The Role of MR Imaging in the Assessment of Clinical Outcomes in Children with X-Linked Adrenoleukodystrophy after Allogeneic Haematopoietic Stem Cell Transplantation
The Role of MR Imaging in the Assessment of Clinical Outcomes in Children with X-Linked Adrenoleukodystrophy after Allogeneic Haematopoietic Stem Cell Transplantation
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DOI：
10.12659/pjr.893285
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发表时间：
2015-01-01
影响因子：
1.2
通讯作者：
Sasiadek, Marek J.
中科院分区：
文献类型：
--
作者：
Bladowska, Joanna;Kulej, Dominika;Sasiadek, Marek J.
背景：本研究的目的是分析X-连锁肾上腺脑白质营养不良(X-ALD)患儿在造血干细胞移植(HSCT)前后的MR图像，包括先进的MR技术，如单体素波谱(MRS)和扩散张量成像(DTI)，并建立有助于疾病分期、HSCT资格和随访的成像标准。材料/方法：7名男孩，年龄5~10岁，平均8.14岁，生化证实X-ALD。使用MRI严重程度评分(LOES评分)分析结构图像。在1例患者中，随访检查包括MRS和代谢产物比率(NAA/Cr，Cho/Cr，mi/Cr)，以及DTI，评估几个白质束的分数各向异性(FA)和表观扩散系数(ADC)。结果：2名男孩在HSCT前MRI严重程度评分等于<；8分，HSCT后他们没有临床或放射学进展。在HSCT前严重程度评分较高(8~16分，平均10.9分)的5例患者中，观察了临床和放射学进展(MRI严重程度评分17~25分，平均20.9分)。1例男童随访有代谢改变，所有受测区FA和ADC值均降低。结论：早期X-ALD(MRI严重程度评分低于8分)儿童更有可能从HSCT中受益。DTI和MRS似乎是评估X-ALD进展的更有用的影像方法。
Background: The aim of the study was to analyse MR images of the brain, including advanced MR techniques, such as single voxel spectroscopy (MRS) and diffusion tensor imaging (DTI), in children with X-linked adrenoleukodystrophy (X-ALD) before and after haematopoietic stem cell transplantation (HSCT) and to establish the imaging criteria which may be helpful in the assessment of disease staging, qualification to HSCT and follow-up.Material/Methods: Seven boys, aged 5-10 years, (mean 8.14 years) with biochemically proved X-ALD, underwent plain MR imaging with a 1.5 T unit before and after HSCT. Structural images were analyzed using an MRI severity scale (Loes scale). In one patient the follow-up examinations included MRS with the assessment of metabolite ratios (NAA/Cr, Cho/Cr, mI/Cr), as well as DTI with evaluation of fractional anisotropy (FA) and apparent diffusion coefficient (ADC) in several white matter tracts.Results: Two boys had an MRI severity score before HSCT equal to < 8 points, and after HSCT they showed no clinical or radiological progression. In 5 patients with a higher severity score (from 8 to 16 points, mean 10.9) before HSCT, clinical and radiological progression was observed (MRI severity score from 17 to 25 points, mean 20.9). Follow-up advanced MRI techniques in one boy showed metabolic alterations, as well as decreased FA and ADC values in all evaluated areas.Conclusions: Children at an early stage of X-ALD (below 8 points in MRI severity scale) are more likely to benefit from HSCT. DTI and MRS seem to be more useful imaging methods to assess the progression of X-ALD.