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伊朗南部依赖输血的β地中海贫血患者的眼部检查结果

Ocular findings in patients with transfusion-dependent β-thalassemia in southern Iran.

作者信息

Haghpanah Sezaneh, Zekavat Omid Reza, Bordbar Mohammadreza, Karimi Mehran, Zareifar Soheila, Safaei Sanaz, Ramzi Mani, Ashraf Hossein

机构信息

Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.

Poostchi Ophthalmology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.

出版信息

BMC Ophthalmol. 2020 Sep 22;20(1):376. doi: 10.1186/s12886-020-01647-y.

Abstract

BACKGROUND

Ocular involvement may occur via several mechanisms in patients with transfusion-dependent β-thalassemia (TDT) mainly chronic anemia, iron overload and iron chelator toxicity. We aimed to evaluate the frequency of abnormal ocular findings and their relationship with hematologic parameters in TDT patients.

METHODS

In this cross-sectional study from January 2018 to January 2019, a total of 79 patients with TDT over the age of 18 who were on iron-chelation therapy (ICT) were consecutively investigated. All patients were registered at the Thalassemia Comprehensive Center affiliated with Shiraz University of Medical Sciences, Shiraz, Southern Iran. Complete ophthalmic examination was performed by an expert ophthalmologist. Clinical and hematologic parameters were collected from the patients´ medical records.

RESULTS

The mean age ± standard deviation (SD) of the patients was 28.4 ± 5.6 years (range: 18-43). Twenty-four patients (30.4%) were male and 29 (36.7%) were splenectomized. The mean ± SD of the best-corrected visual acuity (VA) was 0.960 ± 0.086 decimal, (range: 0.6-1), 0.016 ± 0.046 logMar, (range: 0-0.2). The frequency of patients with VA > 0.1 logMar was 3 (3.8%). The mean intraocular pressure (IOP) was 14.88 ± 3.34 (6-25) mmHg. Fundus abnormalities were observed in 8 patients (10.1%), consisting of increased cup-disk ratio (3.8%), vessel tortuosity (2.5%), retinal pigment epithelium degeneration (2.5%), myelinated nerve fiber layer (1.3%), and internal limiting membrane wrinkling (1.3%). No significant association was observed between fundus abnormalities, VA, or IOP with hematologic parameters (P > 0.05). TDT patients with diabetes mellitus had significantly higher IOP (P = 0.010) but similar frequency of fundus abnormalities with non-diabetic patients (P > 0.05).

CONCLUSIONS

The frequency of ocular abnormalities in our patients was lower than the previous reports. The frequency of fundus abnormalities were similar in diabetic and non-diabetic thalassemia patients indicating close monitoring and proper management of the disease and comorbidities in these patients.

摘要

背景

在依赖输血的β地中海贫血(TDT)患者中,眼部受累可能通过多种机制发生,主要包括慢性贫血、铁过载和铁螯合剂毒性。我们旨在评估TDT患者眼部异常发现的频率及其与血液学参数的关系。

方法

在这项2018年1月至2019年1月的横断面研究中,连续调查了79名年龄在18岁以上且正在接受铁螯合治疗(ICT)的TDT患者。所有患者均在伊朗南部设拉子医科大学附属的地中海贫血综合中心登记。由一名专业眼科医生进行全面的眼科检查。从患者的病历中收集临床和血液学参数。

结果

患者的平均年龄±标准差(SD)为28.4±5.6岁(范围:18 - 43岁)。24名患者(30.4%)为男性,29名(36.7%)进行了脾切除术。最佳矫正视力(VA)的平均值±SD为0.960±0.086小数(范围:0.6 - 1),0.016±0.046 logMar(范围:0 - 0.2)。VA>0.1 logMar的患者频率为3名(3.8%)。平均眼压(IOP)为14.88±3.34(6 - 25)mmHg。8名患者(10.1%)观察到眼底异常,包括杯盘比增加(3.8%)、血管迂曲(2.5%)、视网膜色素上皮变性(2.5%)、有髓神经纤维层(1.3%)和内界膜皱襞(1.3%)。未观察到眼底异常、VA或IOP与血液学参数之间存在显著关联(P > 0.05)。患有糖尿病的TDT患者眼压显著更高(P = 0.010),但与非糖尿病患者的眼底异常频率相似(P > 0.05)。

结论

我们患者的眼部异常频率低于先前的报告。糖尿病和非糖尿病地中海贫血患者的眼底异常频率相似,表明对这些患者的疾病和合并症进行密切监测和适当管理。

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Ophthalmic Evaluation in Beta-Thalassemia.眼科评估在β-地中海贫血症中的应用。
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