磁共振成像(T2*)检测重型β-地中海贫血患儿心脏、肝脏铁负荷及左心室射血分数的研究  

Magnetic Resonance Imaging(MRI T2*)Detection of Left Ventricular Ejection Fraction(LVEF) and Iron Overload in the Heart and Liver of Children with Severe β-thalassemia

作　　者：旷文勇[1] 向永华[1] 李婉丽[1] 杨海霞[1] 张本山[1] 吴攀[1] 何姗[1] 郑敏翠[1] 

机构地区：[1]湖南省儿童医院血液科,长沙410007

出　　处：《中国小儿血液与肿瘤杂志》2016年第1期37-40,44,共5页Journal of China Pediatric Blood and Cancer

摘　　要：目的观察我院重型β-地中海贫血（β-TM）患儿血清铁蛋白（SF）和体内铁沉积状况及对心脏功能的影响。方法检测我院28例重型β-TM患儿SF水平,并由我院放射科运用磁共振成像T2＊（MRI T2＊）技术检测心脏及肝脏铁沉积状况及左室射血分数（LVEF）。结果本组28例患儿中男16例,女12例,中位年龄11.2岁（7岁1个月~16岁11个月）。28例患儿中SF轻度升高者4例（14.3%）,中度升高者8例（28.6%）,高度升高者16例（57.1%）。肝脏MRI T2＊检测结果 ：肝铁轻度沉积2例（7.1%）,中度沉积8例（28.6%）,重度沉积18例（54.8%）;心脏MRI T2＊检测结果 ：心铁正常16例（57.1%）,轻度铁沉积4例（14.3%）,中度铁沉积4例（14.3%）,重度铁沉积4例（14.3%）;LVEF降低者19例（67.9%）,4例心脏重度铁沉积患儿中有3例（75%）LVEF明显降低（≤55%）。本组患儿SF水平与肝脏T2＊值（r=-0.467,P=0.036）、心脏T2＊值（r=-0.486,P=0.01）呈明显负相关;SF与输血量呈正相关（r=0.634,P=0.043;r=0.067,P=0.124）而与去铁治疗呈负相关（r=-0.526,P=0.043）,SF和心脏T2＊值与LVEF均无相关性（r=-0.154,P=0.306;r=0.067,P=0.124）。结论重型β-TM患儿SF增高及肝脏重度铁沉积发生率高,虽然心脏重度铁沉积患儿发生率不高,但心脏重度铁沉积患儿多有LVEF明显降低。Objective To detect the serum level of ferritin and body iron deposition and to study their impact on the cardiac function in children with severe B-thalassemia in our hospital.Methods Serum ferritin（SF） level was detected in children with severe B-thalassemia in our hospital.Heart and liver iron deposition and left ventricular ejection fraction（LVEF） were detected by magnetic resonance imaging T2 ＊（MRI T2 ＊）.Results We studied 28 pediatric patients with severe B-thalassemia aging from 7 years 1 month to 16 years 11 months（median age 11.2 yr）,including 16 males and 12 females.Mild overload of SF was detected in 4 cases（14.3%）,moderate overload of SF in 8 cases（28.6%）and high overload of SF in 16 cases（57.1%）.The liver MRI T2 ＊ test showed that 2 cases（7.1%）had mild hepatic iron deposition,8 cases（28.6%） had moderate deposition and 18 cases（54.8%）had severe deposits.The cardiac MRI T2 ＊ test demonstrated that 16 cases had normal cardiac iron（57.1%） and 4 cases（14.3%） had mild iron deposition,moderate iron deposition and severe iron deposition respectively.The LVEF decreased in 19 patients（67.9%）,and significantly reduced（≤55%） in 3 cases（75%） of 4 patients with severe cardiac iron deposition.The SF level was negatively correlated with both liver T2 ＊ values（r =-0.467,P = 0.036） and cardiac T2 ＊ values（r=-0.486,P=0.01） in the 28 patients and it was positively correlated with the amount of blood transfusion（r = 0.634,P = 0.043;r = 0.067,P = 0.124）.Treatment with deferiprone was negatively correlated with SF level（r =-0.526,P = 0.043）,proving its therapeutic effect.LVEF was not correlated with SF and cardiac T2 ＊ values（r =- 0.154,P = 0.306;r = 0.067,P = 0.124）.Conclusions Children with severe β-thalassemia have high incidence of SF overload and severe liver iron deposition.However,severe heart iron deposition does not often occur in these patients.The LVEF decreases significantly in most patients with severe heart iron d

关 键 词：Β-地中海贫血 铁蛋白 铁沉积 左室射血分数 磁共振成像T2＊ 

分 类 号：R725.5[医药卫生—儿科]