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首页|期刊导航|中国当代儿科杂志|MRI T2*技术对中、重型β地中海贫血患者心脏及肝脏铁沉积状态的评估

MRI T2*技术对中、重型β地中海贫血患者心脏及肝脏铁沉积状态的评估

李长钢 刘四喜 麦惠容 王缨 文飞球 刘日阳 张新华 朱昭颖 区永仁

中国当代儿科杂志2012,Vol.14Issue(2):110-113,4.
中国当代儿科杂志2012,Vol.14Issue(2):110-113,4.

MRI T2*技术对中、重型β地中海贫血患者心脏及肝脏铁沉积状态的评估

Evaluation of heart and liver iron deposition status in patients with β- thalassemia intermedia and major with MRI T2 * technique

李长钢 1刘四喜 1麦惠容 1王缨 1文飞球 1刘日阳 2张新华 3朱昭颖 4区永仁5

作者信息

  • 1. 深圳市儿童医院,广东 深圳 518026
  • 2. 惠州市中心人民医院,广东 惠州 516001
  • 3. 解放军第 303 医院,广西 南宁 530021
  • 4. 香港中文大学威尔斯亲王医院,中国 香港
  • 5. 香港大学玛丽医院,中国 香港
  • 折叠

摘要

Abstract

Objective To study the status of iron deposition in patients with β-thalassemia intermedia and major in mainland China. Methods The status of transfusion and chelation was examined in 39 patients with p-thalassemia intermedia or major. Serum ferritin levels were measured. MRI T2 * technique was used to detect cardiac and hepatic iron deposition. Results Serum ferritin levels ranged from the minimum of 1500 ng/mL up to a maximum of 11491 ng/mL. From liver MRI T2 * measurement, 15 cases had severe hepatic iron deposition ( 38% ) and moderate deposition was found in 15 cases (38% ) , mild in 7 cases (18% ) , and normal in 2 cases (5% ). Heart MRI T2 * showed severe heart iron deposition in 7 cases ( 18% ) , mild in 5 cases ( 13% ) , and normal in 27 cases (69% ). One case had cardiac arrhythmia. Four cases were over 20 years of age, and presented with gonadal function hypoplasia. The majority of patients did not receive regular transfusion and they had delayed, suboptimal chelation due to financial problems. Serum ferritin level was closely related with timing and dosage of chelation. Conclusions In patients with p-thalassemia who do not receive early regular transfusion and iron chelation therapy, iron deposition may occur at an early age. Important organs and tissue functional lesions and related complications also result. Relevant agencies and family members should be aware of this trend and develop appropriate strategies to improve the medical condition and quality of life of patients with this disorder.

关键词

地中海贫血/MRI T2*/铁沉积/儿童

Key words

Thalassemia/ MRI T2*/ Iron deposition/ Child

分类

医药卫生

引用本文复制引用

李长钢,刘四喜,麦惠容,王缨,文飞球,刘日阳,张新华,朱昭颖,区永仁..MRI T2*技术对中、重型β地中海贫血患者心脏及肝脏铁沉积状态的评估[J].中国当代儿科杂志,2012,14(2):110-113,4.

基金项目

深圳市科技计划项目(重点)(No.201001019). (重点)

中国当代儿科杂志

OA北大核心CSCDCSTPCDMEDLINE

1008-8830

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