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1.
Artigo em Chinês | WPRIM (Pacífico Ocidental) | ID: wpr-995631

RESUMO

Objective:To observe the histopathological changes in peripheral retinal lesions under intraoperative optical coherence tomography (iOCT).Methods:A retrospective case series study. Eighty-eight patients (194 eyes) who underwent vitreoretinal surgery in the Department of Ophthalmology at the East Ward of the First Affiliated Hospital of Zhengzhou University from October 2021 to May 2022 in 94 eyes were included in the study. Among them, 49 cases were male and 39 cases were female, with the mean age of (50.93±17.55) years. Ninety-four eyes included 32 eyes with retinal detachment, 6 eyes with proliferative diabetic retinopathy, 28 eyes with vitreous hemorrhage, 8 eyes with ocular trauma, 14 eyes with the macular lesion, 1 eye with uveitis, 1 eye with family exudative vitreoretinopathy (FEVR), 1 eye with acute retinal necrosis (ARN), and 3 eyes with lens dislocation. All affected eyes were examined with iOCT during vitreoretinal surgery. The iOCT scanning of the peripheral retina was performed with the help of episcleral pressure. The pre-equatorial and serrated edge anterior and posterior of retinas were scanned according to the characteristics of different fundus diseases. Various abnormal fundus manifestations were recorded.Results:In 94 eyes, 53 eyes (56.38%, 53/94) have different types of retinopathy in the peripheral retina. Of these, 7 eyes (7.45%) have retinal cystoid degeneration; 19 eyes (20.21%) have lattice degeneration; and 8 eyes (8.51%) have pigment degeneration; 9 eyes (9.57%) have pavement-like degeneration; 7 eyes (7.45%) have small occult holes; 1 eye (1.06%) has familial exudative vitreoretinopathy (FEVR) serrated edge "dyke-like" proliferative degeneration; 4 eyes (4.26%) have vitreous and retinopathy adhesions; and one eye (1.06%) has ARN.Conclusion:With clear refractive media, iOCT can provide clear scans of different peripheral retinal lesions.

2.
Genes (Basel) ; 11(11)2020 11 16.
Artigo em Inglês | MEDLINE | ID: mdl-33207664

RESUMO

BACKGROUND: Peripheral pigmentary changes are common amongst women with Danon disease; however, there is currently a lack of longitudinal observational studies of the retinal changes in this condition, and the long-term visual prognosis is not well understood. METHODS AND RESULTS: In this report, we present long-term follow-up data (12 years of follow-up) regarding peripheral retinopathy in an Asian woman and her mother who were both diagnosed with Danon disease. Both patients showed a novel nonsense mutation of the LAMP2 gene (c.123 of exon 2). During the follow-up period, no evident extension of peripheral pigmented lesions or visual field progression was observed. CONCLUSIONS: We report, for the first time, the long-term longitudinal follow-up of Danon disease-related retinopathy in an Asian patient featuring an indolent macular-sparing peripheral lesion.


Assuntos
Doença de Depósito de Glicogênio Tipo IIb/complicações , Proteína 2 de Membrana Associada ao Lisossomo/genética , Retinose Pigmentar/etiologia , Códon sem Sentido , Feminino , Seguimentos , Doença de Depósito de Glicogênio Tipo IIb/genética , Humanos , Pessoa de Meia-Idade , Retinose Pigmentar/diagnóstico por imagem , Adulto Jovem
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