重型β地中海贫血眼部变化的研究现状

Research status of ocular changes in betathalassemia major

重型β地中海贫血(β-TM)是一种由珠蛋白合成缺陷引起的遗传性疾病。该病需要长期输血和服用祛铁剂药物治疗,可引起眼部组织的各种继发性变化。与正常同龄人相比,β-TM患者的眼部表现为角膜曲率更为陡峭、前房更浅、晶状体厚度增加、眼轴更短和泪液分泌减少等改变;同时,营养缺乏和使用铁螯合剂药物治疗会增加罹患并发性白内障及视网膜变性的风险,从而影响β-TM患者的生存质量。本文结合国内外相关文献,对β-TM患者眼部相关变化进行探讨和综述,旨在为临床实践提供有价值的见解。

Beta-thalassemia major(β-TM)is an inherited disease caused by a defect in the synthesis of globin.The disease requires long-term blood transfusion and iron chelator treatment,which can cause various secondary changes in the body and eye tissues.Compared with normal peers,β-TM patients will show changes in the eye such as steeper corneal curvature,shallower anterior chamber,increased lens thickness,shorter axial length,and reduced tear secretion.At the same time,nutritional deficiencies and the use of iron chelator drugs will increase the risk of complicated cataract and retinal degeneration,thus affecting the quality of life ofβ-TM patients.This article combines relevant domestic and foreign literatures to explore and review the changes in the eye ofβ-TM patients,with a view to providing valuable insights for clinical practice.