重型β地中海贫血眼底变化的研究进展  

Research progress of fundus changes in severe beta thalassemia

作　　者：李雯文 陈丹娜 魏永灿 赵铨文 赵桂玲 Li Wenwen;Chen Danna;Wei Yongcan;Zhao Quanwen;Zhao Guiling(Department of Ophthalmology,Affiliated Hospital of Guangdong Medical University,Guangzhou 524002,China)

机构地区：[1]广东医科大学附属医院眼科,广州524002

出　　处：《中华眼底病杂志》2024年第4期329-333,共5页Chinese Journal of Ocular Fundus Diseases

基　　金：广东省基础与应用基础研究基金项目(2022A1515220122);广东医科大学青年科学培育基金项目(GDMUQ2022033)。

摘　　要：β地中海贫血是由于β珠蛋白基因缺陷导致的遗传性溶血性疾病,其中输血依赖型β地中海贫血患者需要长期输血才能存活,其疾病本身及长期输血会出现一系列的全身及眼部并发症,眼底可因长期贫血、铁螯合剂副作用出现视网膜血流密度下降、视网膜厚度变薄、弹性假黄瘤综合征等。目前国内外对于地中海贫血患者的眼部改变的相关报道较少,认知相对匮乏,因此眼科医师需要提高警惕,应深入探究对因、对症治疗,结合个体疾病特征,为临床治疗提供更科学、更精确的方案。β thalassemia is a hereditary hemolytic disease caused by the defect of β globin gene.Transfusion-dependent β thalassemia patients need long-term blood transfusion to survive,and a series of systemic and ocular complications will occur in the disease itself and long-term blood transfusion.Retinal blood vessel density decreases,retinal thickness thinned and elastic pseudoxanthoxanoma syndrome are found in fundus due to long-term anemia and side effects of iron chelating agent.At present,there are few reports about eye changes in thalassemia patients,and the cognition is relatively scarce.Therefore,it is necessary to be vigilant for physicians,deeply explore the cause and symptomatic treatment,combined with individual disease characteristics,to provide a more scientific and accurate plan for clinical treatment.

关 键 词：地中海贫血 视网膜 视神经 眼底变化 综述 

分 类 号：R773.4[医药卫生—眼科]