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聚硫酸戊聚糖钠相关性色素性视网膜病变:危险因素与眼底表现

Pentosan Polysulfate Sodium-Associated Pigmentary Retinopathy: Risk Factors and Fundus Findings.

作者信息

Leung Ella H, Sharma Sahana, Levie-Sprick Ana, Lee Gregory D, Cho Hyung, Mukkamala Krishna

机构信息

Georgia Retina, Atlanta, GA, USA.

Vanderbilt University, Nashville, TN, USA.

出版信息

Clin Ophthalmol. 2021 Dec 24;15:4809-4816. doi: 10.2147/OPTH.S340041. eCollection 2021.

DOI:10.2147/OPTH.S340041
PMID:34992341
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8714003/
Abstract

PURPOSE

To evaluate the risk factors and fundus findings of patients with potential PPS-associated retinopathy.

PATIENTS AND METHODS

A retrospective chart review was performed of patients exposed to PPS who had a dilated fundus examination at a large retina-only practice from 2018-21. Multimodal images were evaluated by masked reviewers.

RESULTS

A total of 148 patients were included, of whom 33 (22%) had PPS-associated retinopathy, and 115 (78%) did not. The mean age was 60.3 years old, and the mean follow-up was 11.8 months. The PPS-associated retinopathy group had higher mean cumulative doses of PPS (1600g±849 vs 864g±852, < 0.0001, Mann-Whitney test) and longer duration of PPS use (13.6 years vs 7.48, < 0.0001). There was no statistically significant difference based on a history of kidney or liver disease or the dosage per day for the weight, body mass index, body surface area, or lean body weight. Of the patients with PPS-associated retinopathy whose genetic results were available, 15 of 16 (93%) were heterozygous for variants of uncertain significance.

CONCLUSION

A longer duration of PPS use and higher cumulative dosage of PPS were associated with an increased risk of developing PPS-associated pigmentary retinopathy. The role of genetic mutations in patients exposed to PPS is still to be determined.

摘要

目的

评估潜在的与聚苯乙烯磺酸钠(PPS)相关视网膜病变患者的危险因素和眼底表现。

患者与方法

对2018年至2021年在一家大型仅专注于视网膜疾病的医疗机构接受散瞳眼底检查的接触PPS患者进行回顾性病历审查。多模态图像由不知情的评审人员进行评估。

结果

共纳入148例患者,其中33例(22%)患有与PPS相关的视网膜病变,115例(78%)未患病。平均年龄为60.3岁,平均随访时间为11.8个月。与PPS相关的视网膜病变组的PPS平均累积剂量更高(1600克±849 vs 864克±852,<0.0001,曼-惠特尼检验),PPS使用时间更长(13.6年 vs 7.48年,<0.0001)。基于肾脏或肝脏疾病史或按体重、体重指数、体表面积或瘦体重计算的每日剂量,差异无统计学意义。在可获得基因检测结果的与PPS相关视网膜病变患者中,16例中有15例(93%)为意义未明变异的杂合子。

结论

PPS使用时间延长和PPS累积剂量增加与发生PPS相关色素性视网膜病变的风险增加有关。基因突变在接触PPS患者中的作用仍有待确定。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1f5b/8714003/bf21ef7680e6/OPTH-15-4809-g0002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1f5b/8714003/6220be8f328d/OPTH-15-4809-g0001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1f5b/8714003/bf21ef7680e6/OPTH-15-4809-g0002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1f5b/8714003/6220be8f328d/OPTH-15-4809-g0001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1f5b/8714003/bf21ef7680e6/OPTH-15-4809-g0002.jpg

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