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心脏磁共振评估地中海贫血患者的心脏铁负荷。

Evaluation of cardiac iron load by cardiac magnetic resonance in thalassemia.

机构信息

Department of Pediatrics, Dr Balabhai Nanavati Hospital, Mumbai, India.

出版信息

Indian Pediatr. 2011 Sep;48(9):697-701. doi: 10.1007/s13312-011-0115-9. Epub 2010 Nov 30.

Abstract

OBJECTIVE

To quantify myocardial iron stores by Cardiac Magnetic Resonance (CMR).

DESIGN

Prospective cohort study.

SETTING

Thalassemia center in a teaching hospital.

PARTICIPANTS

60 transfusion dependant thalassemia major patients and 10 controls during 2008-2009.

METHODS

MRI T2* for cardiac iron load and cardiac functions was performed on a 1.5 Tesla Siemens Sonata machine using the thalassemia tools software. Ejection fraction (EF) was measured using standard CMR sequence and EF <56% considered as cardiac dysfunction. Quantification of iron deposition was categorized as T2* <10 milliseconds (ms) as high risk, 10-20 ms as intermediate risk and >20 ms as low risk. Simultaneous liver iron T2* values were categorized into normal i.e. >6.3 ms, mild iron overload 6.3-2.7 ms , moderate iron overload 2.7- 1.4 ms and severe iron overload <1.4 ms. Pretransfusion serum ferritin levels were simultaneously determined. Data was analyzed by paired and unpaired t test of mean.

RESULTS

Of 60 patients, 50% had no cardiac siderosis; 33.3% had mild to moderate and while 16.7% had severe cardiac siderosis . In contrast, only 8.3% had normal liver iron values, 55.7% had mild to moderate and 36% had severe iron stores. The mean serum ferritin of all 60 cases was 3528.6 ± 1958.6 ng/mL. There was a statistically significant difference in the mean cardiac T2* of patients (23.45 ± 13.4 ms) as compared to controls (32.67 ± 2.68 ms) (P<0.01).

CONCLUSIONS

Thalassemia patients had significantly higher cardiac iron stores as compared to controls. Serum ferritin and liver iron values did not correlate with cardiac iron values. Three of 10 patients <10 years showed evidence of myocardial siderosis.

摘要

目的

通过心脏磁共振(CMR)定量心肌铁储存量。

设计

前瞻性队列研究。

地点

教学医院的地中海贫血中心。

参与者

2008 年至 2009 年期间,60 名依赖输血的地中海贫血症患者和 10 名对照者。

方法

使用地中海贫血工具软件在 1.5T 西门子 Sonata 机器上进行心脏铁负荷和心脏功能的 MRI T2检查。使用标准 CMR 序列测量射血分数(EF),EF<56%被认为是心功能障碍。铁沉积的定量分类为 T2<10 毫秒(ms)为高风险,10-20 ms 为中风险,>20 ms 为低风险。同时对肝脏铁 T2*值进行分类,正常即>6.3 ms,轻度铁过载 6.3-2.7 ms,中度铁过载 2.7-1.4 ms,重度铁过载<1.4 ms。同时测定转铁蛋白前血清铁蛋白水平。数据通过配对和非配对 t 检验进行分析。

结果

60 例患者中,50%无心脏含铁血黄素沉着症;33.3%有轻度至中度,而 16.7%有重度心脏含铁血黄素沉着症。相比之下,只有 8.3%的患者肝脏铁值正常,55.7%有轻度至中度,36%有重度铁储存。所有 60 例患者的平均血清铁蛋白为 3528.6±1958.6ng/ml。与对照组(32.67±2.68 ms)相比,患者的平均心脏 T2*值(23.45±13.4 ms)存在统计学显著差异(P<0.01)。

结论

与对照组相比,地中海贫血症患者的心脏铁储存量明显更高。血清铁蛋白和肝脏铁值与心脏铁值不相关。10 名<10 岁的患者中有 3 名出现心肌含铁血黄素沉着的证据。

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