一名杀手现身：中间型β地中海贫血的10年经验
A killer revealed: 10-year experience with beta-thalassemia intermedia.
作者信息
Matta Bassem N, Musallam Khaled M, Maakaron Joseph E, Koussa Suzanne, Taher Ali T
出版信息
Hematology. 2014 Jun;19(4):196-8. doi: 10.1179/1607845413Y.0000000120. Epub 2013 Nov 25.
OBJECTIVES
Patients with beta-thalassemia intermedia tend to present later in life with milder anemia than beta-thalassemia major patients. The incidence of mortality and its causes in this patient population remains unknown. We aim to reveal the incidence and most common causes of death in this population.
METHODS AND RESULTS
We reviewed the charts of all of the beta-thalassemia intermedia patients who had been followed at the Chronic Care Center in Hazmieh, Lebanon during a 10-year period. A total of 18 patients out of 127 had died during the follow-up period giving a cumulative 10-year mortality incidence of 14%. The most common causes of cardiac deaths were due to renal and cardiac causes.
DISCUSSION
Most causes of death have been linked to the high levels of iron coupled with anemia present in this patient population. Many of deaths could be prevented by adequate treatment.
CONCLUSION
Larger studies with more comprehensive data capture on risk factors of mortality in this patient population are called for.
目的
中间型β地中海贫血患者往往比重型β地中海贫血患者在生命后期出现症状，贫血症状也较轻。该患者群体的死亡率及其原因尚不清楚。我们旨在揭示该群体的死亡率及最常见死因。
方法与结果
我们回顾了黎巴嫩哈兹米亚慢性病护理中心在10年期间随访的所有中间型β地中海贫血患者的病历。在127名患者中，共有18名在随访期间死亡，10年累积死亡率为14%。心脏死亡的最常见原因是肾脏和心脏问题。
讨论
大多数死亡原因与该患者群体中高水平的铁以及贫血有关。通过适当治疗，许多死亡是可以预防的。
结论
需要开展更大规模的研究，更全面地收集该患者群体死亡率风险因素的数据。
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