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对重型地中海贫血患者体内铁负荷评估的非侵入性检查方法的批判性综述。

A critical review of non invasive procedures for the evaluation of body iron burden in thalassemia major patients.

作者信息

Maggio Aurelio, Capra Marcello, Pepe Alessia, Mancuso Luigi, Cracolici Eliana, Vitabile Salvatore, Rigano Paolo, Cassarà Filippo, Midiri Massimo

机构信息

Division of Haematology II with Thalassemia, Hospital V. Cervello, Via Trabucco n'180, and Radiology Institute, University of Palermo, Policlinico P. Giaccone, Palermo, Italy.

出版信息

Pediatr Endocrinol Rev. 2008 Oct;6 Suppl 1:193-203.

PMID:19337178
Abstract

It is evident that different non invasive methodologies have been implemented for the detection of organ specific iron burden in patients with thalassemia major. Among these MR relaxometry has the potential to become the method of choice for non-invasive, safe and accurate assessment of organ-specific iron load, although further theoretical research, along with studies monitoring wider age groups of patients, is needed. Moreover, the possibility of detecting organ-specific iron burden is relevant for tailoring specific chelation treatment in different patients or in the same patient during different periods of life. In fact, while heart organ-specific effect has been suggested by MR relaxometry for some chelation treatments, it is possible to suppose that another single chelator or association of other chelators may show different organ-specific effects. For these reasons, the future of our clinical research will be to understand, mainly by MR relaxometry, whether it would be possible to set up a tailored organ-specific chelation treatment, according to this supposed difference in organ efficacy of the current chelation therapies.

摘要

显然,已经采用了不同的非侵入性方法来检测重型地中海贫血患者器官特异性铁负荷。其中,磁共振弛豫测量法有潜力成为非侵入性、安全且准确评估器官特异性铁负荷的首选方法,不过还需要进一步的理论研究以及对更广泛年龄组患者的监测研究。此外,检测器官特异性铁负荷的可能性对于为不同患者或同一患者在生命不同阶段量身定制特定的螯合治疗具有重要意义。事实上,虽然磁共振弛豫测量法已表明某些螯合治疗对心脏有器官特异性影响,但可以推测另一种单一螯合剂或其他螯合剂的组合可能会显示出不同的器官特异性影响。出于这些原因,我们临床研究的未来将主要通过磁共振弛豫测量法来了解,根据当前螯合疗法在器官疗效上的这种假定差异,是否有可能建立一种量身定制的器官特异性螯合治疗方法。

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