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Stargardt 病表型的临床特征和人口统计学分布:来自多层次眼科保健网络的电子病历驱动的大数据分析。

Clinical profile and demographic distribution of Stargardt disease phenotypes: An Electronic medical record-driven big data analytics from a multitier eye care network.

机构信息

Department of EyeSmart EMR and AEye; Department of Ophthalmology, Indian Health Outcomes, Public Health, and Economics Research Center, L V Prasad Eye Institute, Hyderabad, Telangana, India, Telangana.

Department of Ophthalmology, Indian Health Outcomes, Public Health, and Economics Research Center; Department of Ophthalmology, Anant Bajaj Retina Institute, L V Prasad Eye Institute, Hyderabad, Telangana, India.

出版信息

Indian J Ophthalmol. 2023 Oct;71(10):3407-3411. doi: 10.4103/IJO.IJO_3290_22.

DOI:10.4103/IJO.IJO_3290_22
PMID:37787244
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10683681/
Abstract

PURPOSE

To describe the demographics and clinical profile of Stargardt disease in patients presenting a multitier ophthalmology hospital network in India.

METHODS

This cross-sectional hospital-based study was performed among 2,834,616 new patients presenting between August 2010 and June 2021 in our network. Patients with a clinical diagnosis of Stargardt disease in at least one eye were included as cases. The data were collected using an electronic medical record system.

RESULTS

Overall, 1,934 (0.069%) patients were diagnosed with Stargardt disease. Most of the patients were male (63.14%). The most common age group at presentation was during the second decade of life, with 626 (31.87%) patients. The overall prevalence was higher in patients from a higher socioeconomic status (0.077%), in those presenting from the urban geography (0.079%), and in students (0.197%). Systemic history of hypertension was seen in 56 (2.85%) patients, while diabetes mellitus was seen in (2.49%) patients. Of the 3,917 eyes, 1,910 (48.76%) eyes had moderate visual impairment (>20/70-20/200) followed by severe visual impairment (>20/200 to 20/400) in 646 (16.49%) eyes. The most commonly associated retinal signs were retinal flecks in 1,260 (32.17%) eyes, followed by RPE changes in 945 (24.13%) eyes. The most documented investigations were autofluorescence (39.85%), followed by optical coherence tomography (23.90). Cataract surgery was the commonest performed surgical intervention in (0.66%) eyes, followed by intravitreal injection in 4 (0.10%) eyes. The family history of parent consanguinity marriage was reported by 212 (10.79%) patients.

CONCLUSION

Stargardt disease was seen more commonly in males presenting during the second decade of life. It is predominantly a bilateral disease, with the majority of the eyes having moderate visual impairment.

摘要

目的

描述在印度一家多级别眼科医院网络就诊的 Stargardt 病患者的人口统计学和临床特征。

方法

本横断面医院病例研究纳入了 2010 年 8 月至 2021 年 6 月期间在我们网络就诊的 2834616 名新患者。将至少一只眼临床诊断为 Stargardt 病的患者作为病例纳入研究。数据通过电子病历系统收集。

结果

总体而言,1934 名(0.069%)患者被诊断为 Stargardt 病。大多数患者为男性(63.14%)。就诊时最常见的年龄组为第二个十年,有 626 名(31.87%)患者。来自较高社会经济地位(0.077%)、来自城市地区(0.079%)和学生(0.197%)的患者总体患病率更高。56 名(2.85%)患者有高血压病史,24 名(2.49%)患者有糖尿病病史。在 3917 只眼中,1910 只(48.76%)眼有中度视力障碍(>20/70-20/200),其次是 646 只(16.49%)眼有严重视力障碍(>20/200 至 20/400)。最常见的视网膜病变体征是视网膜斑点 1260 只(32.17%),其次是 RPE 改变 945 只(24.13%)。最常见的检查是自发荧光(39.85%),其次是光学相干断层扫描(23.90%)。白内障手术是最常见的眼部手术干预措施(0.66%),其次是 4 只眼(0.10%)进行玻璃体腔内注射。212 名(10.79%)患者报告有父母近亲结婚史。

结论

Stargardt 病在第二十年纪就诊的男性中更为常见。它主要是一种双侧疾病,大多数眼睛有中度视力障碍。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0602/10683681/5d20ed5828e1/IJO-71-3407-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0602/10683681/8904e4283b32/IJO-71-3407-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0602/10683681/5d20ed5828e1/IJO-71-3407-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0602/10683681/8904e4283b32/IJO-71-3407-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0602/10683681/5d20ed5828e1/IJO-71-3407-g002.jpg

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