Background Uveitis is a group of intraocular inflammatory diseases whose primary treatment involves immunosuppression. Although corticosteroids (CSs) remain the mainstay therapy, sirolimus is among the recently studied immunomodulatory drugs for treating noninfectious uveitis (NIU). Objective The aim of this review was to assess and summarize the updated evidence on the outcomes of treatment with sirolimus for NIU. Materials and methods Two reviewers conducted a systematic search on November 5, 2018, of electronic databases (EMBASE, MEDLINE, and The Cochrane Library) and clinical trial registers having no restrictions on language or publication date. The primary outcome was uveitis activity as measured by vitreous haze (VH), while the secondary outcomes included central macular thickness (CMT), best-corrected visual acuity (BCVA), CS-sparing effect, IOP elevation, and other adverse events. A meta-analysis was conducted on selected studies with appropriate clinical and methodological homogeneity. Results Seven studies were included and reviewed. Four randomized clinical trials were eligible for meta-analysis: SAVE 2013, One-year outcomes of the SAVE study, SAVE 2 2016, SAKURA 2016. The pooled proportions of inflammation control (VH improvement) were 38% (95% CI 16.19%–62.66%) during a 6-month follow-up and 49.97% (95% CI 16.19%–83.03%) during a 6- to 12-month follow-up with the latter showing a significantly higher response rate ( p =0.0472). BCVA improvement was 62.2% (95% CI 33.17%–87.11%) during a 6-month follow-up and 56.86% (95% CI 20.91%–89.05%) during a 6- to 12-month follow-up with no significant difference between the two ( p =0.3705). Increased IOP remained at 7.11% (95% CI 3.46%–12.68%) for both a 6-month follow-up and up to a 12-month follow-up duration. The CS-sparing effect of sirolimus was also well demonstrated. A reduction in CMT was observed, and only minor drug-related adverse events were reported in all the studies reviewed. Conclusion This review provided evidence that sirolimus is a promising treatment option for controlling inflammatory activity, improving visual acuity, and sparing CS use with minor adverse events for NIU.
AIM: The study aims to identify if mutations in the SLC4A11 gene are present in Filipino families affected with Congenital Hereditary Endothelial Dystrophy (CHED). METHODS: This is a family cohort study that investigated the genetic profile of a selected family in Northern Luzon, Philippines, whose members were diagnosed with Congenital Hereditary Endothelial Dystrophy (CHED). A patient who was diagnosed with CHED prior to this study, served as the proband for this family. A detailed family history and a complete ophthalmologic examination were done to each of the family members. A total of six affected members and three unaffected members were included in this study. DNA was isolated from peripheral blood samples of family members, and polymerase chain reaction (PCR) was used to amplify the gene’s entire coding region, (19 exons and 2 putative promoter regions), and finally, the amplified regions were analyzed using DNA sequencing. RESULTS: Consanguinity was not present in the family. Corneal haze was reported to be present since birth or shortly thereafter in all affected patients. Slit-lamp examination showed edematous corneas. Molecular studies of the SLC4A11 gene revealed four novel homozygous point mutations variably present in six affected members as well as three unaffected members. One unaffected family member (I-1) had a novel sense mutation absent in other family members. All affected siblings showed little phenotypic variability. CONCLUSION: This is the first report that gives us a genetic profile of a Northern Luzon family with members affected by CHED. This study supports earlier findings that the mutations in the SLC4A11 gene are not consistently the same among different ethnic groups worldwide, probably due to the disease’s genetic heterogeneity. Our study documented five novel mutations adding to the growing list of mutations probably responsible for acquiring the CHED phenotype. It is possible that there are more novel mutations waiting to be discovered in this hereditary disease. Screening for these specific mutations in other families may prove useful for genetic counseling, prenatal diagnosis and the future development of gene therapy.
OBJECTIVE To report clinical outcomes of a 25-gauge, beveled-tip, 10000 cuts-per-minute (cpm) microincisional vitrectomy surgery (MIVS) system. METHODS Prospective case series of eyes undergoing primary pars plana vitrectomy (PPV) for common indications. Main outcome measures were: rate of achieving surgical objectives, operative time, number of surgical steps, use of ancillary instruments, corrected distance visual acuity (CDVA), and adverse events (AE). RESULTS Surgical objectives were achieved in all eyes. Mean total operative, core, shave and total vitrectomy times were 1891 ± 890, 204 ± 120, 330 ± 320, 534 ± 389 seconds, respectively. Mean number of surgical steps was 4.3 ± 1.5. Mean number of ancillary instruments used was 4.5 ± 1.9. Mean CDVA improved by 0.53 ± 0.56 logMAR units (P < 0.001) after 3 months. Postoperative AE included elevated IOP (8%), hypotony (6%), and re-detachment (2%). Majority (82%) had no postoperative discomfort. CONCLUSION Beveled-tip, 10000 cpm MIVS system effectively and safely performs common vitreoretinal procedures and may reduce operative time and ancillary instrumentation.
Objective To report the clinical outcomes of a 25-gauge, beveled-tip, 10,000 cuts-per-minute (cpm) microincisional vitrectomy surgery (MIVS) system. Methods Prospective case series of eyes undergoing primary pars plana vitrectomy (PPV) for common vitreoretinal indications. Main outcome measures were: rate of achieving surgical objectives, operative times, number of surgical steps, use of ancillary instruments, corrected distance visual acuity (CDVA), and adverse events (AE). Results The surgical objectives were achieved in all eyes. Mean total operative time (TOT), core, shave and total vitrectomy times were 1891 ± 890, 204 ± 120, 330 ± 320, 534 ± 389 s, respectively. Mean number of surgical steps was 4.3 ± 1.5. Mean number of ancillary instruments used was 4.5 ± 1.9. Mean CDVA improved by 0.53 ± 0.56 logMAR units (P < 0.001) 3 months postoperatively. AE included elevated IOP (8%), hypotony (6%), and re-detachment (2%). Majority (82%) had no postoperative discomfort. The number of surgical steps demonstrated a positive correlation with TOT (p < 0.05), number of ancillary instruments used (p < 0.05), and postoperative Day 1 IOP (p < 0.05). The number of times ancillary instrumentation was used demonstrated a positive correlation with TOT (p < 0.05). Conclusion Beveled-tip, 10,000 cpm MIVS system effectively and safely performs common VR procedures of varying complexity and may reduce operative times and use of ancillary instrumentation.
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