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Introduction: Long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency, typically inherited as a recessive trait, is a genetic condition predominantly observed in Central and Eastern Europe, with birth prevalence in Poland amounting to 1/118,336. In most European countries, e.g., in Poland since 2014, this disorder is included in newborn screening. Case Presentation: This paper presents the ophthalmic symptoms of long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency in three pediatric patients. Visual acuity testing, fundus photography, and optical coherence tomography (OCT) were performed and data were collected over several years (2017-2022). In case 1, a female born in 2010, exhibited abnormalities in the central part of the posterior pole, mainly in the macula, and included choriocapillaris atrophy and severe disruption of the outer retinal layer. Case 2, a female born in 2012, presented with progressive shortsightedness and choroid atrophy documented with angio-OCT. Case 3, a male born in 2013, experienced recurrent hospitalizations due to metabolic decompensations and presented with mild myopia, thinning of the choroid layer, and slight pigment dispersion with macular sparing. Conclusion: The main ophthalmic symptoms of LCHAD deficiency were choroidal atrophy, disorganization of the outer retinal layer, and myopia. Choroidal atrophy and pigment dispersion were consistently the earliest signs of LCHAD-associated chorioretinopathy. Although the progression of chorioretinopathy in each case resulted from metabolic decompensation, one documented case revealed that not every metabolic crisis results in ophthalmological changes. Nonetheless, strict adherence to a low-fat, high-carbohydrate diet remains crucial to prevent gradual deterioration and vision loss.
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INTRODUCTION: The process of scientific publishing changed greatly in the past decades. The authors aimed to get insight into the time required for articles to be accepted and released online in high-impacted ophthalmology journals. METHODS: Comprehensive review of all original articles published by eight ophthalmology journals during a one-year period was performed for 2020 and 2005. Time taken from submission to acceptance and the first online release of the article was abstracted and analyzed. RESULTS: A total of 3110 articles were reviewed. In 2020, the overall median time from submission to acceptance (AT) was 119 days (IQR 83-168) and 30 days (10-71) from acceptance to the first online release of the article (OP). AT increased by 7.3% from 2005 to 2020, whereas OP reduced by 73%. Publications, which the corresponding author was affiliated with US-located institution had shorter both AT and OP in 2005 and 2020. The author's specialty in ophthalmology had an inconclusive impact on AT and OP. Papers with multiple affiliated institutions had shorter AT and OP in both 2005 and 2020; however, these differences were not statistically significant. CONCLUSION: This study demonstrated that increasing pressure on authors, editors, and reviewers to publish articles and journals with high impact factor (IF) significantly influenced publication times in ophthalmology journals. Inflation of research papers was associated with rising AT time. A significant decrease in OP time was potentially explained by the editor's demand to achieve decent journal IF. This article brings to light relative publication times in the ophthalmology scientific journals.
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Introduction This single-center study aimed to compare the 12-month treatment outcomes of ranibizumab with that of aflibercept in routine clinical practice. Methods Cohort of patients diagnosed with treatment-naïve neovascular age-related macular degeneration (AMD), treated using either ranibizumab (n = 33 eyes) or aflibercept (n = 44 eyes) monotherapy over a 12-month follow-up period was analyzed. Anonymous data were extracted from the electronic database dedicated to the drug program. Results In the ranibizumab group, there were no statistically significant changes in best-corrected visual acuity (BCVA) (Early Treatment Diabetic Retinopathy Study [ETDRS] letters) and central retina thickness (CRT) (µm), between baseline (67.9 ± 8.6 & 384.9 ± 97.9) and at 12 months (67.9 ± 12.1 & 398.9 ± 127.1; P = 0.372 & P = 0.884, respectively). In the aflibercept, there was an improvement in BCVA and reduction in CRT between baseline (64.2 ± 8.1 & 414.3 ± 97.8) and at 12 months (70.7 ± 7.4 & 342.3 ± 71.6; P < 0.001 & P < 0.001, respectively). There was no difference in BCVA between the two groups at either diagnosis (P = 0.101) or 12 months (P = 0.917). Mean number of injections in the ranibizumab group was significantly lower (4.9 ± 1.5) than in the aflibercept group (6.7 ± 1; P < 0.001). Conclusions One initial injection of ranibizumab and then pro re nata (PRN) regimen resulted in stabilization of disease progression. Drug selection and treatment scheme could influence twelve-months outcomes. In the aflibercept group, three initial monthly injections and then every two months provided both significant BCVA improvement and CRT reduction at 12 months of treatment.
