Department of Pediatrics, Dr Balabhai Nanavati Hospital, Mumbai, India.
Indian Pediatr. 2011 Sep;48(9):697-701. doi: 10.1007/s13312-011-0115-9. Epub 2010 Nov 30.
To quantify myocardial iron stores by Cardiac Magnetic Resonance (CMR).
Prospective cohort study.
Thalassemia center in a teaching hospital.
60 transfusion dependant thalassemia major patients and 10 controls during 2008-2009.
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.
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).
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.
通过心脏磁共振(CMR)定量心肌铁储存量。
前瞻性队列研究。
教学医院的地中海贫血中心。
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。
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。
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)。
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。