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Acta Biomed. 2022 May 11;93(2):e2022022. doi: 10.23750/abm.v93i2.10736.
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本文引用的文献

1
Prepubertal Hypertransfusion in Thalassemia Intermedia: A Case Report of Sustained Positive Effects on Growth, Splenic Function and Endocrine Parameters.中间型地中海贫血青春期前大量输血:对生长、脾功能和内分泌参数持续产生积极影响的病例报告
Oman Med J. 2012 Nov;27(6):e012. doi: 10.5001/omj.2012.125.
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Nomograms for mitral inflow Doppler and tissue Doppler velocities in Caucasian children.白种儿童二尖瓣流入道多普勒和组织多普勒速度的列线图。
J Cardiol. 2016 Oct;68(4):288-99. doi: 10.1016/j.jjcc.2015.10.004. Epub 2015 Nov 10.
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A stepwise α-thalassemia screening strategy in high-prevalence areas.在高发地区采用逐步的α-地中海贫血筛查策略。
Eur J Haematol. 2013 Aug;91(2):164-9. doi: 10.1111/ejh.12136. Epub 2013 Jun 15.
4
T2* MRI in regularly transfused children with thalassemia intermedia: serum ferritin does not reflect liver iron stores.中间型地中海贫血定期输血患儿的T2*磁共振成像:血清铁蛋白不能反映肝脏铁储备。
Pediatr Hematol Oncol. 2012 Sep;29(6):579-84. doi: 10.3109/08880018.2012.708891. Epub 2012 Jul 27.
5
Risk factors for pulmonary hypertension in patients with β thalassemia intermedia.β 中间型地中海贫血患者肺动脉高压的危险因素。
Eur J Intern Med. 2011 Dec;22(6):607-10. doi: 10.1016/j.ejim.2011.05.013. Epub 2011 Jun 29.
6
Optimal management of β thalassaemia intermedia.β 中间型地中海贫血的最佳管理。
Br J Haematol. 2011 Mar;152(5):512-23. doi: 10.1111/j.1365-2141.2010.08486.x. Epub 2011 Jan 20.
7
Early echocardiographic findings in β-thalassemia intermedia patients using standard and tissue Doppler methods.使用标准和组织多普勒方法对中间型β地中海贫血患者进行早期超声心动图检查的结果
Pediatr Cardiol. 2011 Feb;32(2):154-9. doi: 10.1007/s00246-010-9834-2. Epub 2010 Nov 17.
8
Recommendations for quantification methods during the performance of a pediatric echocardiogram: a report from the Pediatric Measurements Writing Group of the American Society of Echocardiography Pediatric and Congenital Heart Disease Council.小儿超声心动图检查期间定量方法的建议:美国超声心动图学会小儿与先天性心脏病委员会小儿测量写作组的报告
J Am Soc Echocardiogr. 2010 May;23(5):465-95; quiz 576-7. doi: 10.1016/j.echo.2010.03.019.
9
Overview on practices in thalassemia intermedia management aiming for lowering complication rates across a region of endemicity: the OPTIMAL CARE study.地中海贫血中间型管理实践概述,旨在降低流行地区的并发症发生率:OPTIMAL CARE 研究。
Blood. 2010 Mar 11;115(10):1886-92. doi: 10.1182/blood-2009-09-243154. Epub 2009 Dec 23.
10
Relation between iron-overload indices, cardiac echo-Doppler, and biochemical markers in thalassemia intermedia.中间型地中海贫血中铁过载指标、心脏超声多普勒与生化标志物之间的关系。
Am J Cardiol. 2008 Aug 1;102(3):363-7. doi: 10.1016/j.amjcard.2008.03.066. Epub 2008 May 29.

定期输血对非输血依赖型地中海贫血(NTDT)患儿的心脏保护作用:一项队列研究。

Cardio-protective effect of regular transfusion in children with non-transfusion dependent thalassemia (NTDT): A cohort study.

机构信息

Sultan Qaboos University Hospital.

Faculty of Medicine, Alexandria University.

出版信息

Acta Biomed. 2022 May 11;93(2):e2022022. doi: 10.23750/abm.v93i2.10736.

DOI:10.23750/abm.v93i2.10736
PMID:35546036
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9171854/
Abstract

BACKGROUND AND AIM OF THE WORK

Cardiac complications occur in patients with non-transfusion dependent thalassemia (NTDT). The study aimed to evaluate transfusion effect on systolic and diastolic cardiac function in young NTDT patients.  Methods:  Study design: Cohort study. Seventeen regularly-transfused patients with NTDT (12.5±5.3 years; group 1) and 15 none/minimally transfused patients (13.2±4.8 years; group 2) were followed up for 5 years and compared as regards their clinical parameters, echocardiographic and Tissue-Doppler-Imaging.

RESULTS

Group 2 patients had significantly higher peak late-diastolic velocity of the left-ventricular-inflow Doppler (Am). Mitral-valve A-wave duration/pulmonary-veins, A-wave duration-ratio and pulmonary-vein S/D velocities-ratio were larger in group 2 as well (p = < 0.01). The diameters of right and left outflow-tract were larger with a higher cardiac-index in patients of group 2. Systolic-function was similar in the 2 studied groups.

CONCLUSION

Diastolic function assessment revealed indicators of an abnormal relaxation of left-ventricle in non-transfused patients, which suggests a diastolic dysfunction. An increase in the diameter of the outflow-tract is likely attributed to high cardiac-output status in non-transfused NTDT patients as they have a higher cardiac index. Early start of regular transfusion for NTDT patients might prevent serious long-term cardiac complications.

摘要

背景与目的

非输血依赖型地中海贫血(NTDT)患者可发生心脏并发症。本研究旨在评估输血对年轻 NTDT 患者收缩和舒张心脏功能的影响。

方法

研究设计:队列研究。17 例定期输血的 NTDT 患者(12.5±5.3 岁;第 1 组)和 15 例非输血或最小输血患者(13.2±4.8 岁;第 2 组)接受了 5 年的随访,并对其临床参数、超声心动图和组织多普勒成像进行了比较。

结果

第 2 组患者的左心室流入多普勒(Am)峰值晚期舒张速度显著更高。二尖瓣 A 波持续时间/肺静脉、A 波持续时间比和肺静脉 S/D 速度比在第 2 组也更大(p < 0.01)。第 2 组患者的右和左流出道直径较大,心指数较高。两组患者的收缩功能相似。

结论

舒张功能评估显示非输血患者左心室舒张功能异常的指标,提示舒张功能障碍。非输血 NTDT 患者流出道直径增加可能归因于高心输出量状态,因为他们的心指数较高。早期开始对 NTDT 患者进行常规输血可能预防严重的长期心脏并发症。