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KMID : 1151620210060010014
Journal of Retina
2021 Volume.6 No. 1 p.14 ~ p.20
Characteristics and Clinical Course of Korean Patients Diagnosed with Stargardt Disease
Nah Seung-Kwan

Kim Jae-Hui
Lew Young-Ju
Kim Jong-Woo
Kim Chul-Gu
Lee Dong-Won
Abstract
Purpose: The aim of the study was to investigate clinical characteristics and visual prognosis of Korean patients with Stargardt disease.

Methods: Medical records of patients diagnosed with Stargardt disease between 2008 and 2019 were analyzed. In patients followed for more than 12 months, visual acuities measured at diagnosis were compared with those measured at the final visit.

Results: Twenty-five patients (12 males, 13 females) were included in this study, and the mean age was 28.9 ¡¾ 15.8 years. The mean logarithm of minimal angle of resolution (logMAR) best-corrected visual acuity (BCVA) was 0.54 ¡¾ 0.46 at diagnosis. In 12 eyes (24.0%), the photoreceptor layer at the fovea was preserved. Nineteen patients (38 eyes) were followed for more than 12 months, with a mean follow- up period of 70.1 ¡¾ 47.9 months. The logMAR BCVA deteriorated from 0.63 ¡¾ 0.45 at diagnosis to 0.98 ¡¾ 0.58 at the final visit (p < 0.001). LogMAR 0.3 or greater deterioration in BCVA was noted in 19 eyes (50.0%).

Conclusions: Stargardt disease is diagnosed in relatively young people and shows no sex prevalence. The visual acuity at diagnosis was relatively good. However, during follow-up, logMAR 0.3 or greater visual deterioration was noted in half of the patients. Further studies investigating the associations between genetic features and clinical characteristics are needed.
KEYWORD
Characteristics, Outcome, Stargardt¡¯s disease
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