地中海贫血症的治疗管理。
Management of the thalassemias.
机构信息
Hemoglobinopathy Research University Health Network, Toronto, Ontario M5G 2C4, Canada.
出版信息
Cold Spring Harb Perspect Med. 2013 Jun 1;3(6):a011767. doi: 10.1101/cshperspect.a011767.
Abstract
During the last 30 years, in addition to the considerable progress made in control and prevention of thalassemias(3), there have also been major advances in their symptomatic management, at least in wealthier countries where appropriate facilities are available. Remarkable improvements in survival in the severe forms of thalassemia have followed the more judicious use of blood transfusion and, in particular, the ability to manage the iron accumulation resulting from transfusion with its severe and ultimately lethal effects on endocrine and cardiac function.
摘要
在过去的 30 年中，除了在地中海贫血症的控制和预防方面取得了相当大的进展（3）之外，在症状管理方面也取得了重大进展，至少在有适当设施的富裕国家是如此。更明智地使用输血，特别是能够管理输血引起的铁积累，以及其对内分泌和心脏功能的严重和最终致命影响，使得严重地中海贫血症的存活率显著提高。
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