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输血依赖型地中海贫血儿童的眼部受累。

Ocular Involvement in Transfusion Dependent Thalassemia Children.

机构信息

Dr Mohammed Jamal Uddin, Assistant Professor, Department of Pediatrics, Jahurul Islam Medical College Hospital, Kishoreganj, Bangladesh; E-mail:

出版信息

Mymensingh Med J. 2023 Jul;32(3):807-811.

Abstract

Thalassemia is one of the most common haemoglobinopathies in the world. Transfusion dependent thalassemia patients need regular blood transfusion. Repeated blood transfusions lead to iron overloads which can affect various organs in the body including eyes. The present study aims to evaluate the ocular involvements in transfusion dependent thalassemia children and their relationship with duration of the disease and serum ferritin level. This cross-sectional observational study included 46 multi-transfused thalassemia children aged 3-18 years. Detailed ophthalmological examination including visual acuity, slit lamp bio microscopy, direct and indirect ophthalmoscopy was done. Statistical analysis was done using SPSS version (IBM) 23.0. Student's t-test and chi-square (χ²) test was done and p value of <0.05 was considered significant. Out of 46 thalassemia children male were 25(54.3%) and female were 21(45.7%). Mean age of the children was 8.94±5.04 years, mean duration of disease was 7.02±3.5 years and mean serum ferritin level 1543.68±914.43ng/dl. Ocular involvements were detected in 19(41.3%) children. Among them 8(17.39%) children had more than one ocular involvements. The ocular manifestations were decreased visual acuity in 17(36.95%), corneal dryness in 7(15.21%), lens opacity in 6(13.04%), optic disc atrophy in 7(15.21%), peripheral retinal pigmentation in 5(10.86%) and retinal vessels tortuosity in 3(6.52%) children. Higher serum ferritin level and increased duration of the disease were significantly (p<0.001) related with ocular involvement. Various ocular involvements were found in transfusion dependent thalassemia children. Therefore, transfusion dependent thalassemia children should be screened periodically for early detection and proper management of ocular changes.

摘要

地中海贫血是世界上最常见的血红蛋白病之一。依赖输血的地中海贫血患者需要定期输血。反复输血会导致铁过载,从而影响体内的各种器官,包括眼睛。本研究旨在评估依赖输血的地中海贫血儿童的眼部并发症及其与疾病持续时间和血清铁蛋白水平的关系。这项横断面观察性研究纳入了 46 名年龄在 3-18 岁的多次输血的地中海贫血儿童。进行了详细的眼科检查,包括视力、裂隙灯生物显微镜、直接和间接检眼镜检查。使用 IBM SPSS 版本 23.0 进行统计分析。采用学生 t 检验和卡方(χ²)检验,p 值<0.05 认为有统计学意义。46 例地中海贫血儿童中,男性 25 例(54.3%),女性 21 例(45.7%)。儿童的平均年龄为 8.94±5.04 岁,疾病平均持续时间为 7.02±3.5 年,平均血清铁蛋白水平为 1543.68±914.43ng/dl。19 例(41.3%)儿童存在眼部并发症。其中 8 例(17.39%)儿童有多种眼部并发症。眼部表现为视力下降 17 例(36.95%)、角膜干燥 7 例(15.21%)、晶状体混浊 6 例(13.04%)、视盘萎缩 7 例(15.21%)、周边视网膜色素沉着 5 例(10.86%)和视网膜血管迂曲 3 例(6.52%)。较高的血清铁蛋白水平和较长的疾病持续时间与眼部受累显著相关(p<0.001)。依赖输血的地中海贫血儿童存在多种眼部并发症。因此,应定期对依赖输血的地中海贫血儿童进行筛查,以早期发现和适当管理眼部变化。

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