterventional means that no influence was made upon the decision of the physicians to include specific patients and upon the treatment algorithm used. German law for observational studies does not allow any influence on the choice of drugs used, patient selection, masking, and comparator treatment regimens. The main aim of this observational study was to collect "real-life data" on the efficacy and safety of a new medical treatment after approval in a large patient population. Participating ophthalmologists were asked to provide anonymous patient data collected during regular visits by filling a simple data entry form. Intraocular pressure (IOP) readings were recorded at baseline (previous therapy or untreated) and 6-12 weeks after changing medical treatment to or initiating treatment with preservative-free tafluprost once daily. Changes in the IOP were evaluated over the study period for all patients as well as for specific pretreatment subgroups. Local comfort was determined using a five-point scale (very good, good, satisfactory, less satisfactory, not acceptable) before and after the change of medical treatment. All adverse events were recorded. RESULTS: Data from 2123 patients with glaucoma or ocular hypertension were considered for the final evaluation. Medication was changed in 41.1% of patients due to tolerability issues and in 25.6% of patients due to insufficient efficacy with prior medication. In all patients preservative-free tafluprost 0.0015% lowered IOP from 19.5 ± 4.4 mmHg (baseline) to 16.4 ± 2.9 mmHg after 6-12 weeks. Preservativefree tafluprost also significantly lowered the IOP in all monotherapy subgroups: treatment-naïve patients (n=440): 22.6 ± 3.9 mmHg (baseline) to 16.7 ± 2.7 mmHg (week 6-12); beta blockers (n=307): 20.3 ± 3.5 mmHg (baseline) to 16.7 ± 2.6 mmHg (week 6-12); carbonic anhydrase inhibitors (n=158): 19.0 ± 3.6 mmHg (baseline) to 16.0 ± 2.6 mmHg (week 6-12); prostaglandin analogs (PGAs; n=447): 16.8 ± 2.9 mmHg (baseline) to 15.8 ± 2.6 mmHg (week 6-12). Local comfort was rated as "very good" or "good" by 85.6% of patients at the final visit (P<0.001). Only few adverse events occurred during the treatment period: 18 patients (0.8%) discontinued medical treatment with preservative-free tafluprost due to local intolerance; six patients (0.3%) due to efficacy issues; four patients complained about systemic side effects (0.2%); and two patients preferred to use a multidose treatment regimen (0.2%). CONCLUSION: Although this study was limited by its observational design the results demonstrate that preservative-free tafluprost 0.0015% was effective, generally well tolerated, and safe in a broad and heterogeneous patient population.

Dynamic retinal vessel response to flicker in age-related macular degeneration patients before and after vascular endothelial growth factor inhibitor injection.

PURPOSE: Retinal vessel responses to flickering light are different in various systemic and ocular diseases and can be improved after successful therapy. We investigated retinal vessel response to flickering light in age-related macular degeneration (AMD) patients before and after treatment with a single intravitreal bevacizumab (Avastin(®) ) injection. METHODS: In 10 patients with exudative AMD [age: median (1.quartile; 3.quartile) 76.0 (73.5; 80.0) years], retinal vessel reactions were examined by Dynamic Vessel Analyser (DVA) before and 3 months after a single intravitreal application of bevacizumab (1.25 mg). A baseline measurement was followed by three consecutive monochromatic flicker stimulations (530-600 nm, 12.5 Hz, 20 seconds). Temporal retinal vessel reaction was analysed and compared with the reaction in healthy controls. RESULTS: Mean arterial dilation at the end of flicker was not different in all groups. For veins this parameter amounted to: pre-treatment, 2.6 (1.7; 3.9)%; post-treatment, 2.9 (2.4; 4.0)%; control, 4.3 (3.2; 5.7)%; significant: pre-treatment - control (Dunnett's procedure, p < 0.05). Maximal dilation occurred in arteries at: pre-treatment, 17.5 (14.8; 32.5) seconds; post-treatment, 18.0 (16.6; 30.6) seconds; control, 14.5 (10.8; 17.3) seconds. Both AMD groups were slower (p < 0.05): in veins at 17.0 (14.5; 20.0) seconds, 12.8 (8.6; 14.8) seconds and 18.5 (17.1; 19.9) seconds, respectively; significant post-treatment - control (p < 0.05). In the post-treatment AMD group arterial constriction after stimulation occurred more slowly compared with the control group (p < 0.05). CONCLUSION: Dynamic retinal arterial and venous reactions to flickering light are altered in AMD compared with controls. Three months after a single injection of a vascular endothelial growth factor inhibitor, the investigated retinal dynamic vascular parameters were not altered in our study.

Microstructural alterations of retinal arterial blood column along the vessel axis in systemic hypertension.

Purpose. Image analysis by the retinal vessel analyzer (RVA) observes retinal vessels in their dynamic state online noninvasively along a chosen vessel segment. It has been found that high-frequency diameter changes in the retinal artery blood column along the vessel increase significantly in anamnestically healthy volunteers with increasing age and in patients with glaucoma during vascular dilation. This study was undertaken to investigate whether longitudinal sections of the retinal artery blood column are altered in systemic hypertension. Methods. Retinal arteries of 15 untreated patients with essential arterial hypertension (age, 50.9 +/- 11.9 years) and of 15 age-matched anamnestically healthy volunteers were examined by RVA. After baseline assessment, a monochromatic luminance flicker (530-600 nm; 12.5 Hz; 20 s) was applied to evoke retinal vasodilation. Differences in amplitude and frequency of spatial artery blood column diameter change along segments (longitudinal arterial profiles) of 1 mm in length were measured and analyzed using Fourier transformation. Results. In the control group, average reduced power spectra (ARPS) of longitudinal arterial profiles did not differ when arteries changed from constriction to dilation. In the systemic hypertension group, ARPS during constriction, baseline, and restoration were identical and differed from ARPS during dilation (P < 0.05). Longitudinal arterial profiles in both groups showed significant dissimilitude at baseline and restoration (P < 0.05). Conclusions. The retinal artery blood column demonstrates microstructural alterations in systemic hypertension and is less irregular along the vessel axis during vessel dilation. These microstructural changes may be an indication of alterations in vessel wall rigidity, vascular endothelial function, and smooth muscle cells in this disease, leading to impaired perfusion and regulation.