Archivos De La Sociedad Espanola De Oftalmologia最新文献

Background and objective

Objective

Materials and methods

Results

Conclusion

Purpose

Methods

Results

Conclusions

Objective

Methods

Results

Conclusion

Gyrate atrophy of the choroid and retina (GACR) is a rare autosomal recessive disease characterised by elevated plasma ornithine levels due to deficiency of the enzyme ornithine aminotransferase (OAT). The accumulation of this amino acid in plasma leads to the development of patches of chorioretinal atrophy in the peripheral retina extending into the macular area. Patients usually present with night blindness followed by constriction of the visual field and, finally, decreased central vision and blindness. The disease is diagnosed by the presence of the characteristic clinical picture, the presence of hyperornithinaemia in plasma and the detection of mutations in the OAT enzyme gene. There is currently no effective gene therapy and the most common therapeutic intervention mainly involves dietary modifications with arginine restriction.

This article aims to summarise the pathogenesis, clinical and diagnostic findings and treatment options in patients with GACR.

We describe a 28-year-old Caucasian female with vigorexy, who had no previous ocular history. She presented with bilateral gradual painless reduction in vision over the past 3 weeks. She had been taking niacin supplements, averaging 500 mg daily, for 7 years. Fundus examination revealed bilateral CME, which was confirmed by ocular coherence tomography scan. Fundus fluorescein angiography did not reveal any fluid leakage. Niacin supplementation was discontinued, and after 2 months, the CME had completely resolved, and the best corrected visual acuities improved to 1 in both eyes.

Objectives

To show donation data, number of keratoplasties and the changes in transplant indications and techniques that occurred in Andalusia in the period from 2013 to 2022.

Materials and methods

The present work is a retrospective and descriptive study that included all keratoplasties performed between January 2013 and December 2022 in Andalusia, as well as the evolution of the cornea donation and transplant activity of the public and private hospitals pertaining to the waiting list management system of the Public Health System of Andalusia. Transplants performed in private centers with corneas from outside Andalusia were excluded.

Results

Cornea donation activity in Andalusia in the decade 2013-2022 has experienced a growth of more than 126%, while overall transplant activity has increased by 157% in public hospitals. Penetrating keratoplasty has decreased from 83% in 2013 to 43% in 2022, while lamellar techniques have increased from 17% to 57% in this same period. Since 2018, more lamellar transplants are performed than penetrating transplants. Regarding indications, endothelial conditions already represent the first cause of transplantation. In 2022 alone, the public Andalusian Eye Banks evaluated 1,054 corneas and prepared 281 endothelial grafts.

Conclusion

In the decade from 2013 to 2022 in Andalusia there has been an increase in donation activity and the number of keratoplasties. The public Eye Banks implementation in this period has played a key role in the widespread adoption of lamellar keratoplasty techniques and has enabled the transition to perform a greater number of lamellar keratoplasties compared to penetrating keratoplasty.

In retinopathy of prematurity (ROP) type I, the use of intravitreal bevacizumab (IVB), which is an inhibitor of endothelial growth factor (VEGF), has become popular despite not being a therapy approved by regulatory agencies. However, IVB has shown positive effects in halting disease progression at lower costs compared to other anti-VEGF therapies (ranibizumab or aflibercept). In this report, we present the experience during the treatment with IVB of 102 Colombian children with ROP type I, with a success rate of 98% (100). Complications occurred in 3.9% (4). Finally, we conclude that a single dose of IVB is an effective therapy for the management of ROP type I, with a lower risk of complications and retreatment.