重型β-地中海贫血患儿体内铁负荷磁共振成像T2*检测的临床分析

doi:10.3877/cma.j.issn.1673-5250.2011.05.006

目的

探讨磁共振成像T2* (MRI T2*)技术检测β-地中海贫血患儿体内铁负荷的应用价值。

方法

2010年1月至12月本院儿科共收治19例重型β-地中海贫血患儿，采用回顾性分析方法记录患儿输血及去铁治疗情况。分离患儿血清，检测血清铁蛋白含量。人组患儿赴香港玛丽医院进行体内各脏器(肝脏、心脏、胰腺及垂体)铁负荷MRI T2*检测，对研究结果进行评估和相关性分析。

结果

入组的地中海贫血患儿均曾接受不规则输血及去铁治疗，血清铁蛋白值为(3114~14 550)μg/L。患儿各脏器MRI T2*测定结果显示，体内各脏器均出现不同程度的铁沉积。血清铁蛋白值与肝脏MRI T2*呈负相关(r= -0.569, P= 0.011)，与左心室射血分数(EF)及胰腺MRI T2* 呈负相关(r=-0.633, P= 0.004；r= -0.592, P= 0.008)。进一步研究发现，心脏MRI T2*与胰腺MRI T2*及胰腺MRI T2*与垂体MRI T2* 呈正相关(r=0.696，P= 0.001; r=0.491，P=0.033)。

结论

MRI T2*检测方法在评估重型β-地中海贫血患儿体内铁负荷方面有重要临床意义。

关键词: 地中海贫血, 铁负荷, 血清铁蛋白, 磁共振成像

Objective

To investigate the clinical significance of magnetic resonance imaging T2-star (MRI T2*) tests on iron overload in children with β-thalassemia major.

Methods

Nineteen children with β-thalassemia major were recruited in this study during January to December 2010. Retrospective analytical study on the history of blood transfusion and chelation therapy were recorded. Sera were obtained from the patients and were tested for serum ferritin. MRI T2* tests on iron overload of liver, heart, pancreas and pituitary in these children were performed in Queen Mary Hospital, Hong Kong. The results were evaluated, and the relationships between different values of iron overload were analyzed. The study protocol was approved by the Ethical Review Board of Investigation in Human Being of Sun Yat-sen Memorial Hospital, Sun Yat-sen University. Informed consent was obtained from the parents of each participating child.

Results

All the patients received irregular blood transfusion and chelation therapy. The value of serum ferritin ranged from 3114 μg/L to 14 550 μg/L. The organs of the patients showed different degrees of iron overload by MRI T2* detection. Levels of serum ferritin were negatively correlated with liver MRI T2*, left ventricular ejection fraction, and pancreas MRI T2* (r=-0.569, P= 0.011; r=—0.633, P= 0.004;r=- 0.592, P= 0.008). In addition, there were positive correlation between heart MRI T2* and pancreas MRI T2* (r=0.696, P= 0.001), between pancreas MRI T2* and pituitary MRI T2* (r=0.491, P= 0.033) as well.

Conclusion

MRI T2* tests show clinical significance for evaluating iron overload in children with β-thalassemia major.

Key words: thalassemia, iron overload, serum ferritin, magnetic resonance imaging

图2　心脏MRI T2*与胰腺MRI T2*呈正相关

图1　血清铁蛋白水平与肝脏MRI T2*呈负相关

	1　Rund D, Rachmilewitz E. Beta-thalassemia [J]. N Engl J Med, 2005, 353(11): 1135-1146.
	2　Fang JP, Xu LH. Standardization of diagnosis and treatment for children with beta-thalassemia major [J]. Chin J Pediatr, 2010, 48 (3):166-169. [方建培，许吕宏.规范儿童重型β-地中海贫血的诊治[J].中华儿科杂志，2010,48(3) :166-169.]
	3　Borgna-Pignatti C, Gamberini MR. Complications of thalassemia major and their treatment [J]. Expert Rev Hematol, 2011, 4(3):353-366.
	4　Perera NJ, Lau NS, Mathews S, et al. Overview of endocrinopathies associated with β-thalassaemia major [J]. Intern Med J, 2010,40(10):689-696.
	5　Weng WJ, Fang JP, Xu HG, et al. A pilot study on the detection and clinical significance of PRA in children with β-thalassemia major[J/CD]. Chin J Obstet Gynecol Pediatr ( Electron Ed), 2007, 3(3): 134-137. [翁文骏，方建培，徐宏贵，等.重型β地中海贫血患儿群体反应性抗体检测及临床意义[J/CD].中华妇幼临床医学杂志:电子版，2007，3(3)：134-137.]
	6　Lam WW, Au WY, Chu WC, et al. One-stop measurement of iron deposition in the anterior pituitary, liver, and heart in thalassemia patients [J]. J Magn Reson Imaging, 2008,28(1):29-33.
	7　Busca A, Falda M, Manzini P, et al. Iron overload in patients receiving allogeneic hematopoietic stem cell transplantation:Quantification of iron burden by a superconducting quantum interference device ( SQUID) and therapeutic effectiveness of phlebotomy[J]. Biol Blood Marrow Transplant, 2010,16(1): 115-122.
	8　Deborah-Chirnomas S, Geukes-Foppen M, Barry K, et al. Practical implications of liver and heart iron load assessment by T2*-MRI in children and adults with transfusion-dependent anemias [J]. Am J Hematol,2008,83(10):781-783.
	9　Anderson LJ, Holden S, Davis B, et al. Cardiovascular T2-star (T2*) magnetic resonance for the early diagnosis of myocardial iron overload [J]. Eur Heart J, 2001, 22(23):2171-2179.
	10　Au WY, Lam WW, Chu WW, et al. A cross-sectional magnetic resonance imaging assessment of organ specific hemosiderosis in 180 thalassemia major patients in Hong Kong [ J]. Haematologica, 2008, 93(5):784-786.
	11　St Pierre TG, Tran KC, Webb J, et al. Organ-specific crystalline structures of ferritin cores in beta-thalassemia/hemoglobin E [J]. Biol Met, 1991,4(3):162-165.
	12　Oudit GY, Sun H, Trivieri MG, et al. L-type Ca2+ channels provide a major pathway for iron entry into cardiomyocytes in ironoverload cardiomyopathy [J]. Nat Med, 2003,9(9): 1187-1194.

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Clinical Analysis of Magnetic Resonance Imaging T2* Tests on Iron Overload in Children With β-Thalassemia Major

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Clinical Analysis of Magnetic Resonance Imaging T2* Tests on Iron Overload in Children With β-Thalassemia Major