重型β地中海贫血中的心脏疾病：它可逆吗？
Cardiac disease in beta-thalassaemia major: Is it reversible?
作者信息
Atiq M, Bana M, Ahmed U S, Bano S, Yousuf M, Fadoo Z, Khurshid M
机构信息
Department of Paediatrics, The Aga Khan University Hospital, PO Box 3500, Stadium Road, Karachi, Pakistan.
出版信息
Singapore Med J. 2006 Aug;47(8):693-6.
INTRODUCTION
The aim of this study was to evaluate the spectrum of cardiac involvement and its outcome in beta-thalassaemia major.
METHODS
There were 75 patients with a mean age of 13.8 (+/- 5.5) years, of whom 33 were male and 42 were female. Clinical history, examination and laboratory investigations were assessed. Electrocardiograms, chest radiographs and echocardiograms were reviewed.
RESULTS
44 patients had cardiac involvement in the form of left ventricular systolic dysfunction in 17, diastolic dysfunction in 22, pericardial effusion in 12 and pulmonary hypertension in 12 patients. With intense chelation therapy and cardiac medications, the condition of 13 of 17 patients with systolic dysfunction, and four of 22 with diastolic dysfunction, improved.
CONCLUSION
Cardiac disease is a common complication of siderotic disease in thalassaemia major and it can be prevented with regular chelation. This study has shown improved systolic function after regular chelation therapy.
引言
本研究旨在评估重型β地中海贫血患者心脏受累的范围及其预后。
方法
75例患者，平均年龄13.8（±5.5）岁，其中男性33例，女性42例。评估临床病史、检查及实验室检查结果。回顾心电图、胸部X线片及超声心动图。
结果
44例患者存在心脏受累，表现为左心室收缩功能障碍17例、舒张功能障碍22例、心包积液12例及肺动脉高压12例。通过强化螯合治疗及心脏药物治疗，17例收缩功能障碍患者中有13例、22例舒张功能障碍患者中有4例病情改善。
结论
心脏病是重型地中海贫血铁沉着症的常见并发症，通过定期螯合治疗可预防。本研究显示定期螯合治疗后收缩功能有所改善。
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