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加纳阿克拉科勒布医院镰状细胞病的眼部表现。

Ocular manifestations of sickle cell disease at the Korle-bu Hospital, Accra, Ghana.

作者信息

Osafo-Kwaako Alfred, Kimani Kahaki, Ilako Dunera, Akafo Stephen, Ekem Ivy, Rodrigues Onike, Enweronu-Laryea Christabel, Nentwich Martin M

机构信息

Department of Ophthalmology, University of Nairobi, Nairobi, Kenya.

出版信息

Eur J Ophthalmol. 2011 Jul-Aug;21(4):484-9. doi: 10.5301/EJO.2010.5977.

Abstract

PURPOSE

To determine the magnitude and pattern of ocular manifestations in sickle cell disease at Korle-bu Hospital, Accra, Ghana.

METHODS

Hospital-based cross-sectional study including all patients with sickle cell disease reporting for routine follow-up at the Sickle Cell Clinic at Korle-bu Hospital, Accra, Ghana.

RESULTS

A total of 201 patients with sickle cell disease (67 male and 134 female) were enrolled, comprising 114 subjects with genotype HbSS, aged 6-58 years, mean 19.26 (SD 11.70), and 87 with genotype HbSC, aged 6-65 years, mean 31.4 (SD 16.76). Visual impairment was found in 5.6% of eyes examined. Causes were cataract, proliferative sickle retinopathy (PSR), optic atrophy, phthisis bulbi, and central retinal artery occlusion. Common anterior segment signs of sickle cell disease, which were more common in HbSC patients, were tortuous corkscrew conjunctival vessels, iris atrophy, and cataract. Eyes with iris atrophy or depigmentation were 1.8 times more at risk of PSR than eyes without. Overall, PSR was found in 12.9% of subjects examined (3.5% of HbSS, 25.3% of HbSC; 15.9% of males and 11.2% of females). The prevalence of proliferative sickle retinopathy increased with age and increased systemic severity of sickle cell disease; sex did not have an influence.

CONCLUSIONS

There is a high prevalence of ocular morbidity in sickle cell disease patients at Korle-bu Hospital. Prevalence increased with age, systemic severity of sickle cell disease, and HbSC genotype.

摘要

目的

确定加纳阿克拉科尔勒-布医院镰状细胞病患者眼部表现的程度和模式。

方法

基于医院的横断面研究,纳入所有在加纳阿克拉科尔勒-布医院镰状细胞病诊所进行常规随访的镰状细胞病患者。

结果

共纳入201例镰状细胞病患者(67例男性和134例女性),其中114例为HbSS基因型,年龄6 - 58岁,平均19.26岁(标准差11.70),87例为HbSC基因型,年龄6 - 65岁,平均31.4岁(标准差16.76)。在所检查的眼睛中,5.6%存在视力障碍。病因包括白内障、增殖性镰状视网膜病变(PSR)、视神经萎缩、眼球痨和视网膜中央动脉阻塞。镰状细胞病常见的眼前节体征在HbSC患者中更为常见,包括迂曲的螺旋状结膜血管、虹膜萎缩和白内障。有虹膜萎缩或色素脱失的眼睛发生PSR的风险是没有这些情况的眼睛的1.8倍。总体而言,在所检查的受试者中,12.9%发现有PSR(HbSS患者中为3.5%,HbSC患者中为25.3%;男性中为15.9%,女性中为11.2%)。增殖性镰状视网膜病变的患病率随年龄和镰状细胞病全身严重程度的增加而升高;性别无影响。

结论

科尔勒-布医院镰状细胞病患者眼部疾病的患病率很高。患病率随年龄、镰状细胞病全身严重程度和HbSC基因型的增加而升高。

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