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镰状细胞性状患者急性视力丧失的罕见病因

Rare Cause of Acute Loss of Vision in a Patient With Sickle Cell Trait.

作者信息

Bojja Srikaran, Javed Nismat, Allena Nishant, Bojja Shreya, Khaja Misbahuddin

机构信息

Internal Medicine, BronxCare Health System, Icahn School of Medicine at Mount Sinai, New York City, USA.

Pulmonology, BronxCare Health System, Icahn School of Medicine at Mount Sinai, New York City, USA.

出版信息

Cureus. 2023 Jul 27;15(7):e42535. doi: 10.7759/cureus.42535. eCollection 2023 Jul.

Abstract

Sickle cell disease (SCD) is a prevalent inherited blood disorder with various ocular manifestations, including sickle cell retinopathy (SCR), characterized by retinal microcirculation impairment and ischemic complications. We present the case of a 21-year-old male with sickle cell trait who experienced a sudden, painless loss of vision in his left eye. Ophthalmologic examination revealed vitreous hemorrhage and neovascularization, indicating SCR. Initial treatment with hydroxyurea and exchange transfusions led to partial improvement. However, due to persistent vitreous hemorrhage, the patient underwent a vitrectomy. The sickle cell trait affects a large global population, and its retinopathy is a rare but severe complication. The pathogenesis and risk factors for SCR are similar to those for SCD. The diagnosis of SCR is established through fundoscopic examination and graded based on Goldberg's classification. Management involves a multidisciplinary approach targeting systemic illness and visual defects, including hydroxyurea, photocoagulation, anti-vascular endothelial growth factors, and vitrectomy. Awareness, early diagnosis, and timely intervention are essential to preventing vision-threatening complications in sickle cell trait patients with SCR.

摘要

镰状细胞病(SCD)是一种常见的遗传性血液疾病,有多种眼部表现,包括镰状细胞视网膜病变(SCR),其特征为视网膜微循环障碍和缺血性并发症。我们报告一例21岁患有镰状细胞性状的男性,其左眼突然无痛性视力丧失。眼科检查发现玻璃体出血和新生血管形成,提示为SCR。初始使用羟基脲和换血治疗后病情有部分改善。然而,由于玻璃体持续出血,该患者接受了玻璃体切除术。镰状细胞性状影响着全球大量人口,其视网膜病变是一种罕见但严重的并发症。SCR的发病机制和危险因素与SCD相似。SCR通过眼底镜检查确诊,并根据戈德堡分类法进行分级。治疗采用多学科方法,针对全身疾病和视觉缺陷,包括使用羟基脲、光凝治疗、抗血管内皮生长因子和玻璃体切除术。提高认识、早期诊断和及时干预对于预防患有SCR的镰状细胞性状患者发生威胁视力的并发症至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5168/10460114/32ac899ab484/cureus-0015-00000042535-i01.jpg

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