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重型地中海贫血患者肺动脉压与血清铁蛋白水平及心脏磁共振成像T2*之间关系的评估

Evaluation of the Relationship Among Pulmonary Artery Pressure with Serum Ferritin Level and CMR T2* in Thalassemia Major.

作者信息

Ostad-Hosini Fatemeh, Qomi Mohammad Reza Safaei, Eghbali Aziz, Falahati Vahid, Mehrabi Sarvenaz, Ghandi Yazdan

机构信息

School of Medicine, Arak University of Medical Sciences, Arak, Iran.

Pediatric Oncology, Iran University of Medical Sciences, Tehran, Iran.

出版信息

Curr Health Sci J. 2021 Apr-Jun;47(2):184-189. doi: 10.12865/CHSJ.47.02.06. Epub 2021 Jun 30.

Abstract

BACKGROUND

Pulmonary hypertension is one of the most common cardiac complications among patients with β-thalassemia major (β-TM). The aim of this study is to investigate the correlation of pulmonary artery pressure (PAP), serum ferritin level, and cardiac T2* MRI in patients with β-TM.

METHOD

This cross-sectional study was performed on 50 patients older than 7 years old. Echocardiography, electrocardiography, and cardiac T2* MRI were performed on all patients and their serum ferritin levels were measured. Based on the echocardiographic the patients were divided into two groups of PAP>30 and <30 mmHg.

RESULTS

40% had PAP higher than 30 mmHg, 32% had abnormal T2* MRI, and 36% had serum ferritin levels higher than 1500ng/dl. there were significant negative correlations between CMRI *T2 and PAP (-0.36) and the P-pulmonary (-0.29). the serum ferritin level was positively correlated with PAP (0.44) and the P pulmonary in ECG (0.30). in the patients with PAP>30, the means of age, serum ferritin level, and P-pulmonary were significantly higher than patients with PAP<30 (P=0.001). In the patients with PAP>30mmHg, CMRI *T2 had higher accuracy (80%), sensitivity (65%), and specificity (90%) compared to the measurement of serum ferritin levels.

CONCLUSION

The PAP was positively correlated with serum ferritin levels and negatively with T2MRI. The accuracy of 80% for T2MRI as a method to diagnose or even anticipate PH among patients with TM suggested that this method is a useful and accurate method of PH diagnosis and can be used as an alternative among clinicians.

摘要

背景

肺动脉高压是重型β地中海贫血(β-TM)患者中最常见的心脏并发症之一。本研究旨在探讨β-TM患者的肺动脉压(PAP)、血清铁蛋白水平与心脏T2*磁共振成像(MRI)之间的相关性。

方法

本横断面研究对50例7岁以上患者进行。对所有患者进行超声心动图、心电图和心脏T2*MRI检查,并测量其血清铁蛋白水平。根据超声心动图结果,将患者分为PAP>30和<30 mmHg两组。

结果

40%的患者PAP高于30 mmHg,32%的患者T2MRI异常,36%的患者血清铁蛋白水平高于1500 ng/dl。心脏MRI的T2与PAP(-0.36)和肺动脉P波(-0.29)之间存在显著负相关。血清铁蛋白水平与PAP(0.44)和心电图中的肺动脉P波(0.30)呈正相关。在PAP>30的患者中,年龄、血清铁蛋白水平和肺动脉P波的平均值显著高于PAP<30的患者(P=0.001)。在PAP>30 mmHg的患者中,与血清铁蛋白水平测量相比,心脏MRI的T2*具有更高的准确性(80%)、敏感性(65%)和特异性(90%)。

结论

PAP与血清铁蛋白水平呈正相关,与T2MRI呈负相关。T2MRI作为诊断甚至预测TM患者肺动脉高压(PH)的方法,其80%的准确性表明该方法是一种有用且准确的PH诊断方法,可作为临床医生的一种选择。

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Assessment of cardiac parameters in evaluation of cardiac functions in patients with thalassemia major.
Pediatr Hematol Oncol. 2012 Apr;29(3):220-34. doi: 10.3109/08880018.2012.671449.
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Pulmonary hypertension in thalassemia.地中海贫血相关肺动脉高压。
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