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印度尼西亚的地中海贫血症。

Thalassemia in Indonesia.

机构信息

Department of Child Health, Cipto Mangunkusumo Hospital, Jakarta, Indonesia.

The Eijkman Institute for Molecular Biology, Jakarta, Indonesia.

出版信息

Hemoglobin. 2022 Jan;46(1):39-44. doi: 10.1080/03630269.2021.2023565.

DOI:10.1080/03630269.2021.2023565
PMID:35950580
Abstract

Indonesia is located along the 'Thalassemia Belt' and a hotspot for hemoglobinopathies. Around 3.0-10.0% of the population carry β-thalassemia (β-thal) and 2.6-11.0% of the population carry α-thalassemia (α-thal). It is estimated that around 2500 babies are born with β-thal major (β-TM) each year. At present, the cornerstone of treatment for β-TM in Indonesia remains supportive, including blood transfusions and iron chelation therapy. Hemovigilance systems in some cities are poor and it increases the risk of transfusion-transmitted infections and transfusion reactions. The availability of iron chelators remains uncertain, even in some rural areas, iron chelators do not exist. The poor adherence to iron chelation therapy and maintaining pretransfusion hemoglobin (Hb) levels above 9.0 g/dL are still a major issue in Indonesia. The cost of blood transfusion and iron chelation are covered by national health insurance. In line with the rise of life expectancy, the financial burden of thalassemia in Indonesia is increasing sharply. Thus, optimizing preventive programs may be the most suitable option for the current thalassemia condition in Indonesia.

摘要

印度尼西亚位于“地中海贫血带”,是血红蛋白病的热点地区。大约 3.0-10.0%的人口携带β-地中海贫血(β-thal),2.6-11.0%的人口携带α-地中海贫血(α-thal)。据估计,印度尼西亚每年大约有 2500 名婴儿患有重型β-地中海贫血(β-TM)。目前,印度尼西亚β-TM 的治疗基石仍然是支持性治疗,包括输血和铁螯合治疗。一些城市的血液监测系统较差,这增加了输血传播感染和输血反应的风险。即使在一些农村地区,铁螯合剂的供应也不确定,因为根本不存在铁螯合剂。印度尼西亚仍存在铁螯合治疗的依从性差和维持输血前血红蛋白(Hb)水平在 9.0g/dL 以上的问题。输血和铁螯合治疗的费用由国家健康保险覆盖。随着预期寿命的延长,印度尼西亚地中海贫血的财务负担急剧增加。因此,优化预防计划可能是目前印度尼西亚地中海贫血状况的最佳选择。

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