摘要
地中海贫血(地贫)是由于珠蛋白肽链基因缺陷而导致的遗传性溶血性贫血,临床以β和α肽链合成障碍而出现的β地贫和α地贫最为常见。这2类地贫的中间型和重型患者均出现明显的贫血,对患儿生长发育和生活质量影响极大。特别是重型地贫患者若不接受造血干细胞移植,需终生依赖输血及祛铁治疗以维持生命,医疗费用高,给家庭、社会和国家带来极大的负担。因此,如何加强地贫人群的管理,规范中、重型地贫患者的输血及祛铁治疗,有效改善地贫患者生存质量,做好地贫基因携带人群的监测和健康教育及出生干预至关重要。
Thalassemia is a hereditary hemolytic anemia caused by globin peptide gene deficiency. β-thalassemia and α-thalassemia are the most common clinical disorders of β-and α-peptide chain synthesis. Both thalassemia intermedia and major may significantly effect on patient’s growth develop-ment and quality of life,especially in patients with thalassemia major. If not receiving hematopoietic stem cell transplanta-tion,they need rely on life-long blood transfusion and chelation therapy. That leads a high medical costs and a great burden on families,society and the country. Therefore,it is essencial in how to strengthen the management of thalassemic population standardize thalassemic patient’s blood transfusion and iron chelation treatment,effectively to improve the quality life of thalassemic patients and to monitor the population of thalassemia gene carrier,health education and birth intervention.
作者
王晓东
李长钢
WANG Xiao-dong;LI Chang-gang(Department of Blood Oncology,Shenzhen Children's Hospital,Shenzhen 518026,China)
出处
《中国实用儿科杂志》
CSCD
北大核心
2018年第12期965-970,共6页
Chinese Journal of Practical Pediatrics
关键词
地中海贫血
输血
祛铁
综合管理
thalassemia
transfusion
iron chelation
comprehensive management
