| 儿童甲基丙二酸血症颅脑常规MRI影像分析 |
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| 引用本文： | 李素荣,袁新宇,朱彦丽,王立文.儿童甲基丙二酸血症颅脑常规MRI影像分析[J].临床放射学杂志,2006,25(12):1143-1146. |
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| 作者姓名： | 李素荣 袁新宇 朱彦丽 王立文 |
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| 作者单位： | 1. 100020,北京,首都儿科研究所放射科
2. 100020,北京,首都儿科研究所内科 |
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| 摘 要： | 目的探讨甲基丙二酸血症（MMA）患儿颅脑常规MRI特点及其临床应用价值。资料与方法对13例MMA患儿的常规MRI图像进行回顾性分析。全部病例均经尿有机酸分析确诊。结果MRI主要表现为脑发育延迟和脑组织受损。其中，脑沟、脑池增宽者12例，合并侧脑室增宽者9例；脑自质容积减少者8例，脑自质髓鞘化延迟7例；胼胝体形态幼稚6例，另有1例胼胝体压部信号异常；苍白球显示长T1、长T2信号者1例。结论颅脑常规MRI可反映MMA对脑发育的影响和脑损伤，有助于了解病情和评估预后。
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| 关 键 词： | 甲基丙二酸血症 儿童 磁共振成像 |
| 收稿时间： | 2006-06-16 |
| 修稿时间： | 2006-06-162006-08-13 |
MRI Diagnosis of Methylmalonic Acidemia in Children |
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| LI Surong, YUAN Xinyu, ZHU Yanli,et al..MRI Diagnosis of Methylmalonic Acidemia in Children[J].Journal of Clinical Radiology,2006,25(12):1143-1146. |
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| Authors: | LI Surong YUAN Xinyu ZHU Yanli |
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| Institution: | Department of Radiology, Capital Institute of Pediatrics, Beijing 100020, P, R, China |
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| Abstract: | Objective To discuss the conventional brain MR findings and its role in evaluation of methylmalonic acidemia(MMA) in children.Materials and Methods MR data of 13 patients with MMA confirmed by analysis of urine were reviewed retrospectively.Results Brain conventional MRI findings mainly reflected cerebral development retardation and injuries.The presentations included widening of sulci and cisterns(12 cases),lateral ventriculomegaly(9 cases), bulk loss in the white matter(8 cases),and immature form of corpus callosum(6 cases).In addition,abnormal signal intensity was found in white matter(7 cases),splenium of the corpus callosum(1 case),and bilateral globus pallidus(1 case) respectively.Conclusion Brain conventional MR can reflect cerebral development retardation and injuries in children with MMA,it is helpful in assessing the status and prognosis of the disease. |
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| Keywords: | Methylmalonic acidemia Children Magnetic resonance imaging |
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